Parmeggiani A, Posar A, Scaduto M C, Chiodo S, Santucci M, Rossi P G
Department of Child Neurology and Psychiatry, Neurological Institute, University of Bologna, Italy.
J Child Neurol. 1999 Feb;14(2):113-7. doi: 10.1177/088307389901400209.
The association between posterior fossa malformations and epilepsy is rarely reported in the literature. We describe 54 cases with posterior fossa malformations, according to embryogenesis classification, divided into two groups on the basis of presence or absence of epilepsy. Epilepsy occurred in 22 cases (40.7%) and was not related to the type of posterior fossa malformation or to supratentorial cerebral lesions associated with the malformation. Familial antecedents for epilepsy and/or febrile convulsions influenced the presence of epilepsy in patients with posterior fossa malformations (P < .01). Epilepsy was mainly partial (77.3%); benign partial/generalized epilepsies and febrile convulsions occurred in 27.3% of cases. Seizures disappeared for 2 or more years at the end of follow-up in 36.4% of patients. Good epilepsy prognosis was not related to the age at onset of seizures, familial antecedents for epilepsy and/or febrile convulsions, supratentorial associated lesions, or age of patients at the last observation. Profound mental retardation prevailed in patients with epilepsy (P < .01), as did pathologic electroencephalograms (EEG) (P < .0001), with paroxysmal abnormalities (P < .001) and asymmetry (P < .01). In our 54 cases of posterior fossa malformation, we identified two risk factors for epilepsy: familial antecedents for epilepsy and/or febrile convulsions and the involvement of the cerebellum in the malformation.
后颅窝畸形与癫痫之间的关联在文献中鲜有报道。我们描述了54例后颅窝畸形病例，根据胚胎发生分类，基于癫痫的有无分为两组。22例（40.7%）发生癫痫，且癫痫与后颅窝畸形类型或与该畸形相关的幕上脑病变无关。癫痫和/或热性惊厥的家族史影响后颅窝畸形患者癫痫的发生（P <.01）。癫痫主要为部分性发作（77.3%）；27.3%的病例出现良性部分性/全身性癫痫和热性惊厥。36.4%的患者在随访结束时癫痫发作消失达2年或更长时间。癫痫预后良好与癫痫发作起始年龄、癫痫和/或热性惊厥的家族史、幕上相关病变或最后一次观察时患者的年龄无关。癫痫患者中严重智力发育迟缓普遍存在（P <.01），病理性脑电图（EEG）也是如此（P <.0001），伴有阵发性异常（P <.001）和不对称性（P <.01）。在我们的54例后颅窝畸形病例中，我们确定了癫痫的两个危险因素：癫痫和/或热性惊厥的家族史以及小脑受累于畸形。
