BSN PSP/CBD Conference – Larry Golbe

A Cluster of PSP in Northern France: Is This the Key?

Larry Golbe, MD, Rutgers Robert Wood Johnson

  • There is a cluster of people with PSP in Northern France
    • Dominique Caparros-Lefebvre, MD (Wattrelos)
    • Arrived in 2005, had done research in PSP before that, noticed there were a lot more people there with PSP than expected
    • Incidence of PSP in Wattrelos and nearby towns
    • 98 patients studied, symptom onset measured
    • Discovered that it took about 3-4 years for someone to reach medical attention after their symptoms arose
    • PSP incidence over the 5 years – 27 cases with onset during those 5 years (A lot!)
    • PSP incidence measured in Olmsted County, MN and estimated in London, Newcastle, and Yonago 1.1/100,000
    • Population of Wattrelos (2007) 42,077 (i.e., there was a high number of patients there with PSP)
    • Large piles of metal ore behind the canal/factories, all patients with PSP diagnosed lived within very close proximity to this location
    • Another factory that used dyes made from arsenical are there too
    • Only 2 things that differentiated these patients from PSP in general, and that is that their onset age was a bit later, more likely to lose sense of smell, and ration of classic Richards to less common PSP was reverse to what it is with regular PSP
    • No molecular difference
    • Could this be a genetic founder effect? No. None of their families were related to each other as far as they knew
    • Are metals causing this? People would grow vegetables in their yards with this. Metals such as arsenic, nickel, and chromium. Vegetables have a high percentage of these metals, so yes there is a very good chance that these metals played a part in this disease.
    • Analyzing samples of metals, trying to compare addresses of where people worked/lived, trying to collect blood/urine/household dust/nail to test, genetic analysis, genetic testing, and testing for PSP
    • Trying to test which metals are most toxic (PSP wise)