Ogawa Mizue, Uchida Kazuyuki, Isobe Kyoko, Saito Miyoko, Harada Tomoyuki, Chambers James K, Nakayama Hiroyuki
Neuropathology. 2014 Apr;34(2):190-6. doi: 10.1111/neup.12076.
A male Japanese domestic cat developed progressive limb paralysis from 4 months of age. The cat showed visual disorder, trismus and cognitive impairment and died at 9 months of age. At necropsy, significant discoloration of the white matter was observed throughout the brain and spinal cord. Histologically, severe myelin loss and gliosis were observed, especially in the internal capsule and cerebellum.In the lesions, severe infiltration of macrophages with broad cytoplasm filled with PAS-positive and nonmetachromatic granules (globoid cells) was evident. On the basis of these findings, the case was diagnosed as feline globoid cell leukodystrophy (Krabbe’s disease). Immunohistochemical observation indicated the involvement of oxidative stress and small HSP in the disease.
一只雄性日本家猫从4个月大时开始出现进行性肢体麻痹。这只猫表现出视觉障碍、牙关紧闭和认知障碍,于9个月大时死亡。尸检时,在整个脑和脊髓中观察到白质明显变色。组织学检查发现,尤其是在内囊和小脑中,有严重的髓鞘脱失和胶质细胞增生。在病变中,可见大量巨噬细胞浸润,其宽大的胞质内充满PAS阳性且非异染性的颗粒(球状细胞)。基于这些发现,该病例被诊断为猫球状细胞脑白质营养不良(克拉伯病)。免疫组织化学观察表明氧化应激和小分子热休克蛋白参与了该疾病。