Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome

被引:96
作者
Liukkonen, Elina [1 ]
Kantola-Sorsa, Elisa [1 ]
Paetau, Ritva [1 ]
Gaily, Eija [1 ]
Peltola, Maria [2 ]
Granstrom, Marja-Liisa [1 ]
机构
[1] Helsinki Univ Cent Hosp, Epilepsy Unit, Dept Pediat Neurol, Helsinki 00029, Finland
[2] Turku Univ Hosp, Dept Clin Neurophysiol, Turku, Finland
关键词
Epilepsy; LKS; Rolandic; Treatment; Cognitive; Follow-up; ELECTRICAL STATUS EPILEPTICUS; LANDAU-KLEFFNER-SYNDROME; CONTINUOUS SPIKE-WAVE; SLOW SLEEP; CONVULSIVE DISORDER; ROLANDIC EPILEPSY; ACQUIRED APHASIA; FOLLOW-UP; SEIZURES; CLASSIFICATION;
D O I
10.1111/j.1528-1167.2010.02578.x
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
P>Purpose: To prospectively evaluate the efficacy of drug treatment and long-term cognitive outcome in children with encephalopathy with status epilepticus during sleep (ESESS). Methods: Thirty-two children were diagnosed and prospectively followed up for at least 3 years at our unit between 1991 and 2007. Twenty-seven children were included in the prospective treatment study with valproate (VPA) and 17 with VPA combined with ethosuximide (ESM). Treatment response of disappearance of electrical status epilepticus during sleep (SES) was documented with overnight EEG recordings. Neuropsychological follow up for at least 5 years was available in 18 patients. Results: Six children had atypical rolandic (AR) epilepsy, nine Landau-Kleffner syndrome (LKS), and 17 symptomatic epilepsy. Before ESESS, 20 children were cognitively normal. Prospective treatment with VPA and ESM was effective in 3 of the 17 children (18%) treated. Abolition of SES with drug treatment was observed in 16 patients. In all, 10 children (31%), 4 with AR (67%), 3 with LKS (33%), and 3 with symptomatic etiology (19%), including 9 with treatment response regained the pre-ESESS cognitive level. Unfavorable cognitive outcome was predicted by younger age at ESESS diagnosis, lower IQ at the time of the diagnosis, and no response to drug treatment when compared with those with favorable cognitive outcome. Eight of the 16 nonresponders underwent epilepsy surgery. Discussion: Treatment response with VPA combined with ESM was observed more often than with other drug combinations. Most children with ESESS experienced permanent cognitive impairment. Cognitive outcome depends on treatment response on electroencephalography (EEG) and seizures, and on underlying etiology.
引用
收藏
页码:2023 / 2032
页数:10
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