Myoclonic-astatic epilepsy. Doose syndrome 2014

被引：3

作者：

Doege C. ^{[2
]}

Kleiss R. ^{[1
]}

Stephani U. ^{[1
,2
]}

Von Spiczak S. ^{[1
,2
]}

机构：

[1] Klinik für Neuropädiatrie, Christian-Albrechts-Universität, Kiel

[2] Norddeutsches Epilepsiezentrum für Kinder und Jugendliche, 24233 Raisdorf

来源：

Zeitschrift für Epileptologie | 2014年 / 27卷 / 2期

关键词：

Age of onset; Anticonvulsants; Electroencephalography; Obtundation state; Seizures;

D O I：

10.1007/s10309-013-0357-8

中图分类号：

学科分类号：

摘要：

Myoclonic-astatic epilepsy (MAE, Doose syndrome, epilepsy with myoclonic-atonic seizures) is a rare generalized electroclinical syndrome of early childhood usually affecting previously normally developed children with a male preponderance (approximately 70%). Drop attacks, myoclonic, myoclonic-atonic, generalized tonic-clonic seizures or short absences as well as a non-convulsive status epilepticus (obtundation state) constitute the common clinical presentation of MAE. Patterns of generalized spike and wave activity, 4-7/s rhythms as well as photoparoxysmal reaction can be observed in the electroencephalograph (EEG). In this review the clinical picture is described in detail and therapeutic strategies and prognostic criteria are delineated and discussed. © Springer-Verlag 2014.

引用

页码：105 / 111

页数：6

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