75. The answer is c. (Swaiman, pp 1019-1033.) This is a common presentation for primary generalized epilepsy of childhood. An EEG showing the classic 3 Hz spike-and-wave pattern would confirm this diagnosis. 1-2 Hz would be consistent with severe neurological dysfunction and symptomatic generalized epilepsy. Brain MRI would be normal or show an incidental finding. Anatomic problems can cause seizures, but these tests will not provide any information about brain electrical activity. Lumbar puncture is useful for measuring cerebrospinal fluid (CSF) pressure and looking for central nervous system (CNS) inflammation or infection. CNS inflammation or infection may cause seizures. Conners Rating Scale is useful for the evaluation of inattention and hyperactivity.
76. The answer is b. (Ropper, pp 304-335.) Lack of sleep is a common seizure trigger. There is no reason to believe that the patient faked the seizure. It is impossible to predict his future seizure course based on this one event; having one seizure does not necessarily mean that his seizures are getting worse, and even if they are, many treatments are available. There is no reason for the patient to take a prolonged leave of absence from school because of one seizure. This may even have detrimental psychological consequences. Other common seizure triggers include missing antiseizure medication doses, drugs (prescription or illicit), and psychological stress.
77. The answer is c. (Ropper, pp 304-335.) At one time the most popular benzodiazepine for use in status epilepticus was diazepam, which has a rapid onset of action in the brain, but is redistributed in the body relatively quickly. Because of this property, patients needed additional medication to protect themselves from recurrent seizure activity as early as 20 minutes after diazepam injection. A less fat-soluble benzodiazepine, lorazepam, has the advantage of acting rapidly but being cleared more slowly from the brain.
78. The answer is e. (Ropper, pp 304-335.) Rapid infusion of phenytoin may produce a cardiac arrhythmia or hypotension. Phenytoin should not be administered at rates greater than 50 mg/minute in adults or 1 mg/(kg-min) in children to reduce the chances of this reaction occurring. Thus it usually requires approximately 20 minutes to administer a 1000-to 1500-mg standard loading dose of phenytoin in an emergent setting such as status epilepticus. Fosphenytoin, a water-soluble prodrug of phenytoin, has the advantage of causing fewer infusion site reactions. It can be given at doses of up to 150 mg/minute in an adult, with risks of cardiac dysrhythmia similar to those of phenytoin. Another advantage of fosphenytoin is that it can be administered intramuscularly when intravenous access is problematic. Carbamazepine is not administered intravenously at all. Rapid infusion of phenobarbital may produce hypotension or respiratory arrest, but is much less likely to depress cardiac activity. Diazepam and clonazepam are safer than phenobarbital, but rapid infusion of excessively high doses may depress blood pressure and other autonomic functions.
79. The answer is b. (Fauci, pp 2504-2506.) This history is typical of a simple partial seizure. A focal brain lesion must be ruled out. It would be wrong to discharge the patient to follow up in clinic in 2 weeks without at least a CT scan and preferably an MRI. Although he probably had a seizure, obtaining an EEG at this point will not be as helpful as an MRI. The diagnosis of seizure has already been made clinically, now the etiology should be investigated. This is unlikely to be a peripheral nerve problem, and therefore an orthopedic consult or EMG and NCS are not indicated.
80. The answer is a. (Ropper, pp 1135-1139.) This is a typical example of alcohol withdrawal seizure. The greatest risk for alcohol withdrawal seizures occurs within the first day after drinking cessation, in contrast to delirium tremens, which usually occurs within 2-to-4 days of drinking cessation. By 7-to-14 days all signs of withdrawal have largely resolved.
81. The answer is b. (Swaiman, pp 1031-1033.) Lennox-Gastaut syndrome is characterized by mental dysfunction, multiple seizure types, and 1-to 2-Hz generalized spike-wave discharges on EEG. It is often difficult to control the seizures that develop in children with this syndrome. Many affected children have a history of infantile spasms (West syndrome). Infants and children with infantile spasms exhibit paroxysmal flexions of the body, waist, or neck and usually have a profoundly disorganized EEG pattern called hypsarrhythmia. Landau-Kleffner syndrome is associated with loss of language function and an abnormal EEG during sleep. Juvenile myoclonic epilepsy is a relatively benign epilepsy syndrome with onset in late adolescence or early adulthood. Febrile seizures are generalized tonic-clonic seizures associated with fever in a child who is otherwise healthy.
82. The answer is d. (Ropper, pp 310-313.) Many patients with complex partial seizures have a preseizure phenomenon (the aura) that alerts them to an impending seizure. The aura is in fact a simple partial seizure. This patient’s aura includes an olfactory hallucination, which is usually associated with lesions of the mesial temporal lobe, particularly the hippocampus or parahippocampal gyrus. Diseases that can affect that region include tumors, trauma, and MTS. Hippocampus means “seahorse” in Latin.
83. The answer is e. (Ropper, pp 864-865.) There is evidence that prophylactic phenytoin reduces the incidence of seizures after head injury. Because early posttraumatic seizures may lead to increased morbidity and prolonged hospital stays, it is reasonable in some situations to treat patients prophylactically. There is no evidence that prophylactic treatment reduces the long-term (months-to-years later) risk of developing posttraumatic epilepsy.
84. The answer is a. (Patten, p 334.) The most common complication of temporal lobectomy is a visual field defect caused by interruption of fibers from the optic tracts passing over the temporal horn of the lateral ventricles. Superior quadrantanopsia is more common than hemianopsia. Some deficits may improve if the injury does not completely damage the nerves. Language deficits, particularly dysnomia, occur less frequently. Hemiparesis is uncommon (< 2%), because the surgery is performed at a distance from the motor fibers of the corticospinal tract. Other neurological problems that can occur include diplopia caused by extraocular nerve deficits and facial paresis.
85. The answer is a. (Ropper, pp 310-313.) The history and MRI are typical for MTS. The arrow in the MRI is specifically pointing at the sclerotic right hippocampus. This is the most common cause of intractable complex partial seizures in adults. The prognosis for improved seizure control with additional medications is poor; however, surgical resection of the right anterior temporal lobe may produce seizure freedom in up to 80% of cases. If this patient had a high-grade malignant brain tumor, he would probably die within 2 years. A cerebral angiogram may confirm the diagnosis of a vascular malformation.
86. The answer is f. (Ropper, pp 309-310.) With a Jacksonian march, or sequential seizure, the patient develops focal seizure activity that is primarily motor and spreads. This type of seizure often secondarily generalizes, at which point the patient loses consciousness and may have a generalized tonic-clonic seizure. The hand is a common site for the start of a Jacksonian march. The face may be involved early because the thumb and the mouth are situated near each other on the motor strip of the cerebral cortex.
87. The answer is j. (Ropper, pp 97, 98, 308.) Myoclonic seizures may be generalized or partial. They are most commonly seen in the epilepsy syndrome called benign juvenile myoclonic epilepsy (BJME). Unlike sleep myoclonus, the episodes occur when the affected person wakes up rather than when he or she is falling asleep. Myoclonic jerks may be triggered by light flashes or loud sounds. Benign juvenile myoclonic epilepsy accounts for 4% of all cases of epilepsy. More than half of those with BJME have generalized tonic-clonic seizures as well as myoclonic seizures.
88. The answer is a. (Ropper, pp 305-308.) With generalized tonic-clonic seizures, the EEG develops abnormalities all over the cortex simultaneously. The patient may recall a strange sensation before the attack, but it is equally likely that no premonitory sign or aura will occur. Partial seizures may secondarily generalize to this type of seizure. If the patient has frequent generalized tonic-clonic seizures, he or she will be at high risk for a variety of injuries, such as dislocated shoulders, broken bones, and head trauma. Patients with this type of seizure always lose consciousness during the attack and may be confused for minutes or hours after the ictus, the most obvious segment of the seizure.
89. The answer is c. (Ropper, pp 310-313.) Complex partial seizures may be mistaken for a psychiatric problem, especially if the partial seizures do not generalize and produce tonic-clonic seizures. This patient has a typical aura involving an unpleasant smell or taste. These were once called uncinate fits, because they were ascribed to abnormal activity in the uncus of the temporal lobe. Complex partial seizures may arise from a focus of abnormal electrical activity in the temporal lobe, but they do not invariably arise from a temporal lobe focus.
90. The answer is h. (Ropper, pp 304, 307, 332-334.) Status epilepticus is defined as a seizure that lasts continuously for 30 minutes or a series of seizures over a 30-minute period without the patient regaining full consciousness between them. Status epilepticus constitutes a medical emergency, because the longer the seizures last, the worse the morbidity and mortality. Complications of status epilepticus include respiratory failure, aspiration, acidosis, hypotension, rhabdomyolysis, renal failure, and cognitive impairment.
91. The answer is d. (Ropper, pp 314-315.) Epilepsia partialis continua refers to a condition of persistent focal motor seizure activity—in essence, a focal motor status epilepticus. The distal hand and foot muscles are most frequently affected. Active or passive movement of the limb may exacerbate the seizure activity. The seizures may persist for hours or for months. The response to therapy is often poor.
92. The answer is f. (Ropper, p 332.) This young woman is having complex partial seizures without secondary generalization. She has episodic altered consciousness associated with a temporal lobe seizure focus and antedated by febrile seizures. Levetiracetam is the best choice because of its relatively good efficacy and adverse effect profile. Felbamate, phenobarbital, primidone, and divalproex sodium may also be effective at controlling the seizures, but all have side effect profiles making them poor first choices in this case.
93. The answer is i. (Ropper, pp 313-314.) This child has West syndrome, a generalized seizure disorder of infants characterized by recurrent spasms, the EEG pattern of hypsarrhythmia, and retardation. Several different diseases cause West syndrome. The family history in this case suggests tuberous sclerosis as the underlying problem. ACTH is the best of the given choices.
94. The answer is g. (Ropper, pp 307-308.) This girl has generalized absence attacks. This may be a manifestation of a more complex epilepsy syndrome or may occur as an isolated finding. Generalized absence attacks have no aura and no postictal period. The affected child has no warning that an attack is about to occur and is usually unaware that one has occurred unless it is more than a few seconds long. In fact, generalized absence seizures are most often only a few seconds long. Ethosuximide is the drug of choice, but it may cause gastrointestinal distress. Divalproex sodium is effective in many of the children who cannot tolerate ethosuximide or who are not well controlled on that antiepileptic. If the absence seizures are associated with generalized tonic-clonic seizures, divalproex sodium is a better choice. Some antiepileptic drugs, such as the sodium channel blockers phenytoin and carbamazepine, can actually worsen generalized from onset seizures.
95. The answer is b. (Ropper, pp 823-824.) Recent studies have established that magnesium sulfate (MgSO4) is the optimal treatment both to prevent seizures in women with hypertension at the time of admission for delivery (preeclampsia) and to treat seizures in established eclampsia. The dose is 4-to-5 g intravenously, followed by a 1 g/h intravenous infusion. Magnesium sulfate was shown to result in a reduction in recurrent seizures and in maternal morbidity and mortality compared with both diazepam and phenytoin. In addition, the fetus should be delivered as quickly as possible, using caesarean section if necessary. Eclampsia is an example of the posterior leukoencephalopathy syndrome.