Touiti Boutayna, Souirti Zouhayr
Neurology, Hassan II University Hospital, Fez, MAR.
Cureus. 2024 Dec 10;16(12):e75445. doi: 10.7759/cureus.75445. eCollection 2024 Dec.
Hirayama disease, also known as non-progressive juvenile spinal muscular atrophy of the upper limbs, brachial monomelic amyotrophy, or benign focal atrophy, affects the C7 D1 myotomes; an electromyogram (EMG) shows neurogenic damage in the C7-C8-T1 territories. It causes weakness and amyotrophy of the distal upper limb. Although it usually occurs on one side only, bilateral symmetric cases of Hirayama disease have occasionally been described. It is a slow, progressive disease, and its evolution can have a good prognosis when detected early in the process. We describe a clinical observation of Hirayama disease, including its clinical and paraclinical peculiarities, and compare it to data from previous studies.
平山病，也称为上肢非进行性青少年脊髓性肌萎缩症、臂丛单肢肌萎缩症或良性局灶性萎缩症，累及C7D1肌节；肌电图（EMG）显示C7C8~T1区域存在神经源性损伤。它会导致上肢远端无力和肌萎缩。尽管通常仅发生于一侧，但偶尔也有双侧对称的平山病病例报道。这是一种缓慢进展性疾病，若在病程早期被发现，其病情发展可能会有较好的预后。我们描述了一例平山病的临床观察情况，包括其临床和辅助检查特点，并与既往研究数据进行比较。
