抗-Tr/DNER抗体相关的小脑性共济失调：三例罕见病例报告及文献复习
Anti-Tr/DNER antibody-associated cerebellar ataxia: three rare cases report and literature review.
作者信息
Cai Yun, Liu Jie, Ren Haitao, Shi Fuping, Che Jing, Geng Qiang, Tan Yanli, Guan Hongzhi, Di Weiying
机构信息
Department of Neurology, Affiliated Hospital of Hebei University, Baoding, 071000, China.
Department of Neurology, People's Hospital of Sichuan Province, Chengdu, 610072, China.
出版信息
Neurol Sci. 2023 Jan;44(1):397-403. doi: 10.1007/s10072-022-06389-x. Epub 2022 Sep 12.
BACKGROUND
To report three cases of autoimmune cerebellar ataxia related to anti-delta/notch-like epidermal growth factor-related receptor (Tr/DNER) antibodies.
CASE PRESENTATION
Patients with unknown cerebellar ataxia were screened with autoimmune cerebellar ataxia (ACA)-related antibody panel. The anti-Tr antibody was positive in three female patients in whom the onset ages were 43 years, 35 years and 43 years old. The antibody titres of serum and cerebrospinal fluid were all 1:32. Cerebral ataxia was the most prominent presentation. Mild cerebellar atrophy was found in one of the patients. Immunotherapy was effective in all three patients.
CONCLUSION
The Tr antibody is associated with autoimmune ataxia, and it has been suggested that the anti-Tr antibody should be tested in patients with cerebellar ataxia who are negative for routine ACA antibodies. Early immunotherapy may improve patient prognoses.
背景
报告3例与抗δ/Notch样表皮生长因子相关受体（Tr/DNER）抗体相关的自身免疫性小脑共济失调病例。
病例报告
对不明原因的小脑共济失调患者进行自身免疫性小脑共济失调（ACA）相关抗体检测。3例女性患者抗Tr抗体呈阳性，发病年龄分别为43岁、35岁和43岁。血清和脑脊液抗体滴度均为1:32。小脑性共济失调是最突出的表现。其中1例患者发现轻度小脑萎缩。免疫治疗对所有3例患者均有效。
结论
Tr抗体与自身免疫性共济失调有关，提示对常规ACA抗体阴性的小脑共济失调患者应检测抗Tr抗体。早期免疫治疗可能改善患者预后。
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