Wakshlag J J, de Lahunta A
Department of Clinical Sciences, Cornell University, College of Veterinary Medicine, Ithaca, NY 14853-6401, USA.
J Small Anim Pract. 2009 Dec;50(12):670-4. doi: 10.1111/j.1748-5827.2009.00857.x.
An Alaskan husky puppy was examined for a neurologic disease which began at six weeks of age with generalised paresis that progressed resulting in recumbency by 18 weeks. Thoracic limbs primarily exhibited lower motor neuron signs that included distal muscle atrophy and persistent elbow and carpal flexion that resisted manual extension. Pelvic limb signs primarily exhibited upper motor neuron and general proprioceptive deficits, but also included lower motor neuron signs. Abnormal vocalisation suggested a laryngeal paresis. Histopathologic lesions included a diffuse axonopathy and secondary demyelination in the nerves of the limbs and larynx and a similar bilaterally symmetrical degeneration in the spinal cord white matter suggestive of a dying back axonopathy. In addition, a degenerative process was present in nuclei in the brain stem and cerebellum. Recognition of this disease through clinical and pathologic examination in other related Alaskan Huskies suggested an autosomal recessive inherited disorder.
一只阿拉斯加哈士奇幼犬因神经系统疾病接受检查，该疾病始于六周龄，最初表现为全身性轻瘫，病情进展，到18周龄时导致卧地不起。胸肢主要表现为下运动神经元体征，包括远端肌肉萎缩以及持续的肘部和腕关节屈曲，手动伸展时无法伸直。盆腔肢体征主要表现为上运动神经元和一般本体感觉缺陷，但也包括下运动神经元体征。异常发声提示喉轻瘫。组织病理学病变包括四肢和喉部神经的弥漫性轴索性神经病和继发性脱髓鞘，以及脊髓白质中类似的双侧对称性变性，提示轴索性神经病逆行性变性。此外，脑干和小脑的核团存在退行性病变。通过对其他相关阿拉斯加哈士奇进行临床和病理检查来识别这种疾病，提示这是一种常染色体隐性遗传性疾病。
