Kutoku Yumiko, Inoue Ken, Murakami Tatsufumi, Sunada Yoshihide
Department of Neurology, Kawasaki Medical School.
Rinsho Shinkeigaku. 2010 May;50(5):325-8. doi: 10.5692/clinicalneurol.50.325.
A 65-year-old man developed urinary impairment and gait disturbance over a period of four months. On admission, neurological examinations revealed paraplegia, decreased deep tendon reflexes in the extremities, bilateral positive Babinski and Chaddock signs, superficial and deep sensory disturbances and neurogenic bladder. Cerebrospinal fluid examination disclosed a total cell count of 70/mm3, and protein of 76 mg/dl. Nerve conduction studies and somatosensory evoked potential suggested demyelinating neuropathy and myelopathy. Brain MRI revealed irregular-shaped white matter lesions distributed over the bilateral cerebral hemispheres and the brain stem. In addition spinal MRI disclosed long spinal cord lesions disseminated from the higher cervical to the lower thoracic spine. A 1 microm-thick epon-embedded section and teased fiber preparations of a biopsied sural nerve showed segmental demyelination and remyelination. Treatments using intravenous methylprednisolone and IVIg were both effective. The positive responses to immunological treatment, along with the findings, strongly suggested that the demyelinating lesions occurred in both the central and peripheral nervous systems. We regarded this case as one of chronic progression of Encephalo-myelo-radiculo-neuropathy.
一名65岁男性在四个月的时间里出现了排尿障碍和步态紊乱。入院时，神经系统检查发现截瘫、四肢腱反射减弱、双侧巴宾斯基征和查多克征阳性、深浅感觉障碍以及神经源性膀胱。脑脊液检查显示细胞总数为70/mm³，蛋白质为76mg/dl。神经传导研究和体感诱发电位提示脱髓鞘性神经病和脊髓病。脑部MRI显示双侧大脑半球和脑干分布有不规则形状的白质病变。此外，脊髓MRI显示从高位颈段到低位胸段有散在的长脊髓病变。对一条活检的腓肠神经进行的1微米厚的环氧树脂包埋切片和 teased纤维制备显示节段性脱髓鞘和再髓鞘化。静脉注射甲基强的松龙和静脉注射免疫球蛋白治疗均有效。对免疫治疗的阳性反应以及这些发现强烈提示脱髓鞘病变发生在中枢和周围神经系统。我们将此病例视为脑脊髓神经根神经病慢性进展的病例之一。
