Clinical characteristics of double negative atypical inflammatory demyelinating disease: A prospective study

IF 4.4 2区医学 Q1 CLINICAL NEUROLOGY

Annals of Clinical and Translational Neurology Pub Date : 2024-09-02 DOI:10.1002/acn3.52191

Fei Jiang, Haobing Cai, Hongliang Li, Weifan Yin, Song Ouyang, Jue Hu, Ewen Tu, Ke Fu, Junjie Yin, Zhen Zhao, Jieyu Yang, Qiuming Zeng, Huan Yang

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Abstract

Objective

This study aimed to investigate the clinical characteristics and predictors of relapse in double negative atypical inflammatory demyelinating disease (IDD) and to explore potential antigenic targets by tissue-based assays (TBA) using rat brain indirect immunofluorescence.

Methods

We compared the clinical, laboratory, and MRI data of double negative atypical IDD with other IDD patients. Serum samples were collected for TBA. The predictors of relapse were examined over a minimum of 24 months follow-up.

Results

In our cohort of 98 patients with double negative atypical IDD, there was no significant female predominance (58.2%, 57/98). The lesions primarily affected the spinal cord and brain stem, with fewer cases of involvement in the area postrema (5.1%, 5/98) and longitudinally extensive transverse myelitis (43.9%, 43/98). A total of 62.5% (50/80) patients tested positive for anti-astrocyte antibodies based on rat brain TBA. Over a median duration of 39.5 months, 80 patients completed the entire follow-up, and 47.5% (38/80) patients exhibited monophasic course. A total of 36% (18/50) patients positively for anti-astrocyte antibodies had a monophasic course, which is significantly lower than patients negatively for anti-astrocyte antibodies (66.7%, 20/30) (p = 0.008). The presence of anti-astrocyte antibodies (hazard ratio (HR), 2.243; 95% CI, 1.087–4.627; p = 0.029) and ≥4 cerebrum lesions at first attack (HR, 2.494; 95% CI, 1.224–5.078; p = 0.012) were risk factors for disease relapse, while maintenance immunotherapy during remission (HR, 0.361; 95% CI, 0.150–0.869; p = 0.023) was protective factor.

Interpretation

Double negative atypical IDD are unique demyelinating diseases with a high relapse rate. Maintenance immunotherapy is helpful to the prevention of relapse, particularly in patients with anti-astrocyte antibodies or ≥4 cerebrum lesions at first attack.

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双阴性非典型炎性脱髓鞘疾病的临床特征：前瞻性研究

研究目的本研究旨在调查双阴性非典型炎性脱髓鞘病（IDD）的临床特征和复发预测因素，并利用大鼠脑部间接免疫荧光法（TBA）通过组织基础测定（TBA）探索潜在的抗原靶点：我们比较了双阴性非典型 IDD 与其他 IDD 患者的临床、实验室和 MRI 数据。采集血清样本进行 TBA 分析。在至少 24 个月的随访期间，对复发的预测因素进行了研究：在我们的 98 例双阴性非典型 IDD 患者中，女性患者并不占多数（58.2%，57/98）。病变主要累及脊髓和脑干，较少累及后遗区（5.1%，5/98）和纵向广泛横贯性脊髓炎（43.9%，43/98）。根据大鼠脑TBA检测，共有62.5%（50/80）的患者抗胃肠细胞抗体呈阳性。中位病程为 39.5 个月，80 名患者完成了整个随访，47.5%（38/80）的患者表现为单相病程。抗胃泌素细胞抗体阳性的患者中，36%（18/50）的病程为单相，明显低于抗胃泌素细胞抗体阴性的患者（66.7%，20/30）（P = 0.008）。抗胃肠细胞抗体的存在（危险比（HR），2.243；95% CI，1.087-4.627；p = 0.029）和首次发病时脑部病灶≥4个（HR，2.494；95% CI，1.224-5.078；p = 0.012）是疾病复发的危险因素，而缓解期维持免疫治疗（HR，0.361；95% CI，0.150-0.869；p = 0.023）则是保护因素：双阴性非典型IDD是一种独特的脱髓鞘疾病，复发率很高。解读：双阴性非典型IDD是一种独特的脱髓鞘疾病，复发率很高。维持性免疫治疗有助于预防复发，尤其是对首次发病时有抗胃蛋白酶抗体或脑部病灶≥4个的患者。

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来源期刊

Annals of Clinical and Translational Neurology Medicine-Neurology (clinical)

CiteScore

9.10

自引率

1.90%

发文量

218

审稿时长

8 weeks

期刊介绍： Annals of Clinical and Translational Neurology is a peer-reviewed journal for rapid dissemination of high-quality research related to all areas of neurology. The journal publishes original research and scholarly reviews focused on the mechanisms and treatments of diseases of the nervous system; high-impact topics in neurologic education; and other topics of interest to the clinical neuroscience community.