Serial EEG study of patients affected with SLE

Abstract

Holoprosencephaly (HPE) is a complex congenital brain malformation characterized by the failure of the forebrain to bifurcate into two hemispheres, a process normally complete by the fifth week of gestation. HPE is the most common developmental defect of the forebrain and midface in humans and occurs in 1 in 250 pregnancies. HPE has traditionally been classified according to DeMyer's division into three grades of severity: alobar, semilobar, and lobar. These classifications have been based on neuropathological series, but neuroimaging series have more recently contributed to the understanding of the neuroanatomical abnormalities in HPE. In addition to these classic forms, there is another milder subtype of HPE, called “middle inter-hemispheric variant (MIH)” or “syntelencephaly.” Advances in neuroimaging, genetics, and neuropathology have dramatically improved the understanding of the etiology and pathogenesis of HPE. Careful assessment of each affected individual and neuroimaging studies are essential when dealing with the cases of brain malformations such as HPE.