Metsähonkala Liisa, Gaily Eija, Valanne Leena, Blomstedt Göran
Epilepsy Unit, Department of Pediatric Neurology, Helsinki University Hospital, Helsinki, Finland.
Medical Imaging Center, Helsinki University Hospital, Helsinki, Finland.
Neuropediatrics. 2015 Aug;46(4):269-76. doi: 10.1055/s-0035-1554099. Epub 2015 Jun 9.
The purpose of the study was to evaluate the etiology and long-term outcomes of late-onset epileptic spasms (LOS).
This is a retrospective analysis of all consecutive patients seen at our center with onset of clusters of epileptic spasms between 1 and 3 years of age in 1995 through 2005.
Overall, 17 children with LOS were identified. Overall, 14 children (82%) had structural etiology. Six patients received resective surgical treatment. Five had focal cortical dysplasia type 1 (FCD1) histology (29% of all the patients). Overall, 16 children were followed for 2 to 18 years. At the latest follow-up, seizure freedom was observed in 67% of the operated and in 50% of the nonoperated patients. Normal cognition or only mild mental deficiency was observed in nine patients (56%), of whom eight were seizure-free. All patients with intractable spasms had a severe mental deficiency.
The overall cognitive outcome of LOS was more favorable than in the previous reports and was associated with seizure freedom. FCD1 is a frequent etiology for LOS and the cognitive outcome of patients with FCD1 seemed to be favorable.
本研究旨在评估晚发性癫痫性痉挛（LOS）的病因及长期预后。
这是一项对1995年至2005年间在本中心就诊的所有1至3岁起病的癫痫性痉挛丛集发作的连续患者的回顾性分析。
总体而言，共识别出17例LOS患儿。总体上，14例患儿（82%）有结构性病因。6例患者接受了切除性手术治疗。5例组织学检查为1型局灶性皮质发育不良（FCD1）（占所有患者的29%）。总体上，16例患儿随访了2至18年。在最近一次随访时，67%的手术患者和50%的非手术患者无癫痫发作。9例患者（56%）认知正常或仅有轻度智力缺陷，其中8例无癫痫发作。所有难治性痉挛患者均有严重智力缺陷。
LOS的总体认知预后比以往报告的更有利，且与无癫痫发作相关。FCD1是LOS的常见病因，FCD1患者的认知预后似乎较好。
