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两只斗牛獒犬的少突胶质细胞发育异常

Oligodendroglial dysplasia in two bullmastiff dogs.

作者信息

Morrison J P, Schatzberg S J, De Lahunta A, Ross J T, Bookbinder P, Summers B A

机构信息

Department of Biomedical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853, USA.

出版信息

Vet Pathol. 2006 Jan;43(1):29-35. doi: 10.1354/vp.43-1-29.

Abstract

Leukodystrophies are inherited neurological disorders involving central nervous system white matter. They are uncommon in animals but a few, breed-specific entities have been described. In 2002, two young-adult, purebred Bullmastiff dogs from central New York State presented to their referring veterinarians displaying moderate to severe ataxia of all limbs, spastic tetraparesis that was worse in the pelvic limbs, and a diffuse, action-related, whole-body tremor. Clinical signs were insidious in onset and slowly progressive. Anatomic diagnoses considered were a C1-C5 lesion or, based on the whole-body tremor, a diffuse central nervous system disorder. No gross lesions were apparent in the brain or spinal cord. Histopathologically, numerous, multifocal, sharply demarcated, small, ovoid to angular areas of myelin pallor (plaques) were present throughout the major white matter tracts of the brainstem and spinal cord. These plaques, which often were traversed by axons, did not stain with luxol fast blue for myelin and were associated with minimal astrocytosis. Ultrastructural findings include occasional hypertrophic glia in white matter, rare unmyelinated segments of axons, and focal proliferation of tubule-containing cytoplasmic glial cell processes (oligodendroglial). The described clinical and morphological findings and age of onset are similar to the well-characterized, presumably hereditary, bovine syndrome known as Charolais ataxia or oligodendroglial dysplasia. This article presents the first description of a leukodystrophy in the Bullmastiff breed and the first report of oligodendroglial dysplasia in animals other than Charolais cattle.

摘要

脑白质营养不良是一种涉及中枢神经系统白质的遗传性神经疾病。它们在动物中并不常见,但已经描述了一些特定品种的病例。2002年,来自纽约州中部的两只成年纯种斗牛獒犬被转诊至兽医处,表现出四肢中度至重度共济失调、痉挛性四肢轻瘫(后肢更严重)以及弥漫性、与动作相关的全身震颤。临床症状起病隐匿且进展缓慢。初步考虑的解剖学诊断为C1 - C5病变,或者基于全身震颤考虑为弥漫性中枢神经系统疾病。在脑和脊髓中未发现明显的肉眼可见病变。组织病理学检查显示,在脑干和脊髓的主要白质束中存在大量、多灶性、界限清晰的小的卵圆形至角形髓鞘苍白区(斑块)。这些斑块常有轴突穿过,对髓鞘用卢戈氏坚牢蓝染色不着色,且伴有轻微的星形细胞增多。超微结构发现包括白质中偶尔出现的肥大神经胶质细胞、罕见的无髓鞘轴突节段以及含微管的细胞质神经胶质细胞突起(少突胶质细胞)的局灶性增生。所描述的临床和形态学发现以及发病年龄与特征明确的、可能为遗传性的牛综合征(称为夏洛来共济失调或少突胶质细胞发育异常)相似。本文首次描述了斗牛獒品种中的脑白质营养不良,也是首次报道除夏洛来牛以外的动物中出现少突胶质细胞发育异常。

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