Background Patients positive for anti-glutamic acid decarboxylase 65 (GAD65) antibodies have attracted increasing attention. Their clinical manifestations are highly heterogeneous and can be comorbid with tumors. Currently, there is no consensus on the therapeutic regimen for anti-GAD65-associated neurological diseases due to the clinical complexity, rarity and sporadic distribution. We reported six anti-GAD65 autoimmune encephalitis (AE) patients who received intravenous methylprednisolone (IVMP) or immunoglobulin (IVIG) or both. Then, we evaluated the therapeutic effect of both by summarizing results in previous anti-GAD65 AE patients from 70 published references. Results Our six patients all achieved clinical improvements in the short term. Unfortunately, there was no significant difference between IVMP and IVIG in terms of therapeutic response according to the previous references, and the effectiveness of IVMP and IVIG was 45.56% and 36.71%, respectively. We further divided the patients into different subgroups according to their prominent clinical manifestations. The response rates of IVMP and IVIG were 42.65% and 32.69%, respectively, in epilepsy patients; 60.00% and 77.78%, respectively, in patients with stiff-person syndrome; and 28.57% and 55.56%, respectively, in cerebellar ataxia patients. Among 29 anti-GAD65 AE patients with tumors, the response rates of IVMP and IVIG were 29.41% and 42.11%, respectively. There was no significant difference in effectiveness between the two regimens among the different subgroups. Conclusion Except for stiff-person syndrome, we found that this kind of AE generally has a poor response to IVMP or IVIG. Larger prospective studies enrolling large numbers of patients are required to identify the optimal therapeutic strategy in the future.

中文翻译：

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抗谷氨酸脱羧酶65抗体自身免疫性脑炎静脉注射甲泼尼龙或免疫球蛋白：哪个更好？

背景 抗谷氨酸脱羧酶 65 (GAD65) 抗体阳性的患者越来越受到关注。它们的临床表现高度异质性，可能与肿瘤共病。目前，由于临床复杂性、罕见性和散发性，抗 GAD65 相关神经系统疾病的治疗方案尚未达成共识。我们报告了六名接受静脉注射甲基强的松龙 (IVMP) 或免疫球蛋白 (IVIG) 或两者的抗 GAD65 自身免疫性脑炎 (AE) 患者。然后，我们通过总结来自 70 篇已发表参考文献的先前抗 GAD65 AE 患者的结果来评估两者的治疗效果。结果 我们的 6 名患者均在短期内实现了临床改善。很遗憾，根据之前的参考文献，IVMP和IVIG在治疗反应方面没有显着差异，IVMP和IVIG的有效率分别为45.56%和36.71%。我们根据患者突出的临床表现进一步将患者分为不同的亚组。IVMP和IVIG在癫痫患者中的反应率分别为42.65%和32.69%；僵人综合征患者分别为 60.00% 和 77.78%；在小脑性共济失调患者中分别为 28.57% 和 55.56%。在29例抗GAD65 AE肿瘤患者中，IVMP和IVIG的反应率分别为29.41%和42.11%。两种方案在不同亚组之间的有效性没有显着差异。结论 除了僵人综合症，我们发现这种AE一般对IVMP或IVIG的反应较差。需要招募大量患者进行更大规模的前瞻性研究，以确定未来的最佳治疗策略。