REFLEX WRITING SEIZURES IN TWO SIBLINGS WITH JUVENILE MYOCLONIC EPILEPSY
Abstract number :
G.05
Submission category :
Year :
2002
Submission ID :
1545
Source :
www.aesnet.org
Presentation date :
12/7/2002 12:00:00 AM
Published date :
Dec 1, 2002, 06:00 AM
Authors :
Rosanna Chifari, Ada Piazzini, Vincenzo Sgro[ssquote], Raffaele Canger, Maira Paola Canevini. Regional Centre of Epilepsy, San Paolo Hospital, Milano, Italy
RATIONALE: There have been very few reports on reflex epilepsy induced by writing, indicating that such reflex seizures may be very rare, even though, like many rare disorders, they may be underdiagnosed. The pathophysiologic mechanism of this curious disorder is actually unknown, and controversy remains whether writing epilepsy should be included among localization-related epilepsies or it represents a peculiar form of idiopathic generalized epilepsy with seizure precipitate by specific modes of activation. Here we report on two sisters with juvenile myoclonic epilepsy (JME) where writing activity triggered epileptic myoclonic jerks of right arm and hand
METHODS: The patients are two girls of 20 and 18 years of age respectively. Both girls are right-handed, the product of a normal pregnancy, delivery, with normal psychomotor development. At the age of 14 and 13 years respectively, they began to experience early morning seizures while awaking, which were characterized by myoclonus in both arms. Their interictal EEG showed normal background activity and rare generalized spike-wave complexes. A diagnosis of idiopathic generalized epilepsy was made, treatment with valproate (VPA), at the dosage of 400 mg/day), was started, and almost complete control of seizures was achieved. After 3-4 years, they began to present rare myoclonic jerks occasionally triggered by writing Both patients underwent porlonged video-EEG monitoring using writing-tasks of increasing difficulty that required a parallel increased degree of concentration.
RESULTS: In both patients, on video-EEG monitoring we depicted reflex epileptic myoclonus triggered by writing. Most important, myoconic jerks were more easily triggered by writing that required a higher degree of concentration. Conversely, in both patients other cognitive tasks, such as reading, typing, thinking, calculation never elicited any seizures or myoclonus. Valproate was effective in controlling both spontaneous and reflex epileptic seizures.
CONCLUSIONS: The results of this study further support the notion that [dsquote]praxis-induced[dsquote] reflex epilepsy precipitated by specific stimuli occurs in the context of idiopathic generalized epilepsy. Our results also illustrate that writing tasks are more effective in eliciting seizures, when they require higher levels of concentration and mental elaboration.