Department of Neurology, Children's Healthcare of Atlanta, Atlanta, GA, USA.
Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Neurocase. 2023 Feb-Apr;29(1):14-17. doi: 10.1080/13554794.2023.2199936. Epub 2023 Apr 6.
The piriform cortex (PC) is part of the olfactory system, principally receiving input from the lateral olfactory tract and projecting to downstream components of the olfactory network, including the amygdala. Based on preclinical studies, PC is vulnerable to injury and can be easily kindled as an onset site for seizures. While the role of PC in human epilepsy has been studied indirectly and the subject of speculation, cases of demonstrated PC seizure onset from direct intracranial recording are rare. We present a pediatric patient with drug-resistant focal reflex epilepsy and right mesial temporal sclerosis with habitual seizures triggered by coconut aroma. The patient underwent stereoelectroencephalography with implantation of olfactory cortices including PC, through which we identified PC seizure onset, mapped high-frequency activity associated with presentation of olfactory stimuli and performance on cognitive tasks, and reproduced habitual seizures via cortical stimulation of PC. Coconut odor did not trigger seizures in our work with the patient. Surgical workup resulted in resection of the patient's right amygdala, PC, and mesial temporal pole, following which she has been seizure free for 20 months without functional decline in cognition or smell. Histological findings from resected tissue showed astrogliosis and subpial gliosis.
梨状皮层(PC)是嗅觉系统的一部分,主要接收来自外侧嗅束的输入,并投射到嗅觉网络的下游成分,包括杏仁核。基于临床前研究,PC 容易受到损伤,并可轻易被点燃作为癫痫发作的起始部位。虽然 PC 在人类癫痫中的作用已被间接研究,并成为推测的主题,但从直接颅内记录中证明 PC 起始发作的病例很少见。我们报告了一名患有耐药性局灶性反射性癫痫的儿科患者,其右侧内侧颞叶硬化,伴有椰子气味触发的习惯性发作。该患者接受了立体脑电图检查,包括 PC 的嗅觉皮层植入,通过该检查我们确定了 PC 起始发作,绘制了与嗅觉刺激呈现和认知任务表现相关的高频活动图,并通过 PC 的皮层刺激再现了习惯性发作。在我们与患者的合作中,椰子气味并未引发癫痫发作。手术检查结果导致患者右侧杏仁核、PC 和内侧颞极被切除,此后她已经无癫痫发作 20 个月,认知或嗅觉功能没有下降。切除组织的组织学发现显示星形胶质细胞增生和软脑膜下胶质增生。