Challenges in the treatment of West syndrome
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2018, Vol 6, Issue 2
Abstract
West syndrome is a rare and severe infant epileptic encephalopathy, covering 25-30% of infantile epilepsies. It’s incidence is 2-3.5/ 10000 live births. The clinical picture usually starts with infantile spasms, which can occur in clusters and is often associated with developmental arrest or regression. In 75% of the cases the interictal EEG shows hypsarrhythmia, which is chaotic, high amplitude, slow wave activity. The underlying diseases can be a variety of different neurological etiologies, such as metabolic, structural developmental, perinatal lesions, genetic or neurocutan disorders. Despite the widening scale of diagnostic possibilities, detection of the underlying disease is unsuccessful in case of at least a quarter of the patients [1]. Early diagnosis and adequate therapy are key factors in achieving better outcomes [2]. Treatment of this clinical picture is still one of the unresolved questions of pediatric epileptology [3]. There is consensus on using adrenocorticotropic hormon (ACTH), steroid and vigabatrin as first line, standard therapy, but the dosage and the duration of such treatments are still a matter of debate. More recent studies recommend using combined vigabatrin and ACTH therapy, showing to achieve, in relatively short time, the highest outcome ratio of patients without spasms [4]. In our current study, we reviewed the outcomes resulting from various treatments applied at our institution and compared it with the available recommendations given in the relevant literature. Based on these findings, we have compiled a comprehensive guideline of therapy, aimed at optimizing care of patients with West syndrome.In this current study we reviewed the cases of patients treated with West syndrome in our Neurology Department between 2013- 2015, in Bethesda Children’s Hospital, Hungary. Children selected for the study were diagnosed with West syndrome showed infantile spasms and hypsarrythmia on their EEG register and later attended our care system for at least one year. Altogether 35 children were selected into the study, 16 girls and 19 boys. Patients were divided into two groups, based on the underlying disease. Symptomatic group included those patients, where the diagnostic evaluation found any abnormalities possibly responsible for the epilepsy. The idiopathic group held those cases where all available diagnostic tests gave negative results, so no underlying disease was detected. Priour to our current research, no first line treatment had been established in our hospital for West syndrome. With the personal clinical experiences and the international recommendations we usually started the treatment with vigabatrin (100-150 mg/kg, reaching the doses within 3-4 days). In resistant cases we either increased the dosage or added on nitrazepam (0,3-0,5mg/kg). If we still could not reach a spasm free state, intramuscular ACTH therapy or other types of antiepileptic drugs were given. The patients were told spasm free if they were seizure free for at least 3 months, spasms reoccurring after this period were considered as relapses.
Authors and Affiliations
Andrási Fogaras, Fruzsina Erdélyi, Zsuzsa Siegler, Márta Hegyi, Rita Jakus, Tímea Bodó, Bence Ambrus
Keywords
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- EP ID EP588469
- DOI 10.26717/BJSTR.2018.06.001335
- Views 168
- Downloads 0
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