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

F Gökçem Yıldız; Gülay Nurlu; Sevim Erdem Özdamar; Çağrı Mesut Temuçin

doi:10.4274/tnd.70446

F Gökçem Yıldız¹, Gülay Nurlu², Sevim Erdem Özdamar², Çağrı Mesut Temuçin²

¹Hacettepe University Institute of Neurological Sciences and Psychiatry, Ankara, Turkey
²Hacettepe University Faculty of Medicine, Department of Neurology, Ankara, Turkey

Keywords: Hereditary neuropathy with liability to pressure palsy, entrapment neuropathy, peripheral myelin protein 22

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.