Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings
Identifieur interne : 004012 ( Main/Exploration ); précédent : 004011; suivant : 004013Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings
Auteurs : Andrew Lees (neurologue) [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 2003-09.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Atypical, Brain (pathology), Diagnosis, Differential, Human, Humans, Mutation (genetics), Neurodegenerative Diseases (genetics), Neurodegenerative Diseases (pathology), Neurofibrillary tangle, Neurofibrils (genetics), Neurofibrils (pathology), PSP, Parkinson disease, Parkinsonian Disorders (genetics), Parkinsonian Disorders (pathology), Pathophysiology, Phenotype, Progressive, Supranuclear ophthalmoplegia, Symptomatology, Tau protein, Tauopathies (genetics), Tauopathies (pathology), atypical parkinsonism, bodig, bradykinesia, tau, tau Proteins (genetics), tau Proteins (metabolism).
- MESH :
- chemical , genetics : tau Proteins.
- genetics : Mutation, Neurodegenerative Diseases, Neurofibrils, Parkinsonian Disorders, Tauopathies.
- chemical , metabolism : tau Proteins.
- pathology : Brain, Neurodegenerative Diseases, Neurofibrils, Parkinsonian Disorders, Tauopathies.
- Diagnosis, Differential, Humans, Phenotype.
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
Url:
DOI: 10.1002/mds.10560
Affiliations:
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Le document en format XML
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<series><title level="j">Movement Disorders</title>
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<term>Brain (pathology)</term>
<term>Diagnosis, Differential</term>
<term>Human</term>
<term>Humans</term>
<term>Mutation (genetics)</term>
<term>Neurodegenerative Diseases (genetics)</term>
<term>Neurodegenerative Diseases (pathology)</term>
<term>Neurofibrillary tangle</term>
<term>Neurofibrils (genetics)</term>
<term>Neurofibrils (pathology)</term>
<term>PSP</term>
<term>Parkinson disease</term>
<term>Parkinsonian Disorders (genetics)</term>
<term>Parkinsonian Disorders (pathology)</term>
<term>Pathophysiology</term>
<term>Phenotype</term>
<term>Progressive</term>
<term>Supranuclear ophthalmoplegia</term>
<term>Symptomatology</term>
<term>Tau protein</term>
<term>Tauopathies (genetics)</term>
<term>Tauopathies (pathology)</term>
<term>atypical parkinsonism</term>
<term>bodig</term>
<term>bradykinesia</term>
<term>tau</term>
<term>tau Proteins (genetics)</term>
<term>tau Proteins (metabolism)</term>
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<term>Neurofibrils</term>
<term>Parkinsonian Disorders</term>
<term>Tauopathies</term>
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<term>Neurodegenerative Diseases</term>
<term>Neurofibrils</term>
<term>Parkinsonian Disorders</term>
<term>Tauopathies</term>
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<term>Phenotype</term>
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<term>Homme</term>
<term>Ophtalmoplégie supranucléaire</term>
<term>Parkinson maladie</term>
<term>Physiopathologie</term>
<term>Progressif</term>
<term>Protéine tau</term>
<term>Structure neurofibrillaire</term>
<term>Symptomatologie</term>
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<front><div type="abstract" xml:lang="en">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</div>
</front>
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