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

• Published in: Parkinsonism & related disorders Vol. 17; no. 5; pp. 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: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.06.2011
• 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; Parkinsonism; PSP; Progressive supranuclear palsy; Autopsy; Neuropathology; Incidental
• ISSN: 1353-8020; 1873-5126; 1873-5126
• DOI: 10.1016/j.parkreldis.2011.02.017

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. 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. 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. 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.