ANSWERS
Neurology:Question 1
The correct answer is B
Educational Objectives
Manage a patient with a single seizure.
Critique
Most neurologists would not start antiepileptic drug therapy after a single seizure unless the patient has one or more risk factors associated with seizure recurrence: abnormal neurologic examination, abnormal electroencephalography or magnetic resonance imaging of the brain, partial onset, a history of neurologic injury (such as stroke, hemorrhage, or tumor), family history of seizures, or postictal motor paralysis. This patient has no risk factor for recurrence of seizures. Her seizure was provoked by sleep deprivation. Seizures caused by external events (drug toxicity, drug withdrawal, sleep deprivation, hypoglycemia, hypoxia, hyponatremia) are considered provoked and do not require chronic antiepileptic drug treatment. Patients with a single provoked seizure should have electroencephalography and magnetic resonance imaging to rule out underlying focal pathology that could lower the seizure threshold and precipitate a seizure. Not ordering any tests and just treating the patient with an antiepileptic drug can lead to missing an early diagnosis of brain tumor, small stroke, hemorrhage, or other focal cortical abnormality.
The correct answer is B
Educational Objectives
Manage a patient with a single seizure.
Critique
Most neurologists would not start antiepileptic drug therapy after a single seizure unless the patient has one or more risk factors associated with seizure recurrence: abnormal neurologic examination, abnormal electroencephalography or magnetic resonance imaging of the brain, partial onset, a history of neurologic injury (such as stroke, hemorrhage, or tumor), family history of seizures, or postictal motor paralysis. This patient has no risk factor for recurrence of seizures. Her seizure was provoked by sleep deprivation. Seizures caused by external events (drug toxicity, drug withdrawal, sleep deprivation, hypoglycemia, hypoxia, hyponatremia) are considered provoked and do not require chronic antiepileptic drug treatment. Patients with a single provoked seizure should have electroencephalography and magnetic resonance imaging to rule out underlying focal pathology that could lower the seizure threshold and precipitate a seizure. Not ordering any tests and just treating the patient with an antiepileptic drug can lead to missing an early diagnosis of brain tumor, small stroke, hemorrhage, or other focal cortical abnormality.
Neurology:Question 2
The correct answer is E
Educational Objectives
Initiate urgent workup for a patient with intermittent atrial fibrillation and transient ischemic attack.
Critique
Patients with transient ischemic attacks (TIA5) should be evaluated and treated urgently. Among patients who come to the emergency department with TIA, 5% will have a stroke within 2 days, and 25% will have a recurrent event within 3 months. Patients with atrial fibrillation and TIA or stroke should receive life-long oral anticoagulation to prevent cerebral ischemia from cardioembolism. Emergency computed tomography should be done in this patient to rule out tumor or hemorrhage as the cause of hemiparesis. Once bleeding is ruled out, anticoagulation should be initiated urgently. Warfarin benefits patients with chronic or intermittent atrial fibrillation regardless of left atrium size, so findings on Holter monitoring and transesophageal echocardiography would not affect initial management. It is appropriate to obtain carotid duplex ultrasonography, since 5% to 10% of patients with atrial fibrillation have concurrent carotid stenosis, but this is less immediately urgent.
The correct answer is E
Educational Objectives
Initiate urgent workup for a patient with intermittent atrial fibrillation and transient ischemic attack.
Critique
Patients with transient ischemic attacks (TIA5) should be evaluated and treated urgently. Among patients who come to the emergency department with TIA, 5% will have a stroke within 2 days, and 25% will have a recurrent event within 3 months. Patients with atrial fibrillation and TIA or stroke should receive life-long oral anticoagulation to prevent cerebral ischemia from cardioembolism. Emergency computed tomography should be done in this patient to rule out tumor or hemorrhage as the cause of hemiparesis. Once bleeding is ruled out, anticoagulation should be initiated urgently. Warfarin benefits patients with chronic or intermittent atrial fibrillation regardless of left atrium size, so findings on Holter monitoring and transesophageal echocardiography would not affect initial management. It is appropriate to obtain carotid duplex ultrasonography, since 5% to 10% of patients with atrial fibrillation have concurrent carotid stenosis, but this is less immediately urgent.
Neurology:Question 3
The correct answer is D
Educational Objectives
Monitor disease activity in a patient with a monosymptomatic demyelinating event.
Critique
According to the new McDonald criteria for diagnosis of multiple sclerosis, new disease activity demonstrable by new T2 white matter lesions, or new gadolinium-enhancing lesions on serial brain or spinal cord magnetic resonance imaging (MRI) at least 3 months after an initial scan qualify as dissemination of demyelination across time. Serial MRI is the best way to monitor for ongoing disease activity. Much of the activity of multiple sclerosis is subclinical and shows up only on MRI without changes in neurologic examination or new symptoms. Monitoring of visual system function would not help because new disease activity must be disseminated across space (in multiple areas of the central nervous system) and the optic nerves have already been affected. Nerve conduction velocities test for abnormalities in the peripheral nervous system, not the central nervous system, which is affected by multiple sclerosis.
The correct answer is D
Educational Objectives
Monitor disease activity in a patient with a monosymptomatic demyelinating event.
Critique
According to the new McDonald criteria for diagnosis of multiple sclerosis, new disease activity demonstrable by new T2 white matter lesions, or new gadolinium-enhancing lesions on serial brain or spinal cord magnetic resonance imaging (MRI) at least 3 months after an initial scan qualify as dissemination of demyelination across time. Serial MRI is the best way to monitor for ongoing disease activity. Much of the activity of multiple sclerosis is subclinical and shows up only on MRI without changes in neurologic examination or new symptoms. Monitoring of visual system function would not help because new disease activity must be disseminated across space (in multiple areas of the central nervous system) and the optic nerves have already been affected. Nerve conduction velocities test for abnormalities in the peripheral nervous system, not the central nervous system, which is affected by multiple sclerosis.
Neurology:Question 5
The correct answer is B
Educational Objectives
Recognize medication overuse as a cause of chronic daily headache.
Critique
This patient has developed a form of daily headache known as chronic migraine (transformed migraine). In many patients the change in headache pattern is caused by overuse of analgesics (more than 2 to 3 days per week). Combination analgesics are particularly likely to lead to chronic daily headache. Accordingly, the first intervention is to discontinue use of the combination analgesic. Discontinuing the offending agent usually results in improvement within 1 month, but occasionally takes longer. Patients should be advised that headaches may transiently worsen when the medication is discontinued. Amitriptyline may prove to be an effective preventive agent once the medication overuse headache has been eliminated. Increased amitriptyline or substitution with divalproex sodium is not likely to improve the daily headaches as long as the overuse of analgesics persists. Overuse headache from triptan agents is unlikely to occur when the medication is taken at a frequency of 2 days per week, as in this patient.
The correct answer is B
Educational Objectives
Recognize medication overuse as a cause of chronic daily headache.
Critique
This patient has developed a form of daily headache known as chronic migraine (transformed migraine). In many patients the change in headache pattern is caused by overuse of analgesics (more than 2 to 3 days per week). Combination analgesics are particularly likely to lead to chronic daily headache. Accordingly, the first intervention is to discontinue use of the combination analgesic. Discontinuing the offending agent usually results in improvement within 1 month, but occasionally takes longer. Patients should be advised that headaches may transiently worsen when the medication is discontinued. Amitriptyline may prove to be an effective preventive agent once the medication overuse headache has been eliminated. Increased amitriptyline or substitution with divalproex sodium is not likely to improve the daily headaches as long as the overuse of analgesics persists. Overuse headache from triptan agents is unlikely to occur when the medication is taken at a frequency of 2 days per week, as in this patient.
Neurology:Question 6
The correct answer is D
Educational Objectives
Recognize the clinical presentation of amyotrophic lateral sclerosis.
Critique
This patient has progressive, painless weakness and atrophy in the absence of sensory symptoms. He has upper and lower motor neuron signs in the symptomatic left arm, and essentially asymptomatic weakness in the left leg. The presentation suggests motor neuron disease (amyotrophic sclerosis). EMG would most likely confirm the diagnosis with findings of denervation in multiple extremities. As only 5% to 10% of cases of amyotrophic lateral sclerosis are familial, genetic testing at this stage is not indicated. The distribution of weakness is not proximal, as would occur with most myopathies, so muscle biopsy would not be helpful. Although creatine kinase levels may be elevated in rapidly progressive motor neuron disease, it is a nonspecific finding. The absence of sensory symptoms or signs argues against a peripheral neuropathy, so nerve biopsy, while likely showing axonal loss, would not be of high yield.
The correct answer is D
Educational Objectives
Recognize the clinical presentation of amyotrophic lateral sclerosis.
Critique
This patient has progressive, painless weakness and atrophy in the absence of sensory symptoms. He has upper and lower motor neuron signs in the symptomatic left arm, and essentially asymptomatic weakness in the left leg. The presentation suggests motor neuron disease (amyotrophic sclerosis). EMG would most likely confirm the diagnosis with findings of denervation in multiple extremities. As only 5% to 10% of cases of amyotrophic lateral sclerosis are familial, genetic testing at this stage is not indicated. The distribution of weakness is not proximal, as would occur with most myopathies, so muscle biopsy would not be helpful. Although creatine kinase levels may be elevated in rapidly progressive motor neuron disease, it is a nonspecific finding. The absence of sensory symptoms or signs argues against a peripheral neuropathy, so nerve biopsy, while likely showing axonal loss, would not be of high yield.
Neurology:Question 7
The correct answer is C
Educational Objectives
Manage incidental unruptured aneurysm.
Critique
This patient has a 4-mm asymptomatic intracranial aneurysm. The natural history of asymptomatic aneurysms less than 10 mm in diameter is usually benign, with a very low risk of rupture. In general, these patients should probably not undergo surgical therapy unless the aneurysm enlarges or changes shape. Periodic noninvasive testing is appropriate. Computed tomographic angiography has little to add to the magnetic resonance angiography that has already been performed in this patient. Catheter angiography is associated with additional risk and does not improve assessment of asymptomatic aneurysm.
The correct answer is C
Educational Objectives
Manage incidental unruptured aneurysm.
Critique
This patient has a 4-mm asymptomatic intracranial aneurysm. The natural history of asymptomatic aneurysms less than 10 mm in diameter is usually benign, with a very low risk of rupture. In general, these patients should probably not undergo surgical therapy unless the aneurysm enlarges or changes shape. Periodic noninvasive testing is appropriate. Computed tomographic angiography has little to add to the magnetic resonance angiography that has already been performed in this patient. Catheter angiography is associated with additional risk and does not improve assessment of asymptomatic aneurysm.
Neurology:Question 8
The correct answer is D
Educational Objectives
Initiate treatment of symptomatic Parkinson’s disease.
Critique
This man has signs and symptoms of parkinsonism, and very likely Parkinson’s disease, that are causing him to restrict his lifestyle. Treatment is indicated to keep him engaged in activities appropriate for his age. Carbidopa/levodopa is the appropriate first choice of medication. Dopamine agonist medications, such as pramipexole, ropinirole, pergolide, or bromocriptine, are not as effective as carbidopa/levodopa and may be insufficient to counter his increasing parkinsonian symptoms. Selegiline and amantadine are unlikely to have a major impact upon his symptoms. Entacapone is ineffective in the absence of levodopa treatment and hence has no role in initial treatment.
The correct answer is D
Educational Objectives
Initiate treatment of symptomatic Parkinson’s disease.
Critique
This man has signs and symptoms of parkinsonism, and very likely Parkinson’s disease, that are causing him to restrict his lifestyle. Treatment is indicated to keep him engaged in activities appropriate for his age. Carbidopa/levodopa is the appropriate first choice of medication. Dopamine agonist medications, such as pramipexole, ropinirole, pergolide, or bromocriptine, are not as effective as carbidopa/levodopa and may be insufficient to counter his increasing parkinsonian symptoms. Selegiline and amantadine are unlikely to have a major impact upon his symptoms. Entacapone is ineffective in the absence of levodopa treatment and hence has no role in initial treatment.
Neurology:Question 9
The correct answer is A
Educational Objectives
Recognize Alzheimer’s disease.
Critique
This patients progressive cognitive impairment affects both multiple aspects of intellect and his ability to perform daily tasks. The findings are consistent with a progressive dementia. Prominent recent memory loss suggests early involvement of the temporal lobes. The lack of other neurologic signs suggests sparing of the basal ganglia and primary motor and sensory areas. The findings are most compatible with dementia due to Alzheimer’s disease. Frontotemporal dementia may cause a similar profile, but usually has more prominent early personality change and less visuospatial impairment. Imaging often shows focal frontotemporal brain atrophy. Dementia with Lewy bodies is accompanied by parkinsonism, hallucinations, and fluctuating symptoms, none of which is present here. Creutzfeldt-Jakob disease has a much more rapid course and is usually associated with motor signs such as rigidity, ataxia, or myoclonus. Vascular dementia is unlikely in the absence of vascular risk factors, focal abnormalities on imaging, or a stroke-like (abrupt or stepwise) course of illness.
The correct answer is A
Educational Objectives
Recognize Alzheimer’s disease.
Critique
This patients progressive cognitive impairment affects both multiple aspects of intellect and his ability to perform daily tasks. The findings are consistent with a progressive dementia. Prominent recent memory loss suggests early involvement of the temporal lobes. The lack of other neurologic signs suggests sparing of the basal ganglia and primary motor and sensory areas. The findings are most compatible with dementia due to Alzheimer’s disease. Frontotemporal dementia may cause a similar profile, but usually has more prominent early personality change and less visuospatial impairment. Imaging often shows focal frontotemporal brain atrophy. Dementia with Lewy bodies is accompanied by parkinsonism, hallucinations, and fluctuating symptoms, none of which is present here. Creutzfeldt-Jakob disease has a much more rapid course and is usually associated with motor signs such as rigidity, ataxia, or myoclonus. Vascular dementia is unlikely in the absence of vascular risk factors, focal abnormalities on imaging, or a stroke-like (abrupt or stepwise) course of illness.
Neurology:Question 10
The correct answer is B
Educational Objectives
Recognize the clinical presentation of nonepileptic events (pseudoseizures).
Critique
This is a typical presentation of a nonepileptic event (pseudoseizure). Pseudoseizures usually start in an emotionally charged setting, with a gradual buildup of symptoms. Symptoms and duration are not stereotypical. The movements are not synchronous and are usually prolonged, starting and stopping many times throughout the event. Women are more likely than men to have nonepileptic events; associated factors include health care occupation, major life stressors, medical history of unexplained physical symptoms, psychiatric comorbidity, and traumatic childhood, including history of abuse. Many patients with nonepileptic episodes have family members or friends with seizures or have witnessed a seizure.
Syncope often causes tonic posturing and myoclonic jerks, but the episodes are brief and there is fast and full recovery of function (no postictal confusion). True generalized tonic-clonic seizures are characterized by tonic extension of the whole body, followed by synchronous, rhythmic clonic movements of the limbs. Partial seizures with secondary generalization have a focal onset. Basilar migraine is characterized by recurrent stereotypical episodes of transient brainstem symptoms such as vertigo or diplopia, and is usually associated with headache.
The correct answer is B
Educational Objectives
Recognize the clinical presentation of nonepileptic events (pseudoseizures).
Critique
This is a typical presentation of a nonepileptic event (pseudoseizure). Pseudoseizures usually start in an emotionally charged setting, with a gradual buildup of symptoms. Symptoms and duration are not stereotypical. The movements are not synchronous and are usually prolonged, starting and stopping many times throughout the event. Women are more likely than men to have nonepileptic events; associated factors include health care occupation, major life stressors, medical history of unexplained physical symptoms, psychiatric comorbidity, and traumatic childhood, including history of abuse. Many patients with nonepileptic episodes have family members or friends with seizures or have witnessed a seizure.
Syncope often causes tonic posturing and myoclonic jerks, but the episodes are brief and there is fast and full recovery of function (no postictal confusion). True generalized tonic-clonic seizures are characterized by tonic extension of the whole body, followed by synchronous, rhythmic clonic movements of the limbs. Partial seizures with secondary generalization have a focal onset. Basilar migraine is characterized by recurrent stereotypical episodes of transient brainstem symptoms such as vertigo or diplopia, and is usually associated with headache.
Neurology:Question 11
The correct answer is E
Educational Objectives
Initiate etiologic evaluation to determine appropriate therapy for secondary stroke prevention.
Critique
The choice of antithrombotic agent for secondary stroke prevention depends on the cause of stroke; initiating therapy without taking into account the cause of stroke is not appropriate. Patients with atrial fibrillation benefit most from chronic anticoagulation with warfarin (target INR [international normalized ratio] between 2.0 and 3.0). In patients with large- or small-artery disease, antiplatelet therapy decreases the risk of stroke and death, with no additional benefit from warfarin.
The correct answer is E
Educational Objectives
Initiate etiologic evaluation to determine appropriate therapy for secondary stroke prevention.
Critique
The choice of antithrombotic agent for secondary stroke prevention depends on the cause of stroke; initiating therapy without taking into account the cause of stroke is not appropriate. Patients with atrial fibrillation benefit most from chronic anticoagulation with warfarin (target INR [international normalized ratio] between 2.0 and 3.0). In patients with large- or small-artery disease, antiplatelet therapy decreases the risk of stroke and death, with no additional benefit from warfarin.
Neurology:Question 12
The correct answer is C
Educational Objectives
Recognize the ‘locked-in’ syndrome that can occur due to acute pontine lesions.
Critique
This patient is not comatose, but is in a “locked-in” state. Patients in a locked-in state are quadriplegic, have paralysis of horizontal eye movements, and are unable to speak because of paralysis of bulbar muscles; communication is possible only through the patient’s ability to move the eyes vertically and blink. The locked-in syndrome occurs with lesions of the base of the pons, usually infarction (such as from basilar artery occlusion) or hemorrhage. The finding of brisk downward eye movements is consistent with ocular bobbing, a kind of spontaneous eye movement that can be seen in large pontine lesions. Although not entirely specific, another clue to a brainstem localization of this patient’s findings is the presence of bilateral extensor (decerebrate) posturing. The bilaterally small pupils are also commonly associated with pontine lesions. A left middle cerebral artery stroke would cause aphasia and a right hemiparesis, not the bilateral signs or the eye findings seen in this patient. An anoxic encephalopathy would cause diffuse cerebral, and sometimes brainstem, dysfunction but would not explain this patient’s eye movements or the intact alertness in the presence of quadriplegia. The typical findings of a cerebellar infarct are headache, nausea, vomiting, and ataxia; brainstem (including pontine) dysfunction can occur as a secondary phenomenon due to mass effect from infarct-related edema, which is why neurosurgical consultation is important for large cerebellar infarcts as well as for cerebellar hemorrhages.
The correct answer is C
Educational Objectives
Recognize the ‘locked-in’ syndrome that can occur due to acute pontine lesions.
Critique
This patient is not comatose, but is in a “locked-in” state. Patients in a locked-in state are quadriplegic, have paralysis of horizontal eye movements, and are unable to speak because of paralysis of bulbar muscles; communication is possible only through the patient’s ability to move the eyes vertically and blink. The locked-in syndrome occurs with lesions of the base of the pons, usually infarction (such as from basilar artery occlusion) or hemorrhage. The finding of brisk downward eye movements is consistent with ocular bobbing, a kind of spontaneous eye movement that can be seen in large pontine lesions. Although not entirely specific, another clue to a brainstem localization of this patient’s findings is the presence of bilateral extensor (decerebrate) posturing. The bilaterally small pupils are also commonly associated with pontine lesions. A left middle cerebral artery stroke would cause aphasia and a right hemiparesis, not the bilateral signs or the eye findings seen in this patient. An anoxic encephalopathy would cause diffuse cerebral, and sometimes brainstem, dysfunction but would not explain this patient’s eye movements or the intact alertness in the presence of quadriplegia. The typical findings of a cerebellar infarct are headache, nausea, vomiting, and ataxia; brainstem (including pontine) dysfunction can occur as a secondary phenomenon due to mass effect from infarct-related edema, which is why neurosurgical consultation is important for large cerebellar infarcts as well as for cerebellar hemorrhages.
Neurology:Question 13
The correct answer is A
Educational Objectives
Recognize effect of fever on multiple sclerosis.
Critique
Neurologic function often deteriorates when patients with multiple sclerosis are exposed to ambient heat or fever, because raised temperature decreases conduction through demyelinated nerves. This is considered a pseudoexacerbation and is not necessarily evidence of a new exacerbation. Sudden worsening of lower-extremity strength and increased spasticity in any patient with multiple sclerosis, especially if associated with fever, should prompt investigation for urinary tract infection, decubitus ulcer, cellulitis, arthritis, or other irritative conditions. Any painful or irritating stimulus can worsen spasticity. Often the course improves over several days with hydration, appropriate antibiotic therapy, and aggressive reduction of fever. The other treatment options will not directly address this patient’s deterioration due to fever.
The correct answer is A
Educational Objectives
Recognize effect of fever on multiple sclerosis.
Critique
Neurologic function often deteriorates when patients with multiple sclerosis are exposed to ambient heat or fever, because raised temperature decreases conduction through demyelinated nerves. This is considered a pseudoexacerbation and is not necessarily evidence of a new exacerbation. Sudden worsening of lower-extremity strength and increased spasticity in any patient with multiple sclerosis, especially if associated with fever, should prompt investigation for urinary tract infection, decubitus ulcer, cellulitis, arthritis, or other irritative conditions. Any painful or irritating stimulus can worsen spasticity. Often the course improves over several days with hydration, appropriate antibiotic therapy, and aggressive reduction of fever. The other treatment options will not directly address this patient’s deterioration due to fever.
Neurology:Question 14
The correct answer is D
Educational Objectives
Recognize the clinical features of migraine headaches.
Critique
This patients symptoms fulfill the criteria for migraine without aura, despite the bilateral pain and absence of nausea. Rhinorrhea and tearing are autonomic symptoms that frequently accompany migraine and do not usually represent sinus disease. Most patients with a diagnosis of sinus headache in fact have migraine. Acute sinusitis is accompanied by fever and purulent nasal discharge. Episodic tension-type headaches are not pulsatile, do not worsen with physical activity, and are not associated with photophobia and phonophobia. Cluster headaches are unilateral (most commonly retro-orbital), last 1 to 2 hours, and occur in ‘clusters” of recurrent attacks lasting weeks to months. Most patients are agitated and prefer not to lie down during a cluster headache. This patient has no signs or symptoms, such as visual obscurations, that suggest a diagnosis of idiopathic intracranial hypertension, and there is no papilledema on funduscopic examination.
The correct answer is D
Educational Objectives
Recognize the clinical features of migraine headaches.
Critique
This patients symptoms fulfill the criteria for migraine without aura, despite the bilateral pain and absence of nausea. Rhinorrhea and tearing are autonomic symptoms that frequently accompany migraine and do not usually represent sinus disease. Most patients with a diagnosis of sinus headache in fact have migraine. Acute sinusitis is accompanied by fever and purulent nasal discharge. Episodic tension-type headaches are not pulsatile, do not worsen with physical activity, and are not associated with photophobia and phonophobia. Cluster headaches are unilateral (most commonly retro-orbital), last 1 to 2 hours, and occur in ‘clusters” of recurrent attacks lasting weeks to months. Most patients are agitated and prefer not to lie down during a cluster headache. This patient has no signs or symptoms, such as visual obscurations, that suggest a diagnosis of idiopathic intracranial hypertension, and there is no papilledema on funduscopic examination.
Neurology:Question 15
The correct answer is A
Educational Objectives
Recognize brain metastases.
Critique
This patients history of node-positive breast cancer places her at high risk for development of recurrent and metastatic tumor. She presents with progressive signs and symptoms of focal neurologic deficit and raised intracranial pressure. She most likely has multiple brain metastases. Magnetic resonance imaging of the head with contrast would be the most appropriate diagnostic test for this patient. Parenchymal brain metastases are by far the most common neurologic complication of systemic cancer, occurring in 20% to 40% of patients. Leptomeningeal and subdural metastases are less common. Patients with leptomeningeal metastases typically present with headache, multiple cranial nerve palsies, and multiple spinal radiculopathies. Patients with subdural metastases and dural sinus thrombosis primarily present with headache, hemiparesis, and sometimes drowsiness. Hemianopia would be distinctly unusual with a lesion not directly involving brain parenchyma. Paraneoplastic encephalitis is rare. It presents with seizures and rapidly progressive cognitive and personality changes; it is not usually associated with headache or signs of raised intracranial pressure. A cerebral infarct presents as an acute-onset neurologic deficit without symptoms or signs of raised intracranial pressure.
The correct answer is A
Educational Objectives
Recognize brain metastases.
Critique
This patients history of node-positive breast cancer places her at high risk for development of recurrent and metastatic tumor. She presents with progressive signs and symptoms of focal neurologic deficit and raised intracranial pressure. She most likely has multiple brain metastases. Magnetic resonance imaging of the head with contrast would be the most appropriate diagnostic test for this patient. Parenchymal brain metastases are by far the most common neurologic complication of systemic cancer, occurring in 20% to 40% of patients. Leptomeningeal and subdural metastases are less common. Patients with leptomeningeal metastases typically present with headache, multiple cranial nerve palsies, and multiple spinal radiculopathies. Patients with subdural metastases and dural sinus thrombosis primarily present with headache, hemiparesis, and sometimes drowsiness. Hemianopia would be distinctly unusual with a lesion not directly involving brain parenchyma. Paraneoplastic encephalitis is rare. It presents with seizures and rapidly progressive cognitive and personality changes; it is not usually associated with headache or signs of raised intracranial pressure. A cerebral infarct presents as an acute-onset neurologic deficit without symptoms or signs of raised intracranial pressure.
Neurology:Question 16
The correct answer is A
Educational Objectives
Manage antiepileptic drug therapy in a pregnant patient.
Critique
During pregnancy, seizure frequency increases in approximately one third of women with epilepsy. This is a result of increased hepatic metabolism of most antiepileptic drugs and an increased volume of distribution. Therefore, drug levels should be monitored during pregnancy to make sure they remain therapeutic. This patient appears to have good seizure control on the current dose of carbamazepine. There is no need to change the dose or add or switch to another antiepileptic drug at the present time. If the patient starts to have breakthrough seizures, the dose of carbamazepine should be increased instead of adding or changing to another antiepileptic drug. Changing antiepileptic drug therapy during pregnancy can be harmful because during the transition from one drug to another there is a risk of toxicity or breakthrough seizures. Ideally, the patient would have discussed plans to become pregnant in advance, so any necessary changes to her antiepileptic drug regimen could be made before the patient conceives. Since often pregnancies are not planned, every woman of childbearing age should take folate supplements to reduce the risk of neural tube defects from anticonvulsant therapy.
The goal is no seizures on the minimal effective dose of a single antiepileptic drug. Combination of antiepileptic drugs should be avoided if possible since there is an increased risk of congenital malformations and side effects. For many women, withdrawal of antiepileptic drugs before pregnancy results in recurrence or exacerbation of seizures, which can be dangerous for both mother and fetus.
The correct answer is A
Educational Objectives
Manage antiepileptic drug therapy in a pregnant patient.
Critique
During pregnancy, seizure frequency increases in approximately one third of women with epilepsy. This is a result of increased hepatic metabolism of most antiepileptic drugs and an increased volume of distribution. Therefore, drug levels should be monitored during pregnancy to make sure they remain therapeutic. This patient appears to have good seizure control on the current dose of carbamazepine. There is no need to change the dose or add or switch to another antiepileptic drug at the present time. If the patient starts to have breakthrough seizures, the dose of carbamazepine should be increased instead of adding or changing to another antiepileptic drug. Changing antiepileptic drug therapy during pregnancy can be harmful because during the transition from one drug to another there is a risk of toxicity or breakthrough seizures. Ideally, the patient would have discussed plans to become pregnant in advance, so any necessary changes to her antiepileptic drug regimen could be made before the patient conceives. Since often pregnancies are not planned, every woman of childbearing age should take folate supplements to reduce the risk of neural tube defects from anticonvulsant therapy.
The goal is no seizures on the minimal effective dose of a single antiepileptic drug. Combination of antiepileptic drugs should be avoided if possible since there is an increased risk of congenital malformations and side effects. For many women, withdrawal of antiepileptic drugs before pregnancy results in recurrence or exacerbation of seizures, which can be dangerous for both mother and fetus.
Neurology:Question 17
The correct answer is C
Educational Objectives
Recognize cerebellar hemorrhage.
Critique
This patient has classic signs and symptoms of an acute left cerebellar insult, including headache, nausea, vomiting, vertigo, and left-sided ataxia. The etiology is either cerebellar hemorrhage or cerebellar infarction. He requires emergent computed tomography to check for cerebellar hemorrhage; if hemorrhage is present, he would need urgent neurosurgical consult. Cerebellar hemorrhages larger than 3 cm or with mass effect on the brainstem usually require decompression, which is often lifesaving, and patients can recover with minimal neurologic deficits. The other options are peripheral vestibular disorders that are unlikely to cause headache or the cerebellar findings in this patient.
The correct answer is C
Educational Objectives
Recognize cerebellar hemorrhage.
Critique
This patient has classic signs and symptoms of an acute left cerebellar insult, including headache, nausea, vomiting, vertigo, and left-sided ataxia. The etiology is either cerebellar hemorrhage or cerebellar infarction. He requires emergent computed tomography to check for cerebellar hemorrhage; if hemorrhage is present, he would need urgent neurosurgical consult. Cerebellar hemorrhages larger than 3 cm or with mass effect on the brainstem usually require decompression, which is often lifesaving, and patients can recover with minimal neurologic deficits. The other options are peripheral vestibular disorders that are unlikely to cause headache or the cerebellar findings in this patient.
Neurology:Question 18
The correct answer is B
Educational Objectives
Diagnose chronic inflammatory demyelinating polyneuropathy.
Critique
The diagnostic features of chronic inflammatory demyelinating polyneuropathy include roughly symmetric weakness and sensory loss progressing over at least 2 months, with evidence of segmental demyelination on nerve conduction studies. Although results of electrodiagnostic testing are not given in this case, chronic inflammatory demyelinating polyneuropathy is the most likely diagnosis of the options given. Polymyositis and myasthenia gravis would not produce sensory signs or symptoms. The progression of symptoms over 8 months is too long for Guillain-Barré syndrome, which evolves over 4 to 6 weeks.
The correct answer is B
Educational Objectives
Diagnose chronic inflammatory demyelinating polyneuropathy.
Critique
The diagnostic features of chronic inflammatory demyelinating polyneuropathy include roughly symmetric weakness and sensory loss progressing over at least 2 months, with evidence of segmental demyelination on nerve conduction studies. Although results of electrodiagnostic testing are not given in this case, chronic inflammatory demyelinating polyneuropathy is the most likely diagnosis of the options given. Polymyositis and myasthenia gravis would not produce sensory signs or symptoms. The progression of symptoms over 8 months is too long for Guillain-Barré syndrome, which evolves over 4 to 6 weeks.
Neurology:Question 19
The correct answer is C
Educational Objectives
Treat patient with acute ischemic stroke and persistently elevated blood pressure.
Critique
Management of blood pressure is especially complex in patients with acute ischemic stroke who are otherwise eligible for intravenous tissue plasminogen activator (t-PA). In stroke patients who receive intravenous t-PA, elevated blood pressure carries an increased risk for intracranial hemorrhage, hence persistent systolic blood pressure above 185 mm Hg or persistent diastolic blood pressure above 110 mm Hg is a contraindication to intravenous t-PA. However, acute hypertension at the time of stroke is most often a result of—not a cause of—stroke, and is necessary to supply the reversibly ischemic brain (ischemic penumbra) with enough blood to survive. Since the goal of intravenous t-PA therapy is to save the penumbra, it is counterproductive to lower blood pressure excessively in order to administer t-PA. Thus, the need for aggressive therapy to achieve blood pressure below 185/110 mm Hg is also a contraindication to intravenous t-PA. In this patient, the systolic value would need to be lowered by 55 mm Hg in order to administer intravenous t-PA within the next hour. This is considered aggressive therapy.
Intra-arterial t-PA is not currently approved by the U.S. Food and Drug Administration for ischemic stroke; at this time, it is used in specialized centers for patients who present within 3 to 6 hours of onset of symptoms. Recommendations regarding blood pressure management are the same for intra-arterial and intravenous t-PA.
The correct answer is C
Educational Objectives
Treat patient with acute ischemic stroke and persistently elevated blood pressure.
Critique
Management of blood pressure is especially complex in patients with acute ischemic stroke who are otherwise eligible for intravenous tissue plasminogen activator (t-PA). In stroke patients who receive intravenous t-PA, elevated blood pressure carries an increased risk for intracranial hemorrhage, hence persistent systolic blood pressure above 185 mm Hg or persistent diastolic blood pressure above 110 mm Hg is a contraindication to intravenous t-PA. However, acute hypertension at the time of stroke is most often a result of—not a cause of—stroke, and is necessary to supply the reversibly ischemic brain (ischemic penumbra) with enough blood to survive. Since the goal of intravenous t-PA therapy is to save the penumbra, it is counterproductive to lower blood pressure excessively in order to administer t-PA. Thus, the need for aggressive therapy to achieve blood pressure below 185/110 mm Hg is also a contraindication to intravenous t-PA. In this patient, the systolic value would need to be lowered by 55 mm Hg in order to administer intravenous t-PA within the next hour. This is considered aggressive therapy.
Intra-arterial t-PA is not currently approved by the U.S. Food and Drug Administration for ischemic stroke; at this time, it is used in specialized centers for patients who present within 3 to 6 hours of onset of symptoms. Recommendations regarding blood pressure management are the same for intra-arterial and intravenous t-PA.
Neurology:Question 20
The correct answer is B
Educational Objectives
Manage prophylaxis for migraine headaches.
Critique
The patient is tolerating the amitriptyline well; since the dose is currently low, it is appropriate to continue to increase it gradually as planned. Maximal benefit of a prophylactic agent may not be recognized for 2 to 3 months after attaining the target dose. Accordingly, it would be an error to continue with the current dose or to discontinue amitriptyline. Propranolol and divalproex sodium are both useful prophylactic agents, but should not be substituted for amitriptyline at this time. They might be considered if amitriptyline is ultimately not effective or tolerated.
The correct answer is B
Educational Objectives
Manage prophylaxis for migraine headaches.
Critique
The patient is tolerating the amitriptyline well; since the dose is currently low, it is appropriate to continue to increase it gradually as planned. Maximal benefit of a prophylactic agent may not be recognized for 2 to 3 months after attaining the target dose. Accordingly, it would be an error to continue with the current dose or to discontinue amitriptyline. Propranolol and divalproex sodium are both useful prophylactic agents, but should not be substituted for amitriptyline at this time. They might be considered if amitriptyline is ultimately not effective or tolerated.
Neurology:Question 21
The correct answer is A
Educational Objectives
Recognize that antiemetic drugs with dopamine antagonist properties may induce parkinsonism.
Critique
This patient has neurologic symptoms consistent with parkinsonism. Metoclopramide, a commonly prescribed antiemetic that blocks dopamine receptors, may cause parkinsonism and could be responsible for his symptoms.
Gluten sensitivity has been associated with peripheral neuropathies and cerebellar ataxia, but is not a likely cause of parkinsonism. Neither ranitidine nor bupropion has been associated with development of parkinsonism. Selective serotonin reuptake inhibitors (SSRI5) have been reported to cause parkinsonism, but this is extremely rare; they are not contraindicated and usually are well tolerated by patients with Parkinson’s disease. Symptomatic treatment with carbidopa/levodopa or pramipexole may be appropriate later, if discontinuation of metoclopramide fails to resolve parkinsonism.
The correct answer is A
Educational Objectives
Recognize that antiemetic drugs with dopamine antagonist properties may induce parkinsonism.
Critique
This patient has neurologic symptoms consistent with parkinsonism. Metoclopramide, a commonly prescribed antiemetic that blocks dopamine receptors, may cause parkinsonism and could be responsible for his symptoms.
Gluten sensitivity has been associated with peripheral neuropathies and cerebellar ataxia, but is not a likely cause of parkinsonism. Neither ranitidine nor bupropion has been associated with development of parkinsonism. Selective serotonin reuptake inhibitors (SSRI5) have been reported to cause parkinsonism, but this is extremely rare; they are not contraindicated and usually are well tolerated by patients with Parkinson’s disease. Symptomatic treatment with carbidopa/levodopa or pramipexole may be appropriate later, if discontinuation of metoclopramide fails to resolve parkinsonism.
Neurology:Question 22
The correct answer is D
Educational Objectives
Recognize leptomeningeal metastases.
Critique
This patient has known lung cancer and presents with subacute multifocal neurologic deficits affecting multiple levels of the nervous system (ventricular system, cranial nerves, lumbar root). This is the classic presentation of leptomeningeal tumor, which occurs in 3% to 8% of all patients with cancer. Communicating hydrocephalus is common and due to obstruction of the arachnoid granulations. Multifocal cranial neuropathies and radiculopathies are frequent and due to nerve infiltration by tumor cells within the cerebrospinal fluid.
Multiple parenchymal metastases could produce a similar clinical picture but generally would be manifest on computed tomography. Paraneoplastic neuropathy and herpes zoster infection of the central nervous system are extremely rare complications of cancer; paraneoplastic neuropathy would not cause cranial nerve deficits. Venous sinus thrombosis would not produce multifocal cranial neuropathy and lumbar radiculopathy.
The correct answer is D
Educational Objectives
Recognize leptomeningeal metastases.
Critique
This patient has known lung cancer and presents with subacute multifocal neurologic deficits affecting multiple levels of the nervous system (ventricular system, cranial nerves, lumbar root). This is the classic presentation of leptomeningeal tumor, which occurs in 3% to 8% of all patients with cancer. Communicating hydrocephalus is common and due to obstruction of the arachnoid granulations. Multifocal cranial neuropathies and radiculopathies are frequent and due to nerve infiltration by tumor cells within the cerebrospinal fluid.
Multiple parenchymal metastases could produce a similar clinical picture but generally would be manifest on computed tomography. Paraneoplastic neuropathy and herpes zoster infection of the central nervous system are extremely rare complications of cancer; paraneoplastic neuropathy would not cause cranial nerve deficits. Venous sinus thrombosis would not produce multifocal cranial neuropathy and lumbar radiculopathy.
Neurology:Question 23
The correct answer is C
Educational Objectives
Distinguish seizures from syncope.
Critique
This patient’s symptoms are most compatible with vasovagal syncope. There is usually a brief prodrome that may include lightheadedness, warmth, diaphoresis, nausea, pallor, and blurred vision. Abnormal movements (mainly myoclonic jerks or tonic posturing) and urinary incontinence may occur during unconsciousness. Once recumbent, the patient usually recovers consciousness rapidly but may be confused for up to 30 seconds.
Juvenile myoclonic epilepsy is a genetic syndrome characterized by myoclonic jerks and absence and generalized tonic-clonic seizures. Affected patients have no warning prior to the generalized tonic-clonic seizure. Afterward, the patient falls into a deep sleep or is tired and confused for minutes to hours. Complex partial seizures with secondary generalization are associated with auras, but patients are not likely to be pale or diaphoretic or to feel warm. Postictal confusion lasts minutes to hours. Pseudoseizures are not stereotypical in presentation and usually last longer. Atonic seizures consist of sudden loss of postural tone without any prodrome or warning and without loss of consciousness.
The correct answer is C
Educational Objectives
Distinguish seizures from syncope.
Critique
This patient’s symptoms are most compatible with vasovagal syncope. There is usually a brief prodrome that may include lightheadedness, warmth, diaphoresis, nausea, pallor, and blurred vision. Abnormal movements (mainly myoclonic jerks or tonic posturing) and urinary incontinence may occur during unconsciousness. Once recumbent, the patient usually recovers consciousness rapidly but may be confused for up to 30 seconds.
Juvenile myoclonic epilepsy is a genetic syndrome characterized by myoclonic jerks and absence and generalized tonic-clonic seizures. Affected patients have no warning prior to the generalized tonic-clonic seizure. Afterward, the patient falls into a deep sleep or is tired and confused for minutes to hours. Complex partial seizures with secondary generalization are associated with auras, but patients are not likely to be pale or diaphoretic or to feel warm. Postictal confusion lasts minutes to hours. Pseudoseizures are not stereotypical in presentation and usually last longer. Atonic seizures consist of sudden loss of postural tone without any prodrome or warning and without loss of consciousness.
Neurology:Question 24
The correct answer is C
Educational Objectives
Treat pathologic fatigue of multiple sclerosis.
Critique
Amantadine is the first-line pharmacologic agent for treatment of multiple sclerosis fatigue, which is common and disabling. Amantadine affects several neurotransmitter systems and enhances neurotransmitter release; however, the exact mechanism by which it improves fatigue of multiple sclerosis is not known. It is well tolerated in younger patients with multiple sclerosis despite anticholinergic effects at doses such as 100 mg orally each morning or twice a day (morning and afternoon).
Other causes of severe fatigue should be sought in all patients with multiple sclerosis. Typically, patients should be screened for thyroid dysfunction, but thyroid supplementation is used only if laboratory abnormalities suggest thyroid disease. Amphetamines and agents such as ritalin are not first-line therapy because of their addiction potential.
The correct answer is C
Educational Objectives
Treat pathologic fatigue of multiple sclerosis.
Critique
Amantadine is the first-line pharmacologic agent for treatment of multiple sclerosis fatigue, which is common and disabling. Amantadine affects several neurotransmitter systems and enhances neurotransmitter release; however, the exact mechanism by which it improves fatigue of multiple sclerosis is not known. It is well tolerated in younger patients with multiple sclerosis despite anticholinergic effects at doses such as 100 mg orally each morning or twice a day (morning and afternoon).
Other causes of severe fatigue should be sought in all patients with multiple sclerosis. Typically, patients should be screened for thyroid dysfunction, but thyroid supplementation is used only if laboratory abnormalities suggest thyroid disease. Amphetamines and agents such as ritalin are not first-line therapy because of their addiction potential.
No firm data support use of selective serotonin reuptake inhibitors for fatigue of multiple sclerosis. One study suggests an overlap between depression and fatigue in multiple sclerosis, but they are believed to be due to different mechanisms. Pemoline has been used for multiple sclerosis fatigue in the past, but it is now used infrequently because frequent liver function tests are needed to check for hepatotoxicity. Modafinil, a nonamphetamine stimulant used extensively in narcolepsy, is useful in fatigue of multiple sclerosis as well. Its primary limitation is cost. Other approaches to fatigue include energy conservation, graded exercise programs to promote stamina, and elimination of medications that may contribute to sedation.
Neurology:Question 25
The correct answer is A
Educational Objectives
Use cholinergic augmentation in Alzheimers disease.
Critique
This patient has symptoms consistent with progressive dementia, most likely Alzheimer’s dementia. Clinical trials consistently demonstrate modest efficacy of cholinesterase inhibitors, including galantamine, tacrine, donepezil, and rivastigmine, in mild to moderate Alzheimers disease, on both cognitive and global function scales. In addition, cholinesterase inhibitors act favorably on psychiatric symptoms, including agitation, apathy, and hallucinations. There is no documented efficacy of these drugs in delaying the natural history of Alzheimer’s disease. Gingko biloba probably exerts modest effects on cognitive symptoms, but has not been shown to improve global or psychiatric function. Although some studies suggest that vitamin E and selegiline delay the progression of Alzheimer’s disease, they do not directly relieve cognitive or psychiatric symptoms.
The correct answer is A
Educational Objectives
Use cholinergic augmentation in Alzheimers disease.
Critique
This patient has symptoms consistent with progressive dementia, most likely Alzheimer’s dementia. Clinical trials consistently demonstrate modest efficacy of cholinesterase inhibitors, including galantamine, tacrine, donepezil, and rivastigmine, in mild to moderate Alzheimers disease, on both cognitive and global function scales. In addition, cholinesterase inhibitors act favorably on psychiatric symptoms, including agitation, apathy, and hallucinations. There is no documented efficacy of these drugs in delaying the natural history of Alzheimer’s disease. Gingko biloba probably exerts modest effects on cognitive symptoms, but has not been shown to improve global or psychiatric function. Although some studies suggest that vitamin E and selegiline delay the progression of Alzheimer’s disease, they do not directly relieve cognitive or psychiatric symptoms.
Neurology:Question 26
The correct answer is A
Educational Objectives
Administer intravenous tissue plasminogen activator (t-PA)to ischemic stroke patients only within 3
hours of stroke onset.
Critique
Intravenous t-PA decreases the risk of disability in patients with acute ischemic stroke only when given according to the protocol established in 1995 in studies by the National Institute of Neurological Disorders and Stroke (NINDS). Chief among the inclusion criteria is stroke onset within 3 hours of when the patient was last known to be normal. Since patients with acute stroke often cannot give accurate histories, the histories obtained from witnesses and caregivers are essential. According to protocol criteria, this patient’s stroke onset was 3 days earlier. The computed tomography findings lend further evidence that the stroke was not acute: a hypodense (dark) area with mass effect suggests a subacute (days-old) infarction.
Corticosteroids are not indicated and may be harmful in patients with ischemic stroke. The cytotoxic cerebral edema that occurs with ischemic stroke is greatest 2 to 5 days after stroke onset, and does not respond to corticosteroids. Corticosteroids are effective for vasogenic edema due to mass lesions such as tumors. Despite its frequent use in theUnited States
The correct answer is A
Educational Objectives
Administer intravenous tissue plasminogen activator (t-PA)to ischemic stroke patients only within 3
hours of stroke onset.
Critique
Intravenous t-PA decreases the risk of disability in patients with acute ischemic stroke only when given according to the protocol established in 1995 in studies by the National Institute of Neurological Disorders and Stroke (NINDS). Chief among the inclusion criteria is stroke onset within 3 hours of when the patient was last known to be normal. Since patients with acute stroke often cannot give accurate histories, the histories obtained from witnesses and caregivers are essential. According to protocol criteria, this patient’s stroke onset was 3 days earlier. The computed tomography findings lend further evidence that the stroke was not acute: a hypodense (dark) area with mass effect suggests a subacute (days-old) infarction.
Corticosteroids are not indicated and may be harmful in patients with ischemic stroke. The cytotoxic cerebral edema that occurs with ischemic stroke is greatest 2 to 5 days after stroke onset, and does not respond to corticosteroids. Corticosteroids are effective for vasogenic edema due to mass lesions such as tumors. Despite its frequent use in the
Neurology:Question 27
The correct answer is E
Educational Objectives
Recognize multiple system atrophy.
Critique
This patient has evidence of dysautonomia (with orthostatic hypotension and possible neurogenic bladder), parkinsonism, corticospinal tract signs, and ataxia. The constellation of findings is typical of multiple system atrophy. The “screeching” sounds during the night are worrisome for nocturnal stridor, which is highly suggestive of multiple system atrophy. Polysomnography should be obtained to confirm nocturnal stridor because patients with multiple system atrophy and stridor may die during the night, presumably from dysfunction of the brainstem breathing center. Stridor can be treated with tracheostomy or possibly with continuous positive airway pressure. Although Parkinson’s disease is often associated with dysautonomia, the pronounced autonomic symptoms, including symptomatic orthostatic hypotension, are atypical. Further, corticospinal tract signs and ataxia are not expected in Parkinson’s disease. Creutzfeldt-Jakob disease is unlikely, given the clinical picture and the 2-year course.
Although spinocerebellar degenerations may manifest with parkinsonism, corticospinal tract signs, and ataxia, they do not include prominent dysautonomia. Progressive supranuclear palsy manifests with parkinsonism and often corticospinal tract signs, but not with severe dysautonomia with symptomatic orthostatic hypotension. Also, the patient does not have the vertical (downward) gaze palsy suggesting progressive supranuclear palsy.
The correct answer is E
Educational Objectives
Recognize multiple system atrophy.
Critique
This patient has evidence of dysautonomia (with orthostatic hypotension and possible neurogenic bladder), parkinsonism, corticospinal tract signs, and ataxia. The constellation of findings is typical of multiple system atrophy. The “screeching” sounds during the night are worrisome for nocturnal stridor, which is highly suggestive of multiple system atrophy. Polysomnography should be obtained to confirm nocturnal stridor because patients with multiple system atrophy and stridor may die during the night, presumably from dysfunction of the brainstem breathing center. Stridor can be treated with tracheostomy or possibly with continuous positive airway pressure. Although Parkinson’s disease is often associated with dysautonomia, the pronounced autonomic symptoms, including symptomatic orthostatic hypotension, are atypical. Further, corticospinal tract signs and ataxia are not expected in Parkinson’s disease. Creutzfeldt-Jakob disease is unlikely, given the clinical picture and the 2-year course.
Although spinocerebellar degenerations may manifest with parkinsonism, corticospinal tract signs, and ataxia, they do not include prominent dysautonomia. Progressive supranuclear palsy manifests with parkinsonism and often corticospinal tract signs, but not with severe dysautonomia with symptomatic orthostatic hypotension. Also, the patient does not have the vertical (downward) gaze palsy suggesting progressive supranuclear palsy.
Neurology:Question 28
The correct answer is C
Educational Objectives
Recognize vitamin B12 deficiency.
Critique
This man has signs and symptoms of progressive spinal cord dysfunction. He has dense posterior column dysfunction (vibration and proprioception) as well as corticospinal tract motor signs and deficits. This combination suggests subacute combined degeneration, as seen in vitamin B12 deficiency. The normal magnetic resonance image of the spinal cord rules out cord compression. However, other causes of progressive myelopathy should be considered.
The other options are not upper motor neuron syndromes and are not associated with hyperreflexia or extensor plantar responses.
The correct answer is C
Educational Objectives
Recognize vitamin B12 deficiency.
Critique
This man has signs and symptoms of progressive spinal cord dysfunction. He has dense posterior column dysfunction (vibration and proprioception) as well as corticospinal tract motor signs and deficits. This combination suggests subacute combined degeneration, as seen in vitamin B12 deficiency. The normal magnetic resonance image of the spinal cord rules out cord compression. However, other causes of progressive myelopathy should be considered.
The other options are not upper motor neuron syndromes and are not associated with hyperreflexia or extensor plantar responses.
Neurology:Question 29
The correct answer is D
Educational Objectives
Evaluate intracranial hemorrhage.
Critique
This patient has an intracerebral hemorrhage with intraventricular extension. Although the location of the hemorrhage is typical for a hypertensive bleed, patients younger than 45 years who have no prior history of hypertension are at much higher risk for a potential vascular anomaly underlying the hemorrhage. Cocaine use could be solely responsible for the stroke; however, patients with cocaine-related hemorrhage are also more likely to have a vascular anomaly. This patient needs aggressive evaluation for a possible underlying vascular malformation, and the definitive test is cerebral angiography. Electroencephalography is not indicated unless the history raises the possibility of seizure. Echocardiography would provide information about the possibility of chronic hypertension in this patient, but patients younger than 45 years should have vascular imaging after intracranial hemorrhage, even if they have known hypertension.
The correct answer is D
Educational Objectives
Evaluate intracranial hemorrhage.
Critique
This patient has an intracerebral hemorrhage with intraventricular extension. Although the location of the hemorrhage is typical for a hypertensive bleed, patients younger than 45 years who have no prior history of hypertension are at much higher risk for a potential vascular anomaly underlying the hemorrhage. Cocaine use could be solely responsible for the stroke; however, patients with cocaine-related hemorrhage are also more likely to have a vascular anomaly. This patient needs aggressive evaluation for a possible underlying vascular malformation, and the definitive test is cerebral angiography. Electroencephalography is not indicated unless the history raises the possibility of seizure. Echocardiography would provide information about the possibility of chronic hypertension in this patient, but patients younger than 45 years should have vascular imaging after intracranial hemorrhage, even if they have known hypertension.
Neurology:Question 30
The correct answer is B
Educational Objectives
Diagnose acute diabetic proximal neuropathy.
Critique
This patients signs and symptoms are consistent with a lesion of the right upper lumbar root or right lumbar plexus. The normal findings on magnetic resonance imaging argue against a structural or compressive cause. The most likely noncompressive cause of right upper lumbar radiculoneuropathy is diabetes. Diabetic proximal neuropathy (diabetic amyotrophy) may be symmetric or asymmetric, and presents with subacute proximal leg muscle weakness associated with severe pain. Symptoms usually begin after longstanding uncontrolled hyperglycemia and weight loss. The pathogenesis is unclear; nerve biopsy studies have shown a vasculitic inflammatory infiltrate in involved nerves.
Obtaining a creatine kinase level would be appropriate if primary muscle disease were suspected, but this is not likely given the presence of neuropathic pain and sensory loss. Thyroid function tests are often obtained in cases of peripheral neuropathy, but thyroid derangement would not cause a lumbar radiculopathy or plexopathy. Lumbar puncture may help confirm an inflammatory process in the cerebrospinal fluid, but would not result in a specific diagnosis. Similarly, elevations of the erythrocyte sedimentation rate are nonspecific.
The correct answer is B
Educational Objectives
Diagnose acute diabetic proximal neuropathy.
Critique
This patients signs and symptoms are consistent with a lesion of the right upper lumbar root or right lumbar plexus. The normal findings on magnetic resonance imaging argue against a structural or compressive cause. The most likely noncompressive cause of right upper lumbar radiculoneuropathy is diabetes. Diabetic proximal neuropathy (diabetic amyotrophy) may be symmetric or asymmetric, and presents with subacute proximal leg muscle weakness associated with severe pain. Symptoms usually begin after longstanding uncontrolled hyperglycemia and weight loss. The pathogenesis is unclear; nerve biopsy studies have shown a vasculitic inflammatory infiltrate in involved nerves.
Obtaining a creatine kinase level would be appropriate if primary muscle disease were suspected, but this is not likely given the presence of neuropathic pain and sensory loss. Thyroid function tests are often obtained in cases of peripheral neuropathy, but thyroid derangement would not cause a lumbar radiculopathy or plexopathy. Lumbar puncture may help confirm an inflammatory process in the cerebrospinal fluid, but would not result in a specific diagnosis. Similarly, elevations of the erythrocyte sedimentation rate are nonspecific.
Neurology:Question 31
The correct answer is D
Educational Objectives
Diagnose progressive supranuclear palsy.
Critique
This patient’s early falls and vertical gaze paresis make progressive supranuclear palsy the likely diagnosis. Patients with progressive supranuclear palsy often have vision complaints, yet their optometrists are unable to document problems with either visual acuity or peripheral vision. Impaired eye movements, especially the inability to look down, underlie these complaints. Many routine tasks, such as reading and needlework, require down gaze and visual tracking.
Patients with idiopathic Parkinson’s disease do not fall early in the course of disease. Although there are clues that might suggest dystonia, multiple system atrophy, or cerebellar degeneration, the overall clinical picture fits best with progressive supranuclear palsy.
The correct answer is D
Educational Objectives
Diagnose progressive supranuclear palsy.
Critique
This patient’s early falls and vertical gaze paresis make progressive supranuclear palsy the likely diagnosis. Patients with progressive supranuclear palsy often have vision complaints, yet their optometrists are unable to document problems with either visual acuity or peripheral vision. Impaired eye movements, especially the inability to look down, underlie these complaints. Many routine tasks, such as reading and needlework, require down gaze and visual tracking.
Patients with idiopathic Parkinson’s disease do not fall early in the course of disease. Although there are clues that might suggest dystonia, multiple system atrophy, or cerebellar degeneration, the overall clinical picture fits best with progressive supranuclear palsy.
Neurology:Question 32
The correct answer is D
Educational Objectives
Recognize frontotemporal dementia.
Critique
This patients progressive impairment of cognition with prominent personality and behavior changes is most consistent with frontotemporal dementia. Brain imaging would be appropriate to exclude a mass lesion. Brain imaging in patients with frontotemporal dementia typically shows pronounced atrophy of the frontal and temporal lobes. Despite the patients high score on the Mini-Mental State Examination (MMSE), a diagnosis of mild cognitive impairment is inappropriate, since the patient is clearly impaired in his interpersonal and occupational function, and not only in memory. In fact, the paradoxically high score is a clue to the diagnosis, because the MMSE is relatively insensitive to executive dysfunction, the predominant impairment in frontotemporal dementia. The positive family history suggests an autosomal-dominant disorder, which is more likely in frontotemporal dementia than in Alzheimer’s disease. None of the features of dementia with Lewy bodies (hallucinations, parkinsonism, fluctuating symptoms) is present. Creutzfeldt-Jakob disease evolves more rapidly, and usually causes myoclonus and other neurologic signs.
The correct answer is D
Educational Objectives
Recognize frontotemporal dementia.
Critique
This patients progressive impairment of cognition with prominent personality and behavior changes is most consistent with frontotemporal dementia. Brain imaging would be appropriate to exclude a mass lesion. Brain imaging in patients with frontotemporal dementia typically shows pronounced atrophy of the frontal and temporal lobes. Despite the patients high score on the Mini-Mental State Examination (MMSE), a diagnosis of mild cognitive impairment is inappropriate, since the patient is clearly impaired in his interpersonal and occupational function, and not only in memory. In fact, the paradoxically high score is a clue to the diagnosis, because the MMSE is relatively insensitive to executive dysfunction, the predominant impairment in frontotemporal dementia. The positive family history suggests an autosomal-dominant disorder, which is more likely in frontotemporal dementia than in Alzheimer’s disease. None of the features of dementia with Lewy bodies (hallucinations, parkinsonism, fluctuating symptoms) is present. Creutzfeldt-Jakob disease evolves more rapidly, and usually causes myoclonus and other neurologic signs.
Neurology:Question 33
The correct answer is E
Educational Objectives
Recognize the effect of corticosteroids on primary lymphoma of the CNS.
Critique
This is an elderly patient with subacute progressive neurologic deficits referable to the supratentorial level and signs of raised intracranial pressure. The clinical history and imaging suggest neoplasm. Corticosteroid therapy produces rapid clinical and radiographic improvement. This clinical picture is most characteristic of primary central nervous system lymphoma. If primary central nervous system lymphoma is suspected, it is important to refrain from using corticosteroids prior to biopsy, unless the patients clinical situation absolutely requires it. Corticosteroids exert an oncolytic effect on lymphomas and may render the biopsy nondiagnostic.
Glioblastoma and metastatic carcinoma are unlikely to have such a dramatic radiographic and clinical response to corticosteroids. Although vasculitis or multiple sclerosis may respond rapidly to corticosteroids, neither disorder is likely to develop in a 70-year-old patient, and neither would generally present as a mass lesion on brain imaging.
The correct answer is E
Educational Objectives
Recognize the effect of corticosteroids on primary lymphoma of the CNS.
Critique
This is an elderly patient with subacute progressive neurologic deficits referable to the supratentorial level and signs of raised intracranial pressure. The clinical history and imaging suggest neoplasm. Corticosteroid therapy produces rapid clinical and radiographic improvement. This clinical picture is most characteristic of primary central nervous system lymphoma. If primary central nervous system lymphoma is suspected, it is important to refrain from using corticosteroids prior to biopsy, unless the patients clinical situation absolutely requires it. Corticosteroids exert an oncolytic effect on lymphomas and may render the biopsy nondiagnostic.
Glioblastoma and metastatic carcinoma are unlikely to have such a dramatic radiographic and clinical response to corticosteroids. Although vasculitis or multiple sclerosis may respond rapidly to corticosteroids, neither disorder is likely to develop in a 70-year-old patient, and neither would generally present as a mass lesion on brain imaging.
Neurology:Question 34
The correct answer is E
Educational Objectives
Treat secondary progressive multiple sclerosis (MS).
Critique
Mitoxantrone is approved by the U.S. Food and Drug Administration (FDA) to slow progression of secondary progressive or severe relapsing-remitting MS. The speed of this patient’s decline merits consideration of aggressive immunosuppression. The primary concern about treatment with mitoxantrone is that dose-related cardiotoxicity prohibits use beyond a cumulative dose of 120 to 140 mg/m2.
No benefit has been shown for chronic prednisone treatment and this is not recommended. Bone marrow transplantation is investigational only. There is some evidence that plasmapheresis is useful for acute devastating attacks of demyelination that do not respond quickly to standard intravenous corticosteroid treatment, but there is little evidence of benefit for progressive disease. Glatiramer acetate can reduce the frequency of relapses in patients with relapsing-remitting MS, but is not indicated for progressive disease. Results are inconsistent concerning whether interferon beta slows secondary progressive MS. Other agents that have been used for secondary progressive disease include cyclophosphamide, intravenous immunoglobulin, azathioprine, and methotrexate, although none are FDA approved for this purpose.
The correct answer is E
Educational Objectives
Treat secondary progressive multiple sclerosis (MS).
Critique
Mitoxantrone is approved by the U.S. Food and Drug Administration (FDA) to slow progression of secondary progressive or severe relapsing-remitting MS. The speed of this patient’s decline merits consideration of aggressive immunosuppression. The primary concern about treatment with mitoxantrone is that dose-related cardiotoxicity prohibits use beyond a cumulative dose of 120 to 140 mg/m2.
No benefit has been shown for chronic prednisone treatment and this is not recommended. Bone marrow transplantation is investigational only. There is some evidence that plasmapheresis is useful for acute devastating attacks of demyelination that do not respond quickly to standard intravenous corticosteroid treatment, but there is little evidence of benefit for progressive disease. Glatiramer acetate can reduce the frequency of relapses in patients with relapsing-remitting MS, but is not indicated for progressive disease. Results are inconsistent concerning whether interferon beta slows secondary progressive MS. Other agents that have been used for secondary progressive disease include cyclophosphamide, intravenous immunoglobulin, azathioprine, and methotrexate, although none are FDA approved for this purpose.
Neurology:Question 35
The correct answer is A
Educational Objectives
Select prophylaxis for migraine headache.
Critique
This patient has frequent attacks of migraine without aura. She is at risk for developing rebound headaches from overuse of acute medication. Accordingly, a preventive medication is indicated. Amitriptyline is a first-line agent for migraine prophylaxis; its sedating effect may also help her insomnia. Nadolol and propranolol are contraindicated because they may trigger her asthma. Naratriptan is used for treatment of acute migraine and is not approved for prophylactic therapy. Selective serotonin reuptake inhibitors have limited effectiveness in migraine prophylaxis.
The correct answer is A
Educational Objectives
Select prophylaxis for migraine headache.
Critique
This patient has frequent attacks of migraine without aura. She is at risk for developing rebound headaches from overuse of acute medication. Accordingly, a preventive medication is indicated. Amitriptyline is a first-line agent for migraine prophylaxis; its sedating effect may also help her insomnia. Nadolol and propranolol are contraindicated because they may trigger her asthma. Naratriptan is used for treatment of acute migraine and is not approved for prophylactic therapy. Selective serotonin reuptake inhibitors have limited effectiveness in migraine prophylaxis.
Neurology:Question 36
The correct answer is B
Educational Objectives
Determine when to withdraw antiepileptic drug treatment.
Critique
According to recent studies of adult patients who are seizure free for more than 2 years, approximately one third relapse following termination of antiepileptic drugs. Risk factors associated with recurrence of seizures include prolonged duration and high frequency of seizures before they were controlled, abnormal neurologic examination, abnormal magnetic resonance imaging (MRI) of the brain, consistently abnormal electroencephalograms (EEG5), and mental retardation. This patient has consistently abnormal EEGs. He should continue antiepilepsy treatment despite having been seizure free for 4 years. Ordering another EEG and MRI would not change the outcome. Single photon emission computed tomography (SPECT) is not helpful when clinical history, EEG, or MRI provide sufficient localization. Changing to another agent such as carbamazepine would be appropriate if the patient had side effects from phenytoin.
The correct answer is B
Educational Objectives
Determine when to withdraw antiepileptic drug treatment.
Critique
According to recent studies of adult patients who are seizure free for more than 2 years, approximately one third relapse following termination of antiepileptic drugs. Risk factors associated with recurrence of seizures include prolonged duration and high frequency of seizures before they were controlled, abnormal neurologic examination, abnormal magnetic resonance imaging (MRI) of the brain, consistently abnormal electroencephalograms (EEG5), and mental retardation. This patient has consistently abnormal EEGs. He should continue antiepilepsy treatment despite having been seizure free for 4 years. Ordering another EEG and MRI would not change the outcome. Single photon emission computed tomography (SPECT) is not helpful when clinical history, EEG, or MRI provide sufficient localization. Changing to another agent such as carbamazepine would be appropriate if the patient had side effects from phenytoin.
Neurology:Question 37
The correct answer is C
Educational Objectives
Recognize the typical clinical presentation of cerebral meningioma.
Critique
This 50-year-old patient has a chronic progressive history of a scalp/skull mass and symptoms of raised intracranial pressure. Computed tomography demonstrates an extra-axial calcified enhancing mass with overlying skull hyperostosis, a classic picture for meningioma. Meningiomas derive from the arachnoid granulations of the dura, hence their usual occurrence over the cerebral convexities, skull base, or falx cerebri. Calcification is typical and reflects the slow-growing, chronic nature of the tumor. Meningiomas often invade adjacent bone and may produce a palpable or visible hyperostotic mass. Meningiomas most commonly affect middle-aged and elderly women.
Gliomas, including glioblastoma multiforme, are intra-axial tumors that involve the parenchyma of the brain and not the dural surface. Dural metastases are by definition malignant tumors and usually present more acutely. They do not calcify. While chronic subdural hematomas may calcify, they usually do not enhance, nor do they produce hyperostosis of the overlying bone.
The correct answer is C
Educational Objectives
Recognize the typical clinical presentation of cerebral meningioma.
Critique
This 50-year-old patient has a chronic progressive history of a scalp/skull mass and symptoms of raised intracranial pressure. Computed tomography demonstrates an extra-axial calcified enhancing mass with overlying skull hyperostosis, a classic picture for meningioma. Meningiomas derive from the arachnoid granulations of the dura, hence their usual occurrence over the cerebral convexities, skull base, or falx cerebri. Calcification is typical and reflects the slow-growing, chronic nature of the tumor. Meningiomas often invade adjacent bone and may produce a palpable or visible hyperostotic mass. Meningiomas most commonly affect middle-aged and elderly women.
Gliomas, including glioblastoma multiforme, are intra-axial tumors that involve the parenchyma of the brain and not the dural surface. Dural metastases are by definition malignant tumors and usually present more acutely. They do not calcify. While chronic subdural hematomas may calcify, they usually do not enhance, nor do they produce hyperostosis of the overlying bone.
Neurology:Question 38
The correct answer is B
Educational Objectives
Treat an elderly patient with a large cerebral infarction.
Critique
Intravenous tissue plasminogen activator (t-PA) decreases the risk of disability in patients with ischemic stroke when administered within 3 hours of stroke onset (National Institute of Neurological Disorders and Stroke [NINDS] protocol). Although older patients have a higher risk of intracerebral hemorrhage than younger patients, persons within the same age group who receive intravenous t-PA fare better than those who do not. The same is true for patients with large cerebral infarctions (for example, infarcts involving more than one third of the middle cerebral artery territory). At this time, there is insufficient evidence to recommend intra-arterial over intravenous t-PA within 3 hours of stroke onset, and intra-arterial t-PA is not currently approved by the U.S. Food and Drug Administration for ischemic stroke. The NINDS protocol forbids administration of aspirin or any other antithrombotic agent within 24 hours after t-PA administration. To date, all clinical trials assessing emergent administration of aspirin plus thrombolysis in stroke patients have shown poor outcomes.
The correct answer is B
Educational Objectives
Treat an elderly patient with a large cerebral infarction.
Critique
Intravenous tissue plasminogen activator (t-PA) decreases the risk of disability in patients with ischemic stroke when administered within 3 hours of stroke onset (National Institute of Neurological Disorders and Stroke [NINDS] protocol). Although older patients have a higher risk of intracerebral hemorrhage than younger patients, persons within the same age group who receive intravenous t-PA fare better than those who do not. The same is true for patients with large cerebral infarctions (for example, infarcts involving more than one third of the middle cerebral artery territory). At this time, there is insufficient evidence to recommend intra-arterial over intravenous t-PA within 3 hours of stroke onset, and intra-arterial t-PA is not currently approved by the U.S. Food and Drug Administration for ischemic stroke. The NINDS protocol forbids administration of aspirin or any other antithrombotic agent within 24 hours after t-PA administration. To date, all clinical trials assessing emergent administration of aspirin plus thrombolysis in stroke patients have shown poor outcomes.
Neurology:Question 39
The correct answer is A
Educational Objectives
Manage status epilepticus.
Critique
The patient has already received a full dose of lorazepam (0.1 mg/kg). The most appropriate next step is to administer a full loading dose of fosphenytoin or phenytoin (20 mg/kg of body weight; the phenytoin equivalence in option C is too low). Fosphenytoin is preferred over phenytoin because it can be administered faster than phenytoin and has a lower incidence of hypotension and local thrombophlebitis at the site of administration (“purple glove syndrome’). Intravenous infusion of fosphenytoin (ordered in phenytoin-equivalent doses) should be initiated while lorazepam is still being administered because it takes approximately 15 minutes for fosphenytoin to take effect. If there is no risk of respiratory suppression (patient is intubated) and the patient is hemodynamically stable, additional doses of lorazepam (beyond a full dose) can be given if necessary. Appropriate loading doses (mg/kg of body weight) of phenobarbital (after intubation) or valproic acid can be administered if the patient continues to have seizures despite treatment with lorazepam and fosphenytoin. The common practice of routinely giving 1 gram of phenytoin may result in under- or overdosing. Phenytoin should be administered according to patient weight.
The correct answer is A
Educational Objectives
Manage status epilepticus.
Critique
The patient has already received a full dose of lorazepam (0.1 mg/kg). The most appropriate next step is to administer a full loading dose of fosphenytoin or phenytoin (20 mg/kg of body weight; the phenytoin equivalence in option C is too low). Fosphenytoin is preferred over phenytoin because it can be administered faster than phenytoin and has a lower incidence of hypotension and local thrombophlebitis at the site of administration (“purple glove syndrome’). Intravenous infusion of fosphenytoin (ordered in phenytoin-equivalent doses) should be initiated while lorazepam is still being administered because it takes approximately 15 minutes for fosphenytoin to take effect. If there is no risk of respiratory suppression (patient is intubated) and the patient is hemodynamically stable, additional doses of lorazepam (beyond a full dose) can be given if necessary. Appropriate loading doses (mg/kg of body weight) of phenobarbital (after intubation) or valproic acid can be administered if the patient continues to have seizures despite treatment with lorazepam and fosphenytoin. The common practice of routinely giving 1 gram of phenytoin may result in under- or overdosing. Phenytoin should be administered according to patient weight.
Neurology:Question 40
The correct answer is D
Educational Objectives
Recognize dementia with Lewy bodies.
Critique
The patient manifests the cardinal features of dementia with Lewy bodies, including cognitive impairment, fluctuating symptoms, hallucinations, and parkinsonism. Patients who have dementia with Lewy bodies often also have a cholinergic deficit, as suggested in this case by the episode of confusion associated with an anticholinergic medication. The fluctuating and psychiatric symptoms warrant treatment. Treatment with a cholinesterase inhibitor, such as donepezil, may be beneficial.
Mild cognitive impairment is not an appropriate diagnosis, because the disorder is severe enough to impair his daily function, and because the deficits go beyond memory impairment. A primary diagnosis of Alzheimers disease is not appropriate because fluctuating sensorium and parkinsonism are not core features of this disease, as they are in dementia with Lewy bodies. However, a component of Alzheimer pathology is commonly superimposed on the pathology of dementia with Lewy bodies. For similar reasons, a primary diagnosis of frontotemporal dementia is also not appropriate.
The correct answer is D
Educational Objectives
Recognize dementia with Lewy bodies.
Critique
The patient manifests the cardinal features of dementia with Lewy bodies, including cognitive impairment, fluctuating symptoms, hallucinations, and parkinsonism. Patients who have dementia with Lewy bodies often also have a cholinergic deficit, as suggested in this case by the episode of confusion associated with an anticholinergic medication. The fluctuating and psychiatric symptoms warrant treatment. Treatment with a cholinesterase inhibitor, such as donepezil, may be beneficial.
Mild cognitive impairment is not an appropriate diagnosis, because the disorder is severe enough to impair his daily function, and because the deficits go beyond memory impairment. A primary diagnosis of Alzheimers disease is not appropriate because fluctuating sensorium and parkinsonism are not core features of this disease, as they are in dementia with Lewy bodies. However, a component of Alzheimer pathology is commonly superimposed on the pathology of dementia with Lewy bodies. For similar reasons, a primary diagnosis of frontotemporal dementia is also not appropriate.
Neurology:Question 41
The correct answer is A
Educational Objectives
Distinguish chorea from other hyperkinetic movement disorders.
Critique
Rapidly flowing, dancing-like involuntary movements occurring in a chaotic, nonstereotyped fashion define chorea. The differential diagnosis for chorea includes disorders that are inherited (for example,Huntingtons
Chorea does not occur in Parkinsons disease unless provoked by medications (he is taking none). The 3-year course makes Creutzfeldt-Jakob disease unlikely. Tourettes syndrome starts earlier in life with stereotyped movements (tics). This patient’s adventitious movements are chaotic and not stereotyped like tics.
The correct answer is A
Educational Objectives
Distinguish chorea from other hyperkinetic movement disorders.
Critique
Rapidly flowing, dancing-like involuntary movements occurring in a chaotic, nonstereotyped fashion define chorea. The differential diagnosis for chorea includes disorders that are inherited (for example,
Chorea does not occur in Parkinsons disease unless provoked by medications (he is taking none). The 3-year course makes Creutzfeldt-Jakob disease unlikely. Tourettes syndrome starts earlier in life with stereotyped movements (tics). This patient’s adventitious movements are chaotic and not stereotyped like tics.
Neurology:Question 42
The correct answer is D
Educational Objectives
Classify course of multiple sclerosis.
Critique
This woman’s course began with acute exacerbations followed by remissions of variable duration, so her disease was initially relapsing-remitting. However, in the past year, she has had progression of neurologic deficits between acute relapses. In at least 50% of patients with relapsing-remitting multiple sclerosis, disease will evolve to a secondary progressive course, such as in this case. Even a single remote attack followed a decade later by the onset of progression is classified as a secondary progressive course.
Monosymptomatic demyelination refers to a single clinical episode of demyelination, such as isolated optic neuritis. Relapsing-remitting disease refers to multiple attacks of demyelination. Primary progressive multiple sclerosis starts as an insidious spinal cord dysfunction or as ataxia, progressing without distinct acute exacerbations; it tends to have fewer cerebral lesions (as seen on magnetic resonance imaging) than other forms of multiple sclerosis. Benign multiple sclerosis refers to a course in which all neurologic systems are fully functional 15 years after disease onset.
The correct answer is D
Educational Objectives
Classify course of multiple sclerosis.
Critique
This woman’s course began with acute exacerbations followed by remissions of variable duration, so her disease was initially relapsing-remitting. However, in the past year, she has had progression of neurologic deficits between acute relapses. In at least 50% of patients with relapsing-remitting multiple sclerosis, disease will evolve to a secondary progressive course, such as in this case. Even a single remote attack followed a decade later by the onset of progression is classified as a secondary progressive course.
Monosymptomatic demyelination refers to a single clinical episode of demyelination, such as isolated optic neuritis. Relapsing-remitting disease refers to multiple attacks of demyelination. Primary progressive multiple sclerosis starts as an insidious spinal cord dysfunction or as ataxia, progressing without distinct acute exacerbations; it tends to have fewer cerebral lesions (as seen on magnetic resonance imaging) than other forms of multiple sclerosis. Benign multiple sclerosis refers to a course in which all neurologic systems are fully functional 15 years after disease onset.
Neurology:Question 43
The correct answer is D
Educational Objectives
Manage symptomatic extracranial carotid atherosclerosis.
Critique
Sudden right hemiparesis with expressive aphasia suggests a left cortical infarction in the territory of the left middle cerebral artery, which is supplied by the left internal carotid artery. Carotid stenosis of 50% or more that is ipsilateral to the infarction implies that the stroke is due to large artery disease. Carotid endarterectomy decreases risk of future stroke in patients with symptomatic stenosis of 50% or more, and especially in patients with 70% to 99% stenosis, as compared with antiplatelet agents alone. Aspirin, 81 or 325 mg daily, decreases the risk of stroke after endarterectomy. There is no evidence that intravenous heparin is safe and effective in patients with acute ischemic stroke.
The correct answer is D
Educational Objectives
Manage symptomatic extracranial carotid atherosclerosis.
Critique
Sudden right hemiparesis with expressive aphasia suggests a left cortical infarction in the territory of the left middle cerebral artery, which is supplied by the left internal carotid artery. Carotid stenosis of 50% or more that is ipsilateral to the infarction implies that the stroke is due to large artery disease. Carotid endarterectomy decreases risk of future stroke in patients with symptomatic stenosis of 50% or more, and especially in patients with 70% to 99% stenosis, as compared with antiplatelet agents alone. Aspirin, 81 or 325 mg daily, decreases the risk of stroke after endarterectomy. There is no evidence that intravenous heparin is safe and effective in patients with acute ischemic stroke.
Neurology:Question 44
The correct answer is D
Educational Objectives
Manage small, asymptomatic meningiomas.
Critique
This 75-year-old woman has what is most probably a small, asymptomatic convexity meningioma. The calcification suggests that the meningioma has been present for months or years. Calcified meningiomas are less likely to progress than noncalcified lesions. In this setting, the appropriate management is follow-up imaging in 6 months to assess the stability of the lesion, with consideration of further follow-up at increasing intervals if no growth is seen. If the lesion enlarges or becomes symptomatic, then surgical resection is indicated, assuming the patient is a suitable candidate. Biopsy and radiosurgery are not appropriate for patients with incidental, asymptomatic meningioma. In autopsy series, small, asymptomatic meningiomas have been found in 1% to 2% of individuals.
The correct answer is D
Educational Objectives
Manage small, asymptomatic meningiomas.
Critique
This 75-year-old woman has what is most probably a small, asymptomatic convexity meningioma. The calcification suggests that the meningioma has been present for months or years. Calcified meningiomas are less likely to progress than noncalcified lesions. In this setting, the appropriate management is follow-up imaging in 6 months to assess the stability of the lesion, with consideration of further follow-up at increasing intervals if no growth is seen. If the lesion enlarges or becomes symptomatic, then surgical resection is indicated, assuming the patient is a suitable candidate. Biopsy and radiosurgery are not appropriate for patients with incidental, asymptomatic meningioma. In autopsy series, small, asymptomatic meningiomas have been found in 1% to 2% of individuals.
Neurology:Question 45
The correct answer is D
Educational Objectives
Recognize the persistent vegetative state.
Critique
In this patient, severe cerebral anoxia from her cardiac arrest led to diffuse cortical injury with relative preservation of brainstem function, and the development of a vegetative state. A vegetative state is a clinical condition of complete unawareness of self or the environment, accompanied by sleep-wake cycles and preservation of brainstem and hypothalamic autonomic functions. According to the conclusions of a multi-society task force, persistent vegetative state is diagnosed if a vegetative state is present 1 month after acute traumatic or nontraumatic brain injury. Unlike a vegetative state, in which there is no evidence of consciousness, in the minimally conscious state there is severely altered consciousness with minimal but definite behavioral evidence of self or environmental awareness. In the locked-in syndrome, patients are conscious because of normal cortical and upper brainstem function, but are quadriplegic and can communicate only through the ability to move their eyes vertically and blink; this syndrome is caused by lesions at the base of the pons. Brain death is the complete absence of all cerebral hemispheric and brainstem functions, including the absence of respiratory drive.
The correct answer is D
Educational Objectives
Recognize the persistent vegetative state.
Critique
In this patient, severe cerebral anoxia from her cardiac arrest led to diffuse cortical injury with relative preservation of brainstem function, and the development of a vegetative state. A vegetative state is a clinical condition of complete unawareness of self or the environment, accompanied by sleep-wake cycles and preservation of brainstem and hypothalamic autonomic functions. According to the conclusions of a multi-society task force, persistent vegetative state is diagnosed if a vegetative state is present 1 month after acute traumatic or nontraumatic brain injury. Unlike a vegetative state, in which there is no evidence of consciousness, in the minimally conscious state there is severely altered consciousness with minimal but definite behavioral evidence of self or environmental awareness. In the locked-in syndrome, patients are conscious because of normal cortical and upper brainstem function, but are quadriplegic and can communicate only through the ability to move their eyes vertically and blink; this syndrome is caused by lesions at the base of the pons. Brain death is the complete absence of all cerebral hemispheric and brainstem functions, including the absence of respiratory drive.
Neurology:Question 46
The correct answer is A
Educational Objectives
Treat optic neuritis.
Critique
This patient has signs and symptoms consistent with left optic neuritis. Although most patients have good recovery in 6 months with or without treatment, a course of intravenous methylprednisolone (1 g daily for 3 days) followed by an oral prednisone taper speeds recovery of visual acuity in optic neuritis. The decision on whether to use corticosteroids or hold treatment depends on patient preference, severity of the visual deficit, health of the fellow eye, and vocational requirements.
This patient has a single clinical demyelinating event, with no history of previous clinical neurologic events or evidence of silent demyelinating lesions on brain imaging. Therefore, a diagnosis of multiple sclerosis and treatment with disease-modifying therapies (interferon beta or glatiramer acetate) are not warranted. However, surveillance may be appropriate with follow-up magnetic resonance imaging of the brain to watch for development of cerebral white matter lesions. One major study of optic neuritis found that there is no benefit to treatment with oral prednisone alone over no treatment; indeed, the study found an increased risk of optic neuritis in the fellow eye in the next 6 to 12 months with oral prednisone treatment. The presentation is not suggestive of an ischemic event and there is no indication for treatment with aspirin.
The correct answer is A
Educational Objectives
Treat optic neuritis.
Critique
This patient has signs and symptoms consistent with left optic neuritis. Although most patients have good recovery in 6 months with or without treatment, a course of intravenous methylprednisolone (1 g daily for 3 days) followed by an oral prednisone taper speeds recovery of visual acuity in optic neuritis. The decision on whether to use corticosteroids or hold treatment depends on patient preference, severity of the visual deficit, health of the fellow eye, and vocational requirements.
This patient has a single clinical demyelinating event, with no history of previous clinical neurologic events or evidence of silent demyelinating lesions on brain imaging. Therefore, a diagnosis of multiple sclerosis and treatment with disease-modifying therapies (interferon beta or glatiramer acetate) are not warranted. However, surveillance may be appropriate with follow-up magnetic resonance imaging of the brain to watch for development of cerebral white matter lesions. One major study of optic neuritis found that there is no benefit to treatment with oral prednisone alone over no treatment; indeed, the study found an increased risk of optic neuritis in the fellow eye in the next 6 to 12 months with oral prednisone treatment. The presentation is not suggestive of an ischemic event and there is no indication for treatment with aspirin.
Neurology:Question 47
The correct answer is D
Educational Objectives
Manage complex partial seizures.
Critique
This patients episodes are most likely complex partial seizures of frontal lobe origin. Frontal lobe
seizures are brief, usually occur in clusters, and usually are not associated with aura or postictal confusion. Appropriate anticonvulsant management should be initiated, even during the diagnostic work-up. Carbamazepine is approved by the U.S. Food and Drug Administration (FDA) for treatment of partial seizures and is an appropriate initial choice. Phenobarbital is not a first-line agent because of possible associated sedation and cognitive impairment.
The correct answer is D
Educational Objectives
Manage complex partial seizures.
Critique
This patients episodes are most likely complex partial seizures of frontal lobe origin. Frontal lobe
seizures are brief, usually occur in clusters, and usually are not associated with aura or postictal confusion. Appropriate anticonvulsant management should be initiated, even during the diagnostic work-up. Carbamazepine is approved by the U.S. Food and Drug Administration (FDA) for treatment of partial seizures and is an appropriate initial choice. Phenobarbital is not a first-line agent because of possible associated sedation and cognitive impairment.
Gabapentin is approved for adjunctive treatment of partial seizures but should not be used in monotherapy. Ethosuximide is used to treat absence seizures, which this patient does not have. Lamotrigine is FDA approved for adjunctive treatment of partial seizures; many neurologists use it with success as second-line monotherapy.
Neurology:Question 48
The correct answer is C
Educational Objectives
Treat essential tremor.
Critique
This patient has a postural and action tremor (essential tremor), rather than a resting tremor typical of Parkinson’s disease. First-line drugs include primidone or a nonselective β-adrenergic blocker, such as propranolol. Selective β1 blockers, such as atenolol, are not effective against tremor, at least in the lower doses where the β1 effect is predominant. As the clinical description is not that of parkinsonian tremor, carbidopa/levodopa or pramipexole would not be helpful. Vaiproic acid may induce tremor and is not used to treat it.
The correct answer is C
Educational Objectives
Treat essential tremor.
Critique
This patient has a postural and action tremor (essential tremor), rather than a resting tremor typical of Parkinson’s disease. First-line drugs include primidone or a nonselective β-adrenergic blocker, such as propranolol. Selective β1 blockers, such as atenolol, are not effective against tremor, at least in the lower doses where the β1 effect is predominant. As the clinical description is not that of parkinsonian tremor, carbidopa/levodopa or pramipexole would not be helpful. Vaiproic acid may induce tremor and is not used to treat it.
Neurology:Question 49
The correct answer is A
Educational Objectives
Prescribe an angiotensin-converting enzyme (ACE) inhibitor and a diuretic for patients who have had stroke or transient ischemic attack, regardless of baseline blood pressure.
Critique
In PROGRESS, patients with a recent stroke or transient ischemic attack (TIA) had decreased risk of future stroke, heart attack, or death if they took the ACE inhibitor perindopril and the diuretic indapamide—even if they had no previous history of hypertension or if their pretreatment blood pressure levels were in the so-called ‘normotensive” range. However, it is best not to lower blood pressure aggressively soon after a stroke in order to preserve the penumbra and because cerebral autoregulation is dysfunctional. In PROGRESS, low-dose perindopril was begun 2 weeks after the stroke, and gradual increases in antihypertensive therapy were made at 2-week intervals. The results of recent clinical outcome-based trials such as PROGRESS have made it increasingly clear that optimal target blood pressure levels are lower than we previously believed. The target blood pressure for patients with diabetes or end-organ damage, including stroke patients, is 130/80 mm Hg. There are no data regarding the efficacy of β -blockers or calcium channel blockers for secondary stroke prevention.
The correct answer is A
Educational Objectives
Prescribe an angiotensin-converting enzyme (ACE) inhibitor and a diuretic for patients who have had stroke or transient ischemic attack, regardless of baseline blood pressure.
Critique
In PROGRESS, patients with a recent stroke or transient ischemic attack (TIA) had decreased risk of future stroke, heart attack, or death if they took the ACE inhibitor perindopril and the diuretic indapamide—even if they had no previous history of hypertension or if their pretreatment blood pressure levels were in the so-called ‘normotensive” range. However, it is best not to lower blood pressure aggressively soon after a stroke in order to preserve the penumbra and because cerebral autoregulation is dysfunctional. In PROGRESS, low-dose perindopril was begun 2 weeks after the stroke, and gradual increases in antihypertensive therapy were made at 2-week intervals. The results of recent clinical outcome-based trials such as PROGRESS have made it increasingly clear that optimal target blood pressure levels are lower than we previously believed. The target blood pressure for patients with diabetes or end-organ damage, including stroke patients, is 130/80 mm Hg. There are no data regarding the efficacy of β -blockers or calcium channel blockers for secondary stroke prevention.
Neurology:Question 50
The correct answer is E
Educational Objectives
Select the appropriate medication for acute treatment of migraine.
Critique
This patient has episodic severe migraine without aura. Zolmitriptan and other triptans are more likely to be effective for severe migraine headaches than nonspecific agents such as combination analgesics and nonsteroidal anti-inflammatory drugs. Nonspecific agents may be effective for mild to moderate headaches.
The correct answer is E
Educational Objectives
Select the appropriate medication for acute treatment of migraine.
Critique
This patient has episodic severe migraine without aura. Zolmitriptan and other triptans are more likely to be effective for severe migraine headaches than nonspecific agents such as combination analgesics and nonsteroidal anti-inflammatory drugs. Nonspecific agents may be effective for mild to moderate headaches.
Neurology:Question 51
The correct answer is B
Educational Objectives
Recognize the association of Epstein-Barr infection with primary lymphoma of the central nervous system in immunocompromised patients.
Critique
Primary central nervous system (CNS) lymphoma develops in approximately 2% of patients with HIV infection, usually late in the course of infection. Unlike the lesions of immunocompetent patients with primary CNS lymphoma, the lesions in HIV-infected patients often manifest central necrosis with a ring-enhancing appearance that is difficult to distinguish from CNS infections such as toxoplasmosis. Nearly all primary CNS lymphomas in HIV-infected individuals contain Epstein-Barr viral genome. Cerebrospinal fluid polymerase chain reaction is typically negative for Epstein-Barr virus. In the presence of reduced T-cell function, the Epstein-Barr-infected B cells become immortalized and are driven toward a monoclonal malignant B-cell population. Epstein-Barr viral genome is rare in primary CNS lymphoma in immunocompetent patients. Patients with primary CNS lymphoma may be treated with high-dose methotrexate and brain radiation. Unfortunately, treatment of primary CNS lymphoma in patients with HIV is rarely successful, and most patients die within 6 months of diagnosis (usually from complications of other opportunistic infections). Cytomegalovirus, herpes zoster, and herpes simplex may infect the brain of an immunocompromised patient, but none is associated with CNS lymphoma.
The correct answer is B
Educational Objectives
Recognize the association of Epstein-Barr infection with primary lymphoma of the central nervous system in immunocompromised patients.
Critique
Primary central nervous system (CNS) lymphoma develops in approximately 2% of patients with HIV infection, usually late in the course of infection. Unlike the lesions of immunocompetent patients with primary CNS lymphoma, the lesions in HIV-infected patients often manifest central necrosis with a ring-enhancing appearance that is difficult to distinguish from CNS infections such as toxoplasmosis. Nearly all primary CNS lymphomas in HIV-infected individuals contain Epstein-Barr viral genome. Cerebrospinal fluid polymerase chain reaction is typically negative for Epstein-Barr virus. In the presence of reduced T-cell function, the Epstein-Barr-infected B cells become immortalized and are driven toward a monoclonal malignant B-cell population. Epstein-Barr viral genome is rare in primary CNS lymphoma in immunocompetent patients. Patients with primary CNS lymphoma may be treated with high-dose methotrexate and brain radiation. Unfortunately, treatment of primary CNS lymphoma in patients with HIV is rarely successful, and most patients die within 6 months of diagnosis (usually from complications of other opportunistic infections). Cytomegalovirus, herpes zoster, and herpes simplex may infect the brain of an immunocompromised patient, but none is associated with CNS lymphoma.
Neurology:Question 52
The correct answer is A
Educational Objectives
Treat a patient with subarachnoid hemorrhage.
Critique
This patient has a subarachnoid hemorrhage, likely due to aneurysm rupture. In terms of the hemorrhage, the most important intervention at this time is to initiate treatment with nimodipine. Nimodipine helps reduce subsequent development of vasospasm and has been shown to improve outcome in clinical trials. Other antihypertensive medications, such as labetalol and enalapril, are not necessary because nimodipine has an antihypertensive effect. There is no role for corticosteroids in patients with subarachnoid hemorrhage.
The correct answer is A
Educational Objectives
Treat a patient with subarachnoid hemorrhage.
Critique
This patient has a subarachnoid hemorrhage, likely due to aneurysm rupture. In terms of the hemorrhage, the most important intervention at this time is to initiate treatment with nimodipine. Nimodipine helps reduce subsequent development of vasospasm and has been shown to improve outcome in clinical trials. Other antihypertensive medications, such as labetalol and enalapril, are not necessary because nimodipine has an antihypertensive effect. There is no role for corticosteroids in patients with subarachnoid hemorrhage.
Neurology:Question 53
The correct answer is B
Educational Objectives
Diagnose mononeuropathy multiplex.
Critique
This patient has a right peroneal mononeuropathy and a left ulnar mononeuropathy. By definition, this is a case of multiple mononeuropathies (mononeuritis multiplex). Mononeuritis multiplex is usually due to vascular infarction of multiple nerves. Causes of mononeuritis multiplex include diabetes (unlikely in this case because of the normal fasting glucose), connective tissue diseases (such as systemic lupus erythematosus or rheumatoid arthritis), polyarteritis nodosa, Wegeners granulomatosis, HIV infection, and isolated peripheral nervous system vasculitis. The systemic findings in this case suggest connective tissue disease or systemic necrotizing vasculitis.
The effects of amyloidosis on the peripheral nervous system typically involve the autonomic nerves and may be associated with bilateral carpal tunnel syndrome. Guillain-Barré syndrome causes acute symmetric limb or bulbar weakness often preceded by sensory symptoms, but would not cause multiple asymmetric mononeuropathies, as is the case in this patient.
The correct answer is B
Educational Objectives
Diagnose mononeuropathy multiplex.
Critique
This patient has a right peroneal mononeuropathy and a left ulnar mononeuropathy. By definition, this is a case of multiple mononeuropathies (mononeuritis multiplex). Mononeuritis multiplex is usually due to vascular infarction of multiple nerves. Causes of mononeuritis multiplex include diabetes (unlikely in this case because of the normal fasting glucose), connective tissue diseases (such as systemic lupus erythematosus or rheumatoid arthritis), polyarteritis nodosa, Wegeners granulomatosis, HIV infection, and isolated peripheral nervous system vasculitis. The systemic findings in this case suggest connective tissue disease or systemic necrotizing vasculitis.
The effects of amyloidosis on the peripheral nervous system typically involve the autonomic nerves and may be associated with bilateral carpal tunnel syndrome. Guillain-Barré syndrome causes acute symmetric limb or bulbar weakness often preceded by sensory symptoms, but would not cause multiple asymmetric mononeuropathies, as is the case in this patient.
Neurology:Question 54
The correct answer is D
Educational Objectives
Recognize juvenile myoclonic epilepsy.
Critique
The patient had myoclonic jerks that led to a generalized tonic-clonic seizure. This is a typical presentation of juvenile myoclonic epilepsy, an idiopathic epilepsy syndrome. Affected patients have a normal neurologic examination and normal development. Unlike other idiopathic epilepsies, remission is rare even after years without a seizure, so lifelong treatment is necessary. Patients are sensitive to sleep deprivation, alcohol, and stress.
Unlike juvenile myoclonic epilepsy, which is a primary, generalized epilepsy, partial seizures originate from a focal part of the brain, and their clinical presentation depends on their neuroanatomic location. Absence seizures mainly affect children. They manifest with sudden onset of staring, with or without eye blinking or lip smacking. Patients with syncope often have several myoclonic jerks while unconscious, but they recover quickly once blood pressure is reestablished. Nonepileptic events (pseudoseizures) vary in presentation, but they are often associated with moaning, crying, and arrhythmic, on-and-off shaking of the body.
The correct answer is D
Educational Objectives
Recognize juvenile myoclonic epilepsy.
Critique
The patient had myoclonic jerks that led to a generalized tonic-clonic seizure. This is a typical presentation of juvenile myoclonic epilepsy, an idiopathic epilepsy syndrome. Affected patients have a normal neurologic examination and normal development. Unlike other idiopathic epilepsies, remission is rare even after years without a seizure, so lifelong treatment is necessary. Patients are sensitive to sleep deprivation, alcohol, and stress.
Unlike juvenile myoclonic epilepsy, which is a primary, generalized epilepsy, partial seizures originate from a focal part of the brain, and their clinical presentation depends on their neuroanatomic location. Absence seizures mainly affect children. They manifest with sudden onset of staring, with or without eye blinking or lip smacking. Patients with syncope often have several myoclonic jerks while unconscious, but they recover quickly once blood pressure is reestablished. Nonepileptic events (pseudoseizures) vary in presentation, but they are often associated with moaning, crying, and arrhythmic, on-and-off shaking of the body.
Neurology:Question 55
The correct answer is B
Educational Objectives
Assess effect of immunomodulatory therapy on pregnancy.
Critique
Interferon beta is teratogenic in animals; spontaneous abortion is a risk. This patient should be advised to stop interferon beta treatment immediately and see her obstetrician for evaluation and monitoring of fetal development. Women taking disease-modifying treatment for multiple sclerosis should be advised to practice effective contraception and to stop interferon or glatiramer acetate several months before attempting to conceive. There have, however, been successful pregnancies with early exposure to interferon beta.
Pregnancy has a short-term effect on the course of multiple sclerosis. Typically, the frequency of relapse decreases significantly in the third trimester, with a rebound increase in the first 6 months post partum. No studies have demonstrated a long-term effect of pregnancy on disability progression. First-degree relatives of a person with multiple sclerosis have a risk of disease 20 to 50 times greater than that of the general population. However, the transmission of increased susceptibility is believed to be multigenic; no simple one-gene pattern, such as autosomal-recessive transmission, is observed. There is no medical indication for therapeutic abortion in this patient.
The correct answer is B
Educational Objectives
Assess effect of immunomodulatory therapy on pregnancy.
Critique
Interferon beta is teratogenic in animals; spontaneous abortion is a risk. This patient should be advised to stop interferon beta treatment immediately and see her obstetrician for evaluation and monitoring of fetal development. Women taking disease-modifying treatment for multiple sclerosis should be advised to practice effective contraception and to stop interferon or glatiramer acetate several months before attempting to conceive. There have, however, been successful pregnancies with early exposure to interferon beta.
Pregnancy has a short-term effect on the course of multiple sclerosis. Typically, the frequency of relapse decreases significantly in the third trimester, with a rebound increase in the first 6 months post partum. No studies have demonstrated a long-term effect of pregnancy on disability progression. First-degree relatives of a person with multiple sclerosis have a risk of disease 20 to 50 times greater than that of the general population. However, the transmission of increased susceptibility is believed to be multigenic; no simple one-gene pattern, such as autosomal-recessive transmission, is observed. There is no medical indication for therapeutic abortion in this patient.
Neurology:Question 56
The correct answer is D
Educational Objectives
Diagnose hemicrania continua.
Critique
Hemicrania continua is a primary headache disorder characterized by chronic, daily unilateral headache with some autonomic phenomena. Indomethacin is effective in managing the disorder; other preventive agents are usually ineffective. Cluster headaches last 1 to 2 hours and predominantly occur in men. Chronic tension-type headaches are usually bilateral and do not have associated autonomic phenomena. Chronic migraine is typically preceded by years of episodic migraine.
The correct answer is D
Educational Objectives
Diagnose hemicrania continua.
Critique
Hemicrania continua is a primary headache disorder characterized by chronic, daily unilateral headache with some autonomic phenomena. Indomethacin is effective in managing the disorder; other preventive agents are usually ineffective. Cluster headaches last 1 to 2 hours and predominantly occur in men. Chronic tension-type headaches are usually bilateral and do not have associated autonomic phenomena. Chronic migraine is typically preceded by years of episodic migraine.
Neurology:Question 57
The correct answer is B
Educational Objectives
Assess iron levels in patients with restless legs syndrome.
Critique
This patients description is typical of restless legs syndrome. Because restless legs syndrome may occasionally be a manifestation of iron deficiency, it is appropriate to check iron levels. If deficiency is identified, iron supplementation may resolve symptoms. Otherwise, restless legs syndrome usually responds to a dopamine agonist medication (such as pergolide, pramipexole, or ropinirole) or gabapentin. Carbidopa/levodopa is effective against restless legs syndrome, but it is second-line therapy because of an associated rebound phenomenon (‘augmentation’).
Typically, obtaining the serum ferritin level is an adequate work-up if the clinical picture fits with restless legs syndrome, the neurologic examination is normal, and there are no major medical problems. Hence, magnetic resonance imaging of the brain is unnecessary. Serum ceruloplasmin or urine porphyrin levels are not relevant to this clinical presentation. Nerve conduction studies are not helpful in the diagnosis of restless legs syndrome, and this patient has no findings on examination to suggest neuropathy.
The correct answer is B
Educational Objectives
Assess iron levels in patients with restless legs syndrome.
Critique
This patients description is typical of restless legs syndrome. Because restless legs syndrome may occasionally be a manifestation of iron deficiency, it is appropriate to check iron levels. If deficiency is identified, iron supplementation may resolve symptoms. Otherwise, restless legs syndrome usually responds to a dopamine agonist medication (such as pergolide, pramipexole, or ropinirole) or gabapentin. Carbidopa/levodopa is effective against restless legs syndrome, but it is second-line therapy because of an associated rebound phenomenon (‘augmentation’).
Typically, obtaining the serum ferritin level is an adequate work-up if the clinical picture fits with restless legs syndrome, the neurologic examination is normal, and there are no major medical problems. Hence, magnetic resonance imaging of the brain is unnecessary. Serum ceruloplasmin or urine porphyrin levels are not relevant to this clinical presentation. Nerve conduction studies are not helpful in the diagnosis of restless legs syndrome, and this patient has no findings on examination to suggest neuropathy.
Neurology:Question 58
The correct answer is A
Educational Objectives
Recognize Creutzfeldt-Jakob disease and identify the appropriate confirmatory test.
Critique
This patient has signs of a subacute dementing illness. The pace of the illness, normal imaging study, and presence of myoclonus suggest Creutzfeldt-Jakob disease. Treatable inflammatory conditions, such as granulomatous angiitis of the nervous system, are unlikely but remain in the differential diagnosis, and brain biopsy might be required to exclude them. Before considering brain biopsy, a positive diagnosis of Creutzfeldt-Jakob disease can be made if there are periodic sharp waves on the electroencephalogram or if 14-3-3 protein is present in the cerebrospinal fluid. Although the electroencephalogram in this case might evolve over time and become diagnostic, the pace of disease progression makes waiting inadvisable.
Functional imaging can localize dysfunctional tissue, but not characterize it. The presenilin test is indicated only when familial Alzheimer-profile dementia is suspected.
The correct answer is A
Educational Objectives
Recognize Creutzfeldt-Jakob disease and identify the appropriate confirmatory test.
Critique
This patient has signs of a subacute dementing illness. The pace of the illness, normal imaging study, and presence of myoclonus suggest Creutzfeldt-Jakob disease. Treatable inflammatory conditions, such as granulomatous angiitis of the nervous system, are unlikely but remain in the differential diagnosis, and brain biopsy might be required to exclude them. Before considering brain biopsy, a positive diagnosis of Creutzfeldt-Jakob disease can be made if there are periodic sharp waves on the electroencephalogram or if 14-3-3 protein is present in the cerebrospinal fluid. Although the electroencephalogram in this case might evolve over time and become diagnostic, the pace of disease progression makes waiting inadvisable.
Functional imaging can localize dysfunctional tissue, but not characterize it. The presenilin test is indicated only when familial Alzheimer-profile dementia is suspected.
Neurology:Question 59
The correct answer is C
Educational Objectives
Recognize the association of specific tumors with paraneoplastic neurologic disease.
Critique
This patient has paraneoplastic cerebellar degeneration. Paraneoplastic neurologic disorders are rare complications of cancer. In this patient, a structural lesion of the posterior fossa, such as a tumor or infarct, has been appropriately excluded by imaging.
In each of the paraneoplastic syndromes, the systemic tumor cell is believed to express an “onconeural antigen” that produces an immune response in the patient. This onconeural antigen shares similarities to antigens normally expressed by specific neural tissue. The host immune response (cell mediated and humoral) then attacks both the tumor and the specific neural tissue that share antigenic similarity. This theory is strengthened by the fact that patients’ tumors often share antigenic similarities with neural tissue and by the frequent observation that patients with paraneoplastic neurologic disease often have limited or no metastatic disease and small primary tumors. The latter observation suggests that the immune response to tumor may be particularly strong in these patients.
The presence in this patient’s serum of the anti-Yo antibodies is very specific for paraneoplastic cerebellar degeneration in the setting of ovarian, uterine, fallopian tube, or breast cancer. Anti-Hu (anti-neuronal antibody type I) is the antibody associated with small cell lung cancer and paraneoplastic neurologic disease. Paraneoplastic neurologic disorders are very rare in non-Hodgkin’s lymphoma or melanoma, and there is no serologic antibody specifically associated with either tumor.
The correct answer is C
Educational Objectives
Recognize the association of specific tumors with paraneoplastic neurologic disease.
Critique
This patient has paraneoplastic cerebellar degeneration. Paraneoplastic neurologic disorders are rare complications of cancer. In this patient, a structural lesion of the posterior fossa, such as a tumor or infarct, has been appropriately excluded by imaging.
In each of the paraneoplastic syndromes, the systemic tumor cell is believed to express an “onconeural antigen” that produces an immune response in the patient. This onconeural antigen shares similarities to antigens normally expressed by specific neural tissue. The host immune response (cell mediated and humoral) then attacks both the tumor and the specific neural tissue that share antigenic similarity. This theory is strengthened by the fact that patients’ tumors often share antigenic similarities with neural tissue and by the frequent observation that patients with paraneoplastic neurologic disease often have limited or no metastatic disease and small primary tumors. The latter observation suggests that the immune response to tumor may be particularly strong in these patients.
The presence in this patient’s serum of the anti-Yo antibodies is very specific for paraneoplastic cerebellar degeneration in the setting of ovarian, uterine, fallopian tube, or breast cancer. Anti-Hu (anti-neuronal antibody type I) is the antibody associated with small cell lung cancer and paraneoplastic neurologic disease. Paraneoplastic neurologic disorders are very rare in non-Hodgkin’s lymphoma or melanoma, and there is no serologic antibody specifically associated with either tumor.
Neurology:Question 60
The correct answer is C
Educational Objectives
Recognize frontal lobe seizures.
Critique
The patient most likely has simple partial seizures of frontal lobe origin. The clinical presentation of partial seizures depends on their neuroanatomic location. In this case, the seizures originate in Broca’s language area and spread to the primary motor area. Frontal seizures are brief and usually without any aura or postictal confusion. The recent onset of seizures should lead to brain imaging as soon as possible to rule out a space-occupying lesion. Transient ischemic attack, hemiplegic migraine, and hypoglycemia can cause focal neurologic deficits, but would not cause twitching of the face and arm. Anxiety attacks last longer (minutes) and are associated with other symptoms, such as shortness of breath and fear.
The correct answer is C
Educational Objectives
Recognize frontal lobe seizures.
Critique
The patient most likely has simple partial seizures of frontal lobe origin. The clinical presentation of partial seizures depends on their neuroanatomic location. In this case, the seizures originate in Broca’s language area and spread to the primary motor area. Frontal seizures are brief and usually without any aura or postictal confusion. The recent onset of seizures should lead to brain imaging as soon as possible to rule out a space-occupying lesion. Transient ischemic attack, hemiplegic migraine, and hypoglycemia can cause focal neurologic deficits, but would not cause twitching of the face and arm. Anxiety attacks last longer (minutes) and are associated with other symptoms, such as shortness of breath and fear.
Neurology:Question 61
The correct answer is B
Educational Objectives
Diagnose aneurysmal subarachnoid hemorrhage.
Critique
This young patient has sudden onset of severe, debilitating headache without prior history of such events. It is a classic first, worst’ headache scenario that suggests subarachnoid hemorrhage. Further work-up is needed to evaluate for subarachnoid blood. Computed tomography (CT) is sensitive for detecting acute hemorrhage, but its ability to detect subarachnoid hemorrhage is not absolute, and after 3 days, 25% of affected patients have normal CT scans. When subarachnoid hemorrhage is clinically suspected despite a normal CT scan, lumbar puncture is imperative to rule it out. In general, magnetic resonance imaging is inferior to CT for detecting subarachnoid blood. Arteriography is not warranted acutely until the presence of subarachnoid hemorrhage has been established. Abortive treatment for headache should be given only after subarachnoid hemorrhage has been ruled out.
The correct answer is B
Educational Objectives
Diagnose aneurysmal subarachnoid hemorrhage.
Critique
This young patient has sudden onset of severe, debilitating headache without prior history of such events. It is a classic first, worst’ headache scenario that suggests subarachnoid hemorrhage. Further work-up is needed to evaluate for subarachnoid blood. Computed tomography (CT) is sensitive for detecting acute hemorrhage, but its ability to detect subarachnoid hemorrhage is not absolute, and after 3 days, 25% of affected patients have normal CT scans. When subarachnoid hemorrhage is clinically suspected despite a normal CT scan, lumbar puncture is imperative to rule it out. In general, magnetic resonance imaging is inferior to CT for detecting subarachnoid blood. Arteriography is not warranted acutely until the presence of subarachnoid hemorrhage has been established. Abortive treatment for headache should be given only after subarachnoid hemorrhage has been ruled out.
Neurology:Question 62
The correct answer is D
Educational Objectives
Treat acute migraine.
Critique
This patient has migraine. An oral medication will have limited efficacy, because her headaches are accompanied by early nausea and vomiting. Sumatriptan is the only 5HT1 B,D agonist available in a parenteral form and is highly effective. Nasal sumatriptan is another reasonable choice, but it is generally less effective. Subcutaneous or intramuscular dihydroergotamine could also be considered for acute treatment, but intravenous administration is not appropriate in the home setting.
The correct answer is D
Educational Objectives
Treat acute migraine.
Critique
This patient has migraine. An oral medication will have limited efficacy, because her headaches are accompanied by early nausea and vomiting. Sumatriptan is the only 5HT1 B,D agonist available in a parenteral form and is highly effective. Nasal sumatriptan is another reasonable choice, but it is generally less effective. Subcutaneous or intramuscular dihydroergotamine could also be considered for acute treatment, but intravenous administration is not appropriate in the home setting.
Neurology:Question 63
The correct answer is B
Educational Objectives
Diagnose mild cognitive impairment.
Critique
This patients abnormal finding is limited to memory, and he has no impairment of interpersonal, occupational, or daily living activities. Therefore, his condition should be classified as mild cognitive impairment, not dementia. Follow-up evaluation in 1 year is appropriate, as many affected patients may have pre-demential Alzheimers disease. The conversion rate from mild cognitive impairment to mild dementia is 10% to 15% per year.
Although there may be an autosomal-dominant form of Alzheimers disease in this patients family, affected members had disease onset when they were 20 years younger than this patient. Thus, if the patient has Alzheimers disease, it is more likely to be a sporadic than familial case, and genetic testing for presenilin-1 is not likely to be helpful. The specificity of cerebrospinal fluid findings and functional imaging studies (such as positron emission tomography/single photon emission computed tomography [PET/SPECT]) for early dementia has not been determined, so it is unlikely that these tests would be helpful. Diffusion-weighted magnetic resonance imaging is sensitive for acute ischemic stroke and has no role in evaluating degenerative disease.
The correct answer is B
Educational Objectives
Diagnose mild cognitive impairment.
Critique
This patients abnormal finding is limited to memory, and he has no impairment of interpersonal, occupational, or daily living activities. Therefore, his condition should be classified as mild cognitive impairment, not dementia. Follow-up evaluation in 1 year is appropriate, as many affected patients may have pre-demential Alzheimers disease. The conversion rate from mild cognitive impairment to mild dementia is 10% to 15% per year.
Although there may be an autosomal-dominant form of Alzheimers disease in this patients family, affected members had disease onset when they were 20 years younger than this patient. Thus, if the patient has Alzheimers disease, it is more likely to be a sporadic than familial case, and genetic testing for presenilin-1 is not likely to be helpful. The specificity of cerebrospinal fluid findings and functional imaging studies (such as positron emission tomography/single photon emission computed tomography [PET/SPECT]) for early dementia has not been determined, so it is unlikely that these tests would be helpful. Diffusion-weighted magnetic resonance imaging is sensitive for acute ischemic stroke and has no role in evaluating degenerative disease.
Neurology:Question 65
The correct answer is B
Educational Objectives
Use botulinum toxin to treat adult-onset focal dystonia.
Critique
The patient has cervical dystonia (spasmodic torticollis), an idiopathic focal dystonia of adulthood. Although its origins are presumed to be within the brain, imaging studies of the brain are negative, as are studies of the cervical spine. Oral medications for cervical dystonia, including muscle relaxants (diazepam, cyclobenzaprine), are mildly effective at best. Analgesics may be necessary to relieve pain from muscle contraction. The most consistently effective medical treatment is botulinum toxin injected into appropriate neck muscles, sometimes under electromyographic guidance. Within a few days, the injected muscles weaken, tending to release the spasm. Benefits persist for 2 to 4 months, and reinjection every 3 or more months maintains the benefits. Excessive weakness of the injected muscles is rarely a problem.
Although carbidopa/levodopa may improve the dystonia of Parkinson’s disease, it is ineffective in treating primary idiopathic focal dystonias, such as cervical dystonia. Psychiatry referral may improve stress management, but is unlikely to have a major impact on the motor manifestations of cervical dystonia.
The correct answer is B
Educational Objectives
Use botulinum toxin to treat adult-onset focal dystonia.
Critique
The patient has cervical dystonia (spasmodic torticollis), an idiopathic focal dystonia of adulthood. Although its origins are presumed to be within the brain, imaging studies of the brain are negative, as are studies of the cervical spine. Oral medications for cervical dystonia, including muscle relaxants (diazepam, cyclobenzaprine), are mildly effective at best. Analgesics may be necessary to relieve pain from muscle contraction. The most consistently effective medical treatment is botulinum toxin injected into appropriate neck muscles, sometimes under electromyographic guidance. Within a few days, the injected muscles weaken, tending to release the spasm. Benefits persist for 2 to 4 months, and reinjection every 3 or more months maintains the benefits. Excessive weakness of the injected muscles is rarely a problem.
Although carbidopa/levodopa may improve the dystonia of Parkinson’s disease, it is ineffective in treating primary idiopathic focal dystonias, such as cervical dystonia. Psychiatry referral may improve stress management, but is unlikely to have a major impact on the motor manifestations of cervical dystonia.
Neurology:Question 66
The correct answer is A
Educational Objectives
Recognize complex partial seizures of temporal lobe origin.
Critique
This is a typical description of complex partial seizure of temporal lobe origin. The symptoms of complex partial seizures vary, but they are stereotypical for each patient. Patients may have a warning (aura) prior to the seizure. The most common warning is a rising epigastric sensation; other auras may include affective (such as fear), cognitive (such as déjà vu), and sensory symptoms (such as olfactory hallucinations). Complex partial seizures typically last less than 3 minutes. During that time the patient appears awake, but loses contact with the environment and does not respond normally to instructions or questions. Patients usually stare and remain motionless, or engage in repetitive, semi-purposeful behavior (automatisms), such as gesturing, chewing, lip smacking, repeating words or phrases, walking, running, or undressing. Patients do not recall these behaviors. After the seizure the patient is confused or sleepy for minutes to hours.
Absence seizures occur primarily in children, are not preceded by aura, and are not followed by postictal confusion. They last 5 to 10 seconds and occur primarily in clusters. Nonepileptic events (pseudoseizures) are characteristically variable (not stereotypical) in presentation and last longer. Migraine can be associated with neurologic symptoms, including confusion, but there is usually a history of headache or other obvious symptom (for example, visual phenomena). Transient global amnesia is a syndrome of acute memory loss of unknown etiology; it can last for minutes to hours. There is no alteration or loss of consciousness during transient global amnesia.
The correct answer is A
Educational Objectives
Recognize complex partial seizures of temporal lobe origin.
Critique
This is a typical description of complex partial seizure of temporal lobe origin. The symptoms of complex partial seizures vary, but they are stereotypical for each patient. Patients may have a warning (aura) prior to the seizure. The most common warning is a rising epigastric sensation; other auras may include affective (such as fear), cognitive (such as déjà vu), and sensory symptoms (such as olfactory hallucinations). Complex partial seizures typically last less than 3 minutes. During that time the patient appears awake, but loses contact with the environment and does not respond normally to instructions or questions. Patients usually stare and remain motionless, or engage in repetitive, semi-purposeful behavior (automatisms), such as gesturing, chewing, lip smacking, repeating words or phrases, walking, running, or undressing. Patients do not recall these behaviors. After the seizure the patient is confused or sleepy for minutes to hours.
Absence seizures occur primarily in children, are not preceded by aura, and are not followed by postictal confusion. They last 5 to 10 seconds and occur primarily in clusters. Nonepileptic events (pseudoseizures) are characteristically variable (not stereotypical) in presentation and last longer. Migraine can be associated with neurologic symptoms, including confusion, but there is usually a history of headache or other obvious symptom (for example, visual phenomena). Transient global amnesia is a syndrome of acute memory loss of unknown etiology; it can last for minutes to hours. There is no alteration or loss of consciousness during transient global amnesia.
Neurology:Question 67
The correct answer is B
Educational Objectives
Recognize the clinical presentation of Lambert-Eaton myasthenic syndrome.
Critique
This patient has Lambert-Eaton myasthenic syndrome (LEMS), which in 60% of cases occurs in patients harboring small cell lung cancer. LEMS is a disorder of nicotinic and muscarinic cholinergic synapses resulting in impaired acetylcholine release, with muscle weakness and autonomic dysfunction. Affected patients produce antibody to the voltage-gated calcium channel (VGCC), thus impairing calcium influx into the nerve terminal and reducing acetylcholine release. The circulating anti-VGCC antibody is detectable in some but not all patients. Spinal cord compression presents with spine and radicular pain and evidence of myelopathy (signs of upper motor neuron weakness, spasticity, Babinski signs, and sensory loss typically with a defined level). Polymyositis generally produces muscle pain and weakness without symptoms of autonomic nervous system dysfunction. Polyneuropathy is a possibility, although it usually would produce distal more than proximal weakness; the weakness would not improve with repeated effort. Autonomic dysfunction is less commonly associated with polyneu ropathy.
The correct answer is B
Educational Objectives
Recognize the clinical presentation of Lambert-Eaton myasthenic syndrome.
Critique
This patient has Lambert-Eaton myasthenic syndrome (LEMS), which in 60% of cases occurs in patients harboring small cell lung cancer. LEMS is a disorder of nicotinic and muscarinic cholinergic synapses resulting in impaired acetylcholine release, with muscle weakness and autonomic dysfunction. Affected patients produce antibody to the voltage-gated calcium channel (VGCC), thus impairing calcium influx into the nerve terminal and reducing acetylcholine release. The circulating anti-VGCC antibody is detectable in some but not all patients. Spinal cord compression presents with spine and radicular pain and evidence of myelopathy (signs of upper motor neuron weakness, spasticity, Babinski signs, and sensory loss typically with a defined level). Polymyositis generally produces muscle pain and weakness without symptoms of autonomic nervous system dysfunction. Polyneuropathy is a possibility, although it usually would produce distal more than proximal weakness; the weakness would not improve with repeated effort. Autonomic dysfunction is less commonly associated with polyneu ropathy.
Neurology:Question 68
The correct answer is C
Educational Objectives
Treat acute migraine in the emergency department.
Critique
This patient needs parenteral treatment for her prolonged, severe migraine headache that is accompanied by repeated vomiting. The neuroleptic agent chlorpromazine is effective for such a situation. Although dihydroergotamine may be useful when administered intravenously during a severe migraine attack, it should not be used in this situation because the patient has already used rizatriptan within the past 24 hours and both medications have vasoconstrictive effects. Dihydroergotamine may cause nausea and vomiting, so when it can be used intravenously, an antiemetic such as metoclopramide must be administered first. Oral codeine (or any oral agent) is a poor choice since the patient is nauseated and vomiting. Finally, sumatriptan should not be used because the patient has already used rizatriptan within the past 24 hours.
The correct answer is C
Educational Objectives
Treat acute migraine in the emergency department.
Critique
This patient needs parenteral treatment for her prolonged, severe migraine headache that is accompanied by repeated vomiting. The neuroleptic agent chlorpromazine is effective for such a situation. Although dihydroergotamine may be useful when administered intravenously during a severe migraine attack, it should not be used in this situation because the patient has already used rizatriptan within the past 24 hours and both medications have vasoconstrictive effects. Dihydroergotamine may cause nausea and vomiting, so when it can be used intravenously, an antiemetic such as metoclopramide must be administered first. Oral codeine (or any oral agent) is a poor choice since the patient is nauseated and vomiting. Finally, sumatriptan should not be used because the patient has already used rizatriptan within the past 24 hours.
Neurology:Question 69
The correct answer is A
Educational Objectives
Prescribe cholesterol-lowering therapy for secondary prevention of stroke.
Critique
Among patients with known coronary artery disease, statin drugs decrease the risk of ischemic stroke as well as myocardial infarction. The evidence is strongest for pravastatin, 40 mg/d, and simvastatin, 20 to 40 mg/d. Previous studies of lipid-lowering therapy other than statins did not show a decrease in stroke risk (and showed lesser decreases in cholesterol levels). Observational studies suggest that a low-density lipoprotein (LDL) level below 70 mg/dL may increase the risk of hemorrhagic stroke. Patients with diabetes or a history of stroke, coronary artery disease, or peripheral artery disease should take a statin, if at all possible, to achieve a target LDL value between 70 and 100 mg/dL. LDL cholesterol levels decrease in the first 24 to 48 hours after an ischemic event and do not return to baseline for several weeks. It is likely that this patients LDL value of 110 mg/dL in the hospital is lower than his pre-event value, further emphasizing his need for lipid-lowering therapy.
The correct answer is A
Educational Objectives
Prescribe cholesterol-lowering therapy for secondary prevention of stroke.
Critique
Among patients with known coronary artery disease, statin drugs decrease the risk of ischemic stroke as well as myocardial infarction. The evidence is strongest for pravastatin, 40 mg/d, and simvastatin, 20 to 40 mg/d. Previous studies of lipid-lowering therapy other than statins did not show a decrease in stroke risk (and showed lesser decreases in cholesterol levels). Observational studies suggest that a low-density lipoprotein (LDL) level below 70 mg/dL may increase the risk of hemorrhagic stroke. Patients with diabetes or a history of stroke, coronary artery disease, or peripheral artery disease should take a statin, if at all possible, to achieve a target LDL value between 70 and 100 mg/dL. LDL cholesterol levels decrease in the first 24 to 48 hours after an ischemic event and do not return to baseline for several weeks. It is likely that this patients LDL value of 110 mg/dL in the hospital is lower than his pre-event value, further emphasizing his need for lipid-lowering therapy.
Neurology:Question 70
The correct answer is B
Educational Objectives
Recognize treatment options for medically intractable epilepsy.
Critique
This patient has a seizure disorder of right temporal lobe origin. Epilepsy is considered medically intractable when adequate trials of three different antiepileptic drug regimens have not controlled seizures. Excision of the epileptogenic focus is now widely accepted for medically intractable seizure disorders, particularly those of temporal lobe origin.
Vagal nerve stimulation is less efficacious than surgery for medically intractable epilepsy, but should be considered if the patient is not a candidate for temporal lobectomy. Adding a third or fourth antiepileptic drug would probably cause more side effects without significantly improving seizure control. Positron emission tomography is not necessary, because there is enough clinical, electroencephalographic, and radiographic evidence that the patients seizures originate from the right temporal lobe. Repeat magnetic resonance imaging of the brain is not necessary, since mesial temporal sclerosis does not usually progress, and there is no correlation between the degree of sclerosis and seizure frequency.
The correct answer is B
Educational Objectives
Recognize treatment options for medically intractable epilepsy.
Critique
This patient has a seizure disorder of right temporal lobe origin. Epilepsy is considered medically intractable when adequate trials of three different antiepileptic drug regimens have not controlled seizures. Excision of the epileptogenic focus is now widely accepted for medically intractable seizure disorders, particularly those of temporal lobe origin.
Vagal nerve stimulation is less efficacious than surgery for medically intractable epilepsy, but should be considered if the patient is not a candidate for temporal lobectomy. Adding a third or fourth antiepileptic drug would probably cause more side effects without significantly improving seizure control. Positron emission tomography is not necessary, because there is enough clinical, electroencephalographic, and radiographic evidence that the patients seizures originate from the right temporal lobe. Repeat magnetic resonance imaging of the brain is not necessary, since mesial temporal sclerosis does not usually progress, and there is no correlation between the degree of sclerosis and seizure frequency.
Neurology:Question 72
The correct answer is D
Educational Objectives
Use immunomodulatory therapy in multiple sclerosis.
Critique
This patient has clinically definite relapsing-remitting multiple sclerosis. The current consensus of the National Multiple Sclerosis Society is that all patients with definite relapsing multiple sclerosis be considered for treatment with either a form of interferon beta or glatiramer acetate. These immunomodulatory agents decrease the frequency of relapse and the rate of accumulation of new lesions on magnetic resonance imaging.
High-dose intravenous methylprednisolone (for example, 500 to 1000 mg daily for 3 to 5 days) is standard treatment for a significant acute exacerbation of multiple sclerosis. Periodic pulses of high-dose corticosteroids are also given to some patients with progressive disease. However, this treatment is not indicated for patients who are stable between attacks. Chronic oral prednisone is not used in multiple sclerosis, as there is no indication that its benefits outweigh its toxicity. Azathioprine was used to treat relapsing multiple sclerosis in the past, but since the development of interferon, it is used only as an investigational therapy for progressive multiple sclerosis.
The correct answer is D
Educational Objectives
Use immunomodulatory therapy in multiple sclerosis.
Critique
This patient has clinically definite relapsing-remitting multiple sclerosis. The current consensus of the National Multiple Sclerosis Society is that all patients with definite relapsing multiple sclerosis be considered for treatment with either a form of interferon beta or glatiramer acetate. These immunomodulatory agents decrease the frequency of relapse and the rate of accumulation of new lesions on magnetic resonance imaging.
High-dose intravenous methylprednisolone (for example, 500 to 1000 mg daily for 3 to 5 days) is standard treatment for a significant acute exacerbation of multiple sclerosis. Periodic pulses of high-dose corticosteroids are also given to some patients with progressive disease. However, this treatment is not indicated for patients who are stable between attacks. Chronic oral prednisone is not used in multiple sclerosis, as there is no indication that its benefits outweigh its toxicity. Azathioprine was used to treat relapsing multiple sclerosis in the past, but since the development of interferon, it is used only as an investigational therapy for progressive multiple sclerosis.
Neurology:Question 73
The correct answer is D
Educational Objectives
Diagnose idiopathic intracranial hypertension.
Critique
Idiopathic intracranial hypertension most commonly occurs in obese young women and is usually manifested by an unremitting headache; there may be associated visual obscurations. The neurologic examination is typically normal except for papilledema and enlarged blind spots. Its most serious long-term complication is vision loss. The elevated cerebrospinal fluid opening pressure could be due to hydrocephalus, but this diagnosis is excluded by the normal findings on magnetic resonance imaging of the brain. There is no spinal fluid pleocytosis, so meningitis is unlikely in this patient. Chronic migraine is not associated with elevated cerebrospinal fluid pressure and papilledema. The patient is not using medication at a frequency that would lead to medication overuse (analgesic rebound) headache, nor is medication overuse headache associated with evidence of increased intracranial pressure.
The correct answer is D
Educational Objectives
Diagnose idiopathic intracranial hypertension.
Critique
Idiopathic intracranial hypertension most commonly occurs in obese young women and is usually manifested by an unremitting headache; there may be associated visual obscurations. The neurologic examination is typically normal except for papilledema and enlarged blind spots. Its most serious long-term complication is vision loss. The elevated cerebrospinal fluid opening pressure could be due to hydrocephalus, but this diagnosis is excluded by the normal findings on magnetic resonance imaging of the brain. There is no spinal fluid pleocytosis, so meningitis is unlikely in this patient. Chronic migraine is not associated with elevated cerebrospinal fluid pressure and papilledema. The patient is not using medication at a frequency that would lead to medication overuse (analgesic rebound) headache, nor is medication overuse headache associated with evidence of increased intracranial pressure.
Neurology:Question 74
The correct answer is B
Educational Objectives
Recognize status epilepticus.
Critique
This patient’s clinical presentation is consistent with partial status epilepticus. The patient has been having complex partial seizures of right frontal lobe origin without recovery of baseline mental status between seizures. The seizure in the hospital was probably a partial seizure with secondary generalization due to her stroke. She requires urgent neurologic consultation and urgent electroencephalography to confirm partial status epilepticus. The patient’s neurologic examination is consistent with the previous history of right hemispheric stroke. The worsening of the left-sided weakness is more likely due to the frequent seizures (Todd’s paralysis) than to stroke in evolution. Phenytoin toxicity can worsen a previous neurologic deficit, but is unlikely to cause more seizures. Creutzfeldt-Jakob disease is a subacute, degenerative disorder characterized by rapidly progressive dementia, ataxia, and myoclonic jerks; it is unlikely in this patient.
The correct answer is B
Educational Objectives
Recognize status epilepticus.
Critique
This patient’s clinical presentation is consistent with partial status epilepticus. The patient has been having complex partial seizures of right frontal lobe origin without recovery of baseline mental status between seizures. The seizure in the hospital was probably a partial seizure with secondary generalization due to her stroke. She requires urgent neurologic consultation and urgent electroencephalography to confirm partial status epilepticus. The patient’s neurologic examination is consistent with the previous history of right hemispheric stroke. The worsening of the left-sided weakness is more likely due to the frequent seizures (Todd’s paralysis) than to stroke in evolution. Phenytoin toxicity can worsen a previous neurologic deficit, but is unlikely to cause more seizures. Creutzfeldt-Jakob disease is a subacute, degenerative disorder characterized by rapidly progressive dementia, ataxia, and myoclonic jerks; it is unlikely in this patient.
Neurology:Question 75
The correct answer is A
Educational Objectives
Recognize medication-induced cognitive dysfunction.
Critique
The patient’s altered level of consciousness and asterixis suggest a toxic/metabolic etiology of the impairment. The patient is on several medications that might impair cognition. Nortriptyline has anticholinergic properties, and is especially likely to cause impairment in patients with latent cholinergic deficiency (for example, patients with early dementia or Parkinson’s disease). The sedative-hypnotic lorazepam and digoxin may also contribute to cognitive impairment.
Dementia with Lewy bodies is also associated with fluctuation of cognitive status and visual hallucinations, but is less likely given the medication history, absence of parkinsonism, and presence of asterixis. Vascular dementia, particularly that due to small-vessel cerebrovascular disease, is unlikely given the absence of a stepwise course and absence of stroke on computed tomography. Depression may cause chronic cognitive impairment (pseudodementia), but not asterixis and an altered level of consciousness.
The correct answer is A
Educational Objectives
Recognize medication-induced cognitive dysfunction.
Critique
The patient’s altered level of consciousness and asterixis suggest a toxic/metabolic etiology of the impairment. The patient is on several medications that might impair cognition. Nortriptyline has anticholinergic properties, and is especially likely to cause impairment in patients with latent cholinergic deficiency (for example, patients with early dementia or Parkinson’s disease). The sedative-hypnotic lorazepam and digoxin may also contribute to cognitive impairment.
Dementia with Lewy bodies is also associated with fluctuation of cognitive status and visual hallucinations, but is less likely given the medication history, absence of parkinsonism, and presence of asterixis. Vascular dementia, particularly that due to small-vessel cerebrovascular disease, is unlikely given the absence of a stepwise course and absence of stroke on computed tomography. Depression may cause chronic cognitive impairment (pseudodementia), but not asterixis and an altered level of consciousness.
