Bilateral ulnar palsi ile ortaya çikan basinca hassas herediter nöropati olgusu

Abstract

Hereditary neuropathy with liability to pressure palsy (HNPP) is a rare autosomal dominant disorder characterized by mononeuropathies at sites subject to compression. It causes recurrent, painless and transitory acute nerve palcies and rarely presents in two extremities. In this report, we describe a case of HNPP presenting as isolated, bilateral, acute, painless ulnar palsy. Neurologic examination revealed atrophy and weakness limited in intrinsic hand muscles bilaterally. Electrophysiological examination exhibited mild sensorimotor polyneuropathy, bilateral conduction block of the ulnar nerve at the elbow and mild partial acute denervation findings on the abductor digiti minimi and first dorsal interosseus muscles. The diagnosis was based on clinical and electrophysiological features. This case is reported to emphasize the importance of exploring subclinic polyneuropathy in the patients who admit with multiple, acute, painless neuropathies and the value of electrophysiological examination in the diagnosis of HNPP.