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以显著面瘫为特征的格林-巴利综合征变异型。

A Guillain-Barré syndrome variant with prominent facial diplegia.

机构信息

Department of Neurology, Dokkyo Medical University, Tochigi, Japan.

出版信息

J Neurol. 2009 Nov;256(11):1899-905. doi: 10.1007/s00415-009-5254-8. Epub 2009 Jul 25.

Abstract

To determine the clinical features of a Guillain-Barré syndrome variant with prominent facial diplegia, we retrospectively reviewed approximately 8,600 cases referred to our neuroimmunological laboratory for serological tests during the past seven years. Patients' histories, neurological signs, and laboratory and electrophysiological data were clarified based on their clinical records. Sera obtained during the acute phase were tested for prior infectious serology and anti-ganglioside antibodies. In 22 patients, clinical signs such as acute progressive bifacial weakness, paresthesias in the distal dominant limbs, and hypo- or areflexia, were compatible with a Guillain-Barré syndrome variant, facial diplegia and paresthesias. Other cranial nerve involvements, limb weakness, and ataxia were absent or minimal. Clinical courses were monophasic, the nadir being reached within four weeks. Eighteen patients (86%) had had infectious symptoms within the four weeks preceding the onset of neurological illness. In the infection serology tests, anti-cytomegalovirus IgM antibodies were the most frequent (35%). All the patients had cerebrospinal fluid albuminocytologic dissociation. In nerve conduction studies, 14 (64%) showed demyelination in their limbs. Anti-GM2 IgM antibodies were detected in four patients who had anti-cytomegalovirus IgM antibodies. Patients with conditions similar to facial diplegia and paresthesias, but lacking either distal paresthesias or hyporeflexia, were regarded as having marginal facial diplegia and paresthesias, because they also frequently had features of Guillain-Barré syndrome, such as an antecedent infection or cerebrospinal fluid albuminocytologic dissociation. Our findings are further evidence of a facial variant of Guillain-Barré syndrome and provide important information essential for its diagnosis.

摘要

为了确定以显著面瘫为特征的格林-巴利综合征变异型的临床特征,我们回顾性地审查了过去七年中大约 8600 例因血清学检查而转至我们神经免疫实验室的病例。根据临床记录,明确了患者的病史、神经体征以及实验室和电生理数据。在急性期获得的血清用于检测先前的传染病血清学和抗神经节苷脂抗体。在 22 例患者中,急性进行性双侧面部无力、远侧优势肢体感觉异常和反射减退或消失等临床体征与格林-巴利综合征变异型、面瘫和感觉异常相符。其他颅神经受累、肢体无力和共济失调缺失或轻微。临床病程为单相,最低点在四周内达到。18 例患者(86%)在神经系统疾病发病前四周内有感染症状。在感染血清学检测中,抗巨细胞病毒 IgM 抗体最为常见(35%)。所有患者均有脑脊液白蛋白细胞分离。在神经传导研究中,14 例(64%)肢体显示脱髓鞘。在有抗巨细胞病毒 IgM 抗体的 4 例患者中检测到抗 GM2 IgM 抗体。那些存在类似面瘫和感觉异常但缺乏远侧感觉异常或反射减退的患者被认为存在边缘性面瘫和感觉异常,因为他们也经常有格林-巴利综合征的特征,如前驱感染或脑脊液白蛋白细胞分离。我们的发现进一步证明了格林-巴利综合征的面瘫变异型,并提供了对其诊断至关重要的重要信息。

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