Evaluation of psycho-motor development in children with West syndrome

Abstract

Introduction. West Syndrome (WS) is age-related epileptic encephalopathy characterised by a triad of symptoms: specific seizure type, pathognomonic electroencephalographic (EEG) pattern - hypsarrhythmia and delay and/or regression in psychomotor development (PMD). Aetiologically, it occurs in three forms: symptomatic, cryptogenic and idiopathic. Objective. Estimation of PMD in children with WS according to aetiology. Methods. The observed group consisted of 65 children. Age range was between 6 and 30 months. The patients were divided into three groups according to aetiology. All patients underwent psychological examination with Brunet-Lesine test, as well as PMD evaluation based on achieved developmental milestones for the corresponding age. Results. Statistically significant better values in the Human Developmental Index (HDI) had patients with idiopathic compared to other forms of WS, at testing after 12 months (93.0±8.1 vs. 46.8±6.1 vs. 45.6±3.8), as well as after 24 months (93.9±7.7 vs. 51.9±5.5 vs. 50.9±4.4). The best values of HDI after 24 months had patients with improvement in PMD with the average of 66.2±4.4, which was statistically significant compared to those with unchanged PMD (41.5±5.3) and with further regression in PMD (28.3±4.4). Significant correlation was obtained between PMD after 12 and 24 months (r=0.477), as well as a considerable improvement in HDI from the 12th to 24th month (49.4±4.0 vs. 53.7±3.9). Conclusion. The patients with idiopathic WS accomplished the best PMD. Improvement in PMD after 12 and 24 months of treatment was associated with improved HDI. Improvement in PMD was observed in all patients after 2 years of follow-up.

Uvod. Vestov (West) sindrom (VS) je uzrasno zavisna epileptična encefalopatija koju odlikuje trojstvo simptoma: specifičan obrazac napada, tipičan elektroencefalografski (EEG) obrazac - hipsaritmija i zastoj, odnosno regresija u psihomotornom razvoju (PMR). Etiološki, javlja se u tri oblika: simptomatskom, kriptogenom i idiopatskom. Cilj rada. Cilj istraživanja bila je procena PMR dece sa VS u odnosu na etiologiju. Metode rada. Ispitivanu grupu činilo je 65 dece sa VS uzrasta 6-30 meseci. Bolesnici su svrstani u tri grupe prema etiologiji oboljenja. Svi su podvrgnuti psihološkom testiranju Brine-Lesinovim (Brunet- Ljsine) testom i proceni PMR na osnovu ostvarivanja miljokaza razvoja. Rezultati. Statistički značajno bolju vrednost indeksa razvoja (IR) ostvarili su bolesnici s idiopatskim oblikom VS, kako pri testiranju posle 12 meseci (93,0±8,1 prema 46,8±6,1 prema 45,6±3,8), tako i nakon 24 meseca kliničkog praćenja (93,9±7,7 prema 51,9±5,5 prema 50,9±4,4). Najbolje vrednosti IR posle 24 meseca postigli su bolesnici s napretkom u PMR, kod kojih je srednja vrednost ovog parametra bila 66,2±4,4, što je bilo statistički značajno u odnosu na bolesnike s nepromenjenim PMR (41,5±5,3) i regresijom u PMR (28,3±4,4). Dobijena je značajna korelacija između PMR posle godinu dana i dve godine (r=0,477), a uočeno je i značajno poboljšanje vrednosti IR između 12. i 24. meseca (49,2±4,0 prema 53,7±3,9). Zaključak. Deca s idiopatskim oblikom VS ostvaruju najbolji PMR. Napredak u PMR posle 12 meseci i 24 meseca lečenja bio je udružen sa boljim IR. Kod svih bolesnika uočen je napredak u PMR posle dve godine lečenja.