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Cognitive deficits after cryptogenic infantile spasms with benign seizure evolution
E Gaily1, K Appelqvist, E Kantola-Sorsa
1Helsinki University Central Hospital, Hospital for Children and Adolescents, Neurology, Finland. eija.gaily@huch.fi
Insights
Early treatment of infantile spasms in children can lead to positive outcomes. However, some children may still experience cognitive deficits later in life, highlighting the need for ongoing monitoring.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Clinical Pediatrics
Background:
- Infantile spasms are a severe form of epilepsy in infants.
- Cryptogenic infantile spasms have no identifiable cause.
- Long-term cognitive outcomes for these children require further investigation.
Purpose of the Study:
- To assess the risk of cognitive impairment in children with cryptogenic infantile spasms.
- To evaluate the relationship between early developmental status and later cognitive function.
- To analyze the impact of spasm resolution and EEG normalization on cognitive outcomes.
Main Methods:
- Study included 15 children with cryptogenic infantile spasms whose condition resolved within the first year.
- Children received treatments such as adrenocorticotrophic hormone, pyridoxine, vigabatrin, or sodium valproate.
- Neuropsychological assessments were conducted between ages 4.0 and 5.9 years, with early development assessed at presentation and post-spasm resolution.
Main Results:
- Twelve out of 15 children achieved normal intelligence.
- Five children exhibited specific cognitive deficits, and three had mild learning disabilities.
- Abnormal developmental status at 8-15 months post-spasm resolution correlated with later cognitive deficits.
Conclusions:
- Complete resolution of infantile spasms and EEG normalization do not guarantee normal cognitive development.
- Early developmental assessment after spasm resolution is crucial for identifying children at risk of cognitive impairment.
- Further research is needed to understand the factors contributing to cognitive deficits in this population.
Abstract:
Between 1989 and 1994, 18 children with cryptogenic infantile spasms-defined by normal development before onset of spasms, symmetrical hypsarrhythmia or multifocal spikes, and typical spasms on presentation, and no abnormal findings on aetiological studies including neuroradiology-were diagnosed and treated. To assess the risk of cognitive impairment later in life, 15 of these 18 children whose spasms completely resolved within the first year of life were studied. Age at onset of spasms varied between 4.4 and 9.8 months (mean 6.5 months). Children were effectively treated with adrenocorticotrophic hormone (10 children), pyridoxine (three), vigabatrin (one), or sodium valproate (one). Spasms lasted between 11 and 138 days (mean 50 days) and stopped between the age of 6.3 and 10.2 months(mean 8.1 months). EEGs normalized between the age of 7.1 and 13.2 months (mean 9.4 months). Early development was assessed on presentation and within a few months after spasms had stopped. A detailed neuropsychological assessment was performed between the age of 4.0 and 5.9 years. Twelve children had normal intelligence; specific cognitive deficits were found in five. Three children had mild learning disability. Abnormal developmental status at age 8 to 15 months after complete resolution of spasms and EEG abnormalities was associated with cognitive deficits at age 4 to 6 years.