BACKGROUND AND PURPOSE Developmental changes in hippocampal formations (HFs) have been reported in association with agenesis of the corpus callosum, lissencephaly, and holoprosencephaly. The purpose of this study was to evaluate the developmental changes in HFs in patients with a variety of other congenital brain malformations. METHODS MR images of 44 patients with congenital brain malformati...

PURPOSE: To describe the imaging features of a brain anomaly found on studies of three patients, and to speculate on the embryologic basis leading to the development of this abnormality. PATIENTS AND METHODS: Clinical records (three patients) , MR scans (two patients). and CT scans (two patients) of three patients with fusion of the middle portions of the cerebral hemispheres in the presence of...

Holoprosencephaly is a category of congenital brain malformation that is frequently associated with epilepsy. Epileptic spasms and partial seizures are reported with a variety of electrographic ictal and interictal EEG findings. We report a case of severe alobar holoprosencephaly with cortical tissue limited to inferior-anterior-frontal areas and a thin mantle over the posterior areas, and no a...

The first three disorders discussed are abnormalities of bone: too little bone in cleidocranial dysplasia caused by mutations in RUNX2; too much bone in fibrodysplasia ossificans progressiva with overexpression of BMP4; and abnormal bone in McCune-Albright syndrome and fibrous dysplasia caused by mutations in GNAS1. Disorders of the sonic hedgehog signaling network are discussed next, including...