Schizencephaly and agenesis of the corpus callosum (P2.108)

OBJECTIVE: To clarify the MRI protocol required for the exploration of cortical malformations.Cortical malformations are recognized as a frequent cause of drug-resistant epilepsy. BACKGROUND: Mr D, a 50 years old man, has severe epilepsy since he was 18 years old. MRI found a cortical malformation complex involving complete agenesis of the corpus callosum, cleft left parietal schizencephalic, whose walls are lined by looking pachyric cortex malformation. DESIGN/METHODS: Our patient had neurological examination, EEG and neuropsychological investigations. MRI was performed on a 1.5 T GE unit; 6 series were acquired • Sagittal SE-T1 WIs • Volumic gradient-echo T1 WIs (=3D SPGR) with reformatting in coronal axial and sagittal planes (3D-FSPGR-T1) • Coronal SE T2 WIs • Axial fluid-attenuated inversion recovery WIs (=FLAIR) • Axial diffusion (=DWI) b 1000 • Axial gradient echo WIs T2 *.Additional Control MRI was performed including fiber tracks tensor imaging RESULTS: Neurological examination of Mr D. was normal, despite a drug-resistant epilepsy. Neuropsychological explorations did not found mental retardation or cognitive impairment.MRI found a cortical complex malformation: complete agenesis of the corpus callosum with marked hypertrophy of the white commissures especially the anterior one, parietal schizencephalic slot, with signal is similar to CSF, and with rims of pachygyric thickened and irregular gray matter. Absence of any hemorrhagic event was noted on T2 * WI. Fiber track imaging confirm the predominance of anterior white commissure and absence of corpus callosum fibers. CONCLUSIONS: Cognitive and functional consequences of schizencephalic slots, assessed by neuropsychological assessment, are variable and not always completely correlated with the severity of the anatomical damage. MRI is essential for diagnostic workup. The use of 3D sequences-T1 inversion recovery provides excellent mapping of gray matter lesions. Functional MRI study of these defects should allow a better understanding of brain plasticity. Disclosure: Dr. Vlaicu has nothing to disclose. Dr. Gerber has nothing to disclose. Dr. Rodallec has nothing to disclose. Dr. Palfi has nothing to disclose.