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一种自发性发生自身免疫性边缘性脑炎的猫模型。

A feline model of spontaneously occurring autoimmune limbic encephalitis.

机构信息

Clinic for Small Animals, Internal Medicine, University of Veterinary Medicine Vienna, Austria.

Institute for Pathology and Forensic Veterinary Medicine, Universitiy of Veterinary Medicine Vienna, Austria.

出版信息

Vet J. 2023 Jun-Jul;296-297:105974. doi: 10.1016/j.tvjl.2023.105974. Epub 2023 Mar 21.

DOI:10.1016/j.tvjl.2023.105974
PMID:36958405
Abstract

Autoimmune encephalitis (AE) is an important cause of encephalitis in humans and occurs at a similar rate to infectious encephalitis. It is frequently associated with antibodies against the extracellular domain of neuronal proteins. Among human AE, that with antibodies against leucine-rich glioma-inactivated 1 (LGI1) is one of the most prevalent forms, and was recently described in cats with limbic encephalitis (LE). In this study, we describe a large cohort (n = 32) of cats with AE, tested positive for voltage gated potassium channel (VGKC)-antibodies, of which 26 (81%) harboured LGI1-antibodies. We delineate their clinical and paraclinical features as well as long-term outcomes up to 5 years. Similar to human cases, most cats with LGI1-antibodies had a history of focal seizures (83%), clustering in the majority (88%), with interictal behavioural changes (73%). Among feline AE patients, there was no seizure type or other clinical characteristic that could distinguish LGI1-antibody positive from negative cats, unlike the pathognomic faciobrachial dystonic seizures seen in humans. Although six cats were euthanased in the first year for epilepsy-associated reasons, those attaining at least 1-year survival had good seizure control and quality of life with appropriate veterinary care and medication. Acute-phase immunotherapy (prednisolone) was given to the most severely unwell cases and its effect is retrospectively evaluated in 10 cats. Our data show LGI1-antibodies are an important cause of feline encephalitis, sharing many features with human AE. Further research should examine optimal therapeutic management strategies and the cause of LE in seronegative cats, building on paradigms established in the counterpart human disease.

摘要

自身免疫性脑炎 (AE) 是人类脑炎的一个重要病因,其发病率与感染性脑炎相似。它常与神经元蛋白细胞外结构域的抗体有关。在人类 AE 中,针对富亮氨酸胶质瘤失活 1 型 (LGI1) 的抗体是最常见的一种形式,最近在伴有边缘性脑炎 (LE) 的猫中也有描述。在本研究中,我们描述了一组 32 只经检测对电压门控钾通道 (VGKC) 抗体呈阳性的 AE 猫,其中 26 只 (81%) 携带 LGI1 抗体。我们描述了它们的临床和临床前特征以及长达 5 年的长期预后。与人类病例相似,大多数携带 LGI1 抗体的猫有局灶性癫痫发作史 (83%),大部分呈簇状发作 (88%),伴有发作间期行为改变 (73%)。在猫 AE 患者中,没有任何癫痫发作类型或其他临床特征可以区分 LGI1 抗体阳性和阴性的猫,这与人类中特有的面臂肌张力障碍性发作不同。尽管有 6 只猫因癫痫相关原因在第一年被安乐死,但那些至少存活 1 年的猫通过适当的兽医护理和药物治疗,癫痫得到了很好的控制,生活质量也得到了提高。急性期免疫治疗 (泼尼松龙) 用于病情最严重的病例,并对 10 只猫进行了回顾性评估。我们的数据表明,LGI1 抗体是猫脑炎的一个重要病因,与人类 AE 有许多共同特征。进一步的研究应该检查最佳治疗管理策略和血清阴性猫 LE 的病因,建立在对应人类疾病的范例基础上。

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A feline model of spontaneously occurring autoimmune limbic encephalitis.一种自发性发生自身免疫性边缘性脑炎的猫模型。
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引用本文的文献

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J Feline Med Surg. 2025 Sep;27(9):1098612X251365423. doi: 10.1177/1098612X251365423. Epub 2025 Sep 7.
2
Distinctive seizure signature in the first video case-control study of a naturally-occurring feline autoimmune encephalitis model.在一项针对自然发生的猫自身免疫性脑脊髓炎模型的首个视频病例对照研究中独特的癫痫发作特征。
Brain Behav Immun. 2025 May;126:289-296. doi: 10.1016/j.bbi.2025.02.018. Epub 2025 Feb 19.
3
Gliosarcoma associated with bilateral hippocampal sclerosis in a cat presenting complex partial seizures with orofacial involvement: A case report.
一只患有伴有口面部受累的复杂部分性癫痫发作的猫,其胶质肉瘤与双侧海马硬化相关:病例报告。
Clin Case Rep. 2024 Sep 9;12(9):e9123. doi: 10.1002/ccr3.9123. eCollection 2024 Sep.