ArticleJournal of neurology, neurosurgery, and psychiatry2013
TDP-43 frontotemporal lobar degeneration and autoimmune disease.
Article in Journal of neurology, neurosurgery, and psychiatry, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03507257 (Longitudinal Early-onset Alzheimer's Disease Study Protocol), which is not on this map. Cited by 96 papers, 3 of them syntheses that pooled it.
What it found
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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.
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.
Longitudinal Early-onset Alzheimer's Disease Study Protocol
Who cites it
96 citing papers in PubMed, 3 syntheses or guidelines pooled it, 181 citations in OpenAlex.
- Pooled it
- Systematic Review: Genetic, Neuroimaging, and Fluids Biomarkers for Frontotemporal Dementia Across Latin America Countries.Frontiers in neurology · 2021Pooled it
- Immune-related genetic enrichment in frontotemporal dementia: An analysis of genome-wide association studies.PLoS medicine · 2018Pooled it
- Human TDP-43 expression worsens FTD-related phenotypes in progranulin-insufficient mice.Neurobiology of disease · 2026Article
- Neuronal autoantibodies in neurodegenerative dementia: From evidence to clinical framework.The journal of prevention of Alzheimer's disease · 2026Review
- Frontotemporal Dementia, Current Diagnosis and Treatment Emphasis on Natural Products: A Review.Current aging science · 2026Review
- Shared Neuroinflammatory Mechanisms Across Dementia Types: An Integrative Review.International journal of molecular sciences · 2025Review
- Neuronal TDP-43 pathology drives astrocytic interferon response in a mouse model of ALS.Journal of neuroinflammation · 2025Article
- TDP-43: unveiling the hidden key to cellular fate decisions.Cell communication and signaling : CCS · 2025Review
- Deciphering distinct genetic risk factors for FTLD-TDP pathological subtypes via whole-genome sequencing.Nature communications · 2025Article
- Semantic variant primary progressive aphasia with ANXA11 p.D40G.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Frontotemporal Dementia Differential Diagnosis in Clinical Practice: A Single-Center Retrospective Review of Frontal Behavioral Referrals.Neurology. Clinical practice · 2025Article
- Immunity impacts cognitive deficits across neurological disorders.Journal of neurochemistry · 2024Review
- Limbic-predominant age-related TDP-43 encephalopathy (LATE-NC): Co-pathologies and genetic risk factors provide clues about pathogenesis.Journal of neuropathology and experimental neurology · 2024Review
- Immunological aspects of central neurodegeneration.Cell discovery · 2024Review
- The role of interferon signaling in neurodegeneration and neuropsychiatric disorders.Frontiers in psychiatry · 2024Review
- Review
- FAM76B regulates NF-κB-mediated inflammatory pathway by influencing the translocation of hnRNPA2B1.eLife · 2023Article
- Plasma inflammation for predicting phenotypic conversion and clinical progression of autosomal dominant frontotemporal lobar degeneration.Journal of neurology, neurosurgery, and psychiatry · 2023Article
- New Approaches to the Treatment of Frontotemporal Dementia.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2023Article
36 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
24 authors at 6 institutions in 2 countries.
Funding
Abstract
backgroundThe aetiology and pathogenesis of non-genetic forms of frontotemporal dementia (FTD) is unknown and even with the genetic forms of FTD, pathogenesis remains elusive. Given the association between systemic inflammation and other neurodegenerative processes, links between autoimmunity and FTD need to be explored.
objectiveTo describe the prevalence of systemic autoimmune disease in semantic variant primary progressive aphasia (svPPA), a clinical cohort, and in progranulin (PGRN) mutation carriers compared with neurologically healthy normal controls (NC) and Alzheimer's disease (AD) as dementia controls.
designCase control.
settingAcademic medical centres.
participants129 svPPA, 39 PGRN, 186 NC and 158 AD patients underwent chart review for autoimmune conditions. A large subset of svPPA, PGRN and NC cohorts underwent serum analysis for tumour necrosis factor α (TNF-α) levels. OUTCOME MEASURES: χ(2) Comparison of autoimmune prevalence and follow-up logistic regression.
resultsThere was a significantly increased risk of autoimmune disorders clustered around inflammatory arthritides, cutaneous disorders and gastrointestinal conditions in the svPPA and PGRN cohorts. Elevated TNF-α levels were observed in svPPA and PGRN compared with NC.
conclusionssvPPA and PGRN are associated with increased prevalence of specific and related autoimmune diseases compared with NC and AD. These findings suggest a unique pattern of systemic inflammation in svPPA and PGRN and open new research avenues for understanding and treating disorders associated with underlying transactive response DNA-binding protein 43 aggregation.
Indexed as
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What OpenQuestion holds
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.