Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience

Abstract

BACKGROUND:West Syndrome (WS) represents as a specific epileptic encephalopathy characterised with a unique type of attacks, called infantile spasms, severe forms of abnormalities in electroencephalographic (EEG) records as a hypsarythmias and delays in the psychomotoric development. The characteristics of the disease, mostly affecting male gender, are infantile spasms and typical findings in EEG as a hypsarythmia. Infantile spasms are a consequence of many factors in the undeveloped brain.AIM:We aimed: (1) to see the incidence of the illness and the spreading out because of gender in rapport with other syndromes in the epileptic encephalopathies group; (2) to show principles of the treatment for the illness; and (3) to present the effects of the disease in the psycho-motoric development of affected children.METHODS:The study was designed as a cross-sectional study of the patients with epileptic encephalopathies, treated in Paediatric Clinic in Prishtina, from 1st of January 2013 until the 31st of December 2015.RESULTS:From the cohort group of 97 children diagnosed with epileptic encephalopathies, in 14 of them clinical and EEG signs of WS were noted. The earliest age of disease manifestation was 74 days (± 63.8 days). On the group of children with WS, 13 of them with Natrium Valpropat were treated, with the doses of 301.9 mg (± 64.1). From the cohort group, in 89 children (91.8%) psychomotoric retardation was documented, within the higher reoccurrence in the undifferentiated epileptic encephalopathies (96%) and the WS (78.6%).CONCLUSION:WS is a frequent disease of the encephalopathies with the epileptogenic framework. The resistance in anticonvulsive therapy is huge, and psychomotoric retardation follows a big percentage of children with this syndrome.

WDR45, one gene associated with multiple neurodevelopmental disorders

“…As for RLS, none of the four EOEE patients display signs of brain iron accumulation, at the time when they were tested which was at the early stage of EOEE [56,78]. c) WEST SYNDROME West syndrome is the most common subtype of EOEE with an incidence of 2-5 per 10,000 births [79], and it principally manifests at 3-12 months of age, predominantly affecting males, i.e., in 60-70% of the cases [80]. This disease represents as a specific epileptic encephalopathy characterized by unique type of attacks, called infantile spasms, hypsarrhythmia and delays in the psychomotoric development [81,82].…”