Ozawa Eisuke, Fujimoto Takeshi, Motomura Masakatsu, Shirabe Susumu, Yoshimura Toshiro, Tanaka Keiko
First Department of Internal Medicine, Nagasaki University School of Medicine.
Rinsho Shinkeigaku. 2002 Mar;42(3):221-6.
A 46-year-old man developed mild to moderate weakness in the distal muscles of lower limbs and then had gradually progressive weakness and sensory loss in four limbs. He subsequently developed difficulty in walking over a few months. Examination showed severe distal muscle weakness and atrophy, but mild proximal weakness in four limbs. Superficial sensation was decreased in both distal limbs and his vibratory sense was mildly decreased in bilateral feet. All tendon reflexes were absent. Furthermore, he showed four-limb and truncal ataxia with bilateral horizontal gaze-evoked nystagmus in both directions. Nerve conduction study revealed sensorimotor neuropathy, and sural nerve biopsy showed mixed axonal damage and demyelination. Cerebrospinal fluid protein levels were raised 212 mg/dl. Lumbar spine MRI showed marked cauda equina enhancement with gadolinium. Anti-ganglioside antibodies were negative but serum antineuronal antibodies without known antigen specificity were found. Neurootological findings indicated bilateral horizontal gaze-evoked nystagmus was caused by spinocerebellar damage. We diagnosed this case was CIDP with cerebellar ataxia. After administration of high dose steroid therapy, intravenous methylprednisolone 1000 mg/day, his symptoms including ataxia and polyneuropathy were apparently improved.
一名46岁男性下肢远端肌肉出现轻度至中度无力，随后四肢逐渐出现进行性无力和感觉丧失。几个月后，他出现行走困难。检查发现四肢远端肌肉严重无力和萎缩，但近端轻度无力。双下肢浅感觉减退，双足振动觉轻度减退。所有腱反射均消失。此外，他还表现为四肢和躯干共济失调，双侧水平凝视诱发的眼球震颤双向出现。神经传导研究显示感觉运动性神经病变，腓肠神经活检显示混合性轴索损伤和脱髓鞘。脑脊液蛋白水平升高至212mg/dl。腰椎MRI显示钆增强后马尾明显强化。抗神经节苷脂抗体阴性，但发现血清抗神经元抗体，其抗原特异性未知。神经耳科学检查结果表明，双侧水平凝视诱发的眼球震颤是由脊髓小脑损伤引起的。我们诊断该病例为伴有小脑共济失调的慢性炎症性脱髓鞘性多发性神经病（CIDP）。给予高剂量类固醇治疗，静脉注射甲泼尼龙1mg/天，他的共济失调和多发性神经病等症状明显改善。
