SEIZURE SEMIOLOGY, NEUROIMAGING PROFILE AND INTELLECTUAL OUTCOME IN CHILDREN WITH TUBEROUS SCLEROSIS COMPLEX

Objectives: To determine whether seizure prototype, interictal electroencephalograms (EEGs) and tubers burden as seen with magnetic resonance imaging (MRI) are predictive of degree of seizure control and intellectual outcome in children with tuberous sclerosis complex (TSC).

Methods: Setting: The study was conducted on a clinic-based population of patients (n=23) with TSC during the period from July 2003 until September 2005. Patients: The TSC patients must have seizures that started at least 12 months before the date of recruitment. Methods: The patients' seizures pattern, EEGS and MRI images were reviewed and analyzed. All the patients were subjected to an age dependent battery of psychometric tests.

Results: The patients' ages ranged from 1.3 years to 6.2 years with a mean of 2.8±4.2 years. Seizures were infantile spasms in 12 patients (52.2%) and partial seizures in 11 (47.8%) with secondary generalization in 9/11 (81.8%). Interictal EEGS showed modified hypsarrhythmia in 10 patients (43.5%) and multiple independent foci in 13 (56.5%). The commonest features in MRI were the presence of subependymal nodules <1mm in all patients and=3 cortical tubers in 15 patients (65.8%). All patients used antiepileptic drugs associations; at least 2 drugs per therapeutic scheme, each one of the patients have used at least two different schemes. Seizures were stopped in 11 patients (47.9%), reduced=50% in 7 (30.4%), and remained intractable in 5 (21.7%). Mental retardation was mild in 11 (47.8%), moderate to severe in 7 (30.5%) and absent in 5 (21.7%). Abnormal behaviors were in the form of autism in 7 (30.5%) and hyperkinesis in 3 (13%). Poor seizure outcome was significantly related to younger age of onset of seizures, cortical tubers number=3, associated cortical malformations and positive consanguinity. Poor intellectual outcome was significantly related to seizure medical intractability, initial encephalopathic EEG pattern, and cortical tubers number=3.

Conclusion: The significant correlations in this work will facilitate the proper choice of therapeutic modalities and selection of candidates for early intervention programs.