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Von Economo neurons are selectively targeted in frontotemporal dementia.

Santillo, Alexander Frizell ; Nilsson, Christer LU and Englund, Elisabet LU orcid (2013) In Neuropathology & Applied Neurobiology 39(5). p.572-579
Abstract
Von Economo neurons (VEN) are bipolar neurons located in the anterior cingulate cortex (ACC) and the frontoinsular cortex (FI), areas affected early in behavioural variant frontotemporal dementia (bvFTD), in which VEN may constitute a selectively vulnerable cellular population. Aim A previous study has shown a selective loss of VEN in FTD above other neurons in the ACC of FTD. The aim of this study was to confirm this finding in a larger cohort, using a different methodology, and to examine VEN loss in relation to neuropathological severity and molecular pathology. Methods VEN and neighbouring neurons (NN) were quantified in layer Va and Vb of the right dorsal anterior cingulate cortex in 21 cases of behavioural variant FTD, 10 cases of... (More)
Von Economo neurons (VEN) are bipolar neurons located in the anterior cingulate cortex (ACC) and the frontoinsular cortex (FI), areas affected early in behavioural variant frontotemporal dementia (bvFTD), in which VEN may constitute a selectively vulnerable cellular population. Aim A previous study has shown a selective loss of VEN in FTD above other neurons in the ACC of FTD. The aim of this study was to confirm this finding in a larger cohort, using a different methodology, and to examine VEN loss in relation to neuropathological severity and molecular pathology. Methods VEN and neighbouring neurons (NN) were quantified in layer Va and Vb of the right dorsal anterior cingulate cortex in 21 cases of behavioural variant FTD, 10 cases of Alzheimer's disease (AD) and 10 non-demented controls (NDC). Results A marked VEN reduction was seen in all FTD cases. In the neuropathologically early cases of FTD (n=13), VEN/10000 NN was significantly reduced by 53 % compared with NDC (p<0.001) and 41% compared with AD (p=0.019), whereas AD patients showed a non-significant 30% reduction of VEN/10000 NN compared with NDC. VEN reduction was present in all protein pathology subgroups. Discussion In conclusion, this study confirms selective sensitivity of VEN in FTD and suggests that VEN loss is an early event in the neurodegenerative process. (Less)
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Contribution to journal
publication status
published
subject
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Neuropathology & Applied Neurobiology
volume
39
issue
5
pages
572 - 579
publisher
Wiley-Blackwell
external identifiers
  • wos:000328982000009
  • pmid:23346995
  • scopus:84880149144
ISSN
1365-2990
DOI
10.1111/nan.12021
language
English
LU publication?
yes
additional info
The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Department of Psychogeriatrics (013304000), Pathology, (Lund) (013030000)
id
d3eceaf7-0a2f-468a-9e11-e34fa483fda9 (old id 3438375)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/23346995?dopt=Abstract
date added to LUP
2016-04-01 09:51:19
date last changed
2022-05-05 08:19:10
@article{d3eceaf7-0a2f-468a-9e11-e34fa483fda9,
  abstract     = {{Von Economo neurons (VEN) are bipolar neurons located in the anterior cingulate cortex (ACC) and the frontoinsular cortex (FI), areas affected early in behavioural variant frontotemporal dementia (bvFTD), in which VEN may constitute a selectively vulnerable cellular population. Aim A previous study has shown a selective loss of VEN in FTD above other neurons in the ACC of FTD. The aim of this study was to confirm this finding in a larger cohort, using a different methodology, and to examine VEN loss in relation to neuropathological severity and molecular pathology. Methods VEN and neighbouring neurons (NN) were quantified in layer Va and Vb of the right dorsal anterior cingulate cortex in 21 cases of behavioural variant FTD, 10 cases of Alzheimer's disease (AD) and 10 non-demented controls (NDC). Results A marked VEN reduction was seen in all FTD cases. In the neuropathologically early cases of FTD (n=13), VEN/10000 NN was significantly reduced by 53 % compared with NDC (p&lt;0.001) and 41% compared with AD (p=0.019), whereas AD patients showed a non-significant 30% reduction of VEN/10000 NN compared with NDC. VEN reduction was present in all protein pathology subgroups. Discussion In conclusion, this study confirms selective sensitivity of VEN in FTD and suggests that VEN loss is an early event in the neurodegenerative process.}},
  author       = {{Santillo, Alexander Frizell and Nilsson, Christer and Englund, Elisabet}},
  issn         = {{1365-2990}},
  language     = {{eng}},
  number       = {{5}},
  pages        = {{572--579}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Neuropathology & Applied Neurobiology}},
  title        = {{Von Economo neurons are selectively targeted in frontotemporal dementia.}},
  url          = {{http://dx.doi.org/10.1111/nan.12021}},
  doi          = {{10.1111/nan.12021}},
  volume       = {{39}},
  year         = {{2013}},
}