With Epilepsy You Never Know

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Walter van Emde Boas

HISTORY

While living in the tropics, Mrs. F had her first generalized convulsion at the age of 3.5 years in association with a fever, which was possibly malarial. She was born after a normal pregnancy of healthy Caucasian parents and had normal psychomotor development.

Seven years later she had a second generalized convulsion, which was provoked by sleep deprivation. At that time a neurologist examined her. Physical examination was normal. The EEG showed right posterior focal slowing but no epileptiform discharges. A computed tomography (CT) scan showed a small, contrast-enhancing lesion in the right frontal lobe. After a negative angiography study, the tentative diagnosis of angioma was changed to cerebral cysticercosis, although cerebrospinal fluid titers remained negative.

Anticonvulsant treatment with phenytoin and carbamazepine was started but did not prevent occasional generalized seizures, which were consistently provoked by sleep deprivation and jet lag after long flights. Brief absence-like episodes, sometimes with mild automatisms, started at the age of 14 years and continued despite treatment up to maximum tolerated doses with carbamazepine and phenytoin. When she was 16 years old, phenytoin was replaced by valproate because of progressive gingival hyperplasia but this had no effect on the seizures. The possible option of epilepsy surgery was suggested but rejected by the patient and her parents until they could return permanently to The Netherlands.

EXAMINATION AND INVESTIGATIONS

The first time I saw Mrs. F she had just turned 18. Generalized seizures had not occurred for more than 20 months but she reported between one and three brief

"absence" spells a day and complained about drowsiness and impaired concentration caused by her medication (valproate 1000 mg/day in two divided doses and carbamazepine 800 mg/day in two divided doses).

Physical examination was normal, as before, but the interictal EEG now showed irregular slow waves and occasional spike—wave discharges over the right anterior region without consistent focal characteristics. A magnetic resonance imaging scan of the brain showed a small (approximately 2.5 cm), well-demarcated round lesion in the anteriomedian part of the right frontal lobe, probably a cavernoma, without signs of progression in comparison to the previous CT scans.

During EEG with video monitoring, the "absences" turned out to be brief complex partial seizures with arrest reaction, loss of contact, and partial amnesia but without additional clinical signs. An ictal EEG showed some attenuation of ongoing activity, followed by an increase of irregular theta and delta activity over both anterior regions, with slight preponderance to the right but otherwise without convincing lat-eralizing or localizing features.

DIAGNOSIS

Symptomatic frontal lobe epilepsy characterized by complex partial seizures with anterior frontal or frontopolar clinical characteristics.1

TREATMENT AND OUTCOME

Epilepsy surgery was discussed again. At that time (1989) it was believed that intracranial EEG and video monitoring were mandatory in all cases without unequivocal scalp EEG localization, even in the presence of a lesion. Mrs. F and her parents did not consider her sufficiently socially or medically impaired to warrant such studies. Furthermore she wanted to finish high school during that summer and it was decided to postpone further procedures.

She switched to controlled-release carbamazepine, which improved her diurnal alertness, and she successfully took her final examinations. She immediately applied for and obtained a scholarship for a 3-year college education in the USA, starting within 3 months. Presurgical evaluation was thus further postponed.

Over the next 3 years her complex partial seizures gradually became more severe, and secondary generalized seizures recurred occasionally. The dose of controlled-release carbamazepine was increased to 1200 mg/day in two divided doses, and phe-nytoin was added but then withdrawn again when disfiguring hirsutism developed. Repeat EEG with video monitoring during a brief holiday in The Netherlands (in 1991) showed more elaborate complex partial seizures, including vocalizations, oro-alimentary automatisms, and some hypermotor automatisms of the arms.2 The ictal EEG remained poorly localizing.

After graduation, Mrs. F returned to The Netherlands for further consultation. At this time her seizure pattern had become more disabling and included frequent urinary incontinence during complex partial seizures. A third video—EEG suggested clinical involvement of the anteriofrontal area as well as the anterior cingulate, insular-opercular, and mesial frontobasal areas,1 suggesting a progressive type of frontal lobe epilepsy.3 Ictal scalp EEG still remained equivocal but late ictal and postictal slowing predominated over the right frontal lobe. Our surgical approach of lesional epilepsy had changed, following the second Palm Desert Meeting on Epilepsy Surgery,4 and surgery was offered without further invasive procedures.

A partial right frontal lobe resection was performed, including complete lesionec-tomy, and Mrs. F became seizure-free as of December 1992. She successfully completed health care—related studies and started a private practice in yet another faraway country. Treatment was gradually reduced to monotherapy with controlled-release carbamazepine 200 mg/day, and follow-up consultations became rare.

In 1998 she wrote to me saying that she was experiencing a problem with insurance because of her epilepsy, despite having been seizure-free for more than 5 years. I advised her to consider herself healed, and to stop her controlled-release carbamazepine gradually over the next 4 months and then to try again with the insurance company. I included a letter to the company, stating that in her case the epilepsy had been surgically cured and that seizure recurrence was extremely unlikely. Four months later I learned that she had relapsed. Since then she has been on controlled-release carbamazepine 800 mg/day in two divided doses, without significant side effects and thus far without further seizures.

COMMENTARY

What did I learn from this case?

First of all I learnt from her that epilepsy is a disorder that people can cope with. Despite significantly disabling seizures, this brave and motivated young woman, assisted by an understanding and supportive family, managed to pursue and achieve the goals of her own choosing, most often on her own terms.

Secondly, I learnt that epilepsy surgery is indeed an elective type of surgery — medical urgencies can be offset by social urgencies and the process of evaluation and surgery must be optimized accordingly.

From the case of Mrs. F I also learnt that epilepsy can be a progressive disorder, even in lesional epilepsy that is associated with a static, non-malignant structural lesion.3

From her, more than from others, I learnt that epilepsy surgery rarely relieves patients from their seizure disorder. In most patients who undergo surgery, we are able to change an intractable epilepsy into a treatable condition, yet with the need for lifelong drug treatment of a perennial lingering and threatening disorder.

Finally I learnt to realize my own limitations better. I had advised this young woman that she was cured of her epilepsy because I wanted to believe her to be cured, not because I knew that she was. With epilepsy you never know.

REFERENCES

1. van Emde Boas W, Velis DN. Frontal lobe epilepsies. In: Meinardi H, vol ed. The epilepsies, part II. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology (revised series) vol 73(29). Amsterdam: Elsevier;2000:37—52.

2. Lüders H , Acharaya J, Baumgartner C, et al. Semiological seizure classification . Epilepsia 1998;39:1006-13 .

3. van Emde Boas W. Longitudinal evolution of frontal lobe complex partial seizures: a progressive seizure syndrome? In:Wolf P, ed. Epileptic seizures and syndromes. London : John Libbey, 1994 : 408-15 .

4. Engel J (ed.) . Surgical treatment of the epilepsies. 2nd ed. New York: Raven. 1993 .

PART V

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