Abstracts

SYMPTOMATIC INFANTILE SPASMS: RESPONSE TO ANTIEPILEPTIC TREATMENT AND SHORT-TERM OUTCOME

Abstract number : 2.154
Submission category :
Year : 2005
Submission ID : 5458
Source : www.aesnet.org
Presentation date : 12/3/2005 12:00:00 AM
Published date : Dec 2, 2005, 06:00 AM

Authors :
1,2Cigdem I. Akman, 1Claudia A. Chriboga, 1Robert Freyer, 1Joshua Cappell, 1,2Ronald Emerson, and 1,2Linda Leary

To assess the response rate to the antiepileptic treatment and short-term outcome in cases with symptomatic infantile spasm (s-IS). A retrospective chart review, including EEG findings and response to treatment was performed for cases diagnosed with s-IS between 1998-2005 at Comprehensive Epilepsy Center and Division of Pediatric Neurology. Thirty-one cases diagnosed with s-IS were identified. The etiologies included genetic (n=11), infectious(n=3) and other structural or acquired causes. The mean age at onset of s-IS was 7.3 months (1month-29month) and mean follow-up period was 28.9 month. All had abnormal neurological examination and developmental delay at time of diagnosis.
Freedom from spasm was sought as response to antiepeilptic drug treatment. Patients were divided into 2 groups as responders and non-responders. Spasm control was achieved in 89% of s-IS (n:25). Mean number of AED used for the treatment was 2.5 (n:1-5) in the responder group. Response to particular AED was seen within 6 weeks following the introduction. Freedom from spasm occurred with the 1st AED in 32% (n:8/25), 2nd in 28%, 3rd in 24% and 4th in 16%. Vigabatrin (VGB) controlled the spasms in 48% (12/25), followed by ACTH 24% (6/25), and topiramate (TPM) in 20% (5/25). Patient treated with VGB had response rate of 63% (n:12 responded/19 treated), ACTH 40% (n:6/15) and TPM 20% (n:4/20).
Six patients continued to have spasms despite all treatment. This group included severe HIE due to complications of prematurity (n:4), GBS meningitis (n:1) and HSV encephalitis (n:1) as etiologies. Among patients with severe developmental delay 68% (6/9) did not respond to AEDs while all patients with mild to moderate delay achieved spasm-freedom.
Previous history of seizures were noted in 61% of s-IS (n:19/31), of which 89% (n:17/19) had focal onset seizures. Sixty percent of the patients in the responder group had history of previous seizures (n:14/25), versus 83% in the non-responder group (n: 5/6). Even among those whose spasms resolved, 24% (n 6/25) had persistence of other seizure types and 64% (16/25) continued to have epileptiform discharges. All but 2 required continued antiepileptic treatment. Spasm control was achievable in s-IS cases whose developmental delay was not severe. However, many remained at risk of other seizures. Seizures in s-IS vary in refractoriness and chronicity, for which developmental delay may be a useful marker