HNPP: Rare Cause of Entrapment Neuropathies in Young Adults

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Turk Neurosurg. 2008;18(1):82-84.

Hereditary Neuropathy with Liability to Pressure Palsies in a Turkish

Patient (HNPP): A Rare Cause of Entrapment Neuropathies in Young

Adults.

Celýk Y, Kilinçer C, Hamamcioðlu MK, Balci K, Býrgýlý B, Cobanoðlu S,

Utku U.

Trakya University School of Medicine Neurology, Edirne, Turkey.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an

autosomal dominant nerve disease usually caused by 1,5 Mb deletion on

chromosome 17p11.2.2-p12, the region where the PMP-22 gene is

located. The patients with HNPP usually have relapsing and remitting

entrapment neuropathies due to compression. We present a 14-year-old

male who had acute onset, right-sided ulnar nerve entrapment at the

elbow. He had electrophysiological findings of bilateral ulnar nerve

entrapments (more severe at the right side) at the elbow and

bilateral median nerve entrapment at the wrist. Genetic tests of the

patient demonstrated deletions in the 17p11.2 region. The patient

underwent decompressive surgery for ulnar nerve entrapment at the

elbow and completely recovered two months after the event. Although

HNPP is extremely rare, it should be taken into consideration in

young adults with entrapment neuropathies.