[Visual and somatosensory evoked potentials in hereditary motor-sensory neuropathies]

Visual (VEP) and somatosensory evoked potential (SEP) were examined in 31 patients with hereditary motor-sensory neuropathy (HMSN); in the great majority HMSN type I was involved. A prolonged latency of P100 was found only in the two oldest patients, the amplitude of this wave was, however, in adult patients, as compared with the control group, significantly reduced. On examination of SEP a typical finding were N9,N13 waves which were difficult to assess and a spinal wave with markedly prolonged latencies of N20 and P40. The amplitude of these cortical components declined with advancing age. Rather surprisingly the most severe affection of the peripheral sensory and motor neuron was found in the youngest generation of children, while in the older generation of parents the central affection was more marked.