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Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings.

Lees, AJ; (2003) Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings. Mov Disord , 18 Sup S28-S33. 10.1002/mds.10560.

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Abstract

Several distinct clinical syndromes presenting with parkinsonism have been associated with subcortical neurofibrillary degeneration and the abnormal accumulation of hyperphosphorylated tau protein in the brain. Mutations of tau have been linked with a small number of autosomal dominantly inherited families who present with frontolimbic cognitive deficits, behavioural disorders, and Parkinson's syndrome. Some of the sporadic disorders (progressive supranuclear palsy [PSP] and corticobasal degeneration) have been referred to by molecular pathologists as primary tauopathies, implicating abnormalities of tau in their pathogenesis. We have identified a sporadic parkinsonian syndrome characterised by bradykinesia, a variable response to levodopa, and a mean duration of disease of 9 years, which resembles bodig (Parkinson's-dementia of Guam), and histologically has close similarities with both PSP and postencephalitic parkinsonism. Further characterisation of these cases frequently confused with Parkinson's disease may broaden the clinical spectrum of parkinsonian disorders linked with neurofibrillary tangle formation.

Type: Article
Title: Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings.
Location: United States
DOI: 10.1002/mds.10560
Keywords: Brain, Diagnosis, Differential, Humans, Mutation, Neurodegenerative Diseases, Neurofibrils, Parkinsonian Disorders, Phenotype, Tauopathies, tau Proteins
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
URI: http://discovery.ucl.ac.uk/id/eprint/116018
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