Consensus on diagnosis and management of JME: From founder's observations to current trends

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• 作者：Dorothé ; e G.A. Kasteleijn- Nolst Trenité ; ; ^{dkasteleijn@planet.nl} ; Bettina Schmitz ; Dieter Janz ; Antonio V. Delgado-Escueta ; Pierre Thomas ; Edouard Hirsch ; Holger Lerche ; Carol Camfield ; Betul Baykan ; Martha Feucht ; Iris E. Martí ; nez-Juá ; rez ; Reyna M. Duron ; Marco T. Medina ; Guido Rubboli ; Judith Jerney ; Bruce Hermann ; Elza Yacubian ; Michael Koutroumanidis ; Ulrich Stephani ; Javier Salas-Puig ; Ronald C. Reed ; Friedrich Woermann ; Britta Wandschneider ; Michelle Bureau ; Antonio Gambardella ; Matthias J. Koepp ; Philippe Gelisse ; Cardan Gurses ; Arielle Crespel ; Vi Huong Nguyen-Michel ; Edoardo Ferlazzo ; Thierry Grisar ; Ingo Helbig ; Bobby P.C. Koeleman ; Pasquale Striano ; Michael Trimble ; Russel Buono ; Patrick Cossette ; Alfonso Represa ; Charlotte Dravet ; Anna Serafini ; Ivanka Savic- Berglund ; Sanjay M. Sisodiya ; Kazuhiro Yamakawa ; Pierre Genton
• 关键词：JME ; Criteria ; Management ; Consensus
• 刊名：Epilepsy & Behavior
• 出版年：2013
• 出版时间：July, 2013
• 年：2013
• 卷：28
• 期：supp_S1
• 页码：S87-S90
• 全文大小：135 K

文摘

An international workshop on juvenile myoclonic epilepsy (JME) was conducted in Avignon, France in May 2011. During that workshop, a group of 45 experts on JME, together with one of the founding fathers of the syndrome of JME (¡°Janz syndrome¡±), Prof. Dr. Dieter Janz from Berlin, reached a consensus on diagnostic criteria and management of JME.

The international experts on JME proposed two sets of criteria, which will be helpful for both clinical and scientific purposes.

Class I criteria encompass myoclonic jerks without loss of consciousness exclusively occurring on or after awakening and associated with typical generalized epileptiform EEG abnormalities, with an age of onset between 10 and 25. Class II criteria allow the inclusion of myoclonic jerks predominantly occurring after awakening, generalized epileptiform EEG abnormalities with or without concomitant myoclonic jerks, and a greater time window for age at onset (6-25 years).

For both sets of criteria, patients should have a clear history of myoclonic jerks predominantly occurring after awakening and an EEG with generalized epileptiform discharges supporting a diagnosis of idiopathic generalized epilepsy.

Patients with JME require special management because their epilepsy starts in the vulnerable period of adolescence and, accordingly, they have lifestyle issues that typically increase the likelihood of seizures (sleep deprivation, exposure to stroboscopic flashes in discos, alcohol intake, etc.) with poor adherence to antiepileptic drugs (AEDs).

Results of an inventory of the different clinical management strategies are given.

This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?