Mitler M M, Boysen B G, Campbell L, Dement W C
Exp Neurol. 1974 Nov;45(2):332-40. doi: 10.1016/0014-4886(74)90122-8.
Narcolepsy in human beings is a life-long illness afflicting 100,000–150,000 Americans. No current treatment for this neurological disorder is satisfactory and a definitive approach to the disease requires an animal model of narcolepsy. This report summarizes data on a female toy poodle which has a canine form of narcolepsy-cataplexy. At approximately 4 mo of age, the dog presented cataplectic attacks. Physical examination disclosed no systemic factors to account for such atonic episodes. Observation indicated that attacks were frequently partial, involving only the neck musculature and hind legs. However, such attacks could develop into complete cataplexy, causing postural collapse and areflexia, although extrinsic eye muscles and the muscles of respiration were spared. Presentation of food, water, or a plaything were the most frequent elicitors of attacks. During 41 hr of EEG, EOG, and EMG monitoring in conjunction with behavioral observation, the dog exhibited normal polygraphic wakefulness, slow-wave sleep and REM sleep. Unambiguous sleep onset REM periods and cataplectic attacks were also observed. These pathological manifestations are analogous to those characteristic of human narcolepsy. The diagnosis of canine narcolepsy was further confirmed by two negative trials with neostigmine (ruling out myasthenia) and two positive trials with imipramine (cataplexy in human narcolepsy responds to imipramine treatment). The dog will be bred either with a littermate or a similarly afflicted male in an attempt to produce a population of afflicted dogs.
人类发作性睡病是一种终身疾病,折磨着10万至15万美国人。目前针对这种神经紊乱疾病的治疗方法都不尽人意,而要找到治疗该疾病的决定性方法需要建立发作性睡病的动物模型。本报告总结了一只患有犬类发作性睡病-猝倒症的雌性玩具贵宾犬的数据。大约在4月龄时,这只狗出现了猝倒发作。体格检查未发现导致这种张力缺失发作的全身性因素。观察表明,发作通常是局部的,仅累及颈部肌肉和后腿。然而,这种发作可能会发展为完全性猝倒,导致姿势性虚脱和反射消失,不过眼外肌和呼吸肌未受影响。提供食物、水或玩具是最常见的发作诱发因素。在结合行为观察进行41小时的脑电图(EEG)、眼电图(EOG)和肌电图(EMG)监测期间,这只狗表现出正常的多导睡眠图清醒、慢波睡眠和快速眼动睡眠。还观察到明确的睡眠始发快速眼动期和猝倒发作。这些病理表现与人类发作性睡病的特征相似。通过新斯的明的两项阴性试验(排除重症肌无力)和丙咪嗪的两项阳性试验(人类发作性睡病的猝倒对丙咪嗪治疗有反应)进一步证实了犬类发作性睡病的诊断。这只狗将与同窝仔犬或同样患病的雄性犬交配,试图培育出一群患病犬。