ArticleNeurology2004
Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease.
Article in Neurology, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 53 papers.
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Who cites it
53 citing papers in PubMed, 162 citations in OpenAlex.
- Caudate atrophy on MRI is a characteristic feature of FTLD-FUS.European journal of neurology · 2010Trial
- Selective cellular and regional vulnerability in frontotemporal lobar degeneration: a scoping review.Free neuropathology · 2025Review
- Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach.Brain sciences · 2023Review
- Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies.Acta neuropathologica communications · 2023Article
- Neuropathology and emerging biomarkers in corticobasal syndrome.Journal of neurology, neurosurgery, and psychiatry · 2022Review
- Neurodegenerative Implications of Neuronal Cytoplasmic Protein Dysfunction in Response to Environmental Contaminants.Neurotoxicity research · 2021Review
- Development of disease-modifying drugs for frontotemporal dementia spectrum disorders.Nature reviews. Neurology · 2020Review
- Neuronal intermediate filament inclusion disease may be incorrectly classified as a subtype of FTLD-FUS.Free neuropathology · 2020Article
- FTD spectrum: Neuroimaging across the FTD spectrum.Progress in molecular biology and translational science · 2019Review
- Fused in Sarcoma Neuropathology in Neurodegenerative Disease.Cold Spring Harbor perspectives in medicine · 2017Review
- Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype.Brain pathology (Zurich, Switzerland) · 2017Review
- Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis: Neurofilament Light Chain Levels in Definite Subtypes of Disease.JAMA neurology · 2017Article
- Composition of Rosenthal Fibers, the Protein Aggregate Hallmark of Alexander Disease.Journal of proteome research · 2016Article
- Parkinsonism, movement disorders and genetics in frontotemporal dementia.Nature reviews. Neurology · 2016Review
- Comparative quantitative study of 'signature' pathological lesions in the hippocampus and adjacent gyri of 12 neurodegenerative disorders.Journal of neural transmission (Vienna, Austria : 1996) · 2015Article
- PRKAR1B mutations are a rare cause of FUS negative neuronal intermediate filament inclusion disease.Brain : a journal of neurology · 2015Article
- The role of FUS gene variants in neurodegenerative diseases.Nature reviews. Neurology · 2014Review
- Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes.Neuropathology and applied neurobiology · 2013Article
- Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia.Brain : a journal of neurology · 2013Article
- Different molecular pathologies result in similar spatial patterns of cellular inclusions in neurodegenerative disease: a comparative study of eight disorders.Journal of neural transmission (Vienna, Austria : 1996) · 2012Article
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23 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundRecently described neuronal intermediate filament inclusion disease (NIFID) shows considerable clinical heterogeneity.
objectiveTo assess the spectrum of the clinical and neuropathological features in 10 NIFID cases.
methodsRetrospective chart and comprehensive neuropathological review of these NIFID cases was conducted.
resultsThe mean age at onset was 40.8 (range 23 to 56) years, mean disease duration was 4.5 (range 2.7 to 13) years, and mean age at death was 45.3 (range 28 to 61) years. The most common presenting symptoms were behavioral and personality changes in 7 of 10 cases and, less often, memory loss, cognitive impairment, language deficits, and motor weakness. Extrapyramidal features were present in 8 of 10 patients. Language impairment, perseveration, executive dysfunction, hyperreflexia, and primitive reflexes were frequent signs, whereas a minority had buccofacial apraxia, supranuclear ophthalmoplegia, upper motor neuron disease (MND), and limb dystonia. Frontotemporal and caudate atrophy were common. Histologic changes were extensive in many cortical areas, deep gray matter, cerebellum, and spinal cord. The hallmark lesions of NIFID were unique neuronal IF inclusions detected most robustly by antibodies to neurofilament triplet proteins and alpha-internexin.
conclusionNIFID is a neuropathologically distinct, clinically heterogeneous variant of frontotemporal dementia (FTD) that may include parkinsonism or MND. Neuronal IF inclusions are the neuropathological signatures of NIFID that distinguish it from all other FTD variants including FTD with MND and FTD tauopathies.
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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.