Incidence and Trends of Progressive Supranuclear Palsy and Corticobasal Syndrome: A Population-Based Study

Article type: Research Article

Authors: Stang, Cole D.^a | Turcano, Pierpaolo^a | Mielke, Michelle M.^b | Josephs, Keith A.^a | Bower, James H.^a | Ahlskog, J. Eric^a | Boeve, Bradley F.^a | Martin, Peter R.^{b; 1} | Upadhyaya, Sudhindra G.^{b; 1} | Savica, Rodolfo^{a; b; *}

Affiliations: [a] Mayo Clinic Department of Neurology, Rochester, MN, USA | [b] Mayo Clinic Department of Health Sciences Research, Rochester, MN, USA

Correspondence: [] Correspondence to: Rodolfo Savica, MD, PhD, Department of Neurology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA. Tel.: 507 284 2120; E-mail: Savica.Rodolfo@mayo.edu.

Note: [1] Performed statistical analysis.

Abstract: 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.

Keywords: Progressive supranuclear palsy, corticobasal syndrome, incidence, population-based cohort

DOI: 10.3233/JPD-191744

Journal: Journal of Parkinson's Disease, vol. 10, no. 1, pp. 179-184, 2020