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两匹哈福林格马中与维生素E缺乏相关的神经轴突营养不良

Neuroaxonal dystrophy associated with vitamin E deficiency in two Haflinger horses.

作者信息

Baumgärtner W, Frese K, Elmadfa I

机构信息

Institut für Veterinär-Pathologie, Fustus-Liebig-Universität, Giessen, F.R.G.

出版信息

J Comp Pathol. 1990 Jul;103(1):114-9.

PMID:2394844
Abstract

Neuroaxonal dystrophy and minimal diffuse degenerative myelopathy was diagnosed in two female full sibling, 1- and 2-year-old, Haflinger horses. Both animals developed slowly progressive ataxia from the age of 4 months. Clinical signs, which were more prominent in the hind legs, included hypermetria and dysmetria. Histological examination revealed neuroaxonal dystrophy characterized by spheroid formation, vacuolation, astrogliosis and lipofuscin pigment deposition in macrophages and neuronal perikarya. These changes were restricted to the gracilis and cuneate nuclei, nucleus of the solitary tract, nucleus intermediomedialis in the sacral and the seventh segment of the cervical spinal cord and Stilling-Clarke's column in both horses and the medial vestibular nucleus in the older horse. Both diseased Haflingers had significantly reduced serum alpha- and gamma-tocopherol values. No significant alteration in serum total lipid concentrations and serum selenium values were observed. It is likely that the condition has a familial hereditary basis. It is unclear whether there is a link between the observed neuroaxonal dystrophy and vitamin E deficiency and further investigations are warranted.

摘要

在两匹1岁和2岁的雌性同卵哈福林格马中诊断出神经轴突营养不良和轻度弥漫性退行性脊髓病。两匹马从4个月大时开始出现缓慢进展的共济失调。临床症状以后肢更为明显,包括动作过度和辨距不良。组织学检查显示神经轴突营养不良,其特征为巨噬细胞和神经元胞核中出现球状形成、空泡化、星形胶质细胞增生和脂褐素色素沉积。这些变化局限于两匹马的薄束核和楔束核、孤束核、骶段和颈髓第七节段的中间内侧核以及斯廷-克拉克柱,在较年长的马中还累及内侧前庭核。两匹患病的哈福林格马血清α-和γ-生育酚值显著降低。未观察到血清总脂质浓度和血清硒值有明显变化。这种情况可能有家族遗传基础。尚不清楚观察到的神经轴突营养不良与维生素E缺乏之间是否存在联系,因此有必要进行进一步调查。

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Pedigree analysis and exclusion of alpha-tocopherol transfer protein (TTPA) as a candidate gene for neuroaxonal dystrophy in the American Quarter Horse.
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