CNS Malformations

CNS Malformations
By: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
Underdeveloped hypothalamus
Adrenal cortical hyperplasia
Multifactorial-Folic acid supplementation
Anencephaly
SPINA BIFIDA
* myelomenigocele occulta meningocele
* Sacral dimple: dermal sinus track with spina bifida
Myelomeningocele
* Herniation of malformed cord + meninges through vertebral defect
* Usually associated with Arnold-Chiari and hydrocephalus
* Lumbosacral level most common
Chiari II (Arnold Chiari)
* Cerebellar tonsillar herniation
* Small posterior fossa
* Extension of medulla below foramen magnum
* Kinking of medulla (Z-formation)
* Beaking of the quadrigeminal plate
* Hydrocephalus
* Myelomeningocele
* Cerebellar tonsillar herniation
* Small posterior fossa
* Extension of medulla below foramen magnum
* Kinking of medulla (Z-formation)
* Beaking of the quadrigeminal plate
* Hydrocephalus
* Myelomeningocele
Chiari I Malformation
Dandy-Walker Malformation
* Dandy-Walker syndrome
o Agenesis of cerebellar vermis
o cystic dilatation of 4th venticle
o enlargement of posterior fossa
o Variable clinical manifestations
o Hypothesized to result from arrest of cerebellar development prior to the 3rd month

CEREBELLAR MALFORMATIONS: VERMIAN (PALEOCEREBELLUM)
* Joubert syndrome
Holoprosencephaly
Encephalocele
AGENESIS OF CORPUS CALLOSUM
MICROENCEPHALY
MEGALENCEPHALY
CORTICAL DYSPLASIA
Polymicrogyria
TUBEROUS SCLEROSIS
Sturge-Weber Disease
Port-wine stain or nevus
Flammeus tuypical of Sturge-Weber, occurring in V1 distribution.

CNS Malformations.ppt