Monthly Case

A 22-year-old male patient had suffered a first and up to now single generalized tonic-clonic seizure at the age of 11 years. Reports on initial EEGs demonstrated contradictory results with irregular bilateral spike-waves or normal recordings. Cranial MRI was normal. After that index seizure, the patient was treated with valproate, with increasing age the dose was adjusted up to 2,000 mg daily.

When the patient turned to be 18 years, he presented in our epilepsy outpatient clinic for adults. As he had been seizure-free for 7 years and as he had suffered only one seizure, we reduced valproate and – with a further normal EEG – eventually withdrew the antiepileptic.

In the further course, the patient reported new-onset jerks in his legs resulting in falls. On further inquiry, he reported jerks in the upper extremities as well. Jerks only manifested in the first hour after awakening in the morning. Another prerequisite for jerks was a significant reduction of total sleep time to less than 6 h. On nights with conceivably reduced night sleep, he took – on his own decision – 500 mg valproate which completely prevented occurrence of jerks the next morning.

This young patient suffers from juvenile myoclonic epilepsy, myoclonus was suppressed for years by valproate and occurred only after withdrawal of the antiepileptic. Thus, the correct diagnosis could only be made after valproate withdrawal.

Therapeutically, we decided rather pragmatically to continue the path the patient had already taken. If sleep deprivation is foreseeable, he takes 500 mg valpraote the night before. We further recommended to avoid sleep-deprivation and to consume alcohol in moderate amounts only, as excessive alcohol intake may trigger seizures in juvenile myoclonic epilepsy.

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