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[一例针对半乳糖脑苷脂和磷脂混合物抗体的急性自主神经和感觉神经病(AASN)]

[A case of acute autonomic and sensory neuropathy (AASN) with antibody against a mixture of galactocerebroside and phospholipids].

作者信息

Ishikura Teruyuki, Takata Kazushiro, Kinoshita Makoto, Fukada Kei, Sawada Jinichi, Hazama Takanori

机构信息

Department of Neurology, Osaka General Medical Center.

出版信息

Rinsho Shinkeigaku. 2017 Jan 31;57(1):33-36. doi: 10.5692/clinicalneurol.cn-000914. Epub 2016 Dec 23.

Abstract

A 62-year-old woman presented with paresthesia of limbs, gait disturbance, urinary retention and constipation following upper respiratory infection. Neurological examination revealed gait disturbance due to loss of position sense in her extremities with intact muscle power, and autonomic failure represented by orthostatic hypotension, constipation and autonomic bladder. Cerebrospinal fluid analysis showed normal cell counts with elevated protein levels. Nerve conduction study showed sensory nerve impairment with almost normal motor nerve conduction in her upper and lower extremities. Sympathetic skin response of both hands was unresponsive, indicating autonomic nervous dysfunction. We diagnosed her as having acute autonomic and sensory neuropathy (AASN) and treated her with intravenous immunoglobulin, which ameliorated her symptoms enabling her to walk without any assistance at the time of discharge. Screening tests of serum autoantibodies revealed positivity of antibody against a mixture of galactocerebroside (Gal-Cer) and phospholipids. According to previous literature, no specific antibodies have been identified in AASN. This case, therefore, suggests a possible role of anti-Gal-Cer antibody in the pathogenesis of AASN.

摘要

一名62岁女性在出现上呼吸道感染后,出现肢体感觉异常、步态障碍、尿潴留和便秘。神经系统检查发现,由于四肢位置觉丧失导致步态障碍,但肌肉力量正常,且存在以体位性低血压、便秘和自主性膀胱为表现的自主神经功能衰竭。脑脊液分析显示细胞计数正常,但蛋白水平升高。神经传导研究表明,其上下肢感觉神经受损,而运动神经传导基本正常。双手的交感皮肤反应无反应,提示自主神经功能障碍。我们诊断她患有急性自主神经和感觉神经病(AASN),并给予静脉注射免疫球蛋白治疗,症状得到改善,出院时能够无需任何帮助行走。血清自身抗体筛查试验显示,抗半乳糖脑苷脂(Gal-Cer)和磷脂混合物的抗体呈阳性。根据以往文献,AASN中尚未鉴定出特异性抗体。因此,该病例提示抗Gal-Cer抗体在AASN发病机制中可能起作用。

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