Infantile spasms in children with Down syndrome

C E Stafstrom1, R J Konkol

  • 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.

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