A COMPARATIVE ANALYSIS THERAPY FOR WEST SYNDROME: A STUDY OF 35 CHILDREN

Abstract

Objective: comparative evaluation of the effectiveness of hormonal, non-hormonal and combination therapy of West syndrome. 
 Material and methods: a study was conducted of 35 patients aged 3 months to 1.5 years, 23 boys, 12 girls with a primary diagnosed and verified diagnosis of "West syndrome" who had not received any treatment before, as well as with a previously diagnosed West syndrome and resistance to therapy. All patients underwent: EEGvideomonitoring, magnetic resonance imaging (MRI) of the brain, tandem mass-spectrometry, according to indications, DNA sequencing by clinical sequencing (panel "Inherited epilepsies"), chromosomal micromatrix analysis. Tetracosactide or dexamethasone were used as hormone therapy. Non-hormonal therapy was performed with antiepileptic drugs (AED): vigabatrin, topiramate and valproate in mono- or duotherapy regimens. Combination therapy included a combination of tetracosactide or dexamethasone with one of these AED. 
 Research results: the positive result of the therapy was: 1) persistent suppression of hypsarrhythmia on an electroencephalogram (EEG); 2) relief of epileptic spasms and the absence of their recurrence for at least 1 year; 3) restoration, to one degree or another, of normal psychomotor development. Positive results of therapy were obtained in 65,7% of cases. Obtained data showed that starting tetracosactide monotherapy is more effective than starting vigabatrin monotherapy, and combination therapy with tetracosactide and vigabatrin is more effective than starting tetracosactide monotherapy. Combination therapy with tetracosactide and AED showed greater efficacy compared to the combination of dexamethasone and AED: 68,4% and 37,5%, respectively. Of the 65,7% positive treatment results, 45,7% were obtained on combination therapy. 
 Conclusions: the most effective treatment option for West syndrome is a combination of tetracosactide and vigabatrin. It is preferable to start with tetracosactide monotherapy (with the exception of patients with tuberous sclerosis), but if hypsarrhythmia and/or epileptic spasms persist, 2-4 weeks after the start of treatment, it is necessary to switch to combination therapy with tetracosactide and vigabatrin.