Abstracts

Rationale: The development of infantile spasms (IS) in patients with brain injuries of prematurity (periventricular leukomalacia [PVL] and/or intraventricular hemorrhage [IVH]) is a well documented association. Data regarding early treatment-based and long term outcomes is limited.Methods: A retrospective chart review identified children diagnosed with infantile spasms between 2007 and 2012 at a tertiary referral center. Children born prematurely (<37 weeks) and with comorbid periventricular leukomalacia (PVL) and/or intraventricular hemorrhage (IVH) were included. Children not receiving a majority of their treatment for IS at the institution were excluded. Demographic, diagnostic, treatment and outcome data was recorded in a REDCap database including: gestational age, gender, birth weight, history of seizures, dates of IS onset and diagnosis, EEG results, radiologic study results, treatment, dates of treatment initiation, developmental information and presence of subsequent epilepsy. PVL severity was categorized as mild, moderate or severe based on a previously published grading scale using MRI findings and the degree of extent of white matter injury. Response to treatment was defined as resolution of hypsarhythmia on EEG or resolution of clinical spasms. The medication added last was considered the successful therapy. ACTH and vigabatrin were considered standard treatments. Refractory epilepsy was defined by the use of greater than 2 seizure medications or having a diagnosis of Lennox-Gastaut Syndrome (LGS).Results: 15 children met inclusion criteria. 2 were excluded as treatment was received elsewhere, leaving 13 children. The median age at the last follow up was 7 years (IQR 4.6-7.8). Median gestational age was 30 weeks (IQR 25-33). Median birth weight was 1476 grams (IQR 808-1569). IVH was present in 9 children and 10 had PVL. PVL and IVH were present together in 7 children. 11 of 13 children had hypsarhythmia on EEG. The median age of onset was 9 months (IQR 6-9.5) and diagnosis was 9 months (IQR 7-14). 12 of 13 children had resolution of spasms. Of those who responded, the successful medication was ACTH in 7, topiramate in 3, and vigabatrin in 2. When used in succession, ACTH and vigabatrin successfully treated 9 of 10 children. Topiramate was successful in the two children who did not have hypsarythmia. No patients responded to other therapies (prednisolone, valproate, levetiracetam, zonisamide, phenobarbital, or ketogenic diet). All patients had developmental delay. 7 were non-ambulatory and 4 were non-verbal. Although 6 of 13 had subsequent epilepsy, only one child had refractory epilepsy. None had LGS.Conclusions: Standard therapies (ACTH and vigabatrin) appear to be more effective than other treatments. Topiramate was effective in the absence of hypsarhythmia. Developmental delay was ubiquitous in all children with PVL/IVH although these children were largely spared from the development of refractory epilepsy or LGS.