Yoshitoshi M, Shinohara Y, Akiyama K, Yoshii F, Takeoka T
Department of Neurology, Tokai University, School of Medicine.
Rinsho Shinkeigaku. 1992 Oct;32(10):1143-5.
A 43-year-old woman had noticed muscular weakness in her arms for four years before her admission. Muscle weakness and atrophy were prominent in the bilateral deltoid muscles, but muscular strength was almost unimpaired in the bilateral forearms and intrinsic muscles. There was no sign of sensory impairment except vibratory sensation. EMG revealed neuropathic NMU. X-P of the cervical spine showed enlargement of the spinal canal diameter, and MRI of the spinal cord revealed a large syrinx. On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. Despite the presence of a large syrinx extending from C 1 to Th 11, the only detectable neurological sign was proximal weakness of the upper extremities simulating myopathy.
一名43岁女性在入院前四年就已注意到其手臂肌肉无力。双侧三角肌的肌肉无力和萎缩明显，但双侧前臂和固有肌的肌力几乎未受影响。除振动觉外，无感觉障碍迹象。肌电图显示神经性神经肌肉单位异常。颈椎X线片显示椎管直径增大，脊髓磁共振成像显示一个大的空洞。根据甲泛葡胺CT和头颅磁共振成像，诊断为Chiari畸形（I型）合并脊髓空洞症。尽管存在一个从C1延伸至Th11的大空洞，但唯一可检测到的神经学体征是模拟肌病的上肢近端无力。
