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[慢性多发性神经根神经炎。25例]

[Chronic polyradiculoneuritis. 25 cases].

作者信息

Azulay J P, Pouget J, Pellissier J F, Blin O, Serratrice G

机构信息

Clinique des maladies du système nerveux et de l'appareil locomoteur, CHU Timone, Marseille.

出版信息

Rev Neurol (Paris). 1992;148(12):752-61.

PMID:1303569
Abstract

Twenty-five patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were studied in order to define the clinical, biological, electrophysiological and pathological features of this disease. There were 11 men and 14 women ranging in age from 15 to 82 years. The average follow-up was 43 months. Patients fulfilled the criteria laid down by Dyck et al. (1975), except that progression of weakness was at least 2 months and not 6. Fourteen patients had a progressive course and 11 a relapsing one. Weakness was almost constant (24/25), sensory impairment was present in 22/25 with deep sensation predominantly impaired. Areflexia was observed in all patients. A history of previous infection or other possible precipitating event was given by 7 patients. Cerebrospinal fluid examination showed a raised total protein count in 22 cases. Electrophysiological examination revealed a slowing down of nerve conduction velocities to an extent compatible with a demyelinating process in 23 cases; 2 patients only had prolongation of F-waves. Sural nerve biopsy was less informative: inflammatory process--the most specific finding--was observed in only 3 out of 20 biopsies. Six patients had benign forms or spontaneous remission, the others were treated with corticosteroids or with immunosuppressive drugs. Most patients (64%) recovered very well. Only one died during the time of study. Eight other patients were treated with high-dose intravenous immunoglobulins and 4 of them improved with administrations at regular intervals to maintain the benefits observed. The occurrence of CIDP in association with other conditions is reviewed and we discuss its nosological position among the acquired demyelinating neuropathies.

摘要

为明确慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)的临床、生物学、电生理及病理特征,对25例患者进行了研究。患者中男性11例,女性14例,年龄在15至82岁之间。平均随访时间为43个月。患者符合Dyck等人(1975年)制定的标准,但肌无力进展时间至少为2个月而非6个月。14例患者病程呈进行性,11例呈复发型。肌无力几乎持续存在(25例中有24例),25例中有22例存在感觉障碍,主要为深感觉受损。所有患者均无反射。7例患者有既往感染史或其他可能的诱发事件。脑脊液检查显示22例患者总蛋白计数升高。电生理检查显示23例患者神经传导速度减慢,程度与脱髓鞘过程相符;仅2例患者F波延长。腓肠神经活检提供的信息较少:炎症过程(最具特异性的发现)仅在20例活检中的3例中观察到。6例患者为良性形式或自发缓解,其他患者接受了皮质类固醇或免疫抑制药物治疗。大多数患者(64%)恢复良好。研究期间仅1例死亡。另外8例患者接受了大剂量静脉注射免疫球蛋白治疗,其中4例通过定期给药病情改善并维持了疗效。本文回顾了CIDP与其他疾病相关的情况,并讨论了其在获得性脱髓鞘性神经病中的分类地位。

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