Photosensitive epilepsies and pathophysiologic mechanisms of the photoparoxysmal response
Photosensitive epilepsies and pathophysiologic mechanisms of the photoparoxysmal response
复制标题
光敏性癫痫和光阵发性反应的病理生理机制
作者：
Y. Takahashi;T. Fujiwara;K. Yagi;M. Seino
目的：目的探讨光敏性癫痫综合征中光阵发性反应（PPR）的病理生理机制及其与癫痫分类和癫痫综合征的关系。背景资料：作者在特发性全身性癫痫和遗传性齿状核红核-苍白球路易体萎缩患者中发现了两种类型的PPR病理生理机制（波长依赖性PPR和光量依赖性PPR）。研究方法：在光敏性癫痫患者中使用特定波长滤光片进行间歇性光刺激：6例患者患有婴儿期严重肌阵挛性癫痫（SMEI），8例患有定位相关性癫痫（LRE），7例患有症状性全身性癫痫（SGE）。结果：6例光敏性SMEI患者中有4例有光量依赖性PPR。8例光敏性LRE患者中有5例具有波长依赖性PPR。7例光敏性SGE患者中有4例具有波长依赖性PPR，2例具有光量依赖性PPR。结论：低亮度光刺激诱发PPR的病理生理机制类型与癫痫综合征的分类密切相关。
Objective: To identify the pathophysiologic mechanisms of the photoparoxysmal response (PPR) in various photosensitive epilepsy syndromes, and to discuss the relation between these pathophysiologic mechanisms and the classification of epilepsies and epileptic syndromes. Background: The authors found two types of pathophysiologic mechanisms of PPRs (wavelength-dependent PPRs and quantity-of-light–dependent PPRs) in patients with idiopathic generalized epilepsy and hereditary dentatorubral-pallidoluysian atrophy. Methods: Intermittent photic stimulation with wavelength-specific optical filters was performed in photosensitive epileptic patients: six patients had severe myoclonic epilepsy in infancy (SMEI), eight had localization-related epilepsy (LRE), and seven had symptomatic generalized epilepsy (SGE). Results: Four of the six photosensitive SMEI patients had quantity-of-light–dependent PPRs. Five of the eight photosensitive LRE patients had wavelength-dependent PPRs. Four of the seven photosensitive SGE patients had wavelength-dependent PPRs, and two had quantity-of-light–dependent PPRs. Conclusions: The type of pathophysiologic mechanism for eliciting PPRs by low-luminance photic stimulation was closely related to the classification of the epilepsy syndrome.