Clinicopathological features of canine neuroaxonal dystrophy and cerebellar cortical abiotrophy in Papillon and Papillon-related dogs
Clinicopathological features of canine neuroaxonal dystrophy and cerebellar cortical abiotrophy in Papillon and Papillon-related dogs
复制标题
DOI：
10.1292/jvms.69.1047
复制
发表时间：
2007-10-01
影响因子：
1.2
通讯作者：
Uchida, Kazuyuki
中科院分区：
文献类型：
--
作者：
Nibe, Kazumi;Kita, Chiaki;Uchida, Kazuyuki
对2只Papillon犬和1只Papillon与奇瓦瓦州杂交犬进行了神经轴索营养不良（NAD）检查。此外，还研究了Papillon犬的小脑皮质营养不良（CCA），该犬具有与NAD相似的临床和磁共振成像（MRI）特征。所有受NAD和CCA影响的犬的常见临床症状为骨盆肢共济失调和小脑共济失调，包括早期发作的意向性震颤、头部震颤和测量过度。这些临床体征进展迅速，根据主人的要求，两只患有NAD的犬被人道处死，另外两只死于吸入性肺炎。尸检时的MRI检查和大体观察结果显示，所有NAD和CCA病例均出现中度至重度小脑萎缩。NAD最典型的组织学改变是脊髓后角、延髓薄核、楔核、橄榄核及其周围的轴索变性和球状体形成。球状体的特征为在扩大的髓鞘内的大嗜酸性或颗粒状球体，有时伴有小胶质细胞和/或巨噬细胞的中度积聚。与此相反，这种球状体的形成是最小的CCA的大脑。在小脑，轻度至中度损失的浦肯野细胞和颗粒细胞被确认在三只狗与NAD，而这些变化更突出的狗与CCA。虽然NAD和CCA的临床体征和MRI表现相对相似，但组织病理学特征被认为有很大差异，提示不同的发病机制和病因。由于NAD和CCA都被认为是常染色体隐性遗传疾病，因此在Papillon和奇瓦瓦州犬的育种中可能需要仔细考虑。
Neuroaxonal dystrophy (NAD) was examined in two Papillon dogs and a mix breed dog between Papillon and Chihuahua. In addition, cerebellar cortical abiotrophy (CCA) in a Papillon dog, which had similar clinical and magnetic resonance imaging (MRI) features to those of NAD, was also investigated. The common clinical symptoms of all dogs affected with NAD and CCA, were pelvic limb ataxia and cerebellar ataxia including intention tremor, head tremor, and hypermetria in the early onset. These clinical signs were progressed rapidly, and two dogs with NAD were euthanized by owner's request and the other two were died by aspiration pneumonia. MRI examinations and gross observations at necropsy revealed moderate to severe cerebellar atrophy in all cases of NAD and CCA. The most typical histological change of NAD was severe axonal degeneration with marked spheroid-formation in the dorsal horn of the spinal cords, the nuclei gracilis, cuneatus, olivalis and its circumference in the medulla oblongata. The spheroids were characterized as large eosinophilic or granular globes within the enlarged myelin sheaths, sometimes accompanied by moderate accumulation of microglias and/ or macrophages. In contrast, such spheroid formation was minimal in the brain of CCA. In the cerebellum, mild to moderate loss of the Purkinje and granular cells were recognized in three dogs with NAD, whereas these changes were more prominent in a dog with CCA. Although the clinical signs and MRI findings relatively resembled between NAD and CCA, the histopathological features considered to be quite differ, suggesting distinct pathogenesis and etiology. Since both NAD and CCA are proposed as the autosomal recessive hereditary disorders, careful considerations might be needed for the breeding of Papillon and Chihuahua dogs.