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Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach. Pediatrics 2006 May;117(5):1494-502

Date

05/03/2006

Pubmed ID

16651302

DOI

10.1542/peds.2005-1206

Scopus ID

2-s2.0-33646851199 (requires institutional sign-in at Scopus site)   153 Citations

Abstract

OBJECTIVE: Tuberous sclerosis complex (TSC) is associated with medically refractory epilepsy and developmental delay in children and usually results from cortical tubers. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved when seizures are controlled at an early age. Previous reports have shown modest benefit from surgical resection of single tubers/seizure foci in older children; however, many children with TSC develop uncontrolled seizures before age 1. To identify patients who might benefit from surgery and to maximize outcome, we used a novel surgical approach in young children that consists of invasive intracranial monitoring, which is typically 3-staged and often bilateral.

METHODS: Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the past 6 years, 25 patients (9 boys and 16 girls) had TSC. At the time of their first surgery at our institution, they were a median age of 4.0 years. A total of 31 separate admissions for epilepsy surgery in these 25 patients were identified. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively, and 22 patients underwent 3-stage surgeries.

RESULTS: At 6 months or longer after the initial resection, 21 (84%) children were class I, 2 (8%) children were class II, and 2 (8%) children were class IV. At a mean follow-up of 28 months, 17 (68%) children were class I, 6 (24%) were class II, and 2 (8%) were class III. Four of the 5 children who initially were rejected as surgical candidates because of multifocality and who required initial bilateral electrode study are now seizure-free.

CONCLUSIONS: This approach can help to identify both primary and secondary epileptogenic zones in young TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery. Long-term follow-up will determine whether this approach has durable effects.

Author List

Weiner HL, Carlson C, Ridgway EB, Zaroff CM, Miles D, LaJoie J, Devinsky O

Author

Chad Carlson MD Interim Chair, Professor in the Neurology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Brain Mapping
Child
Child, Preschool
Electrodes, Implanted
Epilepsies, Partial
Female
Humans
Male
Monitoring, Physiologic
Neurosurgical Procedures
Tuberous Sclerosis