PURPOSE: Myoclonic astatic epilepsy (MAE, Doose syndrome) is a difficult to 
treat idiopathic generalized epilepsy of early childhood. MAE frequently shows 
the course of an epileptic encephalopathy and may result in permanent cognitive 
impairment. Systematic analyses on clinical effects of different AED 
combinations are still needed. The purpose of our study was to analyze the 
therapeutic effect of adjunctive lamotrigine (LTG) in pharmacoresistant MAE 
patients.
PATIENTS AND METHODS: In an exploratory, retrospective study, 10 
pharmacoresistant MAE patients were included who had been admitted to the 
Northern German Epilepsy Center between 07/2007 and 12/2010 and had been treated 
with LTG. Documentation was performed with the electronic seizure diary 
Epivista. A total observation period of 32 weeks was defined: 8-week 'pre LTG 
treatment phase' (before starting with LTG), 16-week 'titration phase' (starting 
with very low LTG doses), 8-week 'follow-up phase'. Seizure frequency, 
medication and adverse events were extracted from the electronic diary and 
evaluated in each particular patient. The individual reduction of seizure 
frequency per day was defined as primary outcome variable. Additionally, a 
dose-effect-relationship was analyzed for each patient.
RESULTS: Six out of ten patients were seizure free during the follow-up phase. 
Statistical analysis indicated a significant seizure reduction in seven patients 
at follow-up compared to the pre LTG treatment phase. Seizure frequency did not 
significantly decrease in two patients and increased in one patient. A 
significant relationship between seizure frequency per day and LTG dosage during 
titration and follow-up phase could be demonstrated in nine patients. Group 
statistics using the exact Wilcoxon test revealed a significant reduction in 
seizure frequency (p = 0.049, two-sided).
CONCLUSION: Our data provide evidence that adjunctive LTG is an eligible 
therapeutic option for the treatment of pharmacoresistant MAE and encourage 
further prospective studies to verify this observation.