Summary: Acta Neuropathol (2008) 116:479­489
DOI 10.1007/s00401-008-0434-7
123
ORIGINAL PAPER
Selective reduction of Von Economo neuron number
in agenesis of the corpus callosum
Jason A. Kaufman · Lynn K. Paul ·
Kebreten F. Manaye · Andrea E. Granstedt ·
Patrick R. Hof · Atiya Y. Hakeem · John M. Allman
Received: 15 January 2008 / Revised: 10 September 2008 / Accepted: 11 September 2008 / Published online: 25 September 2008
© Springer-Verlag 2008
Abstract Von Economo neurons (VENs) are large spin-
dle-shaped neurons localized to anterior cingulate cortex
(ACC) and fronto-insular cortex (FI). VENs appear late in
development in humans, are a recent phylogenetic special-
ization, and are selectively destroyed in frontotemporal
dementia, a disease which profoundly disrupts social
functioning and self-awareness. Agenesis of the corpus
callosum (AgCC) is a congenital disorder that can have
signiWcant eVects on social and emotional behaviors,

Source: Allman, John M. - Division of Biology, California Institute of Technology

Collections: Biology and Medicine