Four cases report of Jeavons syndrome in childhood
10.3969/j.issn.1000-3606.2014.06.021
- VernacularTitle:儿童Jeavons综合征4例报告
- Author:
Zhefeng XU
;
Zhanli LIU
;
Xianmei HUANG
;
Chunming JIANG
;
Liqiong CHEN
- Publication Type:Journal Article
- Keywords:
epilepsy;
Jeavons syndrome;
eyelid myoclonia;
electroencephalography;
child
- From:
Journal of Clinical Pediatrics
2014;(6):583-586
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and thera-peutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimu-lation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.
