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一例罕见的由电压门控钙通道和谷氨酸脱羧酶自身抗体引起的小脑共济失调病例。

A Rare Case of Cerebellar Ataxia Due to Voltage-Gated Calcium Channel and Glutamic Acid Decarboxylase Autoantibodies.

作者信息

Annunziata Giuseppe, Lobo Pamela, Carbuccia Cristian

机构信息

Department of Internal Medicine, St. Barnabas Hospital, Affiliated to Albert Einstein College of Medicine, Bronx, NY, USA.

出版信息

Am J Case Rep. 2017 Nov 27;18:1251-1255. doi: 10.12659/ajcr.905476.

Abstract

BACKGROUND Autoimmune cerebellar ataxia can be paraneoplastic in nature or can occasionally present without evidence of an ongoing malignancy. The detection of specific autoantibodies has been statistically linked to different etiologies. CASE REPORT A 55-year-old African-American woman with hypertension and a past history of morbid obesity and uncontrolled diabetes status post gastric bypass four years prior to the visit (with significantly improved body mass index and hemoglobin A1c controlled at the time of the clinical encounter) presented to the office complaining of gradual onset of unsteadiness and recurrent falls for the past three years, as well as difficulties coordinating routine daily activities. The neurologic exam showed moderate dysarthria and ataxic gait with bilateral dysmetria and positive Romberg test. Routine laboratory test results were only remarkable for a mild elevation of erythrocyte sedimentation rate, and most laboratory and imaging tests for common causes of ataxia failed to demonstrate an etiology. Upon further workup, evidence of anti-voltage-gated calcium channel and anti-glutamic acid decarboxylase antibody was demonstrated. She was then treated with intravenous immunoglobulins with remarkable clinical improvement. CONCLUSIONS We present a case of antibody-mediated ataxia not associated with malignancy. While ataxia is rarely related to autoantibodies, in such cases it is critical to understand the etiology of this disabling condition in order to treat it correctly. Clinicians should be aware of the possible association with specific autoantibodies and the necessity to rule out an occult malignancy in such cases.

摘要

背景

自身免疫性小脑共济失调可能本质上是副肿瘤性的,或者偶尔在没有持续恶性肿瘤证据的情况下出现。特定自身抗体的检测在统计学上与不同病因相关。病例报告:一名55岁的非裔美国女性,有高血压病史,既往有病态肥胖史,就诊前四年行胃旁路手术后糖尿病控制不佳(临床就诊时体重指数显著改善,糖化血红蛋白得到控制),因过去三年逐渐出现的步态不稳和反复跌倒以及日常活动协调困难前来就诊。神经系统检查显示中度构音障碍和共济失调步态,双侧辨距不良,Romberg试验阳性。常规实验室检查结果仅显示红细胞沉降率轻度升高,大多数针对共济失调常见病因的实验室和影像学检查未能明确病因。进一步检查发现抗电压门控钙通道和抗谷氨酸脱羧酶抗体阳性。随后她接受了静脉注射免疫球蛋白治疗,临床症状明显改善。结论:我们报告一例与恶性肿瘤无关的抗体介导的共济失调病例。虽然共济失调很少与自身抗体相关,但在这种情况下,了解这种致残性疾病的病因对于正确治疗至关重要。临床医生应意识到与特定自身抗体的可能关联,以及在这种情况下排除隐匿性恶性肿瘤的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/820c/5715982/dc125f6f243b/amjcaserep-18-1251-g001.jpg

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