Abstracts

Rationale: Lennox-Gastaut syndrome (LGS) is an electro-clinical syndrome composed of the triad of mental retardation, multiple seizure types, and the characteristic generalized slow spike-wave complexes in the EEG. In this article, we report on two patients with (LGS) whose brain MRI showed dysgenesis of corpus callosum (CC). We review the literature and stress on the role of (CC) in the genesis of secondary bilateral synchrony(SBS).Methods: This was a clinical and neuro-imaging study.Results: The EEG was consistent with (LGS) in patient 1 and unilateral slow spike-wave complexes in patient 2 . The MRI showed hypoplasia of the splenium of (CC) in patient 1, and global hypoplasia of (CC) combined with Joubert syndrome in patient 2.Conclusions: Based on the data, we proffer the following hypotheses : 1-Hypoplasia of (CC) interferes with functional integrity of this structure. 2-The genu of (CC) plays a pivotal role in the genesis of secondary bilateral synchrony (SBS). 3-Electrodecremental seizures in (LGS) emanate from pacemakers generated in the brain stem, in particular the mesencephalon projecting abnormal signals to the cortex via thalamic nuclei. 4-Unilateral slow spike-wave complexes in the context of mental retardation and multiple seizure types may represent a variant of (LGS), justifying neuroimaging studies.