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慢性炎症性脱髓鞘性多发性神经病样神经病变作为克罗恩病的首发表现

Chronic inflammatory demyelinating polyneuropathy-like neuropathy as an initial presentation of Crohn's disease.

作者信息

Kim Suji, Kang Seok-Jae, Oh Ki-Wook, Ahn Byung Kyu, Lee Hang Lak, Han Dong Soo, Jang Kiseok, Kim Young Seo

机构信息

Department of Radiology, Kangbuk Samsung Medical Center, Seoul, Republic of Korea.

Department of Neurology, College of Medicine, Hanyang University, 17 Haengdang-dong, Seongdong-gu, Seoul, 133-792, Republic of Korea.

出版信息

BMC Neurol. 2015 Mar 28;15:48. doi: 10.1186/s12883-015-0302-8.

Abstract

BACKGROUND

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare complication of Crohn's disease (CD), and it is uncertain whether it is associated with CD itself or with its treatment. We describe a case of CIDP-like neuropathy as an initial symptom of CD. The neurologic symptoms of the patient which responded partially to intravenous immunoglobulin (IVIG) recovered after resection of the appendiceal CD.

CASE PRESENTATION

A 17-year-old male had experienced three separate attacks of motor weakness and paresthesia of all four extremities over a period of 7 months. The electrophysiologic findings revealed a demyelinating sensory-motor polyneuropathy which was compatible with CIDP. However, repeated intravenous IVIG (2 g/kg) treatment gave only a partial response. Four days after the last discharge, he was diagnosed as appendiceal CD after surgical resection of a periappendiceal abscess. His neurologic symptoms and electrophysiologic findings recovered without any maintenance therapy.

CONCLUSIONS

CIDP-like neuropathy can be an initial presentation of CD, and recovery of the CIDP symptoms may result from resection of the CD. Clinicians should be aware of the possibility of CD in patients with intractable CIDP symptoms.

摘要

背景

慢性炎性脱髓鞘性多发性神经病(CIDP)是克罗恩病(CD)的一种罕见并发症,其是否与CD本身或其治疗相关尚不确定。我们描述了一例以CIDP样神经病为CD首发症状的病例。患者的神经症状对静脉注射免疫球蛋白(IVIG)有部分反应,在阑尾CD切除术后恢复。

病例介绍

一名17岁男性在7个月内经历了3次四肢运动无力和感觉异常的发作。电生理检查结果显示为脱髓鞘性感觉运动性多发性神经病,与CIDP相符。然而,重复静脉注射IVIG(2 g/kg)治疗仅产生部分反应。最后一次出院4天后,在手术切除阑尾周围脓肿后,他被诊断为阑尾CD。其神经症状和电生理检查结果在未进行任何维持治疗的情况下恢复。

结论

CIDP样神经病可能是CD的首发表现,CIDP症状的恢复可能源于CD的切除。临床医生应意识到难治性CIDP症状患者患CD的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dfd/4379591/a98df6521fd1/12883_2015_302_Fig1_HTML.jpg

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