A geographical cluster of progressive supranuclear palsy in northern France.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
We have identified a cluster of PSP in a geographical area with severe environmental contamination by industrial metals.
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. dcaparroslefebvre@gmail.com.
Dominique
D
Caparros-Lefebvre
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.
Lawrence I
LI
Golbe
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.
Vincent
V
Deramecourt
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.
Claude-Alain
CA
Maurage
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.
Vincent
V
Huin
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.
Valerie
V
Buée-Scherrer
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.
Helene
H
Obriot
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.
Bernard
B
Sablonnière
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.
Francois
F
Caparros
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.
Luc
L
Buée
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.
Andrew J
AJ
Lees
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005602:France; D006239:Haplotypes / Q000235:genetics*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D010641:Phenotype; D013494:Supranuclear Palsy, Progressive / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology*; D016875:tau Proteins / Q000235:genetics / Q000378:metabolism
D016875:tau Proteins