- Human pathology

Home > E. Pathology by systems > Nervous system > Central nervous system > exencephaly


Thursday 27 January 2005

Definition: Exencephaly is a rare malformation in which the scalp and calvarium (acrania) are absent ant the cerebral mass is protected by a thick, dura mater-like membrane.

The brain consists of a disorganized, anarchic outgrowth of nervius tissue with polymicrogyria and nodules of heterotopias.

Etiology (examples)

- amniotic band sequence (ABS)


- Katsanis N. Ciliary proteins and exencephaly. Nat Genet. 2006 Feb;38(2):135-6. PMID: 16444248