Peripheral and central conduction times in hereditary pressure-sensitive neuropathy

Abstract

Seven members of a family with histologically proven hereditary pressure-sensitive neuropathy (HPSN) agreed to be examine clinically and electrophysiologically. A sural nerve biopsy specimen taken from the propositus who suffered from a partial brachial plexus palsy showed typical 'sausage-like' myelin sheath thickenings reflecting a failure of axon-adjusted myelination. Reduced motor and sensory conduction velocities involving several nerves were found in the four family members with clinical signs of HPSN. In addition, central conduction times in the auditory and somatosensory pathways were determined measuring the interwave latency I-V in brainstem auditory-evoked potentials and the interpeak latency N14-N20 in median nerve sensory-evoked potentials. Central conduction times in both afferent systems were within normal limits. The absolute delay of peak N14 and N20 in median and P40 in tibial nerve-evoked potentials was probably due to an impaired conduction in the peripheral branch of the bipolar ganglion cell. Whether the central axon branch in the dorsal columns was also involved could not be decided.