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Infantile spasms in children with Down syndrome
1Department of Pediatrics, New England Medical Center Hospital, Boston, MA 02111.
Insights
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.
Abstract:
The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones. The overall neurological prognosis for children with Down syndrome and infantile spasms appears to be better than for children with infantile spasms in the general population.
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