Electrophysiological findings in a family with Hereditary Neuropathy and Liability to Pressure Palsies

Abstract

Hereditary neuropathy with liability to pressure palsies is an autosomal dominant and demyelinative peripheral neuropathy which characterized by reversible episodes of sensorimotor deficits after neural compression injuries. Their clinical hallmarks are recurrent and painless focal neuropathies maintly preceded by minor trauma or compression at entrapment sites of peripheral nerves. We describe multiple compression mononeuropathies in an individual who presented with left sided ulnar palsy after drilling for a period of 8 hours and report neurophysiologic findings in two clinically asymptomatic family members. We believe that this entity may be clinically and neurophysiologically underdiagnosed by orthopaedic surgeons and electromyographers. Electrophysiological abnormalities can be detected even in asymptomatic patients and it should be considered in differential diagnosis of patients with atypical presentations of compression neuropathies.
Key Words: Hereditary Neuropathy with liability to Pressure Palsies- Electrodiagnostic tests