School of Veterinary Medicine, University of Glasgow, Glasgow, UK.
School of Veterinary Medicine and Science, University of Nottingham, Loughborough, UK.
Vet Rec. 2019 Sep 28;185(12):375. doi: 10.1136/vr.105240. Epub 2019 Jul 25.
A novel spongiform leucoencephalomyelopathy was reported in border terrier puppies in 2012 causing a shaking puppy phenotype, but no information regarding clinical progression, imaging or electrophysiological findings were available. The aim of the present study was to describe the clinical, electrophysiological and MRI features of this disease in seven dogs and compare them with human white matter disorders. All cases presented with cerebellar ataxia and severe generalised coarse body tremors, which started at three weeks of age. The three cases that were not euthanased showed slow but progressive improvement over several months. Brainstem auditory evoked response demonstrated a normal wave I, reduced amplitude of wave II and an absence of waves III-VII. MRI revealed bilateral and symmetrical T2-weighted hyperintensities affecting the brainstem and cerebellar white matter. Histological examination of the brain and spinal cord showed spongiform change affecting the white matter of the cerebellum, brainstem and spinal cord with decreased myelin content. In summary, this leucoencephalomyelopathy has a pathognomonic clinical presentation with defining MRI and electrophysiological characteristics, and it is the first report to describe a long-term improvement of this condition.
2012 年，报道了一种在边境梗幼犬中发生的新型海绵状脑白质病，导致幼犬出现颤抖表型，但尚无关于临床进展、影像学或电生理学发现的信息。本研究的目的是描述 7 只犬的这种疾病的临床、电生理和 MRI 特征，并将其与人类白质疾病进行比较。所有病例均表现为小脑共济失调和严重的全身性粗体震颤，始于 3 周龄。未安乐死的 3 例在数月内缓慢但逐渐改善。脑干听觉诱发电位显示 I 波正常，II 波振幅降低，III-VII 波缺失。MRI 显示双侧和对称的 T2 加权高信号，影响脑干和小脑白质。脑和脊髓的组织学检查显示，小脑、脑干和脊髓的白质呈海绵状改变，髓鞘含量减少。总之，这种脑白质病具有特征性的临床表现，具有明确的 MRI 和电生理特征，这是首次描述这种疾病的长期改善。
