Epilepsy Comprehensive.pdf
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5 Adult and Pediatric Epilepsy and Sleep Questions 1. If an antiepileptic drug (AED) is required in pregnancy, which of the following medications would be the best choice in terms of safety...
5 Adult and Pediatric Epilepsy and Sleep Questions 1. If an antiepileptic drug (AED) is required in pregnancy, which of the following medications would be the best choice in terms of safety evidence? a. Phenytoin b. Carbamazepine c. Lamotrigine d. Valproic acid e. Phenobarbital 2. Which of the following AEDs is associated with weight loss? a. Pregabalin b. Gabapentin c. Topiramate d. Carbamazepine e. Valproic acid 3. A 36-year-old man with a history of herpes simplex virus (HSV) infection presents with new-onset epilepsy that you suspect is due to his prior encephalitis affecting the temporal lobes. The clinical features of seizures in mesial temporal lobe epilepsy may have all of the following characteristic symptoms, except: 351 a. Olfactory hallucinations b. Altered consciousness c. Complex partial seizures d. Automatisms e. Tonic posturing of one limb (fencer’s posture) 4. Which of the following AEDs would have the least drug– drug interactions? a. Gabapentin b. Carbamazepine c. Valproic acid d. Lamotrigine e. Phenytoin 5. In a young child with generalized epilepsy refractory to multiple antiepileptic medications, what would be the next best choice of treatment, if tolerated? a. Corpus callosotomy b. Ketogenic diet c. Vagus nerve stimulation d. Phenytoin e. Carbamazepine 6. Which of the following is the best treatment option for simple febrile seizures (FSs)? a. Intravenous (IV) lorazepam b. Rectal diazepam c. Supportive management d. Phenobarbital e. Intranasal midazolam 7. Mutations in which of the following genes is most commonly associated with generalized epilepsy with febrile seizures plus (GEFS+)? a. SCN1A b. SCN1C c. SCN1B 352 d. SCN2A e. GABRD 8. A 13-year-old girl is being evaluated for epilepsia partialis continua. Rasmussen’s syndrome is suspected. What would be the most common finding on brain MRI in Rasmussen’s syndrome? a. Lissencephaly b. Schizencephaly c. Cortical atrophy d. Pachygyria e. Porencephaly 9. A 12-year-old boy presents to your office with a history of progressively worsening frequency and severity of daily myoclonic seizures. His mitochondrial testing has so far been negative, although you suspect a progressive myoclonic epilepsy (PME) of some type. What would be the best antiepileptic medication to try first, given there are no contraindications? a. Carbamazepine b. Phenytoin c. Oxcarbazepine d. Valproic acid e. Gabapentin 10. Which of the following choices is a characteristic associated with fosphenytoin as compared with phenytoin? a. Does not cause dizziness or nystagmus b. More cardiovascular side effects c. Achieves therapeutic plasma concentrations faster d. Faster rate of IV administration is possible e. More infiltration reactions (purple glove syndrome) 11. Which of the following is the least likely to be associated with worsening of myoclonic seizures? 353 a. Topiramate b. Carbamazepine c. Lamotrigine d. Pregabalin e. Vigabatrin 12. Which of the following antiepileptic medications is a hepatic enzyme inhibitor? a. Phenytoin b. Carbamazepine c. Valproic acid d. Phenobarbital e. Primidone 13. Which of the following electroencephalographic findings would be associated with the highest incidence of seizures? a. Small sharp spikes b. 6-Hz spike and wave c. Wicket spikes d. 3-Hz spike and wave e. 14 and 6 positive spikes 14. At what age do human beings attain the predominant α- frequency (posterior background) that is seen in adults? a. 6 to 8 years b. 8 to 10 years c. 10 to 12 years d. 12 to 14 years e. 14 to 16 years 15. A 47-year-old woman presents with confusion, fever, and seizures. Cerebrospinal fluid (CSF) studies are positive for HSV infection. What would be the most likely finding on an electroencephalogram (EEG) in this patient? a. Triphasic waves b. Wicket spikes 354 c. Periodic lateralized epileptiform discharges d. Polyspikes e. Fast spike–wave complexes 16. In a patient with absence epilepsy, which of the following antiepileptic medications is least likely to precipitate absence status epilepticus? a. Phenytoin b. Topiramate c. Carbamazepine d. Lamotrigine e. Gabapentin 17. Which of the following antiepileptic medications is the most likely to have an effect on steroid hormone concentration in the blood in patients taking oral contraceptive pills and, therefore, lead to contraceptive failure? a. Levetiracetam b. Gabapentin c. Topiramate (dose 3-Hz) spike– wave and polyspike and wave discharges on the EEG. Interictal EEG is normal. His seizures are well controlled on valproic acid and his development has been normal. 367 Which of the following is the most likely diagnosis? a. Severe myoclonic epilepsy of infancy (Dravet’s syndrome) b. Ohtahara’s syndrome c. Benign myoclonic epilepsy of infancy d. Generalized epilepsy with febrile seizures plus (GEFS+) e. Myoclonic–astatic epilepsy (Doose’s syndrome) 51. A newborn is being evaluated for seizures, which are characterized by apneic spells associated with unilateral or bilateral clonic movements. Starting on day 5 of life, he has been having multiple spells per day. His neurologic examination is otherwise normal in between seizures. His interictal EEG is normal. Which of the following is the most likely diagnosis? a. West’s syndrome b. Benign neonatal seizures c. Aicardi’s syndrome d. Ohtahara’s syndrome e. Benign myoclonic epilepsy of infancy 52. A 4-year-old boy presents for evaluation of spells. Apparently, the episodes begin with some visual phenomena, which he cannot describe, followed by eye deviation and vomiting. He has had a total of three of these spells. His EEG shows a normal background with high-voltage occipital spikes, which disappear with eye opening. Which of the following is the most likely diagnosis? a. Ohtahara’s syndrome b. Late-onset or Gastaut-type childhood occipital epilepsy c. Early-onset or Panayiotopoulos-type childhood occipital epilepsy d. Dravet’s syndrome e. Doose’s syndrome 53. A 3-year-old boy with cognitive and developmental delay is brought for evaluation of his seizures. He began having 368 drop attacks about a year ago but progressively has developed multiple seizure types, including absences, tonic seizures, and clonic seizures. Multiple antiepileptic agents have been tried with mild improvement, but he still has multiple seizures per day. His EEG shows 2-Hz spike–wave discharges. Which of the following is the most likely diagnosis? a. Panayiotopoulos syndrome b. West’s syndrome c. Landau–Kleffner syndrome d. Lennox–Gastaut syndrome e. Seizures associated with mesial temporal lobe sclerosis 54. The parents of a 5-year-old boy report that he seems to be more withdrawn over the past several months. One year ago, he began having seizures, initially myoclonic seizures and later GTC seizures. About 9 months ago, he was noticed to have some problems understanding verbal communication, and he now appears to be aphasic. His EEG shows multifocal spikes. Which of the following is the most likely diagnosis? a. Panayiotopoulos syndrome b. West’s syndrome c. Landau–Kleffner syndrome d. Lennox–Gastaut syndrome e. Seizures associated with mesial temporal lobe sclerosis 55. An 11-year-old girl is brought to the epilepsy-monitoring unit for evaluation of possible “pseudoseizures.” Her spells occur only at night and are described as large- amplitude and violent movements of all four limbs and her trunk. Given the hyperkinetic bizarre movements and a normal awake EEG, her seizures were thought to be nonepileptic by a local neurologist. In the epilepsy- monitoring unit, a seizure is captured during non-rapid eye movement (REM) sleep. Which of the following is the most likely diagnosis? 369 a. Electrical status epilepticus during slow-wave sleep (ESES) b. Lennox–Gastaut syndrome c. Landau–Kleffner syndrome d. Autosomal dominant nocturnal frontal lobe epilepsy e. Panayiotopoulos syndrome Questions 56–59 56. A 15-year-old girl has seizures that begin with eye and head deviation toward the left, with subsequent generalization. Where is the most likely location of the seizure focus? a. Hypothalamus b. Right frontal lobe c. Left frontal lobe d. Right temporal lobe e. Left temporal lobe 57. An 18-year-old man has seizures that begin with asymmetric tonic posturing in which his right arm is extended at the elbow with the fist clenched, while the left arm is flexed at the elbow. He subsequently has GTC seizures. Where is the most likely location of the seizure focus? a. Right temporal lobe b. Left temporal lobe c. Right supplementary motor area d. Left supplementary motor area e. Right occipital lobe 58. A 32-year-old woman has seizures in which her left arm becomes dystonic, while she exhibits automatisms with the right arm. Where is the most likely location of the seizure focus? a. Right temporal lobe b. Left temporal lobe c. Left frontal lobe d. Hypothalamus 370 e. Left supplementary motor area 59. A 7-year-old boy has seizures that are characterized by uncontrollable episodes of laughter. Where is the most likely origin of his seizures? a. Hypothalamus b. Right frontal lobe c. Left frontal lobe d. Right temporal lobe e. Left temporal lobe 60. A 56-year-old woman has a history of coronary artery disease, atrial fibrillation, and seizures secondary to a stroke that she suffered last year, well controlled on phenytoin. Two weeks ago, she was diagnosed with a urinary tract infection and treated with trimethoprim– sulfamethoxazole and fluconazole. She is now admitted with unsteady gait and frequent falls. On examination, she is lethargic and dysarthric, with nystagmus and ataxia. Which of the following tests you should order next? a. MRI of the brain b. EEG c. Free and total phenytoin level d. Urine culture e. Computed tomography (CT) of the brain Question 61–62 61. A 2-year-old girl without a significant medical or family history has a generalized seizure lasting 5 minutes in the setting of a fever of 39°C. The patient recovered without any residual neurologic deficit. The mother would like to know the risk of recurrence. Which of the following is not a predictor of recurrence of febrile seizures (FS)? a. Family history of FS b. Age less than 18 months at the time of FS c. Shorter duration of fever prior to FS 371 d. Lower peak temperature at the time of FS e. Complex FS 62. In a patient who suffered a febrile seizure (FS), which of the following choices is the least likely predictor of developing subsequent epilepsy? a. Family history of FS b. Complex FS c. Developmental delay d. Family history of epilepsy e. Neurologic abnormality 63. A 14-year-old boy is brought to the clinic for evaluation. He has had stimulus-sensitive myoclonus noticed about 4 years ago, which has become more frequent lately. Over the past 3 months, he has been having GTC seizures, and he is clumsier and having frequent falls and problems with hand coordination. The neurologic examination demonstrates ataxia. His MR image is unremarkable and the EEG shows generalized spikes and waves. Genetic testing demonstrated an EPM1 gene mutation. Which of the following is the most likely diagnosis? a. Lafora body disease b. Unverricht–Lundborg syndrome c. Sialidosis d. Juvenile myoclonic epilepsy e. Neuronal ceroid lipofuscinosis 64. A 12-year-old boy with a history of migraines has short stature, ataxia, proximal weakness, mild cognitive impairment, deafness, and various types of seizures. He is admitted after a viral illness, becomes dehydrated, and is found to have lactic acidosis. He has been diagnosed with a PME; a muscle biopsy has been obtained and shown in Figure 5.6. Which of the following is a feature of this patient’s condition? a. Mutation affecting cystatin B 372 b. Cherry red spot on the fundoscopic examination c. This is a mitochondrial disorder d. EPM2 A mutation e. Lafora bodies on skin biopsy Figure 5.6 Muscle specimen. (Courtesy of Dr. Richard A. Prayson.. Shown also in color plates. 65. A 21-year-old man presents with progressive myoclonic jerks and GTC seizures. He has also had a mild and gradual onset of gait instability, ataxia, hyperreflexia, and decreased visual acuity, which is worse at night. His fundoscopic examination shows a cherry red spot. Which of the following is the most likely diagnosis? a. Unverricht–Lundborg syndrome b. Lafora body disease c. Sialidosis d. Juvenile myoclonic epilepsy e. Neuronal ceroid lipofuscinosis 66. A 14-year-old boy with progressive cognitive decline and ataxia has a history of myoclonic epilepsy and multiple seizure types. His EEG shows spikes and waves with predominance in the occipital region. A diagnosis was made on the basis of skin biopsy, which showed periodic- acid-Schiff–positive intracellular inclusions. Which of the following is the most likely finding in this patient? 373 a. Mutation affecting cystatin B b. Cherry red spot on fundoscopic examination c. Ragged red fibers on muscle biopsy d. EPM1 mutation e. EPM2 A mutation 67. A 12-year-old boy with epilepsy is being evaluated for surgical treatment of Rasmussen’s encephalitis. He has progressive cognitive decline, left hemiparesis, and left visual field defect, and the EEG shows status epilepticus arising from the right hemisphere. Which of the following is correct regarding this condition? a. There are autoantibodies against a GABA receptor subunit b. The seizures are typically well controlled with AED monotherapy c. Histopathology shows perivascular cuffs of lymphocytes and monocytes, as well as glial nodules in the gray and white matter d. This disease usually affects adults more than children e. Magnetic resonance image typically shows cortical hypertrophy and edema 68. Regarding the use of AEDs in the elderly, which of the following statements is correct? a. The distribution of hydrophilic drugs increases b. The distribution of lipophilic drugs increases c. Hepatic blood flow, bile flow, and protein synthesis increase along with hepatic metabolism d. Renal blood flow and glomerular filtration rate increase e. Gastric acidity may decrease, making weakly basic drugs less easily absorbed and weakly acidic drugs more easily absorbed 69. An 18-year-old woman is diagnosed with juvenile myoclonic epilepsy, and her neurologist plans to start valproic acid for this type of epilepsy. She would like to know about the potential side effects of this medication. Which of the following choices would be the least likely 374 side effect of valproic acid? a. Tremor b. Hepatotoxicity c. Hyperammonemia d. Hyponatremia e. Neural tube defects in children of mothers who take this medication 70. A 67-year-old man with a history of bipolar disorder and epilepsy is brought to the clinic by his wife. Over the past 6 months, since he was started on a new medication, he has become “meaner” and his wife states that she cannot stand him anymore, he yells all the time and has become abusive and very aggressive. Which of the following medications is the patient most likely taking that would explain his behavior? a. Lamotrigine b. Valproic acid c. Carbamazepine d. Levetiracetam e. Phenytoin 71. A 59-year-old man presents for evaluation of partial seizures and is prescribed an antiepileptic agent. About 2 weeks later, he wakes up with a painful red eye and decreased visual acuity. He is diagnosed with acute closed-angle glaucoma. Which of the following is the most likely antiepileptic agent that he was prescribed recently? a. Valproic acid b. Levetiracetam c. Phenytoin d. Lamotrigine e. Topiramate Questions 72–74 72. A 42-year-old man with a history of hyperlipidemia and no prior history of epilepsy is brought to the emergency 375 department after a seizure that witnesses described as generalized tonic–clonic, associated with tongue biting and urinary incontinence. The seizure lasted for less than a minute, and the patient was confused for about 20 minutes after the event. His neurologic examination is normal and nonfocal, and he is afebrile. Which of the following statements is true according to the American Academy of Neurology guidelines for an unprovoked first seizure in adults? a. There is good evidence to support an EEG for first seizure b. There is poor evidence to support CT or MRI for first seizure c. There is good evidence to support the need to order routine blood count, glucose, and electrolytes for first seizure d. There is good evidence to support a toxicology screen for first seizure e. There is good evidence to support a lumbar puncture for first seizure, regardless of fever presence 73. The patient later asks you what his chances are of having a seizure recurrence. Within what period of time beginning from the time of the first seizure is he at highest risk of having a recurrent seizure? a. There is no chance of seizure recurrence for this patient b. 2 years c. 3 years d. 4 years e. 5 years 74. While in the emergency department, the patient did have laboratory studies, a brain MRI, and an EEG performed, all of which were normal. The patient then asks you whether he needs to start an AED. Based on his history, normal examination, and normal studies, and after discussing risks and benefits of AEDs with the patient, you decide not to start an AED at this time. Which of the following statements is true regarding initiation of an AED for an unprovoked first seizure? 376 a. Immediate AED therapy as compared with the delay of treatment pending a second seizure is likely to reduce seizure recurrence in the subsequent 2 years b. Immediate AED therapy as compared with the delay of treatment pending a second seizure is likely to improve quality of life c. Over the longer term (>3 years), immediate AED therapy is likely to improve the prognosis for sustained seizure remission d. The potential AED adverse effects are generally severe and some may be irreversible e. Antiepileptic drug therapy should be recommended in any patient with an unprovoked first seizure, regardless of normal or abnormal results of brain imaging or EEG 75. Which of the following is correct regarding the EEG in Figure 5.7? a. There is poor reactivity of the posterior background b. The patient has an occipital seizure c. The posterior background shows δ-frequencies d. An eye closure is recorded during this EEG page e. The patient is sleeping Figure 5.7 Electroencephalogram. (Courtesy of Dr. Joanna Fong.) 377 76. Which of the following is correct regarding EEG frequencies? a. α-frequency is >13 Hz b. β-frequency is 8 to 13 Hz c. δ-frequency is
