Dr. Wilner graduated from Yale University and Brown University School of Medicine. He is board certified in internal medicine and neurology, with added qualifications in clinical neurophysiology. He was medical director of the Carolinas Epilepsy Center, Charlotte, NC, and then Clinical Associate Professor of Neurology at the Brown University School of Medicine, Providence, RI. Dr. Wilner has a lifelong interest in writing fiction and nonfiction, and writes for many medical and other publications. He received the American Academy of Neurology's Creative Expression of Human Values Award (2001) and is the author of two books on epilepsy; Epilepsy: 199 Answers and Epilepsy in Clinical Practice. More information regarding Dr. Wilner and his publications can be found at: www.drwilner.org


History

F came to the office for his first visit when he was 40 years old. His mother's pregnancy was unremarkable, but he was premature by 6 weeks and blue at birth. He did not walk until 16 months of age or speak until 3 years of age.

F's first convulsion occurred at 15 months of age. He also had brief spells where he appeared afraid and ran to his mother. Despite phenobarbital and phenytoin, he continued to have seizures. After his physician switched him to primidone at the age of 12 years, F became violent and attacked a neighbor with a hatchet. He required institutionalization until he was aged 18.

While taking valproate 2 years ago he again became aggressive and was said to have a 'psychotic reaction'. His behavior improved when he began phenytoin. However, he continues to have seizures three times a month, although they can occur as often as nine times a day. According to his parents, he has right hand jerks, which sometimes proceed to a generalized convulsion. He also has other spells where he smacks his lips and stares. His seizures have failed to improve with acetazolamide, carbamazepine, chlordiazepoxide, clorazepate, diazepam and ethosuximide. He had an episode of status epilepticus when an intercurrent illness resulted in protracted nausea and vomiting.

His past medical history includes hyperthyroidism and hypertension, which he treats with medications. As a result of his seizures, he has fractured both wrists and his right foot and has suffered numerous lacerations on his face and scalp. He has no allergies and does not abuse drugs; nor does he drink alcohol or smoke. F is the only one in his family with epilepsy. His social life is very limited because of his seizures and limited cognitive and social skills. His mother is overprotective. His stepfather mostly ignores him. A prior evaluation included a normal EEG and a normal magnetic resonance imaging (MRI) study. He takes phenytoin (100 mg four times daily) and primidone (250 mg four times daily).

Examination and investigations

F answers questions slowly, but appropriately. His Mini Mental State Examination score is 25/30. His physical examination is normal and his neurological examination is non-focal. Video-EEG monitoring of eight seizures failed to reveal lateralization or localization (because of muscle artifact produced by grimacing and chewing at the onset of each seizure). Depth electrode monitoring revealed that 12 of 13 seizures originated from his right temporal lobe. The origin of the remaining seizure was unclear.

F's repeat MRI scan demonstrated right hippocampal atrophy with increased T2-weighted signal consistent with mesial temporal sclerosis. Positron emission tomography was normal. Neuropsychological testing did not lateralize or localize. The IQ was 83. A Wada test determined that he was left hemisphere dominant for language. Using his left hemisphere, he was able to remember 11 of 12 items. Using his right hemisphere, F couldn't answer any questions correctly.

Diagnosis

Intractable epilepsy caused by partial complex seizures and partial seizures with secondary generalization from the right temporal lobe.

Treatment and outcome

F had a right anterior temporal lobectomy without complication. Postoperatively, primidone was discontinued and he had two breakthrough seizures. I restarted the medication and he remains seizure free on two antiepileptic drugs. He attended adult education classes and passed his high school equivalency exam. He has been to vocational rehabilitation and found some part-time work. He also volunteers at the local hospital. He has learned to drive and can do errands, but he continues to live at home. He has made some friends at the epilepsy support group and has a girlfriend. He has been fishing with his stepfather for the first time.

Commentary

F presented with seizures that failed to respond to treatment. His stepfather considered him little more than a nuisance, and his mother worried about him daily. At the age of 40, there seemed little hope that he would ever live independently or become seizure-free. However, after a new and extensive evaluation, I was able to define an epileptic syndrome that was amenable to surgical treatment.

Many patients who appear 'hopeless' can be helped, whether by new antiepileptic drugs, vagus nerve stimulation or epilepsy surgery. In F's case, a right temporal lobectomy resulted in a 99 % reduction of his seizures and a significant improvement in his quality of life.

What did I learn from this case?

First, I learned that although witnessed reports of seizures can be valuable, they pale in comparison to ictal videotapes. F's convulsions beginning with 'right hand jerks' reported by his family suggested Jacksonian seizures from a left frontal lobe lesion, unlikely to be amenable to seizure surgery. But video-EEG monitoring revealed that F's seizures consisted primarily of chewing and bouncing movements, with additional automatisms of right hand trembling, swinging and kicking of the right leg.

Second, I learned that antiepileptic drugs can subtly – or not so subtly – affect a patient's personality. In F's case, two drugs, primidone (which is notorious for its negative affect on behavior) and valproate (which is not usually associated with behavior abnormalities) altered his personality for the worse. When he was not on either of these drugs, F usually had a very placid disposition.

Third, I learned that if an imaging study is more than a few years old and there is reason to believe the patient has a lesion, a MRI scan with state-of-the-art equipment should be performed. F's first MRI was 'normal'. A follow-up scan revealed clear-cut mesial temporal sclerosis. Many older MRI scans did not have the resolution of today's scans, and significant pathology can be missed.

How did this case alter my approach to the care and treatment of my epilepsy patients?

F's case reinforced the concept that one must always ask: 'why does this patient have epilepsy?' I think that physicians tend to neglect this question because the answer in the past usually was 'I don't know,' as in F's case, even after a thorough evaluation. Now, with modern imaging, we can often answer that question and arrive at a more accurate diagnosis and prognosis. Learning why F had epilepsy permitted me to offer him the appropriate treatment.

Further reading

  • Benbadis SR. Is the underlying cause of epilepsy a major prognostic factor for recurrence? Neurology 1999;53:440.
  • Engel J. Etiology as a risk factor for medically refractory epilepsy. Neurology 1998;51:1243–4.
  • Semah F, Picot MC, Adam C, et al. Is the underlying cause of epilepsy a major prognostic factor for recurrence? Neurology 1998;51:1256–62.
  • Mattson RH, Cramer JA, Collins JF. Prognosis for total control of complex partial and secondarily generalized tonic clonic seizures. Neurology 1996;47:68–76.

This selection for "Challenging Cases" is from 110 Puzzling Cases of Epilepsy, edited by Dieter Schmidt, MD, and Steven C. Schachter, MD (Martin Dunitz, Publisher, London, 2002).

Authored by: Andrew N. Wilner | MD on 1/2007
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