Rousseaux C G, Klavano G G, Johnson E S, Shnitka T K, Harries W N, Snyder F F
Vet Pathol. 1985 Mar;22(2):104-11. doi: 10.1177/030098588502200202.
The clinical and pathological features of a newly recognized, inherited neurodegenerative disorder in horned Hereford calves are described. The disorder is expressed in newborns by tremulous shaking of the head, body and tail, difficulty in rising, a wobbly spastic gait, and aphonia. Transient improvement is followed by deterioration and progressive spastic paraplegia. Generalized tremors can be induced easily by a variety of stimuli, and spinal reflexes may be exaggerated or depressed. The major pathological finding is an excessive accumulation of neurofilaments within neurons of the central, peripheral, and autonomic nervous systems. The involvement of multiple systems of neurons and the similarity with some forms of human motor neuron disease and spinocerebellar degeneration suggest that this unique bovine disease may serve as a suitable animal model for these human neurodegenerative disorders.
本文描述了一种新发现的遗传性神经退行性疾病在有角海福特犊牛中的临床和病理特征。该疾病在新生犊牛中表现为头部、身体和尾巴颤抖，起立困难，步态蹒跚痉挛，以及失声。短暂改善后会出现病情恶化和进行性痉挛性截瘫。各种刺激很容易诱发全身性震颤，脊髓反射可能亢进或减弱。主要病理发现是中枢、外周和自主神经系统神经元内神经丝过度积聚。多个神经元系统受累以及与某些形式的人类运动神经元疾病和脊髓小脑变性的相似性表明，这种独特的牛病可能是这些人类神经退行性疾病的合适动物模型。
