A geographical cluster of progressive supranuclear palsy in northern France
Identifieur interne : 001812 ( Ncbi/Curation ); précédent : 001811; suivant : 001813A geographical cluster of progressive supranuclear palsy in northern France
Auteurs : Dominique Caparros-Lefebvre ; Lawrence I. Golbe ; Vincent Deramecourt ; Claude-Alain Maurage ; Vincent Huin ; Valerie Buée-Scherrer ; Helene Obriot ; Bernard Sablonnière ; Francois Caparros ; Luc Buée ; Andrew J. LeesSource :
- Neurology [ 0028-3878 ] ; 2015.
Abstract
To describe a cluster of progressive supranuclear palsy (PSP) in northern France. PSP has not been reported in geographical, temporal, or occupational clusters. A unit of Neurology and Neurogeriatrics opened in 2005 at the Centre Hospitalier de Wattrelos, serving the population of Wattrelos and Leers (combined population 51,551) and parts of neighboring towns. For most of the 20th century, this area was a center for chromate and phosphate ore processing, textile dyeing, and tanning. Significant industrial waste persists close to residential areas.
From 2005 to 2014, 92 patients with PSP at Centre Hospitalier de Wattrelos were identified and studied. Detailed residential data were available in the medical records. Eighty cases have had magnetic resonance head scanning and 60 have died, of whom 13 have been examined neuropathologically.
The ratio of observed to expected PSP incidence over the period 2005 to 2012 was 12.3 (95% confidence interval: 7.4–35.9). Mean onset age was 74.3 years. The Richardson syndrome/PSP-parkinsonism ratio was 43%/42%. Four other phenotypes each occurred in 2% to 5%. Onset was gait/balance difficulty in 52%. None of the 92 affected patients were relatives and 7 were of North African ancestry. MRI was compatible with a clinical diagnostic of PSP in all cases. Histopathologic examination confirmed neurofibrillary degeneration and tufted astrocytes in all autopsied cases. Western blots revealed a typical tau 4R doublet. The tau H1 haplotype occurred in 95.8% of cases' chromosomes.
We have identified a cluster of PSP in a geographical area with severe environmental contamination by industrial metals.
Url:
DOI: 10.1212/WNL.0000000000001997
PubMed: 26354981
PubMed Central: 4617163
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PMC:4617163Le document en format XML
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<author><name sortKey="Caparros Lefebvre, Dominique" sort="Caparros Lefebvre, Dominique" uniqKey="Caparros Lefebvre D" first="Dominique" last="Caparros-Lefebvre">Dominique Caparros-Lefebvre</name>
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<author><name sortKey="Golbe, Lawrence I" sort="Golbe, Lawrence I" uniqKey="Golbe L" first="Lawrence I." last="Golbe">Lawrence I. Golbe</name>
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<author><name sortKey="Deramecourt, Vincent" sort="Deramecourt, Vincent" uniqKey="Deramecourt V" first="Vincent" last="Deramecourt">Vincent Deramecourt</name>
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<author><name sortKey="Maurage, Claude Alain" sort="Maurage, Claude Alain" uniqKey="Maurage C" first="Claude-Alain" last="Maurage">Claude-Alain Maurage</name>
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<author><name sortKey="Huin, Vincent" sort="Huin, Vincent" uniqKey="Huin V" first="Vincent" last="Huin">Vincent Huin</name>
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<author><name sortKey="Buee Scherrer, Valerie" sort="Buee Scherrer, Valerie" uniqKey="Buee Scherrer V" first="Valerie" last="Buée-Scherrer">Valerie Buée-Scherrer</name>
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<author><name sortKey="Obriot, Helene" sort="Obriot, Helene" uniqKey="Obriot H" first="Helene" last="Obriot">Helene Obriot</name>
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<author><name sortKey="Sablonniere, Bernard" sort="Sablonniere, Bernard" uniqKey="Sablonniere B" first="Bernard" last="Sablonnière">Bernard Sablonnière</name>
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<author><name sortKey="Caparros, Francois" sort="Caparros, Francois" uniqKey="Caparros F" first="Francois" last="Caparros">Francois Caparros</name>
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<author><name sortKey="Buee, Luc" sort="Buee, Luc" uniqKey="Buee L" first="Luc" last="Buée">Luc Buée</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">A geographical cluster of progressive supranuclear palsy in northern France</title>
<author><name sortKey="Caparros Lefebvre, Dominique" sort="Caparros Lefebvre, Dominique" uniqKey="Caparros Lefebvre D" first="Dominique" last="Caparros-Lefebvre">Dominique Caparros-Lefebvre</name>
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<author><name sortKey="Golbe, Lawrence I" sort="Golbe, Lawrence I" uniqKey="Golbe L" first="Lawrence I." last="Golbe">Lawrence I. Golbe</name>
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<author><name sortKey="Deramecourt, Vincent" sort="Deramecourt, Vincent" uniqKey="Deramecourt V" first="Vincent" last="Deramecourt">Vincent Deramecourt</name>
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<author><name sortKey="Maurage, Claude Alain" sort="Maurage, Claude Alain" uniqKey="Maurage C" first="Claude-Alain" last="Maurage">Claude-Alain Maurage</name>
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<author><name sortKey="Huin, Vincent" sort="Huin, Vincent" uniqKey="Huin V" first="Vincent" last="Huin">Vincent Huin</name>
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<author><name sortKey="Buee Scherrer, Valerie" sort="Buee Scherrer, Valerie" uniqKey="Buee Scherrer V" first="Valerie" last="Buée-Scherrer">Valerie Buée-Scherrer</name>
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<author><name sortKey="Obriot, Helene" sort="Obriot, Helene" uniqKey="Obriot H" first="Helene" last="Obriot">Helene Obriot</name>
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<author><name sortKey="Sablonniere, Bernard" sort="Sablonniere, Bernard" uniqKey="Sablonniere B" first="Bernard" last="Sablonnière">Bernard Sablonnière</name>
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<author><name sortKey="Caparros, Francois" sort="Caparros, Francois" uniqKey="Caparros F" first="Francois" last="Caparros">Francois Caparros</name>
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<author><name sortKey="Buee, Luc" sort="Buee, Luc" uniqKey="Buee L" first="Luc" last="Buée">Luc Buée</name>
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<author><name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew J. Lees</name>
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<series><title level="j">Neurology</title>
<idno type="ISSN">0028-3878</idno>
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<front><div type="abstract" xml:lang="en"><sec><title>Objective:</title>
<p>To describe a cluster of progressive supranuclear palsy (PSP) in northern France. PSP has not been reported in geographical, temporal, or occupational clusters. A unit of Neurology and Neurogeriatrics opened in 2005 at the Centre Hospitalier de Wattrelos, serving the population of Wattrelos and Leers (combined population 51,551) and parts of neighboring towns. For most of the 20th century, this area was a center for chromate and phosphate ore processing, textile dyeing, and tanning. Significant industrial waste persists close to residential areas.</p>
</sec>
<sec><title>Methods:</title>
<p>From 2005 to 2014, 92 patients with PSP at Centre Hospitalier de Wattrelos were identified and studied. Detailed residential data were available in the medical records. Eighty cases have had magnetic resonance head scanning and 60 have died, of whom 13 have been examined neuropathologically.</p>
</sec>
<sec><title>Results:</title>
<p>The ratio of observed to expected PSP incidence over the period 2005 to 2012 was 12.3 (95% confidence interval: 7.4–35.9). Mean onset age was 74.3 years. The Richardson syndrome/PSP-parkinsonism ratio was 43%/42%. Four other phenotypes each occurred in 2% to 5%. Onset was gait/balance difficulty in 52%. None of the 92 affected patients were relatives and 7 were of North African ancestry. MRI was compatible with a clinical diagnostic of PSP in all cases. Histopathologic examination confirmed neurofibrillary degeneration and tufted astrocytes in all autopsied cases. Western blots revealed a typical tau 4R doublet. The tau H1 haplotype occurred in 95.8% of cases' chromosomes.</p>
</sec>
<sec><title>Conclusions:</title>
<p>We have identified a cluster of PSP in a geographical area with severe environmental contamination by industrial metals.</p>
</sec>
</div>
</front>
</TEI>
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