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[一例与慢性炎症性脱髓鞘性多发性神经根神经病相关的多发性硬化症病例]

[A case of multiple sclerosis associated with chronic inflammatory demyelinating polyradiculoneuropathy].

作者信息

Watanabe Y, Ishikawa Y, Wakai S, Tachi N, Chiba S

机构信息

Department of Pediatrics, Sapporo Medical College.

出版信息

No To Hattatsu. 1993 Jan;25(1):70-5.

PMID:8416699
Abstract

We report a 19-year-old girl with multiple sclerosis (MS) who had chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). At the age of 9 (1981), she was diagnosed as having Devic disease, including optic neuritis and transverse myelitis. For two years after the onset of the disease, she suffered from three relapses of MS and was treated with prednisone. At the age of 11, she noticed muscle weakness and sensory disturbance of four limbs. On examination, all tendon reflexes were absent, CSF protein was elevated, and motor nerve conduction velocity of the ulnar nerves was markedly slowed. Sural nerve biopsy showed decreased density of myelinated fibers and de-re-myelinated fibers with onion-bulb formations. Between 11 and 19 years, she had a number of episodes of the central nervous system (CNS) and peripheral nervous system (PNS) deficits with partial or complete recovery by oral administration of prednisone. The clinical course and laboratory data are consistent with MS associated with CIDP. Such our case of MS with CIDP in childhood is very rare. Patients with MS and CIDP may represent a subpopulation who had a common pathogenetic factor for both CNS and PNS demyelination.

摘要

我们报告了一名患有慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)的19岁多发性硬化症(MS)女孩。9岁(1981年)时,她被诊断患有视神经脊髓炎,包括视神经炎和横贯性脊髓炎。发病后的两年里,她经历了3次MS复发,并接受了泼尼松治疗。11岁时,她注意到四肢肌肉无力和感觉障碍。检查发现所有腱反射消失,脑脊液蛋白升高,尺神经运动神经传导速度明显减慢。腓肠神经活检显示有髓纤维密度降低,出现脱髓鞘纤维及洋葱球样结构。在11岁至19岁期间,她多次出现中枢神经系统(CNS)和周围神经系统(PNS)功能缺损,通过口服泼尼松部分或完全恢复。临床病程和实验室数据与MS合并CIDP相符。我们报道的这种儿童期MS合并CIDP的病例非常罕见。MS和CIDP患者可能代表了一群中枢神经系统和周围神经系统脱髓鞘有共同致病因素的亚人群。

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