title: [Diagnostic differentiation between two syndromes of progressive myoclonus epilepsy].
abstract: The study aimed to differentiate, according to clinical and electroencephalographical criteria, between the most frequent types of myoclonus epilepsy--Unverricht-Lundborg disease (ULD) and Lafora disease (LD). Two patients with ULD and two with LD, aged 13-16 years, have been examined. In all cases, the diagnosis of myoclonus epilepsy has been verified by using molecular genetic analysis. The differential diagnostic criteria have been ascertained for ULD and LD: the earlier age-of-onset in ULD comparing to LD (8.8 and 12.5 years, respectively); tonic clonic paroxysms in ULD and partial ones with visual aura in LD; a presence of nonepileptical subcortical myoclonus, according to EEG data (visual monitoring); negative myoclonus emerging in manifested stage of LD; appearing of regional epileptiform activity for posterior regions on EEG in LD; more rapid progressiveness of extra pyramidal disturbances with organic dementia outcome in myoclonus LD.
authors.forename: K Iu
authors.initials: KIu
authors.lastname: Mukhin
authors.forename: A S
authors.initials: AS
authors.lastname: Petrukhin
authors.forename: E Iu
authors.initials: EIu
authors.lastname: Rogova
authors.forename: S V
authors.initials: SV
authors.lastname: Piliia
authors.forename: E Iu
authors.initials: EIu
authors.lastname: Volkova
authors.forename: L Iu
authors.initials: LIu
authors.lastname: Glukhova
mesh_terms: D000293:Adolescent; D001921:Brain / Q000503:physiopathology; D003937:Diagnosis, Differential; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D012720:Severity of Illness Index; D013577:Syndrome
vernacular_title: Differentsial'nyĭ diagnoz dvukh sindromov progressiruiushcheĭ mioklonus-épilepsii.