CNS Malformations. SCOTT KULICH, M.D., Ph.D. RAFAEL MEDINA-FLORES, M.D. RONALD L. HAMILTON, M.D. Division of Neuropathology. Neural tube defects. Anencephaly. Failure of closure of the anterior neuropore Common malformation Frog-like facies Area cerebrovasculosa
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SCOTT KULICH, M.D., Ph.D.
RAFAEL MEDINA-FLORES, M.D.
RONALD L. HAMILTON, M.D.
Division of Neuropathology
Failure of closure of the anterior neuropore
Adrenal cortical hyperplasia
Multifactorial-Folic acid supplementation
Sagittal MRI (T1) shows cerebellar tonsils 2-3 cm below foramen magnum, where the CSF space is narrow. There is no syrinx in the cervical cord & the 4th ventricle is normal size & configuration.
-Herniation of brain through skull defect
-Usually occipital, occasionally anterior (frontal) at bridge of nose (“nasal glioma”).
-Asymmetric with overlying ulceration
Wide, flat, firm gyri
Port-wine stain or nevus
Flammeus tuypical of
Sturge-Weber, occurring in
CT: Calcification of gyrus in the parietooccipital region with focal cortical atrophy & ipsilateral enlargement of the choroid plexus.