Structure-function correlations in patients with malformations of cortical development

Malformations of cortical development (MCD) are a diverse group of diseases that exhibit both genetic and phenotypic heterogeneity. While previous studies have described the clinical, MRI, and/or EEG features of specific malformations, structure and function have not been correlated across a wide spectrum of these disorders. We reviewed the medical records of 22 patients with MCD due to abnormal neuronal migration and cortical organization, and correlated levels of clinical disability with specific MRI and EEG findings. The extent of abnormality on MRI was significantly correlated with clinical disability. Compared with patients with other lesions, those with lissencephaly were more disabled. Although background slowing, suppression, fast activity, and interictal epileptiform activity were not individually predictive, the overall degree of EEG abnormality was significantly correlated with clinical disability, as were alterations in the posterior head rhythm and physiologic sleep features.