Continuum Questions

Question 1

A 24-year-old man presents with medically intractable seizures since the age of 16. He has a combination of complex partial seizures, characterized by staring and “smelling burning rubber,” and generalized tonic-clonic seizures. He has failed treatment with four anticonvulsants: carbamazepine, phenytoin, lamotrigine, and levetiracetam. An MRI of his brain demonstrates atrophy of the right hippocampal formation. Video-EEG records three of his typical seizures, all of which originate from the anterior right temporal lobe. What is the likelihood that this patient would achieve excellent seizure control (ie, be seizure free, have auras only, or have marked reduction in seizures) with epilepsy surgery?

Answer 1

75%

Question 2

A 65-year-old woman with a history of partial seizures that had been well controlled on phenytoin is evaluated for recurrent seizures. She developed postherpetic neuralgia in the right V2 distribution following a herpes zoster infection 3 months ago. Her MRI shows an old right frontal lobe infarct. Attempts to increase the dose of phenytoin result in drowsiness. Which of the following would be the most appropriate adjunct therapy in this patient?

Answer 2

Pregabalin

In patients with chronic neuropathic pain, pregabalin, gabapentin, oxcarbazepine, and lacosamide are possible options for adjunct treatment of seizures. Topiramate, lamotrigine, and levetiracetam have not yet been proven effective in this setting. Carbamazepine could be helpful but has the risk of drug interactions at the level of hepatic metabolism. F

Question 3

A 30-year-old woman presents with new-onset partial epilepsy and is discovered to have a low-grade glioma. Her seizures persist despite resection of the tumor. In considering anticonvulsant therapy for this patient, which of the following medications is least likely to interact with her oral contraceptive medication?

Answer 3

LVT

Any hepatic enzyme-inducing antiepileptic drug (AED) can accelerate the metabolism of oral contraceptive pills (OCPs)
and render them less effective. Therefore, phenytoin, carbamazepine, and oxcarbazepine should be avoided. Lamotrigine is not metabolized by the hepatic enzymes, but OCPs may significantly reduce the level of lamotrigine. The dose of lamotrigine may need to be adjusted if OCPs are started or stopped during the course of AED treatment. Levetiracetam should be safe to use with OCPs and should not significantly affect drug effectiveness.

Question 4

A 22 year-old-woman with idiopathic generalized epilepsy with generalized tonic-clonic seizures continues to have breakthrough seizures despite use of lamotrigine. Valproate is added to the regimen. Which of the following effects may result from interaction between these drugs?

Answer 4

Lamotrigine toxicity due to inhibition of its glucuronidation.

Valproate inhibits uridine glucosyl transferase, the enzyme that metabolizes lamotrigine. Initiation of valproate therapy may require immediate reduction of the dose of lamotrigine by 50%.

Question 5

A 6-month-old previously healthy boy is evaluated for recurrent bouts of status epilepticus. The first episode occurred 3 months ago during an upper respiratory tract infection and consisted of twitching of the right side of his face and arm that progressed to a generalized tonic-clonic seizure; the whole event lasted approximately 40 minutes. The second, a similar episode lasting approximately 60 minutes, occurred 2 months ago within 24 hours of his routine immunization. A third episode occurred while he was being given a hot bath. It started with left arm twitching and progressed to a generalized tonic-clonic seizure, with a total duration of approximately 70 minutes. Since then, the patient has continued to have episodes of status epilepticus every 2 to 3 weeks. The EEG shows postictal slowing. The MRI of the head and laboratory studies are normal. The episodes decrease in frequency after stiripentol is added to a previously ineffective regime of valproate and clonazepam. This disorder has been linked to a mutation of which of the following genes?

Answer 5

SCN1A, encoding the sodium channel alpha1 subunit.

The history of recurrent, focal-onset, prolonged seizures starting in the first year of life and triggered by fever or other causes of increased body temperature suggests the diagnosis of
Dravet syndrome. Approximately 80% of cases have mutations of the SCN1A gene.

Question 6

A 30-year-old woman presents to the emergency department in convulsive status epilepticus. She has been previously healthy, takes no medications, and uses no recreational drugs. Her spouse reports that she had stayed home from work the previous day because of a flulike illness with headache and mild fever. On examination, her vital signs are temperature 38°C, blood pressure 140/90 mm Hg, and heart rate 100 beats/min. She is given supplemental oxygen, and IV access is secured. The convulsions stop after 4-mg IV lorazepam is administered. She remains unconscious after the convulsions stop, with a nonfocal examination. The brainstem reflexes are intact. No meningismus is present. Serum glucose, electrolytes, aspartate aminotransferase, creatinine, and calcium levels are normal. A noncontrast
CT of the head is negative, and an urgent EEG shows left temporal periodic lateralized epileptiform discharges. Which of the following is the most appropriate next step in management?

Answer 6

Lumbar puncture

he development of status epilepticus in a patient with fever raises immediate concern for encephalitis. In the setting of temporal periodic lateralized epileptiform discharges, the most likely organism is herpes simplex. A patient with this presentation would likely be started on empiric therapy with acyclovir even before proceeding with additional testing. If additional anticonvulsant treatment is required, fosphenytoin would be a better choice than phenobarbital. Neither neuron-specific enolase nor MR angiography would be useful in this setting. IV ceftriaxone is active against bacterial causes of meningitis (eg, pneumococcus and Neisseria meningitidis) and may be started in conjunction with acyclovir in the less likely event of bacterial meningoencephalitis.

Question 7

A 6-year-old boy is evaluated for episodes of sudden-onset behavioral arrest, unresponsiveness, and a blank stare. Which of the following additional
features would most likely argue against the diagnosis of absence seizure and support that of complex partial seizure in this child?

Answer 7

Postictal confusion.

Absence seizures are characterized by sudden behavioral arrest, unresponsiveness, and a blank stare, typically lasting for a few seconds to half a minute. This is referred to as simple absence. Absence seizures may be associated with clonic, tonic, or atonic components, automatisms, and autonomic features, in isolation or in combination (complex absence). The ictal EEG pattern is classically a 3-Hz generalized monomorphic spike and wave with abrupt onset and termination. Unlike the case of complex partial seizures, little or no postictal confusion follows absence seizures.

Question 8

A 12-year-old boy with Lennox-Gastaut syndrome has had medically intractable generalized seizures since the age of 5. Seizure types include atypical absence, tonic, atonic, and tonic-clonic. He currently takes felbamate and rufinamide and has failed multiple other anticonvulsants in the past, including valproic acid, clonazepam, lamotrigine, and topiramate. Because he has fallen frequently as a result of his seizures, he must wear a protective helmet and use a wheelchair for ambulation. Which of the following is most likely to be helpful in reducing the severity of this patient’s seizures?

Answer 8

Corpus callosotomy.

Surgery for symptomatic generalized epilepsy is confined to corpus callosotomy. This procedure will reduce the frequency of epileptic drop attacks and other generalized seizure types. Although a complete callosotomy is the most effective procedure, it carries more operative risk than an anterior two-thirds corpus callosotomy. Potential complications include urinary incontinence, abulia, speech disorder, and difficulty walking. Additional anticonvulsants are unlikely to be helpful given this patient’s history. Focal cortical resection, as with amygdalohippocampectomy, would not be useful in a patient with primary generalized epilepsy. The role of thalamic stimulation remains a subject
of ongoing investigation but has not been proven to be helpful in Lennox-Gastaut syndrome. Vagal nerve stimulation may be useful in reducing seizure frequency in patients with Lennox-Gastaut syndrome but would not be expected to be as useful as corpus callosotomy.

Question 9

A 32-year-old man with temporal lobe seizures and a history of depression and irritability is evaluated for poorly controlled seizures despite maximal tolerated doses of oxcarbazepine. Which of the following would be considered the preferred adjunctive drug for treatment of seizures in this patient?

Answer 9

Lamotrigine

Lamotrigine has demonstrated beneficial efficacy for treatment of depression in epilepsy. Levetiracetam should probably be avoided in patients with a history of irritability. Phenytoin would not be a good choice because of the risk of cumulative side effects. Zonisamide does not have known antidepressant effects. Felbamate has risk of aplastic anemia and hepatitis, so it should only be used when other appropriate medications fail.

Question 10

A 33-year-old previously healthy man is evaluated for recent onset of spells characterized by abrupt onset of pallor, sweating, and palpitations lasting 60 to 90 seconds and recurring 5 to 6 times a day. During the episodes, he remains aware of his environment. He feels lightheaded when the episodes occur in a standing position. The episodes tend to occur early during the morning hours and sometimes awaken him from sleep. Neurologic and general medical examination is normal between the spells. Routine EKG, EEG, MRI of the head, and general laboratory studies are normal. Which of the following tests is most likely to provide a diagnosis of these events?

Answer 10

Video-EEG monitoring.

The stereotyped nature of the spells, their short duration (less than 2 minutes), and the fact that many of them arise from sleep strongly suggest seizures, most likely from the mesial temporal lobe. A normal interictal EEG, particularly if performed without sleep deprivation, does not exclude seizures as a cause of the events. In these cases, long-term video-EEG monitoring may be helpful. Some disorders associated with temporal lobe seizures, such as limbic encephalitis due to potassium channel antibodies, may show no abnormalities on routine MRI. The characteristics of the spells are not typical of orthostatic intolerance, cardiac arrhythmia, pheochromocytoma, or hypoglycemia.

Question 11

A 35-year-old woman presents to the emergency department for evaluation of a 1-minute generalized tonic-clonic seizure occurring during sleep. Her past medical history is remarkable for hypertension, migraine, and a recent urinary tract infection. She has no prior history of seizure or head injury. Her medications include atenolol, lisinopril, sulfamethoxazole, and tramadol, each of which she has been taking daily and at maximal doses. Vital signs are normal. Her general and neurologic examinations are normal. Blood work, including glucose, aspartate aminotransferase, creatinine, electrolytes, and toxicology screen, is normal. An MRI of the brain shows no structural abnormality, and EEG is normal. Which of the following is the most appropriate next step in the management of this patient?

Answer 11

Discontinue tramadol.

his patient has had an acute, apparently unprovoked, generalized seizure. While the seizure may be idiopathic, it is important to consider symptomatic causes. In this patient, neither an obvious structural lesion nor an obvious metabolic abnormality is present. Tramadol has been associated with seizures, particularly in patients who have recently started taking the medication and/or are taking large doses. Tramadol should be discontinued. No clear indication for anticonvulsant medications exists if an acute reversible cause can be identified.

Question 12

A 25-year-old woman presents with several episodes of speech and motor arrest associated with olfactory hallucinations. Her neurologic examination is normal. MRI shows mild left hippocampal atrophy. EEG demonstrates left temporal spike-wave discharges. She has a long history of obesity and bipolar affective disorder with mania and major depression, which has been moderately well controlled on lithium. Which of the following anticonvulsants would be the most appropriate medication for this patient?

Answer 12

Lamotrigine.

This patient has the coexistence of localization epilepsy and psychiatric disease, suggesting that she would benefit most from an anticonvulsant with mood-stabilizing effects and one that would not be likely to worsen her major depression. Valproic acid might be equally useful in this setting, but her obesity problem is more likely to be aggravated by valproic acid than by lamotrigine. Valproic acid, carbamazepine, and lamotrigine all have proven efficacy for the treatment and prevention of manic symptoms. Phenytoin, levetiracetam, and phenobarbital could all worsen her mood disturbance.

Question 13

A 2-week-old girl is evaluated in the emergency department for frequent seizures characterized by brief multifocal jerks, sometimes associated with apnea, occurring 20 to 30 times daily. In between the episodes, she is alert and interactive. Screen for sepsis and metabolic disorder, MRI, and interictal EEG are normal. Her father and paternal aunt had similar episodes that resolved after several months and had no further problems. This disorder has been linked to mutations of which of the following genes?

Answer 13

KCNQ2, encoding a voltage-gated potassium channel.

he clinical picture is that of benign familial neonatal seizures, which have been linked to mutations of the KCNQ2 or KCNQ3 gene, encoding for a voltage-gated potassium channel that has a major role in regulating neuronal excitability.

Question 14

70-year-old woman with a history of osteoporosis presents with newly diagnosed partial epilepsy believed to have resulted from an old right middle cerebral artery infarct. She was started on phenytoin in the emergency department 1 month ago. She is otherwise healthy and takes no medications other than aspirin, simvastatin, and a bisphosphonate. Which of the following anticonvulsants is most appropriate for long-term seizure control in this patient?

Answer 14

Lamotrigine.

ntiepileptic drugs that are inducers of the hepatic cytochrome P450 enzyme system (including phenytoin, phenobarbital, primidone, carbamazepine) increase the metabolism of vitamin D. Valproic acid is an inhibitor of the cytochrome P450 system but is also associated with altered bone metabolism and decreased bone mass. Lamotrigine has not been associated with bone loss and may be a better choice in this patient.

Question 15

A 22-year-old woman with migraine and idiopathic generalized epilepsy well controlled on topiramate 150 mg 2 times a day is prescribed an oral contraceptive agent. Which of the following potential consequences of an interaction between these two drugs should be discussed with the patient?

Answer 15

Contraceptive failure caused by cytochrome (CYP) 3A4 induction by topiramate.

Topiramate at doses higher than 200 mg may induce CYP 3A4 isoenzymes and result in increased metabolism of steroid hormones with consequently reduced contraceptive efficacy.

Question 16

A 33-year-old right-handed man is evaluated for spells characterized by a warm ascending sensation in the epigastrium associated with a sensation
of fear followed by loss of contact with the environment and oromandibular automatisms. During a recorded seizure, his right hand continually rubs his leg and his left arm remains immobile and stiff. The spells last 1 to 2 minutes. He is able to follow commands toward the end of the seizure. Which of the following is the most likely location of the ictal discharge?

Answer 16

Right mesial temporal.

The presence of epigastric aura, ipsilateral hand automatisms, contralateral dystonic posturing, and ictal preservation of speech and responsiveness and absence of postictal dysphasia are typical of nondominant mesial temporal lobe seizures.

Question 17

A 45-year-old man with a long history of localization-related epilepsy develops major depression with suicidal ideation. He has had moderately good seizure control with carbamazepine, with his last seizure occurring 6 months ago. He has a flat affect with depressed mood. The remainder of his neurologic examination is normal. His carbamazepine level is therapeutic at 6 mg/dL. Which of the following antidepressants would be least likely to aggravate his seizures?

Answer 17

Sertraline.

ecause of concerns for interaction with hepatically metabolized antiepileptic drugs, first-line therapy using a selective serotonin reuptake inhibitor with minimal effects on the cytochrome P450 isoenzymes (eg, citalopram, escitalopram, sertraline) is preferred. The other drugs listed have been reported to increase the risk of seizure, particularly at higher doses.

Question 18

A 70-year-old woman has a witnessed cardiac arrest at a shopping center. Cardiopulmonary resuscitation is initiated. When the ambulance arrives
5 minutes later, the rhythm is ventricular fibrillation. After 10 minutes of resuscitation, a pulse is restored. On admission to the intensive care unit (ICU), the patient is comatose with no response to pain. The pupils are 3 mm and sluggishly reactive. Corneal reflexes and oculocephalic responses are reduced. Respiratory and cardiac support is maintained. Twenty-four hours after admission to the ICU, she develops generalized repetitive jerking of muscles
of the face and limbs. An EEG performed at this time is most likely to show which of the following findings?

Answer 18

Burst suppression.

This patient most likely has postanoxic myoclonic status epilepticus (SE), which is usually associated with the EEG finding of burst suppression, characterized by high-voltage bursts of slow, sharp, and spiking activity alternating with a suppressed background. This EEG pattern portends a poor prognosis in postanoxic cerebral injury. The other EEG patterns listed would not be expected to be associated with myoclonic SE.

Question 19

A 30-year-old woman develops complex partial seizures. An EEG demonstrates left temporal sharp waves, and MRI of the brain shows mild atrophy of the left hippocampal formation. She initially takes levetiracetam but fails to achieve good seizure control. Carbamazepine is added and titrated to a maximum dosage with therapeutic drug levels. Despite this, the patient continues to have disabling seizures. What is the likelihood that addition of a third anticonvulsant will provide adequate seizure control?

Answer 19

10%

This patient has medically refractory epilepsy. Most patients who respond to medical therapy do so with the initial anticonvulsant. In a patient who has failed to achieve good seizure control with monotherapy of two appropriate antiepileptic drugs or a combination of two drugs, the likelihood of response to a third drug is only 5% to 10%. This patient should be evaluated for alternative forms of therapy, including epilepsy surgery.

Question 20

A 26-year-old businessman presents with a witnessed generalized tonic-clonic seizure in sleep lasting 1 minute, followed by a 20-minute period of confusion. His past medical history is unremarkable. He has no prior history of seizure or head injury. He uses no medications and denies recreational drug use, including alcohol. The patient’s sister had childhood epilepsy. The patient’s neurologic examination is normal. An MRI of the brain with contrast is normal. An awake EEG recording shows no abnormalities. Blood work, including toxicology screen, is normal. Which of the following is the most accurate estimate of this patient’s risk of seizure recurrence in the next 2 years?

Answer 20

40%

atients with a single unprovoked seizure have a recurrence rate of 31% to 56% over 2 to 5 years of follow-up. Factors that increase recurrence risk include the presence of a remote symptomatic etiology, abnormalities on neurologic examination, and the onset of the first seizure during wakefulness. The risk of subsequent seizures is higher after a second seizure (73%) and a third seizure (76%). In this patient, a good argument could be made for not starting anticonvulsants given what is probably a relatively low risk of recurrence. On the other hand, early treatment may be considered in cases where seizure recurrence would have significant consequences related to driving and work.

Question 21

An 18-year-old college student is evaluated for recurrent episodes of unusual behavior at night. These episodes occur almost weekly, particularly after sleep deprivation or alcohol intake. They occur within 2 to 3 hours of sleep onset. During these episodes he may walk through the dormitory, crawl into someone else’s bed, or be found sitting quietly in the break room. Witnesses report that, during the events, he appears confused and clumsy, his eyes are open, and his speech is soft and slow. He is amnestic from these events upon wakening the next morning. The EEG demonstrates diffuse slowing of the background during the episode. Which of the following is the most likely diagnosis?

Answer 21

Confusional arousal disorder.

The history of nonstereotypic behavior, long duration of the episodes, occurrence during the first half of the night, and amnesia of the events is suggestive of confusional arousal disorder. EEG recording during the event shows exaggerated slowing of background activity. The clinical picture is less likely to be due to nonconvulsive status epilepticus or complex partial seizures and is not consistent with REM sleep behavior disorders or frontal lobe seizures.

Question 22

A 22-year-old man is evaluated for transient episodes during which he adopts a posture characterized by abduction, elevation, and external rotation of the right arm and deviation of the head toward the right. What is the most likely location of the lesion responsible for these episodes?

Answer 22

Left SMA.

Tonic seizures originating from the supplementary motor area involve abrupt bilateral or asymmetric posturing usually of the contralateral arm, including abduction, elevation, and external rotation associated with contralateral deviation of the head (fencing posture or M2e sign).

Question 23

A 24-year-old thin woman with temporal lobe seizures due to mesial temporal sclerosis is evaluated for frequent episodes of unresponsiveness despite maximally tolerated doses of carbamazepine. Video-EEG monitoring shows that the episodes of unresponsiveness are associated with ictal discharges in the left temporal region. Which of the following is the preferred drug for adjunctive treatment of seizures in this patient?

Answer 23

LVT

Levetiracetam is the most appropriate choice in this patient. All of the second-generation drugs are indicated for adjunctive therapy of partial seizures and could be considered in this patient, but levetiracetam is the easiest to use in this situation since it is well-tolerated and can be increased quickly. Lamotrigine has a long dose escalation phase to avoid rash. Topiramate has risk of weight loss and a moderately long dose escalation phase. Valproate should be avoided in women of child-bearing potential. Vigabatrin has risk of visual field defects so it is only indicated for partial seizures that have not responded to other appropriate medications.

Question 24

A 22-year-old woman with medically refractory complex partial seizures who is not a suitable surgical candidate is prescribed vigabatrin. Which of the following adverse effects of the drug should be discussed with the patient?

Answer 24

Irreversible visual field loss.

Vigabatrin is approved by the US Food and Drug Administration for treatment of adults with medically refractory complex partial seizures. The drug may increase the risk of permanent loss of peripheral vision, and patients should be advised to report any vision changes immediately and maintain regular appointments for monitoring visual examinations.

Question 25

A 6-year-old girl is evaluated at the request of her teacher for possible attention deficit disorder. She was observed by her parents to have transient episodes of abrupt onset of unresponsiveness to external stimuli and behavioral arrest lasting approximately 10 seconds, followed by immediate recovery. She experiences at least 30 such episodes per day. Her neurologic examination is normal. Hyperventilation reproduces the spells. The EEG reveals a generalized 3-Hz spike-and-wave discharge. Which of the following is a feature of this syndrome?

Answer 25

The disorder involves abnormal activation of T-type calcium channels.

The clinical and EEG pictures are typical of childhood absence epilepsy (CAE). Seizures result from activation of T-type calcium channels, leading to abnormally synchronized rhythmic activity in thalamocortical circuits. Calcium channel blockers such as ethosuximide, valproate, and lamotrigine are effective for treatment of absence seizures. In contrast, carbamazepine, oxcarbazepine, and phenytoin should be avoided as they may precipitate absence status epilepticus. Children with early-onset CAE have the best prognosis, with remission of epilepsy 2 to 6 years after onset. Only 3% of children with typical CAE will experience a generalized tonic-clonic seizure during the period of active absences, although up to one-third may develop generalized tonic-clonic seizures in their adolescent or young adult years. CAE may precede juvenile myoclonic epilepsy in 11% to 18% of patients.

Question 26

A 25-year-old woman with complex partial seizures notes that her seizures occur exclusively at the time of menstruation. She takes carbamazepine and is fully compliant with that medication. Her neurologic examination is normal. MRI of the brain demonstrates left hippocampal atrophy, and EEG shows rare left temporal spikes. Which of the following is most likely responsible for the perimenstrual exacerbation of her seizures?

Answer 26

Decreased progesterone

This patient has catamenial epilepsy, which in her case is most likely a result of progesterone withdrawal with a subsequent increase in estrogen relative to progesterone that occurs at the time of menstruation. Estrogen has proconvulsant properties whereas progesterone and its metabolite allopregnanolone have anticonvulsant properties. Allopregnanolone acts as a positive allosteric modulator of γ-aminobutyric acid A receptors, the main modulators of inhibition in the CNS

Question 27

A 14-year-old girl has a history of absence seizures beginning at age 6. These are characterized by staring, motor arrest, and fluttering eyelids without loss of postural tone. No other seizure types have been recognized by the family on careful questioning. An EEG shows a 3-Hz spike-and-wave discharge, and an MRI of the head is normal. She has been seizure free on valproic acid for the past 4 years. If the anticonvulsant is tapered, what is the approximate likelihood that she will remain seizure free?

Answer 27

60%

Absence seizures of childhood with the associated 3-Hz spike-and-wave pattern have a high rate of clinical remission in the absence of associated generalized tonic-clonic and myoclonic seizures. The remission rate is up to 60%

Question 28

A 40-year-old man with a history of generalized convulsive seizures is found dead in his bed by his spouse. His seizures have been suboptimally controlled on monotherapy levetiracetam, a medication with which he was not fully compliant, according to his wife. She reports that he had had three seizures over the past month, but she noted no seizure activity the night of his death. He had not been ill in the days preceding his death. He took no other medications and had consumed two beers the evening preceding his death. His postmortem examination of the brain and other viscera showed no abnormalities other than mild right hippocampal atrophy. Postmortem blood work demonstrated a low levetiracetam level. Which of the following factors placed this patient at highest risk for sudden death?

Answer 28

Uncontrolled convulsive seizures

his patient suffered sudden unexplained death in epilepsy (SUDEP). The cause of SUDEP remains a mystery but is far more common in patients with medically intractable generalized convulsive seizures. Polypharmacy also appears to increase the risk of SUDEP. Poor compliance with antiepileptic drugs (AEDs) and low drug levels have been suspected risk factors, although they have not been consistently shown to increase SUDEP risk. Moreover, the determination of AED levels in postmortem blood samples has been known to be inaccurate. The most important measure to reduce SUDEP risk is optimal control of generalized convulsive seizures with the least number of AEDs possible

Question 29

A 16-year-old student is evaluated for recurrent spells of lightheadedness followed in many cases by loss of consciousness. The spells tend to occur after prolonged standing or shortly after exercise, particularly in a hot environment. Whereas, in general, she recovers rapidly after hitting the ground, on occasion the loss of consciousness is associated with
generalized stiffening and mild jerking of both upper extremities. The spells may be preceded by excessive sweating, palpitations, nausea, generalized fatigue, and occasionally blurred vision. Which of the following tests is most likely to be abnormal in this patient?

Answer 29

Tilt table test.

The clinical picture is highly suggestive of neurally mediated (reflex, vasovagal, or neurocardiogenic) syncope. The presence of prodromes would be atypical for cardiogenic syncope. Tonic or clonic events (convulsive syncope) reflect brainstem hypoxia rather than seizures. The lack of postictal confusion would be atypical of temporal lobe seizures, although syncope may sometimes be caused by ictal bradycardia. The presence of symptoms of excessive sympathoexcitation (eg, pallor, sweating, palpitations) prior to syncope would be atypical for orthostatic hypotension caused by autonomic failure, and thus plasma catecholamines are not helpful. The short duration of the events and spontaneous recovery would also be atypical for hypoglycemia.

Question 30

A 55-year-old-man with a history of chronic obstructive pulmonary disease (COPD), hypertension, and complex partial seizures controlled on carbamazepine (CBZ) is evaluated for acute confusion following a respiratory tract infection. Two days ago, he experienced an accidental fall and injured his right elbow. He was prescribed ampicillin, terbutaline, and prednisone to treat the exacerbation of COPD. Propoxyphene was prescribed for elbow pain and eszopiclone for sleep. On examination, he is disoriented and has diplopia, vomiting, and gait ataxia. A plasma carbamazepine level is 15 mg/dL (therapeutic range is 6 mg/dL to 10 mg/dL). Which of the following drugs would be expected to interact and promote acute CBZ toxicity?

Answer 30

Propoxyphene

Propoxyphene inhibits carbamazepine hepatic metabolism through the cytochrome (CYP) 450 isoenzymes and often
results in increasing plasma carbamazepine levels and risk of clinical toxicity.

Other common drug interactions with CYP 3A4 or CYP 2C19 hepatic metabolism that may be relevant include macrolide antibiotics, calcium channel blockers (diltiazem, verapamil), antifungals (fluconazole, ketoconazole), antivirals (indinavir, ritonavir), antidepressants (fluvoxamine, nefazodone), and grapefruit juice

Question 31

A patient with medically intractable epilepsy is being considered for placement of a vagal nerve stimulator. In counseling the patient, which of the following adverse effects is most likely to result from this device?

Answer 31

Hoarseness

Vagal nerve stimulators (VNS) may be used in the treatment of refractory clinical depression as well as in the treatment of seizure disorders. Vomiting and increased gastric acid secretion are not common side effects of VNS. Hoarseness, throat pain, cough, dyspnea, and paresthesias are relatively common side effects. The VNS is always placed on the left vagal nerve to avoid cardiac complications, which could occur if the right vagal nerve were stimulated

Question 32

A 70-year-old woman presents to the emergency department after she was found having a generalized tonic-clonic seizure at home. She has no prior history of seizure, and her past medical history is remarkable only for hypertension and hypercholesterolemia. On arrival in the emergency department, she continues to have generalized tonic-clonic seizure activity. Vital signs are heart rate 100 beats/min, blood pressure 160/80 mm Hg, and temperature 37°C. Finger-stick blood glucose level is 85 mg/dL. Which of the following is the most likely cause of status epilepticus in this patient?

Answer 32

Stroke

Cerebrovascular disease (including remote and acute hemorrhagic and ischemic events) accounts for 60% of all cases of status epilepticus in older adults. In this vignette, there is no additional history to suggest neoplasm, and the degree of hypertension is not sufficient to produce seizures. Nothing in the history suggests encephalitis or hypoglycemia

Question 33

A 40-year-old man has two unprovoked generalized tonic-clonic seizures while awake. MRI, EEG, and metabolic evaluation results are within normal limits. He starts taking carbamazepine and remains seizure free for 2 years. At that point his anticonvulsant is tapered over 6 weeks. Shortly thereafter, he experiences another generalized tonic-clonic seizure and carbamazepine is restarted. In counseling the patient, what is his approximate risk of another seizure if he remains on medication over the next 12 months?

Answer 33

5%

A common concern among physicians has been that seizures may not be as easy to control once the patient has tapered off anticonvulsants and the anticonvulsant is restarted. There appears to be no evidence for worsened long-term control upon resumption of therapy. Following a failed trial of antiepileptic drug withdrawal, 95% of subjects experienced a 1-year remission by 3 years of follow-up, and 90% experienced a 2-year remission by 5 years of follow-up.

Question 34

A 44-year-old previously healthy man is brought to the emergency department by his wife because she noticed that he was confused that morning. At home he had seemed bewildered and asked repeatedly, “Where am I?” despite
being given the answer to this question moments earlier. Neurologic examination performed approximately 2 hours into the event reveals normal self-identification and digit span but inability to recall four words after 1 minute. He gradually recovers over the next 2 hours but is amnestic of the events occurring during the episode. Which of the following conditions should be considered in the differential diagnosis?

Answer 34

Complex partial seizures.

The patient’s clinical picture is consistent with transient global amnesia. However, complex partial seizures should be considered in the differential diagnosis.

Question 35

12-year-old previously healthy boy is evaluated for a generalized tonic-clonic seizure that occurred in the morning after he had been staying up late to finish his homework. The family reported that he experienced brief, irregular jerks of the head and limbs. Which of the following additional features is typical of this disorder?

Answer 35

The EEG shows brief generalized polyspike discharges triggered by photic stimulation.

The clinical picture is that of juvenile myoclonic epilepsy. The episodes typically occur upon awakening in the morning, particularly after sleep deprivation. Approximately 50% of patients are photosensitive. The ictal EEG shows brief generalized polyspike or polyspike-and-wave discharges. The response to antiepileptic drugs is excellent, but most patients require lifelong treatment because of the high rate of relapse.

Question 36

A 30-year-old woman with partial epilepsy has been seizure free for the past 1 year. She takes carbamazepine and is fully compliant with the medication. She wishes to become pregnant. What is the likelihood that she will remain seizure free during her pregnancy?

Answer 36

80%

Women with epilepsy who are seizure free for
1 year prior to pregnancy have an 80% chance of remaining seizure free during the pregnancy. Seizures during labor and delivery are rare (5% or less), and no increased risk of obstetric complications appears to exist in women with epilepsy.

Question 37

A 6-month-old girl with a history of developmental delay is evaluated for recurrent spells characterized by transient contraction of the neck, trunk, and limb muscles followed by a brief tonic component. The spasms may be flexor or extensor and occur several times a day, particularly shortly after waking. The EEG shows disorganized, paroxysmal, high-voltage slowing and multifocal epileptiform discharges that are asynchronous between the hemispheres. Which of the following is an additional feature of this syndrome?

Answer 37

Vigabatrin may suppress spasms in up to two-thirds of cases.

The clinical and EEG picture is that of West syndrome with hypsarrhythmia. Most cases are symptomatic; underlying etiologies include cortical malformations, tuberous sclerosis, chromosomal disorders, and inborn errors of metabolism. Hypsarrhythmia may only occur during non-REM sleep and is reduced or disappears during REM sleep. With age, the EEG shows greater interhemispheric synchronization. Treatment includes corticotropin, corticosteroids, and vigabatrin.

Question 38

A 24-year-old woman with medically intractable complex partial seizures undergoes a right mesial temporal lobectomy. The procedure is uncomplicated. She becomes seizure free and within 1 year is able to taper off her antiepileptic drugs, which include carbamazepine and lamotrigine. Sixteen months after her surgery she develops weight gain, depressed mood, social withdrawal, and suicidal ideation. No obvious personal stressors are present, and she had been functioning well at her job as a legal assistant. Which of the following factors is most likely responsible for this patient’s mood disorder at this time?

Answer 38

Medication discontinuation.

This patient most likely is experiencing a mood disorder related to the withdrawal of two mood-stabilizing agents (lamotrigine and carbamazepine). Temporal lobectomy would not likely produce depression de novo, and the symptoms of medical illness appear to have resolved. Subclinical seizures would not likely present in this fashion, and nothing in the history places her at higher risk of hypothyroidism.

Question 39

A 70-year-old man with a history of obesity, hypertension, hypercholesterolemia, nephrolithiasis, and atrial fibrillation is evaluated for recent-onset seizures. An MRI of the head shows an old right frontal ischemic stroke. Current medications include enalapril, simvastatin, and warfarin. Which of the following is the most appropriate drug for seizure treatment in this patient?

Answer 39

Lamotrigine

In older adults receiving several drugs that undergo liver metabolism, the drugs of choice include lamotrigine, levetiracetam, pregabalin, and gabapentin, which are primarily excreted by the kidney. Topiramate is also a reasonable consideration. However, topiramate would be contraindicated because of the history of kidney stones. Gabapentin and valproate are contraindicated given the presence of obesity. Both phenytoin (an inducer) and valproate (an inhibitor) target liver enzymes and thus increase the risk of drug interactions in this patient.

Question 40

A 40-year-old man with idiopathic generalized epilepsy is brought to the emergency department in convulsive status epilepticus. His wife believes that he had missed at least two doses of phenytoin over the previous 36 hours because he had neglected to refill his prescription on time. He has no other history of illness and takes no other medications. On admission to the emergency department, his vital signs are normal. An airway is established, and supplemental oxygen is administered. IV access is obtained, and IV lorazepam 4 mg is administered. Convulsions stop promptly, and 10 mg/kg IV fosphenytoin is administered. A phenytoin level drawn prior to the administration of IV fosphenytoin is 9 μg/mL. Initial serum electrolytes
are normal. Four hours later, the patient remains unconscious with no convulsive activity. He withdraws all four limbs in response to pain, and brainstem reflexes are intact. Which of the following is the most appropriate next step in management?

Answer 40

EEG

atients presenting with convulsive status epilepticus (SE) who fail to regain consciousness after successful treatment of their convulsions should be suspected of nonconvulsive SE. An EEG is the best way to evaluate for the presence of this problem. A CT head scan and electrolyte levels are unlikely to be useful in this patient whose SE was probably caused by medication noncompliance. Before administering additional anticonvulsants it would be best to confirm the presence of nonconvulsive SE, although a trial of IV lorazepam would be reasonable if a delay in obtaining the EEG recording is anticipated.

Question 41

Which of the following is the major role of the neurologist after a diagnosis of psychogenic nonepileptic events?

Answer 41

Appropriately weaning antiepileptic drugs.

After a diagnosis of psychogenic nonepileptic attacks, one of the
major roles of the neurologist is the rational weaning of antiepileptic medications. This should be done with the caveat that coexistent epilepsy may also be present, so the physician needs to keep an open mind regarding new events (although routine surveillance EEGs are not necessarily indicated). Driving restrictions should be handled on an individual case basis, although most patients can return to driving with little chance of injury. Neurologists should use unambiguous terms when describing the diagnosis to the patient and other providers so that the misdiagnosis of epilepsy is not perpetuated. While mental health care is often needed, the routine prescription of antidepressants is not always indicated.

Question 42

Deep brain stimulation for drug-resistant epilepsy targets which of the following structures?

Answer 42

Anterior nucleus of the thalamus.

The anterior nucleus of the thalamus is part of the Papez circuit and is therefore believed to be a relay station for information passing from the amygdala and hippocampus to the cerebral cortex. Data have shown that inhibition of the anterior nucleus may result in prevention or cessation of seizures.

Question 43

According to the International League Against Epilepsy’s 2010 revision of its seizure classification system, which of the following factors is most helpful in initially focusing the scope of diagnostic possibilities for specific electroclinical syndromes?

Answer 43

Age at onset

While all the other factors mentioned should be part of an initial seizure history, they are not as important
in establishing the ultimate diagnosis.

Question 44

Which of the following conditions would likely preclude the consideration
of epilepsy surgery in a patient?

Answer 44

Primary generalized epilepsy.

Patients who are typically not surgical candidates include those with clear evidence of bilateral onset of habitual seizures, those with severe psychiatric or medical comorbidities increasing surgical risk or compromising recovery, those with rapidly progressive CNS disease, and those with primary generalized epilepsy.

Question 45

A 63-year-old woman is hospitalized with herpes simplex virus encephalitis.
Which of the following EEG patterns is most likely to be seen in this patient?

Answer 45

Periodic lateralizing discharges.

Periodic lateralizing discharges are EEG abnormalities commonly seen in certain scenarios, including herpes encephalitis. Other conditions in which periodic lateralizing discharges are seen include focal brain lesions, nonketotic hyperglycemia, alcohol withdrawal, and theophylline exposure. Generalized periodic discharges are more commonly seen in anoxic brain injury, Creutzfeldt-Jakob disease, and other diffuse processes. Stimulus-induced, rhythmic, periodic, or ictal discharges (SIRPIDs) are most commonly seen in critically ill patients, even those without neurologic illness. Burst suppression is typically seen in anoxic encephalopathy or in the setting of certain medications (eg, propofol, barbiturates).

Question 46

A 60-year-old woman develops generalized seizure activity lasting 10 minutes; seizure activity appears to arrest after administration of 4 mg
of IV lorazepam. She has chronic kidney disease but is otherwise in good health. Which of the following is the best next pharmacological step
in management?

Answer 46

IV fosphenytoin.

Regardless of whether prolonged seizure activity stops after the administration of an appropriate dose of a benzodiazepine, rapid administration of a longer-acting anticonvulsant is generally recommended. This allows for prevention of additional seizures
as the effect of the benzodiazepine wears off over the course of several hours. Of the options listed, fosphenytoin is the preferred option. A continuous infusion of propofol or midazolam is not indicated in this setting unless clinical or EEG evidence of ongoing seizures (ie, refractory status epilepticus) exists.

Question 47

Which of the following antiepileptic drug combinations is most likely to
result in symptoms of diplopia and dizziness?

Answer 47

Phenytoin and carbamazepine.

he combination of phenytoin and carbamazepine frequently results in the adverse effects of dizziness and diplopia. In addition, both medications have enzyme-inducing properties, resulting in complex pharmacologic interactions and difficulty maintaining therapeutic levels.

Note- henobarbital and valproate have a higher likelihood of resulting in weight gain. Combining valproate and lamotrigine may increase the risk of Stevens-Johnson syndrome, so dosing adjustments need to be made carefully.

Question 48

Which of the following EEG patterns is rarely seen in normal patients and has a high specificity for temporal lobe epilepsy?

Answer 48

Rhythmic temporal delta.

Of the options listed above, rhythmic temporal delta activity is most suggestive of temporal lobe epilepsy. While sensitivity for this finding is not especially high, it is rarely seen outside of the context of temporal lobe seizures and therefore carries high specificity. Triphasic waves are most commonly seen in patients
with metabolic encephalopathy. Diffuse slow activity is seen in patients with traumatic brain injury, subarachnoid hemorrhage, toxic-metabolic encephalopathies, and other processes that affect the brain more diffusely. Wicket spikes and 14 and 6 positive spikes are considered normal EEG variants seen in older adults and adolescents, respectively.

Question 49

The cortical hamartomas of tuberous sclerosis display different MRI characteristics depending on a patient’s age. Which of the following pathophysiologic processes is responsible for this age-related change in the MRI characteristics of these lesions?

Answer 49

Myelination

Cortical hamartomas (tubers) are the most characteristic lesions in tuberous sclerosis complex. These lesions can cause focal seizures, which in some patients may be refractory to antiepileptic drugs; however, not all tubers are epileptogenic. The MRI appearance of tubers changes with myelination. In neonates they are hyperintense on T1 and hypointense on T2-weighted images compared to the surrounding white matter. In older children they are hyperintense on T2-weighted images with poorly defined borders.

Question 50

Surgery for which of the following types of epilepsy is least likely to result in seizure freedom?

Answer 50

Nonlesional neocortical epilepsy

The best outcomes from epilepsy surgery are for temporal lobectomy for mesial temporal sclerosis, lesional resections, and hemispherectomy, with lower seizure-free rates in patients with nonlesional neocortical resections.

Question 51

A patient with which of the following conditions should not be placed on a ketogenic diet?

Answer 51

Pancreatitis

Ketogenic diets are contraindicated in patients with pancreatitis, hepatic failure, primary carnitine deficiency, carnitine palmitoyl transferase I and II deficiency, carnitine translocase deficiency, beta-oxidation defects, pyruvate carboxylase deficiency, and porphyria. In the intensive care setting where diet therapy is being considered for treatment of refractory status epilepticus, the ketogenic
diet is also contraindicated in patients who cannot tolerate enteral feeds, including those with ileus, who are receiving a propofol infusion (to avoid fatal propofol infusion syndrome), and in patients who have metabolic, hemodynamic, or cardiorespiratory instability.

Question 52

10-year-old child with epilepsy since age 2 that is refractory to medical treatment has been followed serially with brain MRI scans, which show progressive atrophy of the left hemisphere. What is the most likely diagnosis?

Answer 52

Rasmussen encephalitis

MRI in Rasmussen encephalitis shows progressive atrophy of one of the cerebral hemispheres, usually beginning in the opercular region.

Question 53

A 21-year-old man is seen in clinic for management of seizures. He has had several episodes of generalized tonic-clonic seizure activity with resultant oral trauma and incontinence. Some of his events may have started with focal shaking in the right arm, although this history is unclear and is unable to be corroborated. EEG reveals no epileptiform activity or asymmetry, and brain imaging is unremarkable. Of the following anticonvulsants, which would be most appropriate for this patient?

Answer 53

Zonisamide

he description of this patient’s episodes is consistent with generalized seizures. However, based on the history, EEG, and imaging studies, it is hard to determine whether these events are primary or secondary generalized in nature. In cases where it is difficult to determine whether seizures are primary generalized or focal in onset, a broad-spectrum anticonvulsant medication is preferable. Of the options listed above, zonisamide and phenobarbital are both considered broad-spectrum drugs, although zonisamide would be preferable based on its more favorable side-effect profile. Oxcarbazepine and gabapentin are more narrow-spectrum and would only be indicated if the events were more clearly focal in onset. The exact spectrum of lacosamide is unknown at
this time, since it has only been studied in the setting of focal seizures.

Question 54

Which of the following is a possible predictor of refractory status epilepticus (status epilepticus not responding to two antiepileptic medications)?

Answer 54

Hypoxic-ischemic encephalopathy

ome etiologies of status epilepticus or patient factors may be more predictive of a refractory course that does not respond to initial treatments. Of the options listed above, hypoxic-ischemic encephalopathy (and other etiologies that are not associated with an underlying structural lesion) may be more likely to be refractory. Encephalitis, delays in diagnosis and treatment, and subtle symptoms as a manifestation of status epilepticus are other possible predictors. Physicians should be aware that these etiologies of status epilepticus may require more aggressive treatment.

Question 55

Approximately what percentage of patients with epilepsy will become seizure free on the initial antiepileptic drug regimen?

Answer 55

Data suggest that 35% to 40% of patients will be well controlled on the initial antiepileptic drug regimen chosen for them. Once a patient is well controlled, both the patient and physician
may be reluctant to make changes in the regimen, even if there is good rationale for making an adjustment. This underscores the importance of making a wise initial choice in an antiepileptic medication because there may be limited ability to make changes in the future. This choice must weigh data on effectiveness, adverse effects, teratogenicity, and patient compliance, among other considerations.

Question 56

A 40-year-old woman is admitted to an epilepsy monitoring unit for evaluation of paroxysmal episodes of neurologic dysfunction. Her episodes involve some nonrhythmic shaking of both arms and pelvic thrusting without any correlate on the EEG; psychogenic nonepileptic events are diagnosed. Which of the following characteristics is most associated with a good outcome (ie, resolution of episodes)?

Answer 56

Shorter duration of illness

The description of the events provided above and the lack of EEG findings during her episodes are both suggestive of nonepileptic events. Prognosis after a diagnosis of psychogenic nonepileptic events is variable but may be dependent on certain characteristics. Of the options listed above, a shorter duration of illness (ie, shorter time between the onset of spells and the diagnosis of nonepileptic events) is most predictive of a positive outcome. Some of the other options listed above (older age, prominent/dramatic motor features, lower educational achievement) are associated with a poorer outcome
after a diagnosis of nonepileptic events.

Question 57

66-year-old woman is seen in clinic for a new diagnosis of epilepsy; she has experienced recurrent partial seizures attributed to a left hemispheric stroke. Her medical history is most notable for atrial fibrillation (for which she takes warfarin), osteoporosis, and a history of kidney stones. Her primary care physician started her on phenytoin, which resulted in a rash. Which of the following is the most appropriate antiepileptic medication
for this patient?

Answer 57

Levetiracetam

This patient with epilepsy is medically complex, with several notable comorbid medical conditions and
a prior adverse reaction to phenytoin. Using either carbamazepine or primidone in this case would be challenging given the potential cross-reactivity with phenytoin (to which she has already had a hypersensitive reaction) as well as the potential drug-drug interactions with warfarin. Zonisamide is associated with a risk of kidney stones and should be avoided in this patient with prior calculi. Valproate and enzyme-inducing medications are associated with accelerated loss of bone density, which is already a concern in this patient with osteoporosis. Of the options listed, levetiracetam has the lowest risk of hypersensitivity, drug-drug interactions with warfarin, and potential worsening of her underlying osteoporosis and kidney stones.

Question 58

Which of the following is the leading rationale for withholding
antiepileptic therapy in a child after a first unprovoked seizure?

Answer 58

The long-term prognosis is unchanged.

The recurrence rate for a second unprovoked seizure is not low and can be reduced by initiating antiepileptic therapy. However, delaying treatment until a second event confers the advantage of having better confirmation of epilepsy, while bearing in mind that the recurrence rate approximately doubles after two episodes.

Question 59

In 2010, the International League Against Epilepsy departed from its 1989 classification scheme of epilepsies and revised its seizure classification system. What was the primary goal of this change?

Answer 59

Keeping pace with advancing knowledge of epilepsy and seizures.

While the advancement of a value system for syndromic severity and the modification of billing and coding practices with regard to epilepsy are desired goals, the overarching goal of revising the old classification scheme is to incorporate genetic, neuroimaging, and other new knowledge of epilepsy and seizures into a workable format for correct individual diagnosis.

Question 60

Although not all data are available for all drugs (and keeping in mind that an individual patient may have other specific needs), which of the following antiepileptic drugs would be the best initial choice in an adolescent girl with juvenile myoclonic epilepsy?

Answer 60

Levetiracetam

Levetiracetam is considered to
be effective in juvenile myoclonic epilepsy. Carbamazepine is probably better in partial or generalized tonic-clonic seizures. Ethosuximide has strong evidence in absence seizures. Valproic acid is effective in juvenile myoclonic epilepsy and related seizure types but is usually not the first choice in adolescent girls because of teratogenicity and risk of polycystic ovary syndrome. Lacosamide has no data for myoclonic epilepsy.

Question 61

Fetal exposure to which of the following medications is most likely to be
associated with decreased IQ during childhood?

Answer 61

Valproate

Of the options listed above, in utero exposure to valproate is most likely to be associated with a lower childhood IQ. Longitudinal studies have shown no changes with phenytoin, carbamazepine, and lamotrigine, but a lower mean IQ at age 3 was seen with exposure to valproate. This was also shown to have a dose-effect response, with lower IQs observed in children exposed to higher doses of valproate. There is no data on cognitive outcomes in children with in utero exposure to topiramate. Unlike valproate, topiramate does not cause apoptosis in immature brains of animals.

Question 62

Psychogenic nonepileptic events occurring in which of the following populations are most likely to be misdiagnosed as epileptic seizures?

Answer 62

Military veterans

Of the options listed above, veterans are most likely to have their psychogenic nonepileptic events mischaracterized as epileptic seizures. The exact cause of this is unclear, although some attribute this to an antecedent history of trauma in this population with an assumption that their events are ‘‘post-traumatic’’ in nature.

Note - other populations at risk of having their events inappropriately attributed to epileptic seizures include the elderly and patients with prior epilepsy surgery.

Question 63

A 23-year-old woman is seen for evaluation of intermittent spells concerning for seizures. The presence of which of the following characteristics of her spells would be more suggestive of epileptic seizures as opposed to psychogenic nonepileptic events?

Answer 63

Tongue biting

Characterization of spells as epileptic or psychogenic (ie, nonepileptic) in nature can be challenging and often requires video-EEG monitoring, although aspects of the history
often help to distinguish the etiology of the episodes. Of the options listed above, tongue biting (or other types of injury/trauma) would be most suggestive of an epileptic nature. Other factors that would favor an epileptic etiology include significant postictal confusion, occurrence in sleep, and incontinence. The other choices listed above would all be more suggestive of a psychogenic nonepileptic etiology.

Question 64

Which of the following clinical factors is most predictive of cognitive decline after epilepsy surgery?

Answer 64

Higher preoperative cognitive performance

Cognitive performance may improve in some patients after surgery, presumably related to better seizure control and possible decrease in the burden of anticonvulsants needed for seizure control. However, even with functional imaging or Wada testing to guide treatment decisions, some patients will note worsened cognitive functioning postoperatively. Of the options listed, patients with higher preoperative cognitive functioning are at a higher risk of new cognitive difficulties after surgery. Older age at seizure onset and at time of surgery, the lack of hippocampal atrophy or sclerosis, and surgery on the language-dominant hemisphere are other predictors of postoperative cognitive symptoms.

Question 65

Which of the following medications is most likely to be associated with interictal spikes or epileptiform abnormalities on EEG?

Answer 65

Cefepime

Cefepime is one of several medications that can be associated with interictal spikes on EEG. Other examples include bupropion, lithium, tramadol, and clozapine. Uremia, thyrotoxicosis, and some other medical comorbidities can also be associated with epileptiform abnormalities but are not diagnostic for epilepsy. Abnormal EEGs in these contexts need to be interpreted with appropriate caution.

Question 66

A 27-year-old woman is seen in clinic for ongoing management of her juvenile myoclonic epilepsy. Her seizures have been well controlled on lamotrigine, with no seizures in the past 2 years. She does report some symptoms of depression, as well as anticipatory anxiety regarding the possibility of future seizures. Which of the following options is the best next step in management?

Answer 66

Citalopram

This patient is reporting symptoms of depression and anxiety, both of which are common in patients with epilepsy. In patients requiring pharmacotherapy for these symptoms, selective serotonin reuptake inhibitors (SSRIs) such as citalopram are considered first-line treatments because of lower likelihood of additional side effects and minimal drug-drug interactions. Venlafaxine is another option if an SSRI is not tolerated, and tricyclic antidepressants (such as nortriptyline) could be considered if neither of these first two agents is successful. Lamotrigine is not commonly associated with depression and is often used as a mood stabilizer; it is unlikely to be the cause of these symptoms in this patient and should not be changed to levetiracetam. Bupropion is associated with a higher risk of seizures and should not be used.

Question 67

A 25-year-old woman with a history of complex partial epilepsy has been treated successfully with topiramate for 2 years. She is contemplating initiating birth control and comes to discuss her options with her physician. Which of the following would be a reasonable contraceptive choice for this patient?

Answer 67

Intrauterine device

This patient is content with her antiepileptic drug, and there is no compelling reason to change it. Instead, she can choose a form of contraception that is compatible with an enzyme-inducing antiepileptic drug such as topiramate. Low-dose oral contraceptive pills, patches, and rings may provide insufficient protection. One of the various long-acting reversible contraceptives - which include progestin implants, IM medroxyprogesterone acetate, and intrauterine devices - may be a good choice.

Question 68

Which of the following is the most common side effect seen after
placement of a vagus nerve stimulator?

Answer 68

Voice alteration/hoarseness

After placement
of a vagus nerve stimulator, alteration of voice quality (including hoarseness) and excessive coughing are the most common side effects, seen in one study in about 66% and 45% of patients respectively. The other choices listed above are possible adverse effects of this procedure but are seen much less commonly.

Question 69

A 23-year-old man is admitted to the hospital for long-term video-EEG monitoring. He has multiple events while in the hospital, but no clear abnormality is apparent on the EEG. Which of the following characteristics may still support a diagnosis of epileptic seizures as opposed to psychogenic nonepileptic events?

Answer 69

Stereotyped nature of events

There are some limitations on the ability of video-EEG monitoring to definitively make a diagnosis of epileptic seizures. Events with subtle, subjective symptoms (eg, simple partial seizures or auras) may involve only a small area of cortex
and may not be detected by a surface EEG. Events of this type that are highly stereotyped in nature may still represent epileptic events despite the lack of EEG findings during monitoring. Thrashing motor activity can sometimes obscure the EEG because of motion artifact, but the semiology of these events usually allows for them to be characterized as nonepileptic.
Similarly, in the setting of a reassuring EEG, the other options listed above (suggestibility, prolonged alterations in consciousness, lack of sleep-related events) would all be more suggestive of psychogenic nonepileptic events.

Question 70

A 30-year-old man who has had focal epilepsy for 15 years still experiences four to five episodes per month despite medication. He takes two anticonvulsants; his current medications are the ninth and tenth drugs he has tried. Repeated EEGs have shown a right temporal focus. He is on disability and lives with his parents. What is the next best step in management of this patient?

Answer 70

Surgical evaluation

Failure to respond adequately to proper trials of two anticonvulsants and presence of focal epilepsy should lead to a surgical evaluation. Vagus nerve stimulation is much less effective than surgery in this setting.

Question 71

Which of the following best describes the relationship between vagus nerve stimulation and risk of bradycardia?

Answer 71

Can be seen with stimulation of either nerve.

Efferent fibers of the vagus nerve originating from the nucleus ambiguous include cardiac branches that help control heart rhythms. Since fibers originating from the right vagus nerve primarily innervate the sinoatrial node, it was thought that right nerve stimulation (or bilateral stimulation) may be most likely to cause bradycardia. However, at certain stimulator settings, even stimulation of the left vagus nerve (which primarily innervates the atrioventricular node) may precipitate bradycardia. Neurologists involved
in programming stimulators should be aware that bradycardia can be a risk with stimulation of either nerve.

Question 72

A 27-year-old woman with longstanding complex partial seizures, well controlled on monotherapy with carbamazepine, is seen in the first trimester of pregnancy. She is concerned about seizures becoming more frequent during pregnancy. Which of the following is the most appropriate counseling of this patient regarding her pregnancy and seizure recurrence?

Answer 72

Most women experience no change in seizure frequency

Although the effect of pregnancy on seizure frequency is variable, most women with epilepsy do not experience a significant change in the number or severity of their seizures. Expected physiologic hormonal changes, alterations in sleep and other routines, and other factors can alter the seizure frequency in some cases. Noncompliance can also play a role, since some women will discontinue their medications with the assumption that the teratogenic effects of antiepileptic medications outweigh their potential benefits.

Question 73

Which of the following seizure/epilepsy subtypes appears to respond best to deep brain stimulation techniques?

Answer 73

Temporal lobe epilepsy

In pivotal studies of deep brain stimulation of the anterior nucleus of the thalamus, patients with temporal lobe epilepsy responded best to this treatment approach. Patients with this form of epilepsy had a 44% reduction in seizures (compared to 22% in controls); patients with seizures originating from other lobes had no significant effect from this procedure. One possible explanation for this finding is the connection between the hippocampus and the anterior nucleus as part of the Papez circuit.

Question 74

Which of the following clinical characteristics is associated with an
increased risk of suicidal ideation in patients with epilepsy?

Answer 74

Coexistent depression

Among many potential predictors, only the presence of a coexisting mood disorder such as major depression is associated with a higher incidence of suicidal ideation in epilepsy patients. Other characteristics, including age, sex, seizure type, seizure control, duration of epilepsy, and lower quality of life, have not been associated with a higher risk of suicide. Because of this, routine screening for depression and suicidality in patients with epilepsy is recommended. F

Question 75

A 76-year-old man is admitted with a right frontal intracerebral hemorrhage. After some initial improvement in his neurologic status, he has become a bit less responsive. EEG shows fluctuating right greater than left periodic discharges at 1 to 2 Hz without definite evolution. Which of the following is the best next step in management?

Answer 75

Nonconvulsive status epilepticus is increasingly being identified as a cause of altered mental status in critically ill patients, including patients with structural brain lesions such as intracerebral hemorrhage. The persistent mental status change is suggestive of nonconvulsive status epilepticus. Initial management consists of sequential administration of low-dose benzodiazepines (preferably short-acting agents, such as midazolam) or IV administration of a relatively nonsedating antiepileptic medication (such as levetiracetam or lacosamide). After initiation of these medications, improvement in the clinical state
and EEG is supportive of a diagnosis of nonconvulsive status epilepticus. Initiation of additional antiepileptics should be held until the diagnosis is clearer.

Question 76

A 6-year-old child with Lennox-Gastaut syndrome and medically resistant epilepsy is currently on two anticonvulsants and still has several seizures daily despite trials of more than five antiepileptic drugs. Dietary treatment with a ketogenic diet is proposed. What is the approximate percent chance that this patient will respond with a greater than 50% seizure reduction?

Answer 76

50%

Randomized controlled studies and meta-analyses have shown that approximately 50% of children with pharmacoresistant epilepsy have greater than 50% reduction in seizures on the ketogenic diet.

Question 77

A 25-year-old man with a 10-year history of medically intractable focal epilepsy undergoes an evaluation for possible epilepsy surgery. His clinical symptoms and EEG findings suggest a left mesial temporal focus. Which of the following MRI sequences is most sensitive for diagnosing hippocampal sclerosis in this patient?

Answer 77

Fluid-attenuated inversion recovery (FLAIR)

Both routine T2-weighted images and FLAIR images are useful, with FLAIR images being the most sensitive, for the demonstration of the abnormalities associated with hippocampal sclerosis defined on histopathological examination.

Question 78

A 33-year-old man with known epilepsy is seen in the emergency department after a seizure with an associated fall. IV access is obtained. He is initially at his baseline neurologic status but then develops generalized seizure activity that has lasted 5 minutes. Which of the following is the most appropriate next step in management?

Answer 78

IV lorazepam

This patient with underlying epilepsy has had continuous seizure activity for at least 5 minutes and meets criteria for status epilepticus. In patients with IV access, IV lorazepam is considered the initial treatment of choice. It has been shown
to be more effective than IV diazepam or phenytoin. In patients without IV access, administration of rectal diazepam or buccal midazolam can be considered.

Question 79

The presence of a photoparoxysmal response to photic stimulation
during an EEG is most commonly seen in which of the following settings?

Answer 79

Primary generalized epilepsy

The photoparoxysmal response is an abnormal response seen in the setting of photic stimulation and goes beyond the ‘‘photic driving’’ of the posterior dominant rhythm seen in cognitively normal patients. It can be seen in different types of epilepsy but is most commonly seen in primary generalized epilepsies, especially childhood absence epilepsy.

Question 80

In addition to a risk of major congenital malformations, which of the following antiepileptic drugs is also associated with an elevated risk of autism spectrum disorders and lower IQ at age 6?

Answer 80

Valproate

Women treated with valproate (either monotherapy or in combination with other antiepileptics) are at elevated risk of giving birth to children with major congenital malformations. In addition, of the medications listed above, valproate is also associated with
a higher risk of neurodevelopmental effects in children exposed during pregnancy, as measured by lower IQ at age 6 and a higher risk of autism spectrum disorders.