WHITE-MATTER ABNORMALITIES IN CONGENITAL MUSCULAR-DYSTROPHY

Central nervous system (CNS) characteristics were examined in seventee n patients with autosomal recessive classic or ''pure'' congenital mus cular dystrophy (CMD). In three patients, neuroradiological examinatio n (CT/MRI) indicated hypodense white matter areas. Two out of these th ree patients had epilepsy (seizures and epileptic discharges on their EEG). Only two of the remaining patients had epileptic EEG discharges, but without clinical seizures. By comparing our results to data in th e literature, we could conclude that the classic or ''pure'' form of C MD can be subdivided into two subtypes, i.e. those with and those with out white matter hypodensities. A mild form of epilepsy or an epilepti c predisposition on EEG can be part of the subtype with white matter h ypodensities.