TY - JOUR
T1 - Incidence and Trends of Progressive Supranuclear Palsy and Corticobasal Syndrome
T2 - A Population-Based Study
AU - Stang, Cole D.
AU - Turcano, Pierpaolo
AU - Mielke, Michelle M.
AU - Josephs, Keith A.
AU - Bower, James H.
AU - Ahlskog, J. Eric
AU - Boeve, Bradley F.
AU - Martin, Peter R.
AU - Upadhyaya, Sudhindra G.
AU - Savica, Rodolfo
N1 - Funding Information:
Savica: grant support from the National Institute
Funding Information:
Mielke: grant support from the National Institutes of Health and unrestricted research grants from Biogen and Lundbeck. Consulting fees from Lysosomal Therapeutics, Inc, and Eli Lilly
Funding Information:
Boeve: grant support from the National Institute on Aging, the National Institute of Neurological Disorders and Stroke, the Lewy Body Dementia Association, GE Healthcare, Axovant Sciences, Inc., and Biogen.
Funding Information:
This studywas supported by award R01AG034676 from the National Institute on Aging of the National Institutes of Health and by the Mayo Foundation for Medical Education and Research. The sponsors had no role in the design and conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; or decision to submit the manuscript for publication.
Publisher Copyright:
© 2020-IOS Press and the authors. All rights reserved.
PY - 2020
Y1 - 2020
N2 - Background: Few studies have investigated the incidence of PSP and CBS in the population. Objective: To examine the incidence of and trends in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in a population-based cohort of residents of Olmsted County, MN. Methods: We used the 1991-2005 population-based, Olmsted County Parkinsonism-cohort study, defined via the Rochester Epidemiology Project. A movement-disorder specialist reviewed medical records, to confirm PSP and CBS diagnoses. Results: We identified 21 patients with these diagnoses 1991-2005 : 18 (85.7%), PSP; 3 (14.3%), CBS. The median diagnosis age was 78 (range: 66-88). 13/21 (62.0%) were male. MRI was performed pre-diagnosis in 11 patients (8 PSP and 3 CBD); 10 showed atrophy consistent with clinical diagnoses. We observed concordance between clinical and pathological diagnoses in two PSP patients who underwent autopsy. Combined incidence for PSP and CBS in Olmsted County was 3.1 per 100,000 person-years (2.6 per 100,000 person-years, PSP; 0.4 per 100,000 person-years, CBS). Incidence was higher in men (4.5, 95% CI, 2.0-7.0) than women (1.8, 95% CI, 0.5-2.9). A combined, significant trend of increasing incidence was observed between 1991 and 2005 (B=0.69, 95% CI 0.42, 0.96, p<0.001). Median time from symptom onset to death among both groups was 6 years (range PSP, 1-10 years; range CBS, 3-8 years). Conclusions: The combined incidence for PSP and CBS was 3.1 per 100,000 person-years, higher in men than women. We observed a significant increase in both PSP and CBS, likely due to advancing imaging technology and improved diagnostic ability among physicians.
AB - Background: Few studies have investigated the incidence of PSP and CBS in the population. Objective: To examine the incidence of and trends in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in a population-based cohort of residents of Olmsted County, MN. Methods: We used the 1991-2005 population-based, Olmsted County Parkinsonism-cohort study, defined via the Rochester Epidemiology Project. A movement-disorder specialist reviewed medical records, to confirm PSP and CBS diagnoses. Results: We identified 21 patients with these diagnoses 1991-2005 : 18 (85.7%), PSP; 3 (14.3%), CBS. The median diagnosis age was 78 (range: 66-88). 13/21 (62.0%) were male. MRI was performed pre-diagnosis in 11 patients (8 PSP and 3 CBD); 10 showed atrophy consistent with clinical diagnoses. We observed concordance between clinical and pathological diagnoses in two PSP patients who underwent autopsy. Combined incidence for PSP and CBS in Olmsted County was 3.1 per 100,000 person-years (2.6 per 100,000 person-years, PSP; 0.4 per 100,000 person-years, CBS). Incidence was higher in men (4.5, 95% CI, 2.0-7.0) than women (1.8, 95% CI, 0.5-2.9). A combined, significant trend of increasing incidence was observed between 1991 and 2005 (B=0.69, 95% CI 0.42, 0.96, p<0.001). Median time from symptom onset to death among both groups was 6 years (range PSP, 1-10 years; range CBS, 3-8 years). Conclusions: The combined incidence for PSP and CBS was 3.1 per 100,000 person-years, higher in men than women. We observed a significant increase in both PSP and CBS, likely due to advancing imaging technology and improved diagnostic ability among physicians.
KW - Progressive supranuclear palsy
KW - corticobasal syndrome
KW - incidence
KW - population-based cohort
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U2 - 10.3233/JPD-191744
DO - 10.3233/JPD-191744
M3 - Article
C2 - 31594251
AN - SCOPUS:85078454908
VL - 10
SP - 179
EP - 184
JO - Journal of Parkinson's Disease
JF - Journal of Parkinson's Disease
SN - 1877-7171
IS - 1
ER -