Clinical characteristics and outcomes of patients with antibody-related autoimmune encephalitis presenting with disorders of consciousness: A prospective cohort study

IF 3.1 4区医学 Q3 IMMUNOLOGY Immunity, Inflammation and Disease Pub Date : 2024-09-24 DOI:10.1002/iid3.70019

Dawei Shan, Huimin Zhang, Lili Cui, Shuting Chai, Weibi Chen, Gang Liu, Fei Tian, Linlin Fan, Le Yang, Yan Zhang

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Abstract

Objective

To explore the clinical characteristics, short- and long-term functional outcomes, and risk factors for antibody-related autoimmune encephalitis (AE) in patients with disorders of consciousness (DoC).

Methods

Clinical data were collected from AE patients admitted to Xuanwu Hospital of Capital Medical University from January 2012 to December 2021, and patients were followed up for up to 24 months after immunotherapy.

Results

A total of 312 patients with AE were included: 197 (63.1%) with anti-NMDAR encephalitis, 71 (22.8%) with anti-LGI1 encephalitis, 20 (6.4%) with anti-GABAbR encephalitis, 10 (3.2%) with anti-CASPR2 encephalitis, 10 (3.2%) with anti-GAD65 encephalitis, and 4 (1.3%) with anti-AMPAR2 encephalitis. Among these patients, 32.4% (101/312) presented with DoC, and the median (interquartile range, IQR) time to DoC was 16 (7.5, 32) days. DoC patients had higher rates of various clinical features of AE (p < .05). DoC was associated with elevated lumbar puncture cerebrospinal fluid (CSF) pressure, CSF leukocyte count, and specific antibody titer (p < .05). A high percentage of patients in the DoC group had a poor prognosis at discharge and at 6 months after immunotherapy (p < .001), but no significant difference in prognosis was noted between the DoC group and the non-DoC group at 12 and 24 months after immunotherapy. Dyskinesia (OR = 3.266, 95% CI: 1.550–6.925, p = .002), autonomic dysfunction (OR = 5.871, 95% CI: 2.574–14.096, and p < .001), increased CSF pressure (OR = 1.007, 95% CI: 1.001–1.014, p = .046), and modified Rankin scale (mRS) score ≥3 at the initiation of immunotherapy (OR = 7.457, 95% CI: 3.225–18.839, p < .001) were independent risk factors for DoC in AE patients.

Conclusion

DoC is a relatively common clinical symptom in patients with AE, especially critically ill patients. Despite requiring longer hospitalization, DoC mostly improves with treatment of the primary disease and has a good long-term prognosis after aggressive life support and combination immunotherapy.

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出现意识障碍的抗体相关自身免疫性脑炎患者的临床特征和预后：前瞻性队列研究。

目的探讨意识障碍（DoC）患者抗体相关自身免疫性脑炎（AE）的临床特征、短期和长期功能预后及风险因素：收集2012年1月至2021年12月首都医科大学宣武医院收治的自身免疫性脑炎患者的临床资料，并对患者进行免疫治疗后长达24个月的随访：结果：共纳入312例AE患者：结果：共纳入312例AE患者：197例（63.1%）抗NMDAR脑炎患者，71例（22.8%）抗LGI1脑炎患者，20例（6.4%）抗GABAbR脑炎患者，10例（3.2%）抗CASPR2脑炎患者，10例（3.2%）抗GAD65脑炎患者，4例（1.3%）抗AMPAR2脑炎患者。在这些患者中，32.4%（101/312）出现 DoC，DoC 的中位时间（四分位数间距，IQR）为 16（7.5，32）天。出现 DoC 的患者出现各种 AE 临床特征的比例较高（P 结论：DoC 是一种相对常见的临床症状：DoC是急性呼吸衰竭患者，尤其是重症患者相对常见的临床症状。尽管住院时间较长，但在治疗原发疾病后，DoC 大多会得到改善，在积极的生命支持和联合免疫疗法后，长期预后良好。

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来源期刊

Immunity, Inflammation and Disease Medicine-Immunology and Allergy

CiteScore

3.60

自引率

0.00%

发文量

146

审稿时长

8 weeks

期刊介绍： Immunity, Inflammation and Disease is a peer-reviewed, open access, interdisciplinary journal providing rapid publication of research across the broad field of immunology. Immunity, Inflammation and Disease gives rapid consideration to papers in all areas of clinical and basic research. The journal is indexed in Medline and the Science Citation Index Expanded (part of Web of Science), among others. It welcomes original work that enhances the understanding of immunology in areas including: • cellular and molecular immunology • clinical immunology • allergy • immunochemistry • immunogenetics • immune signalling • immune development • imaging • mathematical modelling • autoimmunity • transplantation immunology • cancer immunology