Carmichael K P, Howerth E W, Oliver J E, Klappenbach K
Athens Veterinary Diagnostic Laboratory, College of Veterinary Medicine, University of Georgia, Athens 30602.
J Vet Diagn Invest. 1993 Oct;5(4):585-90. doi: 10.1177/104063879300500414.
A syndrome resembling previously described feline hereditary neuroaxonal dystrophy (FHND) was diagnosed in a litter of cats. The disorder was characterized by a sudden onset of hind limb ataxia that slowly progressed to hind limb paresis and paralysis. The cats were between 6 and 9 months old when clinical signs were first noted. Histologically, there was marked ballooning of axonal processes, with spheroid formation and vacuolation in specific regions of the brain and spinal cord. Some dystrophic axons contained a central periodic acid-Schiff (PAS)-positive core. Neuronal loss and gliosis were seen in certain brain stem nuclei, spinal cord nuclei, and the cerebellum. Ultrastructurally, there was hypomyelination and dysmyelination of affected axons. The PAS-positive core in dystrophic axons corresponded ultrastructurally with accumulations of electron-dense, flocculent, amorphous material. In addition, these axons contained membrane-bound osmiophilic bodies and large nonmembrane-bound vacuoles. The syndrome in this report differs from the previously described FHND in that no inner ear involvement was seen and onset of clinical signs occurred at a later age. In addition, although some of the affected cats did have diluted coat colors, abnormal coat color was not always associated with clinical disease. This disease is similar to juvenile neuroaxonal dystrophy in children and to neuroaxonal dystrophies described in horses, dogs, cattle, and sheep.
一窝猫被诊断出患有一种类似于先前描述的猫遗传性神经轴索性营养不良（FHND）的综合征。该疾病的特征是后肢共济失调突然发作，随后逐渐发展为后肢轻瘫和瘫痪。首次发现临床症状时，这些猫的年龄在6至9个月之间。组织学上，轴突明显肿胀，在脑和脊髓的特定区域形成球体并出现空泡化。一些营养不良的轴突含有中央过碘酸希夫（PAS）阳性核心。在某些脑干核、脊髓核和小脑中可见神经元丢失和胶质细胞增生。超微结构上，受影响的轴突存在髓鞘形成减少和髓鞘发育异常。营养不良轴突中的PAS阳性核心在超微结构上与电子致密、絮状、无定形物质的积累相对应。此外，这些轴突含有膜结合的嗜锇小体和大的非膜结合空泡。本报告中的综合征与先前描述的FHND不同，在于未发现内耳受累，且临床症状的发作年龄较晚。此外，虽然一些患病猫确实有稀释的毛色，但毛色异常并不总是与临床疾病相关。这种疾病类似于儿童的青少年神经轴索性营养不良以及马、狗、牛和羊中描述的神经轴索性营养不良。
