Abstracts

Rationale: Infantile spasm (IS) is a major form of severe epileptic encephalopathy of early infancy. AAN practice parameters (2004) states that ACTH is probably effective for the short term treatment of IS and resolution of hypsarrhythmia(HA). There is insufficient evidence with regards to the effectiveness of oral steroids. The U.S. consensus report (2010) suggests the high dose prednisolone (PZ) therapy to be suboptimal due to insufficient data. However it recommended further study as it appeared promising. The multicenter randomized open label UKISS study compared hormonal treatment (IM synthetic ACTH versus oral PZ) with vigabatrin for the treatment of IS. This study revealed that oral PZ was equivalent to synthetic ACTH. Kossoff et al reported good outcome on 15 patients treated with PZ and recommended routine use of PZ for IS. We report 5 cases of IS treated at our institution. Methods: 5 infants were treated with high dose PZ. 4 out of 5 infants had symptomatic IS (2 had Down's syndrome), and one had cryptogenic IS. The starting dose for all infants was 40 mg per day .The dose of PZ was increased to 60 mg per day when response was subopitmal. Age at time of diagnosis ranged from 3 to10 months. Results: All 5 infants showed cessation of clinical spasms during therapy, while 3 had recurrence after therapy. PZ was discontinued due to reported lethargy in one patient. The duration of PZ treatment ranged from 3 to 8 weeks.Two patients among five required escalation of PZ dose to 60 mg/day for non-resolution of clinical spasms. The remaining 3 patients who received 40 mg/day of PZ although showed cessation of spasm by the second week of treatment, either had recurrence of IS or showed HA following treatment. Two patients on 60 mg PZ remained spasm free, one achieved complete remission of IS with cessation of both clinical spasm and HA .The second patient had abnormal EEG with sharp waves but remained free of spasms. Side effects like lethargy, irritability and increased appetite was observed in 2 infants.One infant developed hypertension and cushingoid features and required steroid taper for HPA axis suppression. Among the 4 infants who completed PZ therapy, EEG normalized in one, revealed persistent multi-regional sharp waves in 2 and HA in 1. Conclusions: Our study showed that PZ appeared effective mostly at 60 mg/day and overall well tolerated for the treatment of IS. In our patient population, initial dose of 40 mg/day either had no resolution of IS mandating higher dose or had recurrence later (clinical or EEG) after treatment completion. Side effects was seen in some patients and were consistent with effects of steroid .The outcome of this study tends to support use of PZ for IS however was limited by sample size. This warrants a prospective randomized multi center trial comparing both clinical and EEG response of ACTH vs high dose PZ.