[The E.E.G. and E.M.G. findings in sudanophilic leucodystrophy (author's transl)].
Four patients with infantile familial sudanophilic leucodystrophy confirmed histologically and biochemically were investigated by E.E.G. and E.M.G. The E.E.G. showed a severe, non-specific slow wave abnormality, of moderate voltage and disorganized in 2 cases, with some critical' tonic' discharge. E.M.G. study was normal in relaxed muscle; peripheral nerve stimulation, however, revealed marked slowing of motor conduction velocities favouring a segmental demyelinisation process, which was ultimately confirmed histologically. This study underlines the value of investigation of infantile encephalopathies.
D002540:Cerebral Cortex / Q000503:physiopathology*; D002675:Child, Preschool; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000235:genetics / Q000503:physiopathology*; D004569:Electroencephalography; D004576:Electromyography; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009469:Neuromuscular Junction / Q000503:physiopathology; D010525:Peripheral Nerves / Q000503:physiopathology*; D009435:Synaptic Transmission
Données de l'E.E.G. et de l'E.M.G. dans les leucodystrophies soudanophiles.