Abstracts

Rationale: Infantile spasms (IS) constitute an age dependent epileptic encephalopathy with a high risk for adverse developmental outcome. At present ACTH is considered first line treatment for IS in the United States. In the last few years high dose oral prednisolone has also been used as first line treatment. There is very little in the IS literature about developmental outcome in cases treated with high dose oral steroid. This is a report on the developmental outcome of 9 infants with IS who responded to treatment with high dose oral prednisolone . Methods: Details of nine infants with IS (and hypsarrhythmia), who were treated with high dose oral prednisolone (4 6 mg/kg/day), are shown in table 1. Each infant was treated for a total of four weeks. At follow up, data regarding development was gathered and is presented here. Development was assessed by the pediatric neurologist and supplemented with report from community child developmental services. Results: Cessation of spasms in the first week of treatment was seen in all nine patients. This was accompanied by resolution of hypsarrhythmia. One patient, # 6, with early relapse needed a second course of treatment, and was spasm free after that. Table 2 shows development at presentation with IS and at follow-up. The mean duration of follow-up is 2.1 years (range 0.9 - 3.6yrs). Two patients have age appropriate development. Two have mild expressive speech delay. Motor delays are present in the two patients with perinatal stroke. One of them also has speech delay. One patient, with IS of unknown etiology, was diagnosed with autism at age 20 months. In another patient, at 19 months of age, there are concerns about language delay and social interaction. One patients has severe spastic quadriparesis related to HIE . Overall, 4 of the 9 patients have normal development or mild delay (44%). Another 3 have moderate to severe delay related to the underlying etiology (33%). Of two patient with reportedly normal development prior to onset of IS, one has autistic spectrum disorder and concerns regarding language and social skills have been raised in the other (22%). Conclusions: Developmental outcome in this series is similar to that reported in literature for IS treated with ACTH and/or vigabatrin. If larger prospective studies confirm these findings, high dose oral steroid should be considered a viable alternative to ACTH for treatment of IS.