Surgical Management of Hirayama Disease: A Rare Entity With Unusual Clinical Features
Year: 2025
Type: article
Study design: Case Report
Abstract: Hirayama disease (HD) is a rare neurological disorder. It is a focal, lower motor neuron disease, mainly affecting young males in their second and third decades. It is prevalent most commonly in Asian countries like Japan. Magnetic resonance imaging (MRI) of the cervical spine in flexion will reveal the hallmark features of Hirayama disease. A 22-year-old male presented with a history of insidious weakness of the left hand for 2 years. On examination, he had clawing of the left medial three fingers with wasting of left forearm muscles, which was progressive in nature. MRI showed thinning of the spinal cord from C3 to C7 level, suggestive of cord atrophy. Based on these features, a diagnosis of focal amyotrophy was made. As the patient was non-compliant with the use of the cervical collar, resulting in the progression of symptoms, C3–C6 lateral mass fixation without fusion was performed to stop the progression of the disease and to facilitate implant removal at a later stage and regain cervical motion. Early diagnosis is necessary as the use of a simple cervical collar, which will prevent neck flexion, has been shown to stop the progression in the majority of the cases.
Source: Indian Spine Journal
DOI: 10.4103/isj.isj_1_24
Authors: Suresh Cheekatla, Peddineni Anudeep
Institution: Krishna Institute of Medical Sciences
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4406487086