Clinical and Electrophysiologic Features of Infantile Spasm with Malformation of Cortical Development
Abstract number :
1.155
Submission category :
Year :
2001
Submission ID :
927
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
J.C. Park, MD, Dept. of Pediatrics, Inje University, Sang-gye Paik Hospital, Nowon Gu, Seoul, Korea; H.D. Kim, MD, PhD, Dept. of Pediatrics, Inje University, Sang-gye Paik Hospital, Nowon Gu, Seoul, Korea
RATIONALE: Infantile spasm (IS) with malformation of cortical development (MCD) can have characteristic clinical and EEG features than other types of IS, which can provide better identification and more successful treatment in this group.
METHODS: We studied clinical features, EEG, brain MRI, PET, and SPECT, and observed clinical courses, response to treatment, and outcome in 17 patients of IS with MCD.
RESULTS: 1. Six(35.3%) patients started seizures before 3 months of age, 7 patients between 3 and 12 months, and 4 cases started seizure after 1 year of age.
2. Unilateral focal MCD[ssquote]s were seen in 13 cases, bilateral focal MCD[ssquote]s in 2 patients, each of unilateral and bilateral diffuse MCD[ssquote]s are noted 1 case.
3. Cortical dysplasias were noted in 16 cases, and schizencephaly was seen in 1 case.
4. Partial seizures were associated in 6 cases prior to and/or later after IS.
5. EEG was most sensitive diagnostic study for determining the location of MCD[ssquote]s than MRI, SPECT, and PET, by showing asymmetric hypsarrhythmia, localized paroxysmal fast activity, spindle shaped fast activity, localized persistent polymorphic delta waves, and isolated focal seizures in some cases.
6. Sixteen (94.1%) cases were refractory to AED[ssquote]s including steroids.
7. Among 16 intractable cases, 13 cases were treated with ketogenic diet, and 9 (69.2%) cases were completely controlled.
8. Earlier seizure onset cases were more intractable even to ketogenic diet.
9. Among 3 cases of surgical resection, 2 cases became seizure free for the follow-up period from 3 months to 2 years.
CONCLUSIONS: EEG is most sensitive diagnostic tools for IS with MCD[ssquote]s, and most cases were intractable to AED[ssquote]s including steroids treatment. Ketogenic diet was successful 69.2% of intactable IS with MCD[ssquote]s even in the case of unilateral focal MCD[ssquote]s. Surgical resection was also successful in 2 cases out of 3 patients.