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Predictors of outcome among 31 children with infantile spasms syndrome
Ana Kvernadze1, Nana Tatishvili2, Giorgi Lomidze3
1David Tvildiani Medical University, Tbilisi, Georgia.
Insights
Early infantile spasms (IS) onset before seven months, pre-existing developmental delay, and MRI abnormalities predict poor neurodevelopmental outcomes in children. Early intervention is crucial for improving development in infantile spasms syndrome.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Epileptology
Background:
- Infantile spasms syndrome (ISS) is a severe epileptic encephalopathy often associated with neurodevelopmental delay.
- Managing ISS is challenging due to pharmacoresistant epilepsy and seizure relapses.
- Predicting neurodevelopmental outcomes in affected children is critical for timely intervention.
Purpose of the Study:
- To identify predictors of neurodevelopmental outcomes in patients diagnosed with infantile spasms syndrome.
- To establish factors that correlate with developmental trajectories in infants with ISS.
- To guide clinical management and parental counseling regarding prognosis in ISS.
Main Methods:
- Prospective evaluation of 31 patients with infantile spasms from 2014-2017.
- Assessment included demographic data, video-electroencephalography (EEG), brain magnetic resonance imaging (MRI), and neurodevelopmental evaluations (ASQ).
- Parental diaries tracked spasms, and children were followed for one and two years post-assessment.
Main Results:
- Neurodevelopmental deterioration observed in 61.1% (second) and 53% (third) evaluations for infants with spasm onset before seven months.
- Low scores on the Ages and Stages Questionnaire (ASQ) communication domain were noted in patients with structural brain abnormalities.
- Eleven patients with pre-existing developmental delay showed significant developmental regression (p=0.01).
Conclusions:
- Early infantile spasms onset (before seven months), pre-existing developmental delay, low ASQ scores, and structural MRI abnormalities are significant predictors of poor developmental outcomes.
- These findings underscore the importance of early clinical evaluation and prognosis communication to parents.
- Prompt initiation of interventions is recommended to improve developmental trajectories in infants with ISS.
Objective:
Infantile spasms syndrome is a severe epileptic encephalopathy. Management of infantile spasms remains challenging because of pharmacoresistant forms and relapsing seizures. A high number of patients with this syndrome have neurodevelopmental delay. The main objective of our study was to determine predictors to measure the neurodevelopmental outcome of patients with infantile spasms.
Methods:
We prospectively evaluated 31 patients with infantile spasms from 2014 to 2017 at three hospitals in Tbilisi, Georgia. Various demographic data were evaluated at the first visit; video-EEG, brain MRI and neurodevelopmental evaluation were performed upon admission. A diary to record spasms was provided and completed by all parents/caregivers. Seizures were recorded on video and the phenomenology of infantile spasms was studied. Children were followed for one and two years after the first assessment.
Results:
Neurodevelopmental deterioration was revealed in 61.1% on the second and 53% on the third evaluation in patients with onset of spasms before seven months of age. The mean score on the ASQ communication domain was low among structural cases. Eleven patients with pre-existing delay had developmental regression based on the second evaluation (Fisher's exact test: 7.2; df 1; p=0.01).
Significance:
Our study reveals that age at onset of infantile spasms at less than seven months, pre-existing developmental delay, low ASQ scores and structural abnormalities on MRI are predictors of poor developmental outcome. Our data suggest that clinicians should inform parents at the first clinical evaluation about prognosis, and intervention should be started as early as possible in order to improve development.
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