[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.