RECURRENT FAMILIAL NEUROPATHY WITH LIABILITY TO PRESSURE PALSIES - REPORTS OF 2 CASES AND ULTRASTRUCTURAL NERVE STUDY

Electrophysiological, histological and ultrastructural studies on two patients (one family) with familial neuropathy and a liability to pressure palsies are reported. There was slowing of nerve conduction velocities and increase of distal latencies of clinically affected and clinically non-affected nerves. These alterations were confirmed by biopsy of the sural nerve which had the characterized signs of the disease. Pathogenetically there seems to be an endogeneous metabolic defect of the Schwann cell to synthetize a normal myelin sheath, which is responsible for the particular susceptibility to mechanical damage. © 1979 Springer-Verlag.