A geographical cluster of progressive supranuclear palsy in northern France
Identifieur interne : 000047 ( Pmc/Corpus ); précédent : 000046; suivant : 000048A 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
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">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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<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>
<idno type="eISSN">1526-632X</idno>
<imprint><date when="2015">2015</date>
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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>
<pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
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<contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Caparros-Lefebvre</surname>
<given-names>Dominique</given-names>
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<degrees>MD, PhD</degrees>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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</list>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
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</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
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<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
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<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Legal Proceedings:</title>
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</contrib>
<contrib contrib-type="author"><name><surname>Golbe</surname>
<given-names>Lawrence I.</given-names>
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<degrees>MD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>University of California San Francisco</p>
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<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
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</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>Robert Wood Johnson University Hospital Foundation (speaker honorarium), University of Vermont (speaker honorarium and travel), University of Kansas (speaker honorarium and travel), CurePSP (travel), Tau Consortium (travel), CBD Solutions (travel)</p>
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<list-item><p>Parkinsonism and Related Disorders, 2012-present</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
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<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>Bristol-Myers Squibb Sanofi GLG Consulting iPierian</p>
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<p><list list-type="order"><title>Speakers' Bureaus:</title>
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<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
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<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>American Parkinson's Disease Association CurePSP</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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</contrib>
<contrib contrib-type="author"><name><surname>Deramecourt</surname>
<given-names>Vincent</given-names>
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<degrees>MD, PhD</degrees>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
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<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
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<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
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</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
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<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
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<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
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<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
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<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
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<list-item><p>NONE</p>
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<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
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</contrib>
<contrib contrib-type="author"><name><surname>Maurage</surname>
<given-names>Claude-Alain</given-names>
</name>
<degrees>MD, PhD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
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<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
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<p><list list-type="order"><title>Research Support, Government Entities:</title>
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<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
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<list-item><p>NONE</p>
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</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Huin</surname>
<given-names>Vincent</given-names>
</name>
<degrees>MD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Buée-Scherrer</surname>
<given-names>Valerie</given-names>
</name>
<degrees>PhD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Obriot</surname>
<given-names>Helene</given-names>
</name>
<degrees>MS</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Sablonnière</surname>
<given-names>Bernard</given-names>
</name>
<degrees>MD, PhD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Caparros</surname>
<given-names>Francois</given-names>
</name>
<degrees>MS</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Buée</surname>
<given-names>Luc</given-names>
</name>
<degrees>PhD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>(1) Journal of Alzheimers disease, editorial board, 2013-14, (2) Current Alzheimer research, editorial board, 2013-2014</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>Patent applications on tau detection and anti-Alzheimer drug compounds</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>Scientific advisory committee at French NIH (Inserm)</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>Received grant from ANR (French National Research Agency) as PI or co-PI in 2014 and 2015</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>Received annual institutional support from Inserm and University of Lille</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>Received support from French Foundations: France Alzheimer Association, LECMA</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<contrib contrib-type="author"><name><surname>Lees</surname>
<given-names>Andrew J.</given-names>
</name>
<degrees>MD</degrees>
<author-comment content-type="disclosure"><p><list list-type="order"><title>Scientific Advisory Boards:</title>
<list-item><p>Britannia, UCB phrama,Novartis, Teva, Meda, Boehringer Ingelheim, GSK, Ipsen, Lundbeck, Allergan, Orion, BIAL, Noscira, Roche</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Gifts:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Funding for Travel or Speaker Honoraria:</title>
<list-item><p>Britannia, Novartis, Teva, Meda, Boehringer Ingelheim, GSK, Ipsen,Lundbeck, Allergan, Orion, BIAL, Noscira, Roche</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Editorial Boards:</title>
<list-item><p>2006 Archivas de Nevropsiquiatria (Brazil) 1995-03 Co-Editor in Chief, Movement Disorders Journal and Historical Section Editor 1994-98Founder Editor, Behavioural Neurology 1994- Neurologia Espana 1994-00 Revista Neurologica, Argentina 1994-95 European Journal of Neurology 1993- Revue Neurologique 1993-03 Brain 1991-95 Journal Neurology, Neurosurgery & Psychiatry 1989-95 Journal of Neurology 1988-95 Journal of Neural Transmission</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Patents:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Publishing Royalties:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Employment, Commercial Entity:</title>
<list-item><p>Institute of Neurology, UCL</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Consultancies:</title>
<list-item><p>Britannia, BIAL Portela</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Speakers' Bureaus:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Other Activities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Clinical Procedures or Imaging Studies:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Commercial Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Government Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Academic Entities:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Research Support, Foundations and Societies:</title>
<list-item><p>PSP Association, Weston Trust - The Reta Lila Howard Foundation</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options/Board of Directors Compensation:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>License Fee Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Royalty Payments, Technology or Inventions:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Research Sponsor:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Stock/Stock Options, Medical Equipment & Materials:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
<p><list list-type="order"><title>Legal Proceedings:</title>
<list-item><p>NONE</p>
</list-item>
</list>
</p>
</author-comment>
</contrib>
<aff>From the Unit of Neurology (D.C.-L.), Centre Hospitalier de Wattrelos, France; Department of Neurology (L.I.G.), Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ; University of Lille Nord de France (V.D., C.-A.M., V.H., V.B.-S., H.O., B.S., F.C., L.B.), INSERM UMR 1172, Batiment JPARC, France; and Reta Lila Weston Institute for Neurological Studies (A.J.L.), London, UK.</aff>
</contrib-group>
<author-notes><corresp>Correspondence to Dr. Caparros-Lefebvre: <email>dcaparroslefebvre@gmail.com</email>
</corresp>
<fn fn-type="financial-disclosure"><p>Go to <ext-link ext-link-type="uri" xlink:href="http://neurology.org/lookup/doi/10.1212/WNL.0000000000001997">Neurology.org</ext-link>
for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.</p>
</fn>
</author-notes>
<pub-date pub-type="ppub"><day>13</day>
<month>10</month>
<year>2015</year>
</pub-date>
<pub-date pub-type="pmc-release"><day>13</day>
<month>10</month>
<year>2016</year>
</pub-date>
<pmc-comment> PMC Release delay is 12 months and
0 days and was based on the . </pmc-comment>
<volume>85</volume>
<issue>15</issue>
<fpage>1293</fpage>
<lpage>1300</lpage>
<history><date date-type="received"><day>18</day>
<month>1</month>
<year>2015</year>
</date>
<date date-type="accepted"><day>29</day>
<month>5</month>
<year>2015</year>
</date>
</history>
<permissions><copyright-statement>© 2015 American Academy of Neurology</copyright-statement>
<copyright-year>2015</copyright-year>
<copyright-holder>American Academy of Neurology</copyright-holder>
</permissions>
<self-uri xlink:title="pdf" xlink:type="simple" xlink:href="NEUROLOGY2014625632.pdf"></self-uri>
<abstract><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>
</abstract>
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</front>
</pmc>
</record>
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