Longitudinal EEG studies in a kindred with Lafora disease.
We reviewed 18 EEG studies in four members of a family with the Lafora form of progressive myoclonic epilepsy. Each patient was the product of a consanguinous marriage and presented as a teenager with progressive seizures, myoclonus, dementia, and ataxia, and had biopsy proven disease. The EEG early in Lafora disease has spike-wave activity resembling that seen in a primary generalized epilepsy; the background slowing is more typical of a secondary generalized epilepsy. With disease progression, there is increased epileptiform activity, and a striking change in the spike-wave complexes, with a marked increase in frequency up to 6-12 Hz, and many more short duration polyspike components. Unlike some other forms of secondarily generalized epilepsy, the EEG in Lafora disease is distinguished by an increased frequency of the spike-wave complexes with disease progression.
Yen
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C
Department of Neurology, University of Michigan, Ann Arbor.
Beydoun
A
A
Drury
I
I
D000293:Adolescent; D001611:Beta Rhythm; D001921:Brain / Q000503:physiopathology; D002648:Child; D003241:Consanguinity; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male