Cusmai R, Curatolo P, Mangano S, Cheminal R, Echenne B
Institute of Child Neuropsychiatry, Rome University La Sapienza, Italy.
Neuropediatrics. 1990 Nov;21(4):179-82. doi: 10.1055/s-2008-1071490.
Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features: no presence of seizures before one month, seizures controlled or absent, no focal neurological signs and peculiar EEG findings. In our opinion the absence of heterotopias and hamartomas can be related with less severe outcome.
半侧巨脑症以往与以难治性癫痫和严重脑病为特征的不良临床病程相关，但在两名患有神经纤维瘤病的儿童中发现并无这些情况。这些儿童表现出相对相似且良好的预后特征：1个月前无癫痫发作，癫痫发作得到控制或无发作，无局灶性神经体征以及特殊的脑电图表现。我们认为，无异位和错构瘤可能与较轻微的预后相关。
