Hereditary neuropathy with liablity to pressure palsies

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Singapore Med J. 2006 Jul;47(7):625-6.

Hereditary neuropathy with liablity to pressure palsies.

Chua SY, Lim YW, Lam KS, Low CO.

Department of Orthopaedic Surgery, Changi General Hospital, 2 Simei

Street 3, Singapore.

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.