OTCohortDevelopmental medicine and child neurology2022

Response to treatment and outcomes of infantile spasms in Down syndrome.

Susan Harvey, Nicholas M Allen, Mary D King and 7 others

PMID 35092693

WHAT IT FOUND

In children with Down syndrome and infantile spasms, spasms stopped in 88% (46 children), but 85% of those followed up had developmental concerns and 25% had ongoing seizures.

Prednisolone as first medicine stopped spasms in 60%; vigabatrin caused many side effects.

Key findings

01Spasm resolution occurred in 88% (46 children), but 25% of children followed up had ongoing seizures and 85% had developmental concerns.

02Prednisolone was the most common first-line medication and stopped spasms in 60% (12 of 20), while sodium valproate stopped spasms in 1 of 9.

03Vigabatrin caused the most side effects, and MRI restricted diffusion occurred in 6 of 11 children imaged while prescribed vigabatrin.

STILL TO COME

How it was doneWhat they foundWhat it means for OTs

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What it does not show

This was a retrospective chart review, so treatment assignment and outcome recording were not controlled. Medication choice, dose, and timing varied by centre and clinician, so response rates may reflect local practice rather than the medication itself. Developmental outcomes were subjective clinician reports, not standardized tests, and children with Down syndrome already have variable development. Only six children received first-line prednisolone plus vigabatrin, so the 83% response rate is too small to guide treatment. Fifty-four children met the criteria, but long-term outcome percentages are based on 52 children with follow-up because two died unrelated to spasms. Neuroimaging was not standardized and was performed in 78% (42 children), so imaging findings may not apply to all children. The study reported outcomes in one group of children with Down syndrome and infantile spasms, so it cannot show how much spasms or treatment changed development.

The easy way to misread this

Do not read the 83% spasm cessation after first-line prednisolone and vigabatrin as evidence the combination should be preferred. Only six children received it, and three had side effects, so the result is too small to guide treatment.

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