Case 1: Seizure with bilateral arm jerks and loss of consciousness
An 18-year-old female college student is evaluated in the emergency department because of a seizure that occurred that morning. According to her roommate, the patient woke up at her usual time and had several episodes of brief bilateral arm jerks before she fell and lost consciousness. Her whole body stiffened for five to 10 seconds, and then she had rhythmic clonic jerking of her arms, legs and trunk for about two to three minutes. She remained unresponsive for 10 minutes and then gradually regained complete consciousness over the next hour.
The patient remembers the arm jerks but has no recollection of the seizure. She states that she has been having arm jerks in the morning for several months, which have become worse recently. She has been staying up late every night for the last week studying for final examinations. Neurologic examination is normal.
Which of the following is the most likely diagnosis?
A. Partial seizure with secondary generalization
B. Absence epilepsy
D. Juvenile myoclonic epilepsy
Case 2: Convulsive status epilepticus
A 45-year-old man is evaluated in the emergency department for convulsive status epilepticus. A passerby found him having a seizure on the sidewalk, and paramedics were on the scene within five minutes, bringing him to the emergency department in about 10 minutes. His medical history is not known. There are no external signs of trauma.
A finger stick glucose measurement taken by the paramedics was 90 mg/dL (5 mmol/L). Serum is obtained for measurement of glucose and electrolytes and a toxicology screen.
What is the most appropriate initial intervention for this patient?
A. CT of the head
C. Phenytoin, intravenously
D. Phenobarbital, intravenously
Case 3: Generalized tonic-clonic seizure
A 27-year-old-man is evaluated for a first generalized tonic-clonic seizure. He recalls that he has had two episodes in the preceding six months during which he briefly lost awareness and his friends said he stared blankly and hummed. These episodes lasted less than 30 seconds. He takes no medications.
At age 17 years, he sustained a head injury with a 20-minute loss of consciousness. There were no other sequelae. Electro-encephalogram shows focal slowing over the left temporal lobe. MRI of the head is consistent with an area of resolved hemorrhage at the left temporal tip.
What is the most appropriate treatment for this patient?
E. Vagus nerve stimulation
Case 4: Inability to speak
A 34-year-old man is evaluated for a one-month history of episodes during which he is suddenly unable to speak. He has had five such episodes, the last three during the past week. No warning precedes the episodes, and they last 20 to 30 seconds. During some episodes, the patient has twitching of the right side of his face; on one occasion the twitching progressed to involve his right arm.
The patient states that he is fully aware of his surroundings during the episodes and has no other symptoms. He has no significant medical history and takes no medications. Physical examination is normal.
Which of the following is the most likely diagnosis?
A. Transient ischemic attack
B. Frontal lobe seizure
C. Hemiplegic migraine
Case 5: Follow-up for epilepsy
A 24-year-old woman is evaluated in follow-up for epilepsy. She had her first complex partial seizure 20 months ago, which she described as a rising epigastric sensation followed by déjà vu and then loss of awareness. Observers described her as having a blank stare, with chewing and dystonic posturing of the left arm and automatisms of the right. Seizures occurred three times a month during the two months before her diagnosis and during the first month after therapy was started.
She was treated with oxcarbazepine but developed hyponatremia that did not respond to fluid restriction. Therapy was then switched to carbamazepine, but she continued to have one complex partial seizure every one to two months, usually at the time of menses. Therapy was switched to topiramate, which did not alleviate her seizures. In general, she feels well on topiramate therapy except for afternoon fatigue and increased need for sleep.
Risk factors for epilepsy include a 30-minute febrile convulsion at two years of age. She has subjective memory impairment, although she functions well in her job as a financial analyst. Neurologic examination is normal. MRI of the head shows increased signal in the right hippocampus, but there is no obvious atrophy.
Which of the following is the most appropriate intervention at this time?
A. Switch therapy to valproate
B. Increase the dosage of topiramate
C. Add a second antiepileptic medication
D. Consider vagus nerve stimulation
E. Refer for evaluation for epilepsy surgery
Case 6: Recurrent seizures
A 28-year-old woman is evaluated for recurrent seizures. She has a history of absence seizures and two generalized tonic-clonic seizures between ages seven and 12 years. She was subsequently seizure-free until four months ago. She now has seizures that begin with anxiety and are then followed by an inability to speak (although she can hear). Within 30 seconds to one minute she loses awareness. Her husband says that her head moves to and fro, and she will grab onto him or another object and rock back and forth. She often whimpers but does not speak.
These episodes usually last 10 to 15 minutes but may occur in series over one or two hours. The seizures occur two to five times per week. She did not respond to an initial trial of carbamazepine and is now receiving levetiracetam.
Neurologic examination is normal. Electroencephalogram is normal awake, although she was not having an episode during the procedure. MRI of the head is normal.
What is the most likely diagnosis of her current seizures?
A. Complex partial seizure
B. Absence seizure
C. Nonepileptic psychogenic seizure
D. Juvenile myoclonic epilepsy
Answers and commentary
Correct answer: D. Juvenile myoclonic epilepsy.
The patient had myoclonic jerks that led to a generalized tonic-clonic seizure. This is a typical presentation of juvenile myoclonic epilepsy, an idiopathic epilepsy syndrome. Affected patients have a normal neurologic examination and normal development. Unlike other idiopathic epilepsies, remission is rare even after years without seizure, so lifelong treatment is necessary.
Patients are sensitive to sleep deprivation, alcohol and stress. It is often necessary to specifically ask the patient whether there have been any other episodes of jerks involving the head, extremities or trunk. Patients may have been experiencing these for some time but not have associated the myoclonus with seizures.
Unlike juvenile myoclonic epilepsy, which is a primary, genetic, generalized epilepsy, partial seizures originate from a focal part of the brain and their clinical presentation depends on their neuroanatomic location. A partial epilepsy may present with a simple partial seizure (consciousness not impaired) or complex partial seizure (consciousness impaired) before progressing to a generalized tonic-clonic seizure, although some patients with partial epilepsies present with generalized tonic-clonic seizures only. In this instance, the patient with partial epilepsy may show a focal abnormality on electroencephalography or on MRI.
Idiopathic generalized epilepsies present with only generalized onset seizures, which may be convulsive or nonconvulsive. Absence seizures are nonconvulsive generalized onset seizures that mainly affect children. Absence seizures manifest with sudden onset of staring, with or without eye blinking or lip smacking.
The other options are unlikely. Patients with syncope often have several myoclonic jerks while unconscious, but they recover quickly once blood pressure is reestablished. Nonepileptic events (pseudoseizures) vary in presentation but are often associated with moaning, crying and arrhythmic shaking of the body.
- Juvenile myoclonic epilepsy is a primary, genetic, generalized epilepsy that typically manifests with myoclonic jerks followed by a generalized tonic-clonic seizure.
Correct answer: B. Lorazepam.
This patient is in status epilepticus and must be treated urgently. Once his airway is stabilized and glucose is determined to be normal, he should be treated with parenteral antiepileptic medications. The best first medication is a benzodiazepine because of its potent antiepileptic properties and rapid onset of action. Lorazepam is preferred over diazepam because of its longer half-life. Phenytoin (or intravenous fosphenytoin) may be administered concurrently but has a slower onset of action than a benzodiazepine. Therefore, giving phenytoin should not delay benzodiazepine administration.
Phenobarbital is an excellent agent for treatment of status epilepticus but is generally used only in patients who fail to respond to lorazepam and phenytoin, because of its profound pulmonary depressant effects. Diagnostic studies to determine the cause of the status epilepticus should never delay pharmacotherapy.
- In a patient with status epilepticus, after the airway is stabilized and plasma glucose is determined to be normal, parenteral antiepileptic medications should be started.
- Lorazepam is the preferred antiepileptic medication for a patient in status epilepticus.
Correct answer: D. Oxcarbazepine.
The most appropriate treatment for this patient is to begin oxcarbazepine. This patient has partial onset epilepsy with complex partial seizures and a generalized tonic-clonic seizure. He should be started on an antiepileptic drug because he has several risk factors for recurrent seizures, including multiple previous seizures, a history of significant head trauma, focal electroencephalographic abnormalities and a structural abnormality on MRI.
Phenobarbital is not the best choice because of frequent associated sedation and depression. Ethosuximide is used for absence seizures and is not effective for partial onset seizures. The vagus nerve stimulator is appropriate for adjunctive therapy in patients with medically intractable seizures. Oxcarbazepine is the only therapy choice listed here that is effective for partial onset seizures as initial monotherapy. Lamotrigine or valproate may be effective, although neither is approved for initial management of partial onset seizures.
- Oxcarbazepine is effective monotherapy for partial onset seizures.
- Risk factors for recurrent seizures include multiple previous seizures, a history of significant head trauma, focal electroencephalogram abnormalities and structural abnormality on MRI.
Correct answer: B. Frontal lobe seizure.
The patient most likely has simple partial seizures of frontal lobe origin. The clinical presentation of partial seizures depends on their neuroanatomic location. In this case, the seizures originate in Broca's language area and spread to the primary motor area. Frontal seizures are brief and usually without any aura or postictal confusion. The recent onset of seizures should lead to brain imaging as soon as possible to rule out a space-occupying lesion. Transient ischemic attack, hemiplegic migraine and hypoglycemia can cause focal neurologic deficits but would not cause twitching of the face and arm.
- The manifestations of partial seizures depend on their neuroanatomic location.
- Frontal seizures are brief and are usually not associated with aura or postictal confusion.
Correct answer: E. Refer for evaluation for epilepsy surgery.
This patient should be evaluated with video electroencephalography to determine whether she is a candidate for epilepsy resective surgery.
Despite new and generally well-tolerated medications, more than 30% of patients with epilepsy continue to have seizures or medication-associated side effects that limit their quality of life. About 50% of patients treated for epilepsy become seizure free on the first antiepileptic drug tried. An additional 15% become seizure free with the second trial, but only an additional 5% become seizure free with the third trial, whether that is a third drug used as monotherapy or a combination of two antiepileptic drugs. Therefore, patients who have failed to respond to three medication trials are unlikely to ever be seizure free with drug therapy.
Treatment-resistant patients should be evaluated for a surgically remedial epilepsy syndrome, such as mesial temporal sclerosis with partial seizures, or neocortical epilepsy caused by focal, resectable lesions. Surgery can result in the patient being seizure free in about 85% of cases and has an excellent risk profile in patients with these syndromes. Patient selection is critical, and the assessment should be conducted at an experienced epilepsy center. The vagus nerve stimulator is an appropriate option if this patient does not have a surgically remedial epilepsy.
- Patients with epilepsy who fail to respond to three trials of antiepileptic drugs are unlikely to ever become seizure free with drug therapy.
- Treatment-resistant patients with epilepsy should be evaluated for a surgically remedial epilepsy syndrome.
Correct answer: C. Nonepileptic psychogenic seizure.
This patient most likely has nonepileptic seizures of psychogenic origin based on the duration and behavior characterizing the events. The majority of epileptic seizures last less than three minutes; a seizure lasting 10 or more minutes is unusual. To and fro head movements are rare in epileptic seizures (although rocking and whimpering may occur in complex partial seizures). Anxiety is often a prominent component of nonepileptic events of psychogenic origin. The history of epilepsy does not argue against a diagnosis of nonepileptic events. About 30% of persons with nonepileptic seizures of psychogenic origin also have a history of epilepsy.
This patient's presentation is not typical of the other types of seizures. Absence seizures are characterized by a brief loss of awareness with no movement or very subtle movements of the lips and fingers. Juvenile myoclonic epilepsy is characterized by myoclonic seizures and often accompanied by absence and generalized tonic-clonic seizures.
In this setting, video electroencephalographic monitoring or ambulatory electroencephalography to capture an event is the gold standard to establish the diagnosis. A psychiatric or psychological evaluation would also be helpful to assess risk factors for nonepileptic events of psychogenic origin.
- Nonepileptic seizures of psychogenic origin can be differentiated from epilepsy by their longer duration, normal electroencephalogram findings and maintenance of consciousness.
- Nonepileptic psychogenic seizures are often associated with moaning, crying and arrhythmic shaking of the body.
- Absence seizures are characterized by a brief loss of awareness with no movement or very subtle movements of the lips and fingers.
- Juvenile myoclonic epilepsy is characterized by myoclonic seizures and often accompanied by absence and generalized tonic-clonic seizures.