Abstracts

Rationale: ACTH and vigabatrin (VGB) are recommended for the treatment of infantile spasms (IS) and have historically been shown to be more effective than non-standard therapies. There is variability amongst child neurologists regarding optimal treatment for IS. Guidelines for treatment of IS have been established since 2004, but it is unclear how often they are actually followed. We evaluated how often children received treatment consistent with published guidelines as well as outcome differences between those treated per guidelines and those who were not.Methods: Children diagnosed with IS between January 2007 and December 2012 at Children's Hospital of Colorado were retrospectively reviewed. Patients who did not receive their care in its entirety at Children's Hospital of Colorado were excluded from the study. Primary outcome measures were resolution of spasms and hypsarhythmia on EEG based upon last encounter within a 6 week period. If a new medication was started, the prior medication was considered a non- response. Variables collected included EEG data, etiology, medication used including dosing, date of spasms onset, general developmental status before and after treatment, brain imaging, and genetic/metabolic evaluations if applicable. Medication was considered “standard of care” if ACTH dose was 150 u/m2 or VGB was 100-150 mg/kg/day. Chi square and Fisher’s exact test were calculated.Results: 39 children were analyzed between the ages of 2-23 months: 56% of patients were male: 16/39 (41%) had an identified structural lesion of the brain, 15/39 (38%) had an identified genetic abnormality, only 1 child had an identified metabolic disorder (3%), and 7/39 (18%) had no clearly identified etiology. 30/39 (77%) of patients had hypsarhythmia prior to treatment initiation. 21/39 (54%) received standard of care and 18/39 (46%) received non-standard care including anti-seizure medications, ketogenic diet, and oral steroids. 40% of children treated with standard of care had resolution of clinical spasms and 75% had resolution of hypsarhythmia (amongst those with hypsarhythmia prior to treatment). 41% of children treated with non-standard care had clinical resolution of spasms and 85% had resolution of hypsarhythmia. There were no statistical differences in outcomes between the two groups for either clinical resolution of IS (p = 0.98) or resolution of hypsarhythmia (p = 0.66). Overall 41% of children had resolution of clinical spasms and 80% of children with hypsarhythmia had resolution. Children without hypsarhythmia before treatment were unlikely to develop hypsarhythmia regardless of treatment type.Conclusions: There was no significant difference in resolution of clinical IS or hypsarhythmia when treated with standard or nonstandard therapy in this cohort. While more children had resolution of hypsarhythmia, resolution of clinical spasms was low. More research is required to identify novel and more effective treatments for this severe epileptic encephalopathy.