Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410000, Hunan, P.R. China.
Clinical Research Center for Neuroimmune and Neuromuscular disorders, Xiangya Hospital, Central South University, Changsha, 410008, Hunan, P.R. China.
Ann Clin Transl Neurol. 2024 Oct;11(10):2769-2784. doi: 10.1002/acn3.52191. Epub 2024 Sep 2.
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.
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.
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.
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.
本研究旨在探讨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，并通过大鼠脑间接免疫荧光法进行组织基础检测（TBA）来探索潜在的抗原靶点。
我们比较了双阴性非典型 IDD 患者与其他 IDD 患者的临床、实验室和 MRI 数据。收集血清样本进行 TBA。在至少 24 个月的随访中，检查了复发的预测因素。
在我们的 98 例双阴性非典型 IDD 患者中，没有明显的女性优势（58.2%，57/98）。病变主要影响脊髓和脑干，较少累及极后区（5.1%，5/98）和长节段横贯性脊髓炎（43.9%，43/98）。根据大鼠脑 TBA，共有 62.5%（50/80）的患者抗星形胶质细胞抗体检测呈阳性。在中位 39.5 个月的随访期间，80 例患者完成了整个随访，47.5%（38/80）的患者呈单相病程。共有 36%（18/50）抗星形胶质细胞抗体阳性患者呈单相病程，明显低于抗星形胶质细胞抗体阴性患者（66.7%，20/30）（p=0.008）。抗星形胶质细胞抗体的存在（风险比（HR），2.243；95%置信区间，1.087-4.627；p=0.029）和首次发作时≥4 个脑病变（HR，2.494；95%置信区间，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%置信区间，0.150-0.869；p=0.023）是保护因素。
双阴性非典型 IDD 是独特的脱髓鞘疾病，复发率较高。维持免疫治疗有助于预防复发，特别是在首次发作时具有抗星形胶质细胞抗体或≥4 个脑病变的患者中。
