Greater loss of von Economo neurons than loss of layer II and III neurons in behavioral variant frontotemporal dementia.

Previous studies have shown a selective reduction of von Economo neurons (VENs) in behavioral variant frontotemporal dementia (bvFTD). However, the alleged selectivity rests on the comparison between VENs and other neurons in cortical layer V, while it has been established that neurons in the superficial cortical layers (I-III) are particularly affected in bvFTD. The purpose of this study was to examine loss the loss of VENs in comparison with that of non-VEN-neurons of superficial cortical layers. VENs and non-VEN-neurons of cortical layer V and layers II+III were quantified in the anterior cingulate cortex in 16 cases of bvFTD, 12 non-demented controls and 10 cases of Alzheimer's disease (AD). In bvFTD VENs were more depleted than non-VEN-neurons of layers V and II+III. Also, non-VEN-neurons of layer II+III showed a greater density reduction than those of layer V in bvFTD. VEN density was also reduced in AD, albeit to a lesser extent than in bvFTD, and the differences between bvFTD and AD were only significant when relating VEN loss to that of layer V neurons. Our study strengthens the view of VENs as a particularly sensitive neuronal type of bvFTD, and appears to be on a continuum with the loss of other neurons both in bvFTD and between conditions.