Abstract
Five unrelated patients are described with the clinical and electrical features of eyelid myoclonia with absences (EMA). In this syndrome brief, typical absences occur with rapid eyelid myoclonia associated with retropulsive movements of the eyeballs and occasionally of the head. The seizures are of shorter duration than in childhood absence epilepsy, and are accompanied by less profound impairment of consciousness. The electroencephalogram demonstrates high amplitude discharges consisting of spikes, multiple spikes and slow waves at a fluctuating frequency of 3-5 Hz and following eye closure, which disappear in darkness. Photosensitivity is also seen. Onset is in early childhood and EMA appears to persist into adult life. Treatment is sodium valproate in combination with either ethosuximide or a benzodiazepine. On the basis of the clinical features, EEG findings, and the response to treatment and prognosis, it is suggested that EMA be classified as a specific epilepsy syndrome.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Binnie C. D., Darby C. E., De Korte R. A., Wilkins A. J. Self-induction of epileptic seizures by eyeclosure: incidence and recognition. J Neurol Neurosurg Psychiatry. 1980 May;43(5):386–389. doi: 10.1136/jnnp.43.5.386. [DOI] [PMC free article] [PubMed] [Google Scholar]
- DeMarco P. Eyelid myoclonia with absences (EMA) in two monovular twins. Clin Electroencephalogr. 1989 Jul;20(3):193–195. doi: 10.1177/155005948902000313. [DOI] [PubMed] [Google Scholar]
- Dravet C., Bureau M. L'epilepsie myoclonique benigne du nourrisson. Rev Electroencephalogr Neurophysiol Clin. 1981 Dec;11(3-4):438–444. doi: 10.1016/s0370-4475(81)80083-4. [DOI] [PubMed] [Google Scholar]
- Jeavons P. M. Nosological problems of myoclonic epilepsies in childhood and adolescence. Dev Med Child Neurol. 1977 Feb;19(1):3–8. doi: 10.1111/j.1469-8749.1977.tb08014.x. [DOI] [PubMed] [Google Scholar]
- Loiseau P., Pestre M., Dartigues J. F., Commenges D., Barberger-Gateau C., Cohadon S. Long-term prognosis in two forms of childhood epilepsy: typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci. Ann Neurol. 1983 Jun;13(6):642–648. doi: 10.1002/ana.410130610. [DOI] [PubMed] [Google Scholar]
- Panayiotopoulos C. P., Chroni E., Daskalopoulos C., Baker A., Rowlinson S., Walsh P. Typical absence seizures in adults: clinical, EEG, video-EEG findings and diagnostic/syndromic considerations. J Neurol Neurosurg Psychiatry. 1992 Nov;55(11):1002–1008. doi: 10.1136/jnnp.55.11.1002. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Panayiotopoulos C. P. Fixation-off-sensitive epilepsy in eyelid myoclonia with absence seizures. Ann Neurol. 1987 Jul;22(1):87–89. doi: 10.1002/ana.410220120. [DOI] [PubMed] [Google Scholar]
- Panayiotopoulos C. P., Obeid T., Waheed G. Differentiation of typical absence seizures in epileptic syndromes. A video EEG study of 224 seizures in 20 patients. Brain. 1989 Aug;112(Pt 4):1039–1056. doi: 10.1093/brain/112.4.1039. [DOI] [PubMed] [Google Scholar]