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Von Economo Neurons Lost in Frontotemporal Dementia

Study shows loss of unique neurons found in socially complex animal may contribute to disease

FRIDAY, Dec. 29 (HealthDay News) -- Loss of von Economo neurons, which are found in the frontal brain regions of socially complex species including apes and humans, is associated with frontotemporal dementia but not Alzheimer disease, according to a report in the December issue of the Annals of Neurology.

William W. Seeley, M.D., of the University of California San Francisco, and colleagues used unbiased stereology to quantify anterior cingulate von Economo neurons (VENs) in seven patients with frontotemporal dementia, five with Alzheimer disease, and seven control subjects.

The researchers found that frontotemporal dementia was associated with early, severe losses of VENs, showing a 74 percent reduction in numbers compared with controls. Those VENs still present were dysmorphic and showed signs of Pick's disease. VEN counts were normal in patients with Alzheimer disease, however.

"Distinctive functions of these unique cells may prove invaluable in health, yet may also expose us to specific forms of developmental or later-life illness," the authors conclude. "The link forged here between VENs and frontotemporal dementia should spawn further studies of how human brain evolution relates to human brain disease."

Abstract
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