The Neurophysiologic Frequency of Hereditary Neuropathy with Liability to Pressure Palsy in Entrapment Neuropathies

Abstract

Objective: Hereditary neuropathy with liability to pressure palsy (HNPP) needs to be differentiated from entrapment neuropathies due to differences in the treatment management. Materials and Methods: Among 5075 patients with entrapment neuropathy, we retrospectively evaluated the neurophysiologic results of 20 patients with three or more entrapments. Results: Ten patients were diagnosed as having HNPP according to their genetic or nerve biopsy results; eight (80%) had bilateral Carpal tunnel syndrome, nine (90%) had bilateral ulnar entrapment neuropathy, eight (80%) had bilateral median and ulnar entrapment together; and three (30%) had one-sided peroneal neuropathy. Conclusion: Our data suggest that analyzing the neurophysiologic studies and keeping HNPP in mind are essential to characterize underdiagnosed patients with HNPP referred for entrapment neuropathy.

Keywords

• Hereditary neuropathy with liability to pressure palsy
• entrapment neuropathy
• peripheral myelin protein 22