Department of Spinal Surgery, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.
Department of Spinal Surgery, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.
World Neurosurg. 2019 Oct;130:84-88. doi: 10.1016/j.wneu.2019.06.208. Epub 2019 Jul 5.
Hirayama disease (HD), characterized by insidiously progressive muscular atrophy of forearms and hands, is a self-limited cervical myelopathy that predominantly affects male adolescents, with female patients, especially middle-aged women, rarely affected. We present a rare case of HD with severe spinal cord injury in a 34-year-old woman.
A 34-year-old woman presented with progressive weakness of both hands and forearms for a duration of 6 years and spastic gait for 1 year. The patient worked as a teacher and experienced long-term and repetitive neck flexion while writing at a desk, potentially causing cervical loading. Examination showed weakness and muscular atrophy of the bilateral upper limbs and pyramidal signs, including Hoffmann sign and hyperreflexia of the lower limbs. Radiologic studies revealed midcervical kyphosis and spinal cord compression by forward displacement of the dura mater with neck flexion. The clinical presentation and radiologic characteristics confirmed the diagnosis of HD. Anterior cervical corpectomy and fusion was performed owing to severe spinal cord injury, and the patient experienced satisfactory improvement.
The present case clarified the potential involvement of cervical kyphosis and cervical loading-related exercise in the onset and progression of HD. Anterior cervical corpectomy and fusion could serve as a promising treatment of HD with severe spinal cord injury.
平山病（HD）以手臂和手部进行性肌肉萎缩为特征，是一种自限性颈髓病，主要影响男性青少年，女性患者，尤其是中年女性，很少受影响。我们报告了一例罕见的中年女性 HD 病例，其伴有严重的脊髓损伤。
一名 34 岁女性，因双手和前臂进行性无力 6 年，痉挛性步态 1 年就诊。患者是一名教师，在伏案工作时可能因长期重复颈部前屈而导致颈椎负荷增加。检查发现双侧上肢无力和肌肉萎缩，以及锥体束征，包括 Hoffmann 征和下肢反射亢进。影像学研究显示颈椎中段后凸，且在颈部前屈时硬脑膜向前移位导致脊髓受压。临床表现和影像学特征均符合 HD 的诊断。由于严重的脊髓损伤，行前路颈椎椎体切除术和融合术，患者的症状得到了满意的改善。
本病例阐明了颈椎后凸畸形和与颈椎负荷相关的运动可能在 HD 的发病和进展中起作用。前路颈椎椎体切除术和融合术可能是治疗伴有严重脊髓损伤的 HD 的一种有前途的方法。
