5 Cases of Early Stage PSP (Pre-symptomatic)

Banner Sun Health in the Phoenix area encourages retirees in Arizona to donate their bodies and brains upon death. Many are neurologically normal. These neurologically normal brains are tremendous helpful in conducting research.

In this study of 277 brains donated to Banner Sun Health, they found five cases where the pathology seemed like “early PSP.” None of these five cases had any clinical symptoms of dementia, parkinsonism, or PSP. “Incidental” is the term neuropathologists give to such cases. So, incidental Lewy Body Disease is where the brain contains Lewy body pathology but there were no clinical symptoms of Parkinson’s Disease. And “incidental PSP” is where the brain has some PSP pathology but there were no PSP symptoms while the person was alive.

Robin

Parkinsonism & Related Disorders. 2011 Mar 18. [Epub ahead of print]

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

Evidente VG, Adler CH, Sabbagh MN, Connor DJ, Hentz JG, Caviness JN, Sue LI, Beach TG.
Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA.

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.

Copyright © 2011 Elsevier Ltd. All rights reserved.

PubMed ID#: 21420891