Abstracts

RATIONALE: CSWS/ESES and related syndromes have cognitive symptoms as predominant features, aspecific neurocognitive deterioration in some cases or language deterioration as the major sign in other cases. Seizures are usually rare and easy to treat. However, in some atypical CSWS, atypical absences can be very severe and highly refractory. Even though some of these syndromes may regress spontaneously in few patients, a fast and effective treatment has to be found to limit neurocognitive deterioration.
Because of the poor efficacy of standard treatments in CSWS/ESES, including immunomodulatory and surgical treatment, a treatment with TPM has been evaluated.
METHODS: Our cases, 7 boys with age between 4 and 12 years, could be considered as CSWS with lanuage deterioration being less severe and relatively aspecific compared to abnormalities described in Landau-Kleffner (acquired aphasia). TPM was added to the conventional medication. Subsequently co-medication was tapered-off up to TPM monotherapy were possible and evolution of IQ, EEG and seizures were followed.
RESULTS: Small doses of TPM were effective and the follow-up of the children showed that all cases - were other AEDs were ineffective or lost their efficacy after a few weeks - were improved from a clinical point of view; some mildly, others dramatically.
EEG was normalized in several cases and remained normalized after several months.
CONCLUSIONS: TPM should be tried before steroids or other immune treatments and of course before neurosurgery.
To our knowledge this is the first report of the long-term (up to more than one year) efficacy of TPM in CSWS/ESES and related syndromes. Other studies with additional cases are necessary.