Ryvlin Philippe, Ravier C, Bouvard S, Mauguire Franois, Le Bars D, Arzimanoglou Alexis, Petit Jérôme, Kahane Philippe
Department of Functional Neurology and Epileptology, Neurology Hospital, Lyon, France.
Epileptic Disord. 2003 Dec;5(4):219-27.
Whether the intrinsic epileptogenicity of hypothalamic hamartomas (HH) is responsible for the entire clinical spectrum of epileptic, neuropsychological and behavioural disorders associated with HH, remains an open issue, in as much as morphologically similar HH can be associated with dramatically different seizure types and cognitive outcomes. The aim of this study was to investigate brain glucose metabolism in patients with epileptogenic HH, in an attempt to identify signs of focal cortical and subcortical dysfunction which might correlate with other clinical data. We have studied five patients with epileptogenic HH using [18F]-fluoro-desoxyglucose and positron emission tomography (FDG-PET). All our patients also underwent an optimal MRI and a video-EEG monitoring, as well as an intra-cranial EEG recording in one of them. The anatomical distribution of FDG-PET abnormalities was compared to that of interictal and ictal electroclinical findings. All five patients demonstrated focal hypometabolism, ipsilateral to the predominant EEG abnormalities and side of HH. Hypometabolic areas greatly varied between patients, but were grossly concordant with the cortical regions suspected to participate in the ictal discharges in each individual. Epileptogenic hypothalamic hamartomas are usually associated with focal cortical hypometabolism in regions which might participate in the overall HH-driven epileptic network. Whether these cortical abnormalities only reflect the propagation of ictal discharges, or a potentially independent seizure onset zone remains unknown.
下丘脑错构瘤（HH）的内在致痫性是否是与HH相关的癫痫、神经心理和行为障碍的整个临床谱的病因，仍然是一个悬而未决的问题，因为形态学上相似的HH可能与截然不同的癫痫发作类型和认知结果相关。本研究的目的是调查致痫性HH患者的脑葡萄糖代谢，试图识别可能与其他临床数据相关的局灶性皮质和皮质下功能障碍的迹象。我们使用[18F]-氟脱氧葡萄糖和正电子发射断层扫描（FDG-PET）研究了5例致痫性HH患者。我们所有的患者还接受了最佳的MRI和视频脑电图监测，其中1例患者还进行了颅内脑电图记录。将FDG-PET异常的解剖分布与发作间期和发作期的电临床发现进行比较。所有5例患者均表现出与主要脑电图异常和HH侧同侧的局灶性代谢减低。患者之间代谢减低区域差异很大，但总体上与每个个体中怀疑参与发作期放电的皮质区域一致。致痫性下丘脑错构瘤通常与可能参与整个HH驱动的癫痫网络的区域的局灶性皮质代谢减低有关。这些皮质异常仅仅是反映发作期放电的传播，还是一个潜在的独立发作起始区，仍然未知。
