【黄博士罕见病例分享】one rare case of hirayama disease for ten years 平山病一例

Hirayama is a rare nonfamiliar monomelic amyotrophy also known as benign juvenile brachial spinal muscular atrophy, juvenile asymmetric segmental spinal muscular atrophy, juvenile muscular atrophy of the distal upper extremity, monomelic amyotrophy, and oblique amyotrophy. Classically, it presents with muscle atrophy and weakness of bilateral or unilateral forearms and hands in the absence of sensory alterations, progressing for one to two years before plateauing. The condition is caused by a tight dural sac in the cervical canal that leads to chronic ischemic changes to the anterior horn cells. Although commonly considered a non-progressive and self-limiting disease, this has been a source of significant disability for some affected individuals. This activity reviews the evaluation and management of patients with this condition and highlights the role of the interprofessional team in caring for these patients.

小红书链接

一例罕见的上肢肌肉萎缩

平山病(Hirayama disease)是1959年由日本学者平山惠造(Keizo Hirayama)首次报道，累及脊髓前角细胞，主要影响C7-T1脊髓神经及其支配的肌肉，以上肢远端不对称肌萎缩为特征表现的一种少见疾病，且初步认为该病是良性自限性疾病