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一种伴有短暂心脏功能障碍和抗硫酸脂IgM阳性的吉兰-巴雷综合征严重咽感觉共济失调变异型

A Severe Pharyngeal-Sensory-Ataxic Variant of Guillain-Barré Syndrome With Transient Cardiac Dysfunction and a Positive Anti-sulfatide IgM.

作者信息

Lee King Peng, Abdul Halim Sanihah, Sapiai Nur Asma

机构信息

Department of Neurosciences, School of Medical Sciences, Universiti Sains Malaysia, Kelantan, MYS.

Department of Internal Medicine/Neurology, School of Medical Sciences, Universiti Sains Malaysia, Kelantan, MYS.

出版信息

Cureus. 2022 Sep 17;14(9):e29261. doi: 10.7759/cureus.29261. eCollection 2022 Sep.

Abstract

Guillain-Barré syndrome (GBS) is a heterogeneous group of acute immune-mediated polyradiculoneuropathy that typically presents with classic axonal or demyelinating sensory-motor type. However, there are variants of GBS with atypical presentation. We report a rare case of severe pharyngeal-sensory-ataxic variant of GBS associated with poor cardiac systolic function, elevated troponin, and positive anti-sulfatide IgM. The sensory symptom atypically started in the hands in an ascending pattern, which progressed to involve the trunk and face and, later, all limbs. It was associated with severe dysphagia, ataxia, and generalized areflexia but with preserved muscle strength in all extremities. Recognizing the atypical pattern of presentation and the ability to perform an accurate clinical localization are the utmost important initial steps in making the diagnosis. The patient showed complete recovery after immunoglobulin therapy.

摘要

吉兰 - 巴雷综合征(GBS)是一组异质性的急性免疫介导性多发性神经根神经病,通常表现为典型的轴索性或脱髓鞘性感觉运动型。然而,GBS存在非典型表现的变异型。我们报告了1例罕见的GBS严重咽感觉共济失调变异型病例,该病例伴有心脏收缩功能差、肌钙蛋白升高及抗硫酸脂IgM阳性。感觉症状非典型地从手部开始呈上行性发展,进而累及躯干和面部,随后累及所有肢体。它伴有严重吞咽困难、共济失调和全身腱反射消失,但四肢肌力保留。认识到非典型的表现模式以及进行准确临床定位的能力是做出诊断至关重要的初始步骤。该患者经免疫球蛋白治疗后完全康复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/790d/9578660/b90bd00e481a/cureus-0014-00000029261-i01.jpg

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