A Rare Case of Hirayama Disease with Ulnar Nerve Palsy: Expanding the Clinical Spectrum

ABSTRACT Hirayama disease, or monomelic amyotrophy, is a rare, self-limiting cervical myelopathy that primarily affects young males. It leads to muscle atrophy and weakness, especially in the hands and forearms, often due to cervical spinal cord compression during neck flexion. We report a case of a 41-year-old female from rural India who presented with progressive weakness in her left hand, resulting from ulnar nerve compression. The patient was unable to actively extend the metacarpophalangeal joint of her left hand, significantly impairing her hand function. Notably, she did not exhibit autonomic symptoms, which are commonly seen in cervical myelopathies. This case highlights the importance of considering Hirayama disease in females, despite its male predominance, and the need for early diagnosis and targeted rehabilitation. Timely intervention is crucial to managing functional impairments and improving patient outcomes.