感觉性慢性炎症性脱髓鞘多发性神经病:被忽视的免疫疗法反应性感觉神经病

IF 2.9 3区 医学 Q2 CLINICAL NEUROLOGY
Journal of Clinical Neurology Pub Date : 2024-05-01 Epub Date: 2024-02-05 DOI:10.3988/jcn.2023.0469
Shin J Oh, Peter King
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引用次数: 0

摘要

背景和目的:报告在56名感觉性慢性炎症性脱髓鞘多发性神经病(CIDP)患者中,34名(85%)/40名需要接受免疫治疗的患者的病情改善情况:符合以下两个纳入标准的患者可被诊断为感觉性慢性炎症性脱髓鞘多发性神经病:1)获得性、慢性进行性或复发性对称或不对称感觉性多发性神经病,且病程超过2个月;2)明确的脱髓鞘神经病电生理学和/或活检证据:结果:共发现 56 例感觉性 CIDP 患者。39例(70%)患者的脱髓鞘证据来自常规运动神经传导研究(NCS),10例来自神经活检,7例来自近神经针感觉NCS。最突出的实验室异常是脑脊液蛋白质含量过高,43 名受检患者中有 21 人(49%)出现这种情况。52 名随访患者中有 41 人(79%)需要接受免疫治疗。在接受免疫治疗后,36 名患者(88%)/41 名患者的病情有所改善。有 3 名患者在 5-8 年的随访期间出现了运动无力,因此,他们的诊断被改为 CIDP:结论:88%的患者对免疫疗法有反应。70%的患者是通过常规运动神经功能检查诊断出感觉型 CIDP 的,18%的患者是通过鞍神经活检诊断出感觉型 CIDP 的。因此,在不同类型的 "特发性感觉神经病 "中,感觉型 CIDP 应被视为一种可治疗的 CIDP 变异类型。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sensory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Neglected Immunotherapy-Responsive Sensory Neuropathy.

Background and purpose: To report an improvement with immunotherapy in 34 (85%)/40 patients who required an immunotherapy among 56 patients with sensory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods: Sensory CIDP was diagnosed when two inclusion criteria are met: 1) acquired, chronic progressive or relapsing symmetrical or asymmetrical sensory polyneuropathy that had progressed for >2 months; and 2) definite electrophysiological and/or biopsy evidence of demyelinating neuropathy.

Results: Fifty-six patients with sensory CIDP were identified. Evidence of demyelination was obtained from by the routine motor nerve conduction study (NCS) in 39 (70%) patients, from a nerve biopsy in 10, and from a near-nerve needle sensory NCS in 7 patients. The most prominent laboratory abnormality was a high protein level in the cerebrospinal fluid in 21 (49%) of 43 tested patients. Immunotherapy was required in 41 (79%) of the 52 followed-up patients. An improvement with immunotherapy was observed in 36 (88%)/41 patients. In three patients, motor weakness developed in 5-8 years' follow-up period and so, their diagnosis was changed to CIDP.

Conclusions: Sensory CIDP is responded to an immunotherapy in 88% of the treated patients. Sensory CIDP was diagnosed by the routine motor NCS in 70% of patients and by a sural nerve biopsy in 18% of patients. Thus, sensory CIDP should be recognized as a treatable CIDP variant among the different types of "idiopathic sensory neuropathy."

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来源期刊
Journal of Clinical Neurology
Journal of Clinical Neurology 医学-临床神经学
CiteScore
4.50
自引率
6.50%
发文量
0
审稿时长
>12 weeks
期刊介绍: The JCN aims to publish the cutting-edge research from around the world. The JCN covers clinical and translational research for physicians and researchers in the field of neurology. Encompassing the entire neurological diseases, our main focus is on the common disorders including stroke, epilepsy, Parkinson''s disease, dementia, multiple sclerosis, headache, and peripheral neuropathy. Any authors affiliated with an accredited biomedical institution may submit manuscripts of original articles, review articles, and letters to the editor. The JCN will allow clinical neurologists to enrich their knowledge of patient management, education, and clinical or experimental research, and hence their professionalism.
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