A Confirmed Case in the United Kingdom of Hirayama Disease in a Young White Male Presenting with Hand Weakness.

Abstract

We present the first reported case of Hirayama disease in the United Kingdom. A literature review of Hirayama disease in the Western literature shows that this case is unique in being the first in the United Kingdom, constituting one of only a few cases in Europe with supporting magnetic resonance imaging and reported treatment outcome. Our patient was a young Caucasian male who presented with progressive bilateral hand weakness, had confirmatory magnetic resonance imaging findings of Hirayama disease, and experienced improvement of symptoms with cervical collar immobilization. This case lends further evidence to the flexion-induced myelopathy theory of Hirayama disease and supports the findings of other studies in which avoidance of neck flexion and cervical immobilization helped to attenuate disease progression in this patient group.