Dynamic examinations in MRI scanners crucial in diagnosing cervical flexion myelopathy (Hirayama Disease)

Introduction

Hirayama disease (HD), a juvenile distal amyotrophy of the unilateral upper limb, is a rare cervical flexion myelopathy involving C7, C8, and T1 myotomes. It has an insidious and often unilateral onset, with muscular atrophy and weakness in the forearms and hands. It predominantly and disparately affects young Asian males, especially people from the Indian sub-continent, with an approximate male-to-female ratio of 7:1 [Foster E et al. J Clin Neurosci 2015; 22: 951]. HD is a sporadic, benign, non-progressive disorder. Although muscle atrophy initially progresses, spontaneous arrest usually occurs several years after onset. However, there are rare cases in which symptoms progress in adulthood after a stable course [Preethish-Kumar V et al. Neurol India 2018; 66: 1094], emphasizing the importance of long-term follow-ups. Polavarapu et al. documented cases of familial HD [Polavarapu K et al. Amyotroph Lateral Scler Frontotemporal Degener 2018; 19: 38].

Publication History

Received: 30 January 2024

Accepted after revision: 03 June 2024

Article published online:
08 July 2024