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“单侧起病的爪形手”作为吉兰-巴雷综合征的一种区域变异型:一例报告

"Claw hand with a unilateral onset" as a regional variant of Guillain-Barre' syndrome: A case report.

作者信息

Wang Suhong, Zhao Shuxin, Zhang Zhecheng

机构信息

Department of Neurology, Third Central Hospital of Tianjin.

Tianjin Institute of Hepatobiliary Disease.

出版信息

Medicine (Baltimore). 2020 May;99(20):e20227. doi: 10.1097/MD.0000000000020227.

DOI:10.1097/MD.0000000000020227
PMID:32443353
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7253783/
Abstract

RATIONALE

Although distal nerves located at sites prone to compression are susceptible to autoimmune attack, Guillain-Barre' syndrome (GBS) with exclusive hand muscle involvement is rarely found in clinics. All reported patients presented with a special variant - finger extensor weakness, especially claw hand caused by predominant ulnar extensor involvement. Similar to typical GBS, these patients showed bilateral symmetric onset with rapid clinical progression.

PATIENT CONCERNS

A 62-year-old man with GBS was admitted to our hospital with unilateral onset of claw hand. He showed relatively slow progression and did not develop bilateral symmetric claw hands until 6 weeks later.

DIAGNOSES

Eventually the patient was diagnosed as having a regional variant of GBS by neuronal electrophysiology and cerebrospinal fluid examinations.

INTERVENTIONS

This patient was treated with intravenous thrombolysis within 4.5 hours of onset. Eventually he was diagnosed as having a regional variant of GBS and was treated with gamma-globulin (400 mg/kg/d) for 5 consecutive days via intravenous infusion.

OUTCOMES

The patient had a slow recovery with persistent mild finger extensor weakness.

LESSONS

This patient presented with unilateral onset of claw hand, and the diagnosis of acute ischemic stroke could not be excluded because of a short time window; hence, he was treated with intravenous thrombolysis within 4.5 hours of onset. Eventually he was diagnosed as having a regional variant of GBS. It is important that GBS should also be considered in patients with unilateral hand weakness and unknown aetiology in the early stages of disease.

摘要

理论依据

尽管位于易受压部位的远端神经易受自身免疫攻击,但临床上很少发现仅累及手部肌肉的吉兰-巴雷综合征(GBS)。所有报道的患者均表现为一种特殊变体——手指伸肌无力,尤其是主要由尺侧伸肌受累导致的爪形手。与典型GBS相似,这些患者表现为双侧对称起病,临床进展迅速。

患者情况

一名62岁的GBS男性患者因单侧爪形手入院。他的病情进展相对缓慢,直到6周后才发展为双侧对称爪形手。

诊断

最终,通过神经电生理和脑脊液检查,该患者被诊断为GBS的一种局部变体。

干预措施

该患者在发病4.5小时内接受了静脉溶栓治疗。最终他被诊断为GBS的一种局部变体,并通过静脉输注连续5天接受丙种球蛋白(400mg/kg/d)治疗。

结果

患者恢复缓慢,手指伸肌持续存在轻度无力。

经验教训

该患者表现为单侧爪形手起病,由于发病时间窗短,不能排除急性缺血性卒中的诊断;因此,他在发病4.5小时内接受了静脉溶栓治疗。最终他被诊断为GBS的一种局部变体。对于疾病早期出现单侧手部无力且病因不明的患者,早期也应考虑GBS,这一点很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6383/7253783/2171940159a9/medi-99-e20227-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6383/7253783/2171940159a9/medi-99-e20227-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6383/7253783/2171940159a9/medi-99-e20227-g001.jpg

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