Infantile spasms in Down syndrome: A case series

E. Freri, L. Giordano, T. Granata, A. Pozzi, S. Binelli, A. Tiberti

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Abstract

Objective To describe the outcome of infantile spasms in a series of patients affected by Down syndrome. Materials and methods 8 patients now aged from 18 months to 10 years have been prospectively followed since the onset of spasms (mean follow-up, 5.2 years). Results Pre-perinatal history was uneventful in 7 cases; one infam suffered from perinatal hypoxic insult. Symmetrical spasms appeared between 5 and 10 months (mean age, 6.5 months) and were always associated with hypsarrhythmia and mild psychomotor regression. All infants have been treated with ACTH (3-5 ILJ/kg per day for 15 days, tapered over 2 months), in three cases after an unsuccessful trial with AED. Following ACTH treatment, spasms and hypsarrhythmia promptly disappeared in 7 of 8 cases and the infants regained developmental milestones; relapse of spasms occurred only in one infant who, in addition to Down syndrome, had had a perinatal hypoxic insult. Conclusion Our data confirm the good prognosis of infantile spasms in Down syndrome and the efficacy of ACTH treatment in seizure control, EEC normalization and long-term prognosis.

Original languageEnglish
Pages (from-to)359
Number of pages1
JournalItalian Journal of Neurological Sciences
Volume20
Issue number5
Publication statusPublished - 1999

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ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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