title: Longitudinal EEG studies in a kindred with Lafora disease.
abstract: 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.
authors.affiliation: Department of Neurology, University of Michigan, Ann Arbor.
authors.forename: C
authors.initials: C
authors.lastname: Yen
authors.forename: A
authors.initials: A
authors.lastname: Beydoun
authors.forename: I
authors.initials: I
authors.lastname: Drury
mesh_terms: 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