Hirayama Disease: A clinical-radiological review

Abstract

Hirayama disease, also known as monomelic amyotrophic, is a rare neurological entity. The disease is characterized by progressive muscle wasting in the distribution of nerve roots C6, C7 and T1. It is believed to be a result of forward displacement of the dural sac and spinal cord induced by neck flexion. We present the case of a 16-year-old boy with progressive weakness of left upper limb.