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脑脊液蛋白水平正常的复发性吉兰-巴雷综合征罕见病例:一例报告

An unusual case of recurrent Guillain-Barré syndrome with normal cerebrospinal fluid protein levels: a case report.

作者信息

Gunatilake Sonali Sihindi Chapa, Gamlath Rohitha, Wimalaratna Harith

机构信息

Teaching Hospital, Kandy, Sri Lanka.

出版信息

BMC Neurol. 2016 Sep 5;16(1):161. doi: 10.1186/s12883-016-0687-z.

Abstract

BACKGROUND

Guillain-Barré syndrome is an acquired polyradiculo-neuropathy, often preceded by an antecedent event. It is a monophasic disease but a recurrence rate of 1-6 % is documented in a subset group of patients. Patients with Guillain-Barré syndrome show cerebrospinal fluid albuminocytologic dissociation. Normal cerebrospinal fluid protein levels during both initial and recurrent episodes of Guillain-Barré syndrome is a rare occurrence and has not been described earlier in the literature.

CASE PRESENTATION

Twenty-five-year-old Sri Lankan female with past history of complete recovery following an acute inflammatory demyelinating polyneuropathy (AIDP) variant of Guillain-Barré syndrome 12 years back presented with acute, ascending symmetrical flaccid quadriparasis extending to bulbar muscles, bilateral VII cranial nerves and respiratory compromise needing mechanical ventilation. Nerve conduction study revealed AIDP variant of Guillain-Barré syndrome. Cerebrospinal fluid analysis done after 2 weeks were normal during both episodes without albuminocytologic dissociation. She was treated with intravenous immunoglobulin resulting in a remarkable recovery. Both episodes had a complete clinical recovery in three and four months' time respectively, rather a faster recovery than usually expected.

CONCLUSION

Recurrence of Guillain-Barré syndrome can occur in a subset of patients with Guillain-Barré syndrome even after many years of asymptomatic period. Normal cerebrospinal fluid profile does not exclude Guillain-Barré syndrome and may occur in subsequent recurrences of Guillain-Barré syndrome arising the need for further studies to identify the pathophysiology and the possibility of a different subtype of Guillain-Barré syndrome.

摘要

背景

吉兰-巴雷综合征是一种获得性多神经根神经病,通常在发病前有前驱事件。它是一种单相疾病,但在部分患者群体中有1%-6%的复发率记录。吉兰-巴雷综合征患者表现为脑脊液蛋白细胞分离。在吉兰-巴雷综合征的初始发作和复发发作期间脑脊液蛋白水平正常是一种罕见情况,且此前文献中未作描述。

病例介绍

一名25岁的斯里兰卡女性,12年前曾患吉兰-巴雷综合征的急性炎症性脱髓鞘性多发性神经病(AIDP)变异型,完全康复。此次因急性、进行性对称性弛缓性四肢瘫,累及延髓肌、双侧第七对脑神经,并有呼吸功能不全需要机械通气入院。神经传导研究显示为吉兰-巴雷综合征的AIDP变异型。两周后进行的脑脊液分析在两个发作期均正常,无蛋白细胞分离。给予静脉注射免疫球蛋白治疗后恢复显著。两个发作期分别在3个月和4个月时完全临床康复,恢复速度比通常预期的要快。

结论

吉兰-巴雷综合征患者的一个亚组即使在多年无症状期后仍可复发。脑脊液检查结果正常不能排除吉兰-巴雷综合征,且可能出现在吉兰-巴雷综合征的后续复发中,这就需要进一步研究以确定其病理生理学以及吉兰-巴雷综合征不同亚型的可能性。

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