Mervaala E, Andermann F, Quesney L F, Krelina M
Montreal Neurological Institute and Hospital, McGill University, Montreal, Québéc, Canada.
Neurology. 1990 Jan;40(1):53-6. doi: 10.1212/wnl.40.1.53.
We studied the effect of apomorphine, a dopamine receptor agonist, on epileptic photosensitivity in 7 patients with progressive myoclonus epilepsy (PME). Specific diagnoses included Baltic PME (Unverricht-Lundborg disease), Lafora disease, Kufs' disease, juvenile neuroaxonal dystrophy, and action myoclonus-renal failure syndrome; 2 patients had PME of uncertain etiology. Apomorphine blocked the epileptic photosensitivity in all patients and also reduced intention myoclonus in a patient with Baltic PME. There is a common deficit of dopaminergic inhibitory neurotransmission at the level of the striate cortex in patients with PME, regardless of the nature of the specific underlying neuropathologic process.
我们研究了多巴胺受体激动剂阿扑吗啡对7例进行性肌阵挛癫痫（PME）患者癫痫光敏性的影响。具体诊断包括波罗的海PME（翁韦里希特-伦德伯格病）、拉福拉病、库夫斯病、青少年神经轴索性营养不良和动作性肌阵挛-肾衰竭综合征；2例患者病因不明。阿扑吗啡可阻断所有患者的癫痫光敏性，还可减轻1例波罗的海PME患者的意向性肌阵挛。无论具体潜在神经病理过程的性质如何，PME患者在纹状皮质水平均存在多巴胺能抑制性神经传递的共同缺陷。
