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Infantile spasms in children with Down syndrome
1Department of Pediatrics, New England Medical Center Hospital, Boston, MA 02111.
Developmental Medicine and Child Neurology
|July 1, 1994
Summary
Children with Down syndrome experiencing infantile spasms show a better prognosis for seizure control compared to the general population. However, developmental outcomes are poorer for those with hypoxic insults or developmental regression.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Infantile spasms are a severe epilepsy syndrome in infants.
- Down syndrome is associated with an increased risk of epilepsy.
- The prognosis of infantile spasms in the general population is often poor.
Purpose of the Study:
- To analyze the etiology, EEG findings, treatment response, development, and prognosis of infantile spasms in children with Down syndrome.
- To compare the outcomes of infantile spasms in children with Down syndrome to those in the general population.
Main Methods:
- Retrospective identification of 17 children with Down syndrome and infantile spasms.
- Analysis of etiological factors, electroencephalogram (EEG) findings, medication response, developmental status, and long-term prognosis.
Main Results:
- 16 survivors had a relatively benign outcome regarding seizure control, with only 3 still experiencing seizures and 7 on anticonvulsants.
- Poorest developmental outcomes were observed in children with superimposed hypoxic insults or developmental regression.
- Neurological prognosis for this group appears better than for infantile spasms in the general population.
Conclusions:
- Infantile spasms in children with Down syndrome have a generally better seizure control prognosis than in the general population.
- Developmental trajectory is significantly impacted by hypoxic insults and regression at spasm onset.
- The neurological prognosis for children with Down syndrome and infantile spasms is relatively favorable.