Hanzlícek D, Kathmann I, Bley T, Srenk P, Botteron C, Gaillard C, Jaggy A
Institut für Genetik und Ernährung, Universität Bern, Brno, CZ.
Schweiz Arch Tierheilkd. 2003 Aug;145(8):369-75. doi: 10.1024/0036-7281.145.8.369.
Three American Staffordshire Terriers were presented with gait abnormalities and loss of balance at the age of 4.5 (female) and 6 years (2 males). The onset varied between 3 and 5 years of age and the clinical signs were slowly progressive. The neurological examination revealed symmetrical generalized cerebellar ataxia with hypermetria, stiffness, and loss of balance with no evidence of paresis. The menace reflex was decreased in one dog and absent in another. A positional nystagmus was found in two dogs. The dogs were euthanized and a histopathological examination of each brain was performed. Pathological changes were confined to the cerebellum. The main finding was loss of Purkinje cells, as well as depletion of granular cell bodies and shrinkage of the granular and molecular cell layer. These findings are consistent with cerebellar cortical abiotrophy. A genetic basis is supposed, but the mode of inheritance is not determined yet. In contrast to some spinocerebellar ataxias in humans, the cause of Purkinje cell degeneration in cerebellar cortical abiotrophy of dogs is not known.
三只美国斯塔福郡梗犬在4.5岁(雌性)和6岁(两只雄性)时出现步态异常和平衡失调。发病年龄在3至5岁之间,临床症状呈缓慢进展。神经学检查显示对称性全身性小脑共济失调,伴有动作过度、僵硬和平衡失调,无轻瘫迹象。一只犬的威胁反射减弱,另一只则消失。两只犬出现位置性眼球震颤。这些犬被实施安乐死,并对每只犬的大脑进行了组织病理学检查。病理变化局限于小脑。主要发现是浦肯野细胞丢失,以及颗粒细胞体减少和颗粒层与分子层萎缩。这些发现与小脑皮质发育不全相符。推测存在遗传基础,但遗传方式尚未确定。与人类的某些脊髓小脑共济失调不同,犬小脑皮质发育不全中浦肯野细胞变性的原因尚不清楚。