BAEPs in hereditary motor-sensory neuropathies. Involvement of central brain-stem parts of the auditory pathway.
Brain-stem auditory evoked potentials (BAEPs) were tested in 34 patients with hereditary motor and sensory neuropathy (HMSN), most of which with HMSN type I with the autosomal dominant type of inheritance. In only a few cases a recessive or sporadic form of the disease was diagnosed, and one patient was rated as HMSN type III. In 44% complicated forms with signs of CNS involvement (cerebellar manifestation, nystagmus or cranial nerve lesions) were ascertained. BAEPs tests revealed slightly, albeit non-significantly prolonged wave III latency while wave V latency was significantly prolonged and so was the interpeak interval of waves III-V. One third of the records showed a prominent decrease in wave V amplitude.
Nevsímalová
S
S
Department of Neurology, Charles University Medical Faculty, Prague, Czechoslovakia.
Dlouhá
O
O
Roth
B
B
D000293:Adolescent; D000328:Adult; D001306:Auditory Pathways / Q000503:physiopathology; D001933:Brain Stem / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004576:Electromyography; D005072:Evoked Potentials, Auditory; D005260:Female; D005808:Genes, Recessive; D015417:Hereditary Sensory and Motor Neuropathy / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011930:Reaction Time / Q000502:physiology