Infantile spasms in Newfoundland and Labrador, a population based survey over the last 13 years

Abstract

Objective: We review our experience of Infantile Spasms in Newfoundland and Labrador, Canada. This is a population based retrospective review over the last 13 years. Methods: All children with IS were identified from our own database. All charts were reviewed and information collated. Results: 36 patients were identified (17 Male/19 Female) from 2003-2016. Our incidence is 0.6/1000 live births which is higher than reported in other centers in Canada. 25 children had developmental issues prior to the onset of spasms and 28 after spasms were treated. 4 children died. 4 infants were born prematurely. 10 children had epilepsy before onset of spasms. 21 children had ongoing seizures after treatment of their spasms. 32 children were initially treated with Vigabatrin for variable periods of time; one was initially treated with Prednisone. 13 had Prednisone or ACTH as second line treatment. 5 required a repeat treatment of steroids. An etiology was identified in 19 children with CNS abnormalities being the commonest in 7 cases, genetic causes in 6, Tuberous Sclerosis in 3, Hypoxic Ischemic Encephalopathy in 3, non-accidental injury in 1, congenital CMV in 1, Low Vit B12 in 1 and neonatal stroke in 2. The majority of children developed infantile spasms in the first year of life; 12 below 6 months, 20 six months to 1 year, 3 in the second year of life and 1 child in the 3rd year of life. Conclusion: In this population based study CNS malformations were the commonest cause identified. Most children were treated with Vigabatrin. Despite controlling the IS, outcome was not good; the majority remained handicapped to some degree. Treatment of the IS may improve outcome but the underlying etiology remains a significant factor influencing development.