Abstracts

Rationale: Infantile spasms (IS) constitute an age dependent epileptic encephalopathy with a high risk for later epilepsy and adverse developmental outcome. At present ACTH is considered first line treatment for IS in the United States (1) . In the last few years high dose oral prednisolone has also been used as first line treatment (2, 3) . Last year we presented seven cases of IS treated with high dose oral prednisolone and noted cessation of spasms in all seven. This is a report on follow-up of these cases with regards to seizure recurrence and development (as reported by parents +/- reports from community child development services). Methods: Details of the seven infants treated with high dose oral prednisolone for IS are shown in table 1. Each infant was treated for a total of four weeks, two weeks on full dose (minimum 4 mg/kg/day divided tid, to a max of 60 mg/day,) followed by a two week taper. These patients were followed with special attention to recurrence of spasms, occurrence of other seizure types, EEG, and developmental progress. Results: Short term response with cessation of spasms was seen in all patients (one patient, # 7, with early relapse became spasm free on day 2 of a second course, the rest had a single course of high dose oral prednisolone). Follow-up (f/u) ranged from 9 months to 32 months (mean 17 months). During this period there was no recurrence of IS or hypsarrhythmia on EEG, and no other seizure type occurred. Three patients are on no anti-epileptic medication (AED) and four are on one AED. All patients were seizure free at the time of last f/u. One patient (#5) died at 21 months. Cause of death unclear. She was seizure free after initial treatment. Table 2 shows follow-up details. Conclusions: This report on the follow-up of infants treated successfully with high dose oral steroids as initial treatment for IS shows no recurrence of spasms and no hypsarrythmia on EEG on follow-up ranging from 9 - 32 months. Although standardized developmental assessment was not done, parental history and developmental reports from community child development services showed developmental progress in most cases. Developmental delay was related to etiology. References: 1. Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, et al. Infantile Spasms: A U.S. consensus report. Epilepsia 2010;51(10):2175-2189. 2. Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, et al. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre randomized controlled trial. Lancet 2004;364:1773-1778. 3. Kossoff EH, Hartman AL, Rubenstein JE, Vining EP. High-dose oral prednisolone for infantile spasms: An effective and less expensive alternative to ACTH.