Menini C, Silva-Barrat C
Laboratoire de Physiologie Nerveuse du CNRS, GIF-SUR-YVETTE, France.
Pathol Biol (Paris). 1990 Mar;38(3):205-13.
The baboon Papio papio is the only animal model showing a natural photosensitive epilepsy very similar to that observed in some human epileptic patients. In the baboon, intermittent light stimulation (ILS) induces bilateral and synchronous myoclonic twitches which are associated with paroxysmal discharges (PDs) predominating in the frontal cortex, and can be followed by generalized tonic-clonic seizures. We were able to demonstrate the motor cortical origin of all these manifestations since neuronal generators responsible for paroxysmal discharges are localized there and are activated by visual afferents from the occipital lobe. The corpus callosum is the structure determining the interhemispheric synchronization of PDs. An unbalance of neurotransmitter systems such as GABA or excitatory amino acids should be responsible for the hereditary predisposition of baboons to photosensitive epilepsy. Some Papio papio, either photosensitive or not, may show spontaneous truncular myoclonic twitches, different from those induced by intermittent light stimulation, and resembling the intention myoclonus as observed in some human neurological disorders (post-anoxic syndrome, degenerative encephalopathies such as Ramsay-Hunt syndrome...). Because of the absence of any abnormal electrographic discharge, this myoclonus is considered non epileptic. Until now, we were unable to determine the structure generating this myoclonus. The most probable origin is in the lower brain stem. Experimental data suggest that a local unbalance of the cholinergic neurotransmission could be responsible for the predisposition of baboons to show this type of myoclonus.
东非狒狒(Papio papio)是唯一一种表现出与某些人类癫痫患者所观察到的非常相似的自然光敏性癫痫的动物模型。在东非狒狒中,间歇性光刺激(ILS)会诱发双侧同步肌阵挛抽搐,这与额叶皮质中占主导地位的阵发性放电(PDs)相关,并可能继以全身性强直阵挛发作。我们能够证明所有这些表现都起源于运动皮质,因为负责阵发性放电的神经元发生器位于此处,并由来自枕叶的视觉传入神经激活。胼胝体是决定PDs半球间同步的结构。神经递质系统如GABA或兴奋性氨基酸的失衡应是东非狒狒对光敏性癫痫遗传易感性的原因。一些东非狒狒,无论是否光敏,可能会出现自发的躯干肌阵挛抽搐,与间歇性光刺激诱发的抽搐不同,类似于在一些人类神经系统疾病(缺氧后综合征、退行性脑病如拉姆齐-亨特综合征……)中观察到的意向性肌阵挛。由于没有任何异常的脑电图放电,这种肌阵挛被认为是非癫痫性的。到目前为止,我们还无法确定产生这种肌阵挛的结构。最可能的起源是在脑桥下部。实验数据表明,胆碱能神经传递的局部失衡可能是东非狒狒易出现这种类型肌阵挛的原因。