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Ⅱ型纤维蛋白样脑白质营养不良(亚历山大病)在幼年比格犬中的诊断特征。

Diagnostic features of type II fibrinoid leukodystrophy (Alexander disease) in a juvenile Beagle dog.

机构信息

Highcroft Veterinary Referrals, Bristol, UK.

School of Veterinary Medicine and Sciences, University of Nottingham Sutton Bonington Campus, Leicestershire, UK.

出版信息

J Vet Intern Med. 2023 Mar;37(2):670-675. doi: 10.1111/jvim.16655. Epub 2023 Feb 17.

DOI:10.1111/jvim.16655
PMID:36799664
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10061190/
Abstract

A 3-month-old female entire Beagle presented with a progressive history of caudotentorial encephalopathy. Reactive encephalopathies were ruled out and tests for the most common infectious diseases agents were negative. Magnetic resonance imaging of the brain using a 1.5 Tesla scanner showed diffuse, bilateral, T2-weighted and T2-weighted-FLAIR hyperintense, T1-weighted hypointense, noncontrast-enhancing lesions involving the white matter of the cerebellum, brainstem, spinal cord, and forebrain to a lesser extent. There was cerebellar enlargement. Abnormalities were not detected on cerebrospinal fluid examination. Given the progressive nature of the disease and suspected poor prognosis the dog was euthanized. Histopathological analysis of the brain was consistent with fibrinoid leukodystrophy, also known as Alexander disease. Based on the classification used in humans, this is a description of MRI of a case of type II Alexander disease in veterinary medicine, with characteristics different to other described leukoencephalopathies in dogs.

摘要

一只 3 月龄雌性全贝克犬出现进行性颅尾脑病病史。排除反应性脑病,并对最常见的传染病病原体进行了检测,结果均为阴性。使用 1.5T 扫描仪对大脑进行磁共振成像显示,小脑、脑干、脊髓和前脑的白质弥漫性、双侧、T2 加权和 T2 加权-FLAIR 高信号,T1 加权低信号,非增强病变,小脑肿大。脑脊液检查未发现异常。鉴于疾病的进行性和可疑的预后不良,对该犬实施了安乐死。脑的组织病理学分析与纤维蛋白样脑白质营养不良一致,也称为亚历山大病。根据在人类中使用的分类,这是兽医中 II 型亚历山大病的 MRI 描述,与其他描述的犬脑白质疾病不同。

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J Vet Intern Med. 2021 May;35(3):1455-1465. doi: 10.1111/jvim.16085. Epub 2021 Mar 18.
2
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J Vet Med Sci. 2020 Dec 5;82(11):1704-1707. doi: 10.1292/jvms.20-0133. Epub 2020 Oct 15.
3
Spongiform leucoencephalomyelopathy in border terriers: clinical, electrophysiological and imaging features.边境梗的海绵状白质脑病:临床、电生理和影像学特征。
Vet Rec. 2019 Sep 28;185(12):375. doi: 10.1136/vr.105240. Epub 2019 Jul 25.
4
Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe's disease).犬球状细胞脑白质营养不良(克拉伯病)中外周和中枢神经系统疾病的临床、电生理及生化标志物
J Neurosci Res. 2016 Nov;94(11):1007-17. doi: 10.1002/jnr.23838.
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A canine orthologue of the human GFAP c.716G>A (p.Arg239His) variant causes Alexander disease in a Labrador retriever.人类GFAP基因c.716G>A(p.Arg239His)变体的犬类直系同源基因导致一只拉布拉多猎犬患上亚历山大病。
Eur J Hum Genet. 2016 Jun;24(6):852-6. doi: 10.1038/ejhg.2015.223. Epub 2015 Oct 21.
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