Delayed Diagnosis of Hirayama Disease Due to an Antecedent History of Trauma: A Report of Two Cases

Abstract

Hirayama illness is a rare neurological condition that first manifests as gradual muscular weakening and wasting of the distal upper limb in young males, followed within a few years by a spontaneous arrest. The spinal cord and cervical dural sac are thought to have been displaced forward by neck flexion, which is thought to be the cause of the condition. In contrast to the Middle East countries, it is more widespread in Asia. We have described here two cases from India, who presented with increasing weakness and wasting of the distal part of their upper limbs after a traumatic event. Although their symptoms were initially thought to be due to trauma, later, they were diagnosed as cases of Hirayama disease (HD), following detailed history and physical examination, radiological investigations and electrophysiological examination. A review of the literature was done which also described electrophysiological and radiographic findings, which supported our diagnosis. For young male patients who present with weakness and wasting of upper limb distal group muscles, HD should be investigated because it is infrequently seen in clinical settings.

Keywords

• electromyography
• hirayama disease
• magnetic resonance imaging
• monomelic amyotrophy
• trauma