ArticleNeurology2020
Familial language network vulnerability in primary progressive aphasia.
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.
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Who cites it
11 citing papers in PubMed, 24 citations in OpenAlex.
- The causal association between resting state intrinsic functional networks and neurodegeneration.Brain communications · 2025Article
- Primary progressive aphasia: six questions in search of an answer.Journal of neurology · 2024Review
- Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2024Article
- Cerebral hyperactivation across the Alzheimer's disease pathological cascade.Brain communications · 2024Review
- Evaluating the Association Between Genetically Proxied Neurodevelopmental Language Phenotypes and the Risk of Primary Progressive Aphasia.Neurology · 2023Article
- Symptom-led staging for primary progressive aphasia.medRxiv : the preprint server for health sciences · 2023Article
- Temporopolar regions of the human brain.Brain : a journal of neurology · 2023Article
- Article
- Neuropathological fingerprints of survival, atrophy and language in primary progressive aphasia.Brain : a journal of neurology · 2022Article
- Investigation of the Association Between History of Learning Disabilities and Primary Progressive Aphasia in Brazilian Portuguese Speakers.Frontiers in neurology · 2022Article
- Primary Progressive Aphasia: Toward a Pathophysiological Synthesis.Current neurology and neuroscience reports · 2021Review
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
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.
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Registered trials
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.