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[多发性硬化合并脊髓空洞症——病例报告]

[Multiple sclerosis with syringomyelia--case report].

作者信息

Deguchi K, Takeuchi H, Yamada A, Katanaka J, Tsukaguchi M, Sasaki I, Nishioka M

机构信息

Third Department of Internal Medicine, Kagawa Medical School, Japan.

出版信息

No To Shinkei. 1994 Jan;46(1):65-9.

PMID:8136203
Abstract

A 32-year-old woman experienced subacute onset of weakness in her left leg, urinary retention and difficulty in extending her right middle and third finger. She subsequently suffered episodes of myelopathy, optic neuritis and cerebellar ataxia over a period of several years. Brain MRI showed multiple areas of high signal intensity on T2-weighted images, consistent with multiple sclerosis (MS). However spinal MRI revealed no abnormal findings. In her most recent episode, at age 40 she developed paraparesis. Neurologic examination revealed down beat nystagmus on gazing to the right, horizontal jerk nystagmus gazing to the left, weakness of the right middle and third fingers and paraparesis associated with spasticity of the right leg. Sensory disturbance below C3 and diminished vibration and position sense in both legs were also observed. The patient could not stand or walk, and urinary disturbance was present. Spinal MRI revealed syrinx formation at the level of vertebral bodies C2 to C6. The syrinx within the cervical cord diminished in size after four months, but the patient was unable to walk unaided and had moderate sensory disturbance as before. This finding suggests that the prognosis of MS with syrinx formation following repeated episodes of myelopathy is not always favorable. We believe that functional recovery in MS with syringomyelia is affected by the severity of the demyelination and/or gliosis caused by MS rather than by the presence of the syrinx.

摘要

一名32岁女性出现左腿亚急性无力、尿潴留以及右手食指和中指伸展困难。随后几年间,她又出现了脊髓病、视神经炎和小脑共济失调发作。脑部磁共振成像(MRI)显示在T2加权图像上有多个高信号区,符合多发性硬化症(MS)表现。然而,脊髓MRI未发现异常。在她40岁时最近一次发作中,出现了双下肢轻瘫。神经系统检查发现,向右凝视时出现下跳性眼球震颤,向左凝视时出现水平性急动性眼球震颤,右手食指和中指无力,双下肢轻瘫并伴有右腿痉挛。还观察到C3以下感觉障碍以及双下肢振动觉和位置觉减退。患者无法站立或行走,存在排尿障碍。脊髓MRI显示C2至C6椎体水平有脊髓空洞形成。4个月后颈髓内的脊髓空洞缩小,但患者仍无法独立行走,且仍有中度感觉障碍。这一发现表明,反复发生脊髓病后出现脊髓空洞形成的MS预后并非总是良好。我们认为,伴有脊髓空洞症的MS功能恢复受MS引起的脱髓鞘和/或胶质增生严重程度影响,而非脊髓空洞的存在。

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