Hereditary neuropathy with liability to pressure palsy (HNPP) is a rare autosomal dominant disease characterized by focal, recurrent, demyelinating peripheral neuropathies. It is caused by deletions of the gene encoding for peripheral myelin protein 22 (PMP22) on chromosome 17. While it may range widely, the most common clinical presentation is an acute, focal mononeuropathy with numbness or muscle weakness after trauma or compression. Diagnostic tools include electrophysiological studies, genetic tests and nerve biopsies. There is no standard surgical or pharmacological treatment. The course of the disease is usually benign, with spontaneous improvement after most episodes of peripheral nerve palsy. HNPP is best managed by early detection, preventative measures, and subsequent treatment of symptoms. According to the medical literature, operative treatment was undertaken in few cases and limited to decompression of the nerve at the classic entrapment sites of the carpal or cubital tunnels. We present a case of multiple tendon transfer (pronator teres to extensor carpi radialis brevis and flexor carpi radialis to extensor digitorum communis) with a two-year follow-up in a 24-year-old woman with HNPP who was affected by irreversible radial nerve palsy, and conclude with a review of the medical literature related to the disease.
Keywords: hereditary neuropathy with liability to pressure palsies (HNPP); hereditary sensory and motor neuropathy; radial nerve; radial neuropathy; tendon transfer.