Hereditary neuropathy with liability to pressure palsies - A case report

Hereditary neuropathy with liability to pressure palsy is a rare autosomal dominant disorder characterized by multiple episodes of focal demyelinating neuropathies after minor trauma to peripheral nerves. It usually appears i n early adulthood with recurrent attacks of pain, numbness, and muscular we akness along the distribution of the clinically affected nerve. Segmental d emyelination and thickenings of the myelin sheath are the pathologic findin gs. Electrophysiologic studies show a nonuniform mild demyelinating neuropa thy with prolonged distal latencies. Genetic tests are available to aid in diagnosis as molecular analysis has identified a deletion in the chromosome 17p11.2 in the majority of these patients. There is a paucity of informati on in the orthopaedic literature regarding hereditary neuropathy with liabi lity to pressure palsy. A case report is presented of a patient with this d isorder to promote awareness and recognition that this entity should be con sidered in patients with multiple nerve palsies.