Department of Neurology, The Royal Children's Hospital, 50 Flemington Road, Parkville, Victoria, 3052, Australia; Developmental Brain Imaging and Neuroscience Research Groups, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, Victoria, 3052, Australia.
Developmental Brain Imaging and Neuroscience Research Groups, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, Victoria, 3052, Australia.
Epilepsy Res. 2020 Mar;161:106288. doi: 10.1016/j.eplepsyres.2020.106288. Epub 2020 Feb 10.
Atypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism on PET in three children with atypical BRE and investigated the spatial and temporal relationship with their centrotemporal spikes.
EEG, MRI and PET were performed clinically in three children with atypical BRE. The frequency and source localization of centrotemporal spikes was determined and compared with the location of maximal metabolic activity on PET.
Cortical hypermetabolism on thresholded PET t-maps and current density reconstructions of centrotemporal spikes overlapped in each child, in the central sulcus region, the distances between the "centers of maxima" being 2 cm or less. Hypermetabolism was not due to recent seizures or frequent centrotemporal spikes at the time of FDG uptake.
The findings suggest that localized, increased cortical activity, in the region of the EEG focus, underlies the negative clinical manifestations of atypical BRE. Similar findings are reported in the broader group of epileptic encephalopathies associated with electrical status epilepticus in sleep.
非典型良性罗兰多癫痫（BRE）是一种常见癫痫综合征中认识不足且了解甚少的表现。大多数人认为其为局灶性癫痫性脑病，其中频繁的、间发性的中央颞区棘波导致负性运动发作，并干扰运动能力，有时还会影响言语和认知能力。我们在 3 例非典型 BRE 患儿中观察到 PET 上的局灶性皮质代谢亢进，并研究了其与中央颞区棘波的空间和时间关系。
对 3 例非典型 BRE 患儿进行临床 EEG、MRI 和 PET 检查。确定中央颞区棘波的频率和源定位，并与 PET 上最大代谢活性的位置进行比较。
在每个患儿中，皮质代谢亢进的阈值 PET t 图和中央颞区棘波的电流密度重建结果相重叠，在中央沟区域，“最大值中心”之间的距离为 2cm 或更小。代谢亢进不是由于最近的癫痫发作或在 FDG 摄取时中央颞区棘波频繁发生所致。
这些发现表明，在 EEG 焦点区域，局部皮质活动增加是导致非典型 BRE 阴性临床表现的基础。在与睡眠中电癫痫持续状态相关的更广泛的癫痫性脑病组中也有类似的发现。
