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Response to treatment and outcomes of infantile spasms in Down syndrome.
Harvey, Susan Allen, Nicholas M King, Mary D Lynch, Bryan Lynch, Sally A O'Regan, Mary O'Rourke, Declan Shahwan, Amre Webb, David Gorman, Kathleen M
Harvey, Susan
Allen, Nicholas M
King, Mary D
Lynch, Bryan
Lynch, Sally A
O'Regan, Mary
O'Rourke, Declan
Shahwan, Amre
Webb, David
Gorman, Kathleen M
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Date
2022-01-29
Date Submitted
Keywords
DOWN SYNDROME
CHILDREN
SPASMS
CHILDREN
SPASMS
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Start Date
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Principal Investigators
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DMCN-64-780.pdf
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Abstract
Aim: To estimate the prevalence, and evaluate presentation, treatment response, treatment side effects, and long-term seizure outcomes in all known cases of children with Down syndrome and infantile spasms on the island of Ireland.
Method: This was a 10-year retrospective multicentre review of clinical records and investigations, focusing on treatment response, side effects, and long-term outcomes.
Results: The prevalence of infantile spasms in Down syndrome was 3.0% during the study period. Fifty-four infants were identified with median age of spasm onset at 201 days (interquartile range [IQR] 156-242). Spasm cessation was achieved in 88% (n=46) at a median of 110 days (IQR 5-66). The most common first-line medications were prednisolone (n=20, 37%), vigabatrin (n=18, 33.3%), and sodium valproate (n=9, 16.7%). At follow-up (median age 23.7mo; IQR 13.4-40.6), 25% had ongoing seizures and 85% had developmental concerns. Treatment within 60 days did not correlate with spasm cessation. Seventeen children (31%) experienced medication side effects, with vigabatrin accounting for 52%.
Interpretation: Prednisolone is an effective and well-tolerated medication for treating infantile spasms in Down syndrome. Despite the high percentage of spasm cessation, developmental concerns and ongoing seizures were common.
Language
en
Citation
ISSN
eISSN
1469-8749
ISBN
DOI
10.1111/dmcn.15153
PMID
35092693
PMCID
PMC9303415