Evidence map›Paper›PMID 32699140›Full record

ArticleNeurology2020

Familial language network vulnerability in primary progressive aphasia.

Sandra Weintraub, Benjamin Rader, Christina Coventry, Jaiashre Sridhar, Jessica Wood, Kyla A Guillaume, Giovanni Coppola, Eliana Marisa Ramos, Borna Bonakdarpour, Emily J Rogalski and 1 more

Open access · greenAbstract readCase Reports
In one paragraph

Article in Neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
2.2field-weighted citation impact, top 12% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

11 citing papers in PubMed, 24 citations in OpenAlex.

  1. Article
  2. Review
  3. Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2024
    Article
  4. Review
  5. Article
  6. Symptom-led staging for primary progressive aphasia.medRxiv : the preprint server for health sciences · 2023
    Article
  7. Temporopolar regions of the human brain.Brain : a journal of neurology · 2023
    Article
  8. Article
  9. Article
  10. Article
  11. Primary Progressive Aphasia: Toward a Pathophysiological Synthesis.Current neurology and neuroscience reports · 2021
    Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

11 authors at 1 institution in 1 country.

Sandra WeintraubFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles. sweintraub@northwestern.edu.ORCID 0000-0003-2605-5205
Benjamin RaderFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Christina CoventryFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.ORCID 0000-0003-3596-1998
Jaiashre SridharFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Jessica WoodFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.ORCID 0000-0001-7304-5750
Kyla A GuillaumeFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Giovanni CoppolaFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Eliana Marisa RamosFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Borna BonakdarpourFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Emily J RogalskiFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
M Marsel MesulamFrom the Mesulam Cognitive Neurology and Alzheimer's Disease Center (S.W., B.M.R., C.C., J.S., J.W., B.B., E.J.R., M.M.M.), Department of Psychiatry and Behavioral Sciences (S.W., E.J.R.), and Department of Neurology (B.B., M.M.M.), Northwestern Feinberg School of Medicine, Chicago, IL; Department of Epidemiology (B.M.R.), Boston University School of Public Health, MA; Northwestern University Weinberg School of Arts and Sciences (K.A.G.), Evanston, IL; and Department of Psychiatry (G.C., E.M.R.), Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles.
Boston University · US

Funding

Neuropathology CoreP30AG013854 · NIA · NORTHWESTERN UNIVERSITY AT CHICAGO · PI MORHARDT, DARBY · 1996 to 2020
$28.9M
Language in Primary Progressive AphasiaR01DC008552 · NIDCD · NORTHWESTERN UNIVERSITY AT CHICAGO · PI MESULAM, MAREK-MARSEL M · 2007 to 2021
$8.5M
Determinants of neurodegenerative decline in the aphasic variant of Alzheimer's diseaseR01AG056258 · NIA · UNIVERSITY OF CHICAGO · PI ROGALSKI, EMILY J · 2017 to 2022
$4.1M
Determinants of Neurodegenerative Decline in Primary Progressive AphasiaR01NS075075 · NINDS · NORTHWESTERN UNIVERSITY AT CHICAGO · PI ROGALSKI, EMILY J · 2012 to 2016
$1.7M
Atrophy and resting state connectivity in primary progressive aphasiaK23DC014303 · NIDCD · NORTHWESTERN UNIVERSITY AT CHICAGO · PI BONAKDARPOUR, BORNA · 2015 to 2019
$846k
NIA NIH HHS P30 AG013854NIA NIH HHS R01 AG056258NIDCD NIH HHS K23 DC014303NIDCD NIH HHS R01 DC008552NINDS NIH HHS R01 NS075075
6 · The paper itself

Abstract

objectiveTo investigate evidence of the potential role of early cortical vulnerability in the development of primary progressive aphasia (PPA).

methodA woman with a diagnosis of PPA and her 9 adult siblings, 7 with developmental language disabilities, underwent neuropsychological testing, structural MRI, and resting-state fMRI. Whole-exome sequencing was conducted for genes associated with dyslexia or with neurodegenerative dementia.

resultsThe siblings demonstrated lower verbal than nonverbal cognitive test scores in a developmental dyslexia pattern. On structural MRI, although the siblings did not differ from controls in total brain volume, the left hemisphere language area volume was significantly smaller than the right. Furthermore, cortical connectivity between the left superior temporal area, previously identified as the region of peak atrophy in the proband early in the course of illness, and adjacent language network components, including the planum temporale, was decreased in the siblings. No distinctive genetic signatures were identified.

conclusionThis report further supports the hypothesis that at least some cases of PPA may be based on a familial language network vulnerability that interferes with the acquisition of language in some members and that makes the language network a locus of least resistance to the effects of an independently late-arising neurodegenerative disease in others. This association offers a conceptual model to explain why identical neurodegenerative diseases may selectively target the language network in some individuals while targeting networks that regulate memory or behavior in others. The genetic basis for this vulnerability remains to be determined.

Indexed as

LanguageAphasia, Primary ProgressiveAtrophyBrainFemaleHumansMagnetic Resonance ImagingMemoryMiddle AgedNerve NetNeurodegenerative DiseasesNeuropsychological Tests

Identifiers

PMID32699140
PMCPMC7605508
OpenAlexW3044735758

What OpenQuestion holds

Textmetadata
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.