(Indian Society for Health and Advanced Research, 2024-01) Hazra, Sneharghya; Acharyya, Asok Kumar; Jhilik, Dyuti
Uncommon in the clinical setting with no de?nite etio-pathogenesis, Hirayama disease (HD), also referred to as non progressive juvenile spinal muscular atrophy usually unilateral, is characteristically seen in young adolescent males in the east. We report a case of a 19-year-old male with asymmetric amyotrophy of distal upper limb with oblique atrophy and reverse split hand syndrome. He was diagnosed to be a sporadic case of typical HD, in an early plateau phase according to the Tashiro guidelines as well as the latest Huashan Diagnostic Criteria. Early intervention with physiotherapy and hard cervical collar showed no progression of disease after 12 months' follow-up. Clinicians should be vigilant about myriad of unusual presentations of HD in daily practice.