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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Clinical spectrum of glutamic acid decarboxylase antibodies in a Taiwanese population
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Clinical spectrum of glutamic acid decarboxylase antibodies in a Taiwanese population

机译：台湾人群中谷氨酸脱羧酶抗体的临床谱

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Background and purpose High levels of autoantibodies against glutamic acid decarboxylase ( GAD ‐abs) are associated with stiff‐person syndrome ( SPS ). However, the full clinical spectrum associated with GAD ‐abs in Asians is unclear. The clinical and immunological features of patients positive for GAD ‐abs were reviewed in a large Taiwanese series. Methods Retrospective case series and immunological investigations were conducted between July 2007 and July 2017 at a tertiary referral centre in Taiwan. Amongst 361 patients with GAD ‐ab reactivity, 185 with detailed clinical records were included. Results Twenty‐seven patients (14.59%), with a mean age at assessment of 54.8?±?13.9?years, presented with neurological symptoms. The major neurological presentations (mean GAD ‐ab concentrations) were SPS ( n ?=?9, 33.3%; 135.45?±?27.84?U/ml), cerebellar ataxia ( n ?=?3, 11.1%; 95.61?±?49.63?U/ml), encephalopathy ( n ?=?2, 7.4%; 51.8?±?49.64?U/ml) and epilepsy ( n ?=?1, 3.7%; 83.3?U/ml). Notably, eight patients fulfilling the clinical diagnosis of multiple system atrophy had relatively lower GAD ‐ab concentrations (2.57?±?0.82?U/ml), which has not been reported previously. There was no correlation between disease severity and GAD ‐ab concentration. Patients presenting with comorbid endocrinopathies ( n ?=?15, 55.5%) had higher GAD ‐ab concentrations than those without endocrinopathies ( n ?=?12, 44.4%; 104.45?±?22.51?U/ml vs. 34.08?±?21.83?U/ml, P ?=?0.04). Of 158 patients (85.4%) without a neurological presentation, 133 had type 1 diabetes mellitus and 20 had diabetes of other aetiologies (type 2 or gestational diabetes mellitus, or diabetes secondary to pancreatitis); the remaining four patients had pure thyroid disorders. Conclusions A clinical and immunological evaluation of East Asian patients positive for GAD ‐abs is presented and a different clinical spectrum of anti‐ GAD syndrome is identified compared to Caucasians.
机译：背景和目的抗谷氨酸脱羧酶（GAD -Abs）的高水平自身抗体与僵硬的人综合征（SPS）有关。然而，与亚洲人的GAD -Abs相关的全临床谱尚不清楚。大台湾系列综述了GAD -ABs阳性患者的临床和免疫功能。方法回顾性案例系列和免疫学调查于2007年7月至2017年7月在台湾的第三次推荐中心进行。在361例GAD -AB反应患者中，包括185名具有详细的临床记录。结果二十七名患者（14.59％），平均年龄为54.8？±13.9岁，呈神经系统症状。主要的神经陈述（平均GAD -Ab浓度）是SPS（n？=Δ9,33.3％; 135.45？27.84？U / ml），小脑共济失调（n？= 3,11.1％; 95.61？±？ 49.63？U / ml），脑病（n？= 2,7.4％; 51.8？±49.64？U / ml）和癫痫（n？=？1,3.7％; 83.3？U / ml）。值得注意的是，八位符合多种系统萎缩的临床诊断的患者具有相对较低的GAD -Ab浓度（2.57？±0.82μm≤ml），其尚未报道。疾病严重程度与GAD -Ab浓度之间没有相关性。患有合并内分泌的患者（N？= 15，55.5％）的GAD -Ab浓度高于没有内分泌术的浓度（n？=Δ12,44.4％; 104.45？±22.51？U / ml与34.08？±？ 21.83？U / ml，p？=？0.04）。 158例患者（85.4％）没有神经呈现，133型型糖尿病型糖尿病和20型患有其他疾病的糖尿病（2型或妊娠期糖尿病，或胰腺炎患糖尿病）;其余四名患者含有纯甲状腺障碍。结论介绍了东亚患者的临床和免疫学评价，鉴定了与白种人相比鉴定出不同临床综合征的临床谱。

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