Myoclonic astatic epilepsy (Doose-Syndrome) a Lamotrigine Responsive Epilepsy?
Abstract number :
3.232
Submission category :
4. Clinical Epilepsy
Year :
2011
Submission ID :
15298
Source :
www.aesnet.org
Presentation date :
12/2/2011 12:00:00 AM
Published date :
Oct 4, 2011, 07:57 AM
Authors :
R. Boor, C. Doege, T. May, S. von Spiczak, U. Stephani,
Rationale: Myoclonic astatic epilepsy (MAE) is a difficult to treat idiopathic epilepsy of early childhood. MAE frequently shows the course of an epileptic encephalopathy, which may result in permanent cognitive impairment. Therefore systematic analyses on clinical effects of different AED combinations are still needed. Methods: In an exploratory retrospective study, the therapeutic effect of adjunctive lamotrigine (LTG) was analyzed in 9 patients with pharmacoresistant MAE subsequently admitted to the Northern German Epilepsy Center (NGEC) between 07/2007 and 09/2009. Documentation was performed with the electronic seizure diary Epivista , which has been routinely used in all patients from the NGEC since 7/2007. The age at seizure onset was 24 - 50 (median 32) months and the latency between seizure onset and LTG start was 1 - 30 (median 8) months. After a baseline (BL) of 8 weeks and a titration phase of 12 weeks an interventional phase (IP) of 8 weeks was defined. Digitized information on seizure frequency, medication and adverse events were extracted and evaluated in each of patient using nonparametric statistical tests. Patients had a mean of 0,7 4,7 seizures per day during baseline, and 2 - 8 (median 4) AEDs had been used before LTG was added. One boy was excluded from statistical analysis due to incomplete documentation. Results: Seizure reduction per day (2-sided Mann-Whitney-U-Test, p<0,001) and an increase of seizure free days (2-sided exact Fisher-Test, p<0,001) was found in 5 patients during the IP. These 5 patients were free of seizures during the IP. No significant change was found in 2 patients. However, seizures per day increased and seizure free days decreased during the IP in the girl. A dose relationship between LTG and seizure counts per day was found in the other 7 patients (2-sided Jonckheere-Terpstra test). Conclusions: Our data provide evidence that adjunctive lamotrigine may be effective in pharmacoresistant MAE. Prospective studies may test the hypothesis whether MAE may be a LTG responsive epilepsy.
Clinical Epilepsy