Last modified: 2014-04-03
Abstract
Introductión
Genetic migration cortical disorders represent an important cause of epilepsy and developmenat delay. Sucortical band heterotopic (HSB) or double cortex, is a disorders caracterized by band of grey matter within the white matter, between the cortex and ventricular space.
Purpose:
To analyze the clinical and neuroradiological features of patients whit double cortex.
Materials and méthods
A retrospective descriptive study was performed of 10 patients whit diagnosis of HSB from January 2000 until June 2013.
Results
The mean age was 22 years, the average age of onset, 3.5 years, 60% were women. 40% had focal seizures, 20% generalized seizures and 40% both. Most patients had symptomatic focal epilepsy related to localization 50 %, followed by 30 % unclassifiable and 20% Lennox Gastaut syndrome. All had refractory epilepsy and developmental delay. Eight patients with moderate developmental delay and 2 severe. In the electroencephalogram, 50 % had focal discharges, 40% both and 10% not present. The MRI showed diffuse involvement, 3 with anterior predominance and 2 with back predominance. The average number of drugs used was 6. Valproic acid was the most AED indicated (9p), followed by lamotrigine and carbamazepine (8p).
The semiology was related to the focal dominance of the double cortex. (p 0.0039)
Conclusion
We found an association between the seizure semiology and focal predominance double cortex. No significant difference was found in other clinical variables and neuroradiological subclassification.