Fujimori Takahito, Tamura Akiko, Miwa Toshitada, Iwasaki Motoki, Oda Takenori
Departments of Orthopedic Surgery, Sumitomo Hospital, Osaka, Japan.
Departments of Neurology, Sumitomo Hospital, Osaka, Japan.
Spinal Cord Ser Cases. 2017 May 11;3:17016. doi: 10.1038/scsandc.2017.16. eCollection 2017.
Hirayama disease, a type of cervical flexion myelopathy, is a rare neurological disease characterized by muscular atrophy of the forearms and hands. Generally, the pathology is limited to the gray matter of the anterior horns in the lower cervical spinal cord. However, in rare cases the damage can spread to the white matter and present as long tract signs.
We report on a 30-year-old female whose onset presented as unilateral muscle atrophy of the right hand in her teens. Despite conservative treatment using a cervical collar, she developed prolonged bilateral muscle atrophy, sensory disturbance and spastic gait, along with bladder and rectal disturbances. Her hands were frozen into a 'claw-like' gesture and her intrinsic muscles were highly atrophic. She was unable to unclench her hands. Although the space available for the spinal cord was large, the spinal cord was highly atrophic. She had local kyphosis with a large (61°) flexion range of motion. During flexion the spinal cord was stretched, resulting in contact with the posterior wall of the vertebrae. Posterior fusion surgery was performed to prevent progression of the myelopathy. After surgery, she gained mild improvement in both muscle strength and her hand's movement. However, her spastic gait and muscle atrophy remained.
Most cases of cervical flexion myelopathy as represented by Hirayama disease have a self-limiting benign prognosis. However, some cases can develop advanced myelopathy with long tract signs. Long-term follow-up is recommended for these cases because they may require early surgical treatment.
平山病是一种颈屈型脊髓病，是一种罕见的神经系统疾病，其特征为前臂和手部肌肉萎缩。一般来说，病理改变仅限于下颈段脊髓前角灰质。然而，在罕见情况下，损伤可蔓延至白质并出现长束征。
我们报告一例30岁女性，她在十几岁时发病表现为右手单侧肌肉萎缩。尽管使用颈托进行了保守治疗，但她仍出现了双侧肌肉萎缩、感觉障碍和痉挛性步态延长，以及膀胱和直肠功能障碍。她的双手呈“爪状”姿势固定，手部固有肌肉高度萎缩。她无法松开双手。尽管脊髓的可用空间较大，但脊髓高度萎缩。她有局部脊柱后凸，屈伸活动范围大（61°）。在屈曲过程中，脊髓被拉伸，导致与椎体后壁接触。进行了后路融合手术以防止脊髓病进展。术后，她的肌肉力量和手部运动均有轻度改善。然而，她的痉挛性步态和肌肉萎缩仍然存在。
以平山病为代表的大多数颈屈型脊髓病病例预后良好，具有自限性。然而，一些病例可发展为伴有长束征的晚期脊髓病。对于这些病例，建议进行长期随访，因为它们可能需要早期手术治疗。
