Neuropathological findings of PSP in the elderly without clinical PSP: Possible incidental PSP?

• Published in: Parkinsonism & related disorders 2011-06, Vol.17 (5), p.365-371
• Main Authors: Evidente, Virgilio Gerald H, Adler, Charles H, Sabbagh, Marwan N, Connor, Donald J, Hentz, Joseph G, Caviness, John N, Sue, Lucia I, Beach, Thomas G
• Format: Article
• Language: English
• Subjects: Aged, 80 and over; Autopsy; Brain - pathology; Cognition Disorders - etiology; Female; Humans; Incidence; Incidental; Lewy Bodies - pathology; Longitudinal Studies; Male; Neurites - pathology; Neurology; Neurons - pathology; Neuropathology; Parkinsonism; Progressive supranuclear palsy; PSP; Retrospective Studies; Supranuclear Palsy, Progressive - complications; Supranuclear Palsy, Progressive - diagnosis; Supranuclear Palsy, Progressive - epidemiology
• ISSN: 1353-8020; 1873-5126
• DOI: 10.1016/j.parkreldis.2011.02.017

Abstract Aims We aimed to describe cases with incidental neuropathological findings of progressive supranuclear palsy (PSP) from the Banner Sun Health Research Institute Brain and Body Donation Program. Methods We performed a retrospective review of 277 subjects with longitudinal motor and neuropsychological assessments who came to autopsy. The mean Gallyas-positive PSP features grading for subjects with possible incidental neuropathological PSP was compared to those of subjects with clinically manifest disease. Results There were 5 cases with histopathological findings suggestive of PSP, but no parkinsonism, dementia or movement disorder during life. Cognitive evaluation revealed 4 of the 5 cases to be cognitively normal; one case had amnestic mild cognitive impairment (MCI) in her last year of life. The mean age at death of the 5 cases was 88.9 years (range 80–94). All 5 individuals had histopathologic microscopic findings suggestive of PSP. Mean Gallyas-positive PSP features grading was significantly lower in subjects with possible incidental neuropathological PSP than subjects with clinical PSP, particularly in the subthalamic nucleus. Conclusions We present 5 patients with histopathological findings suggestive of PSP, without clinical PSP, dementia or parkinsonism during life. These incidental neuropathological PSP findings may represent the early or pre-symptomatic stage of PSP. The mean Gallyas-positive PSP features grading was significantly lower in possible incidental PSP than in clinical PSP, thus suggesting that a threshold of pathological burden needs to be reached within the typically affected areas in PSP before clinical signs and symptoms appear.