Movement Disorders (revue)

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

Identifieur interne : 001848 ( Istex/Corpus ); précédent : 001847; suivant : 001849

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

Auteurs : Andrew J. Lees

Source :

RBID : ISTEX:103C6596D49525946F75A0063BE71A992FEF5EF0

English descriptors

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. © 2003 Movement Disorder Society

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DOI: 10.1002/mds.10560

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ISTEX:103C6596D49525946F75A0063BE71A992FEF5EF0

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