Hereditary neuropathy with liablity to pressure palsies

Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is a disease that presents with recurrent reversible episodes of neurapraxia that occur typically after trivial trauma. It is an autosomal dominant, demyelinating neuropathy. A 20-year-old man presented with left ulnar nerve palsy after a fall. He had reduced two-point discrimination over his left ulnar nerve distribution, with mild clawing of the ring and little fingers, and accompanying weakness of the first dorsal interrosseus and abductor digiti minimi of grade four power. His Froment's sign was also positive. Careful clinical examination and appropriate tests, including electromyography and genetic testing, confirmed the diagnosis of HNPP. This case report is presented to promote awareness and recognition of this disease in the local and regional context.