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Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia

Overview of attention for article published in Acta Neuropathologica Communications, July 2015
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  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (88th percentile)

Mentioned by

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1 news outlet
twitter
5 tweeters

Citations

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16 Dimensions

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31 Mendeley
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Title
Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia
Published in
Acta Neuropathologica Communications, July 2015
DOI 10.1186/s40478-015-0216-0
Pubmed ID
Authors

Jennifer L. Ziskin, Michael D. Greicius, Wan Zhu, Anna N. Okumu, Christopher M. Adams, Edward D. Plowey

Abstract

Transthyretin/TTR gene mutations usually cause systemic amyloidotic diseases. Few TTR variants preferentially affect the central nervous system, manifesting as oculoleptomeningeal amyloidosis. Patients with TTR meningovascular amyloidosis often show dementia, however the neuropathologic features of dementia in these cases have not been elucidated. We report the neuropathologic findings from a brain autopsy of a 72-year-old man with the rare Tyr69His (Y69H) TTR gene variant, dementia and ataxia. Severe amyloid deposits were observed in the leptomeninges and in a subpial and subependymal distribution. Mass spectrometry analysis demonstrated that the amyloid deposits were comprised of over 80 % of the variant TTR. TTR was undetectable by mass spectrometry in the neocortex subjacent to the subpial amyloid deposits. Subpial TTR amyloid deposits were associated with brisk superficial reactive gliosis and siderosis in the neocortex and cerebellar cortex. Subependymal TTR amyloid deposits were associated with subjacent myelin pallor in the hippocampal outflow tract structures including the alveus, fimbria and fornix. Phospho-tau immunostains demonstrated transentorhinal-stage neurofibrillary degeneration (Braak stage II) which, in the absence of neocortical amyloid-beta and neuritic plaques, was indicative of primary age-related tauopathy (PART). However, distinctive phospho-tau aggregates were observed subjacent to the subpial TTR amyloid deposits in all regions of the neocortex, including the primary motor and striate cortices, suggesting a potential link between TTR amyloid and neocortical tauopathy. Our report reveals novel insights into the potential neuropathologic substrates of dementia in variant TTR amyloidosis that need to be investigated in larger autopsy series.

Twitter Demographics

The data shown below were collected from the profiles of 5 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 31 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 31 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 9 29%
Other 5 16%
Student > Doctoral Student 3 10%
Professor > Associate Professor 2 6%
Student > Ph. D. Student 1 3%
Other 3 10%
Unknown 8 26%
Readers by discipline Count As %
Medicine and Dentistry 11 35%
Neuroscience 3 10%
Biochemistry, Genetics and Molecular Biology 2 6%
Pharmacology, Toxicology and Pharmaceutical Science 1 3%
Agricultural and Biological Sciences 1 3%
Other 4 13%
Unknown 9 29%

Attention Score in Context

This research output has an Altmetric Attention Score of 14. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 01 July 2020.
All research outputs
#1,728,102
of 18,081,658 outputs
Outputs from Acta Neuropathologica Communications
#276
of 1,111 outputs
Outputs of similar age
#21,699
of 189,308 outputs
Outputs of similar age from Acta Neuropathologica Communications
#1
of 1 outputs
Altmetric has tracked 18,081,658 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 90th percentile: it's in the top 10% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,111 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 11.6. This one has done well, scoring higher than 75% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 189,308 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 88% of its contemporaries.
We're also able to compare this research output to 1 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them