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Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions

Lookup NU author(s): Emeritus Professor Robert Perry, Dr Evelyn Jaros, Professor David Burn, Professor Ian McKeith


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We report a new disease, dementia with neurofilament inclusions, characterized clinically by early-onset dementia with frontal lobe signs, focal atrophy of the frontal and temporal lobes, and microscopically by the presence in many brain regions of intraneuronal, cytoplasmic, neurofilament inclusions. The neuronal inclusions are immunoreactive to all three molecular weight neurofilament subunits: heavy (NF-H), light, and medium subunits, including the phosphorylated and non-phosphorylated forms of NF-H. Prion protein and beta-amyloid deposits were absent. The inclusions do not contain tau or alpha-synuclein protein aggregates known to characterize many neurodegenerative disorders. In addition to delineating a new disease entity, the identification of intraneuronal, cytoplasmic, neurofilament inclusions extends the molecular classification of neurodegenerative diseases and implicates new mechanisms of neurodegeneration in diseases affecting the human brain. (C) 2003 Elsevier Science Ireland Ltd. All rights reserved.

Publication metadata

Author(s): Cairns NJ, Perry RH, Jaros E, Burn D, McKeith IG, Lowe JS, Holton J, Rossor MN, Skullerud K, Duyckaerts C, Cruz-Sanchez FF, Lantos PL

Publication type: Article

Publication status: Published

Journal: Neuroscience Letters

Year: 2003

Volume: 341

Issue: 3

Pages: 177-180

ISSN (print): 0304-3940

ISSN (electronic): 1872-7972

Publisher: Elsevier


DOI: 10.1016/S0304-3940(03)00100-9


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