Abstract
Objective Infantile Spasms (IS) are an age-specific epilepsy syndrome associated with poor outcome. Sustained and early spasm-control remains the main goal of therapy. We aimed to evaluate a unique pulsatile dexamethasone therapy regime in children with IS, applying a prolonged first pulse in cases of insufficient spasm-control. Methods Children with IS were treated with oral pulsatile-applied dexamethasone in the Children's Hospital Jena between 2002-2021, regardless of duration since IS onset or previous therapy (except ACTH). We analyzed spasm-reduction, electroencephalographic response, adverse reactions, neurodevelopmental status and epileptic disorders at last follow-up. Results Included were 26 patients with median age of 5.5 months (IQR 4-8) at IS onset and mean follow-up of 6.2 years (SD 3.99). 50% had an unknown etiology. Patients received on average 10.8 pulses (SD 6.0). 69.2% achieved initial seizure-freedom, however 38.9% relapsed. 17 patients had an initial prolonged pulse, of those, 14 got initially seizure-free (82.4%). 64% of the cases had a sustained spasm-cessation after the third pulse. At last follow-up, half the patients had no persisting epileptic disorder; 22.2% had a favourable neurocognitive development. Patients with unknown etiology were more likely to achieve seizure-freedom during therapy (P=0.025), had a more favourable neurocognitive outcome (P=0.049) and were less likely to suffer from epileptic disorders (P=0.037). No serious adverse effects were observed.
Published Version
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