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Published on: January 12, 2019
[Evaluation of psycho-motor development in children with West syndrome]
Insights
Children with idiopathic West Syndrome (WS) show better psychomotor development (PMD). Improvement in PMD correlates with enhanced Human Developmental Index (HDI) scores, indicating positive outcomes in WS patients.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
Context:
- West Syndrome (WS) is an age-related epileptic encephalopathy.
- WS presents with seizures, hypsarrhythmia on EEG, and psychomotor development delay/regression.
- Etiologically, WS is classified into symptomatic, cryptogenic, and idiopathic forms.
Purpose:
- To estimate psychomotor development (PMD) in children with West Syndrome based on etiology.
- To compare PMD outcomes across different etiological subtypes of WS.
Summary:
- This study evaluated 65 children (6-30 months) with WS, categorized by etiology.
- Idiopathic WS patients demonstrated significantly higher Human Developmental Index (HDI) scores at 12 and 24 months compared to other forms.
- Improvement in PMD after treatment was associated with better HDI scores and was observed in all patients after two years.
Impact:
- Idiopathic WS is associated with superior psychomotor development outcomes.
- Early detection and intervention for WS may lead to improved developmental trajectories.
- Findings highlight the importance of etiological classification in predicting and managing PMD in West Syndrome.
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 otherforms 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.
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