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[吉兰-巴雷综合征患者出现急性口咽麻痹伴局部感觉障碍,类似急性咽-颈-臂变异型的症状分布]

[Acute oropharyngeal palsy with localized sensory impairment resembling symptom distribution of acute pharyngeal-cervical-brachial variant in a patient with Guillain-Barré syndrome].

作者信息

Nakajima Nobuhito, Ueda Masayuki, Nomura Koichi, Kusunoki Susumu, Katayama Yasuo

机构信息

Department of Neurology, Kitamurayama Public Hospital, Japan.

出版信息

Rinsho Shinkeigaku. 2013;53(8):630-3. doi: 10.5692/clinicalneurol.53.630.

Abstract

We present the case of a 40-year-old woman who experienced dysarthria and, numbness in her upper extremities and posterior region of her neck. Upon admission to our hospital, neurological examination revealed rhinolalia aperta and an incomplete palatoplegia; however, muscle strength in the neck and limbs was satisfactorily preserved, tendon reflexes were normal, and pathological reflexes were not observed. Cerebrospinal fluid and electrophysiological test results were also normal. On day 3 of hospitalization, a slight backflow of fluid into the nasal cavity was observed upon deglutition, and vibration perception was also impaired in the bilateral arms. Her serum tested positive for immunoglobulin G antibodies against such gangliosides as GT1a, GQ1b, GT1b, and GD1a. Despite normal tendon reflexes, she was diagnosed with a subtype of Guillain-Barré syndrome (GBS), and was treated with intravenous immunoglobulin therapy. Subsequently, her symptoms improved. Due to the combination of oropharyngeal palsy and sensory impairment, it was more likely the GBS subtype in this patient was acute oropharyngeal palsy (AOP) rather than pharyngeal-cervical-brachial (PCB) variant; though interestingly, the patient's sensory disturbance was limited to the posterior neck and upper extremities, which resembles the distribution of motor symptoms in PCB variant. The present case was a rare and important phenotype, demonstrating diversities of GBS variants. We also believe that GBS subtypes may represent a continuum of pathological conditions and not just one static condition. However, further studies involving serological characteristics of anti-ganglioside antibodies and clinical features for GBS are needed to clarify this possibility.

摘要

我们报告了一例40岁女性病例,该患者出现构音障碍、上肢及颈部后部麻木。入院时,神经系统检查发现开放性鼻音和不完全性腭麻痹;然而,颈部和四肢肌肉力量保存良好,腱反射正常,未观察到病理反射。脑脊液和电生理检查结果也正常。住院第3天,吞咽时观察到有少量液体反流至鼻腔,双侧手臂的振动觉也受损。她的血清检测显示,针对GT1a、GQ1b、GT1b和GD1a等神经节苷脂的免疫球蛋白G抗体呈阳性。尽管腱反射正常,但她被诊断为吉兰 - 巴雷综合征(GBS)的一种亚型,并接受了静脉注射免疫球蛋白治疗。随后,她的症状有所改善。由于口咽麻痹和感觉障碍同时存在,该患者更可能的GBS亚型是急性口咽麻痹(AOP)而非咽颈臂型(PCB)变异型;不过有趣的是,患者的感觉障碍仅限于颈部后部和上肢,这与PCB变异型的运动症状分布相似。本病例是一种罕见且重要的表型,展示了GBS变异型的多样性。我们还认为,GBS亚型可能代表了一系列连续的病理状态,而不仅仅是一种静态情况。然而,需要进一步研究GBS的抗神经节苷脂抗体血清学特征和临床特征来阐明这种可能性。

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