Clinical features and neurophysiological follow-up in a case of Brown-Vialetto-Van Laere syndrome.

Brown-Vialetto-Van Laere syndrome is a rare disease of unknown origin commonly considered as part of the large group of motor neuron diseases. The course is quite variable: it may be quickly fatal or protracted, with relapsing phases followed by periods of arrest and even partial improvement. We describe a case of Brown-Vialetto-Van Laere syndrome with strong family history for sensorineural hearing impairment. The patient came to our medical attention for severe respiratory failure and leg weakness. The clinical conditions partially improved with recovery of spontaneous respiration and mild increase in muscle strength. The neurophysiological studies performed on our patient showed evidence of nerve damage with subsequent improvement. Our study raises the possibility that the disorder is due to primary nerve damage, which can better justify the intermittent course of the disease, the partial clinical regression and the neurophysiological improvement, never detected in typical motor neuron disorders.

[Motor conduction velocities and distal latencies in multiple sclerosis]. / Studio della VCM e delle latenze distali nella sclerosi multipla.

Motor conduction velocities (MCV) and distal latencies (DL) of the ulnar nerve and of the external popliteal sciatic nerve have been recorded in 21 cases of multiple sclerosis. Only in 4 cases (19%) all values were found within normal limits. The maximal MCV of one or both nerves appeared to be slightly reduced in 7 cases (33%) and the minimal MCV was reduced more markedly in 13 cases (62%). The MCV alterations (both minimal and maximal) were more frequent among patients with marked hypertonus. The ulnar nerve was the most frequent site of such alterations. LD were markedly altered in 17 cases (81%), independently from the entity of pyramidal signs.