Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology

Authors

Heather B. Rigby, Parkinson's Disease and Movement Disorders Center Department of Neurology Mayo Clinic Scottsdale Arizona USA.
Brittany N. Dugger, Civin Laboratory for Neuropathology Banner Sun Health Research Institute Sun City Arizona USA.
Joseph G. Hentz, Department of Biostatistics Mayo Clinic Scottsdale Arizona USA.
Charles H. Adler, Parkinson's Disease and Movement Disorders Center Department of Neurology Mayo Clinic Scottsdale Arizona USA.
Thomas G. Beach, Civin Laboratory for Neuropathology Banner Sun Health Research Institute Sun City Arizona USA.
Holly A. Shill, Cleo Roberts Center Banner Sun Health Research Institute Sun City Arizona USA.Follow
Erika Driver-Dunckley, Parkinson's Disease and Movement Disorders Center Department of Neurology Mayo Clinic Scottsdale Arizona USA.
Marwan N. Sabbagh, Cleo Roberts Center Banner Sun Health Research Institute Sun City Arizona USA.Follow
Lucia I. Sue, Civin Laboratory for Neuropathology Banner Sun Health Research Institute Sun City Arizona USA.
John N. Caviness, Parkinson's Disease and Movement Disorders Center Department of Neurology Mayo Clinic Scottsdale Arizona USA.

Document Type

Article

Abstract

The pathologic changes of Parkinson's disease (PD) and Progressive Supranuclear Palsy (PSP) have been reported to coexist, but whether PSP pathology modifies the clinical course of those individuals is unknown. The aim of this study was to determine whether clinical features of pathologically confirmed PD subjects with concomitant PSP pathology differ from those with PD alone. Subjects enrolled in the Arizona Study of Aging and Neurodegenerative Disorders had annual movement and cognitive evaluations from enrollment until death/autopsy. All cases between 1997 and 2014 with a final clinicopathological diagnosis of PD with or without PSP at autopsy were analyzed. Overall, 12 of the 125 cases with pathologically confirmed PD had coexisting PSP pathology (9.6%). Those with PD-PSP had more-prominent postural instability, body bradykinesia, difficulty arising from a chair, and falls; asymmetric onset was less common in this group. Downgaze palsy and square wave jerks were infrequent in both groups. Gender, age at death, disease duration, rate of dementia, and presence of rest tremor did not differ between groups. Only 58% of subjects in the PD-PSP group were correctly given a final diagnosis in life of PD, compared to 91% of those with PD alone. The combination of PD and PSP pathology yields a heterogeneous clinical syndrome that often resembles PD, but may be more symmetric at onset and have more-prominent postural instability and falls. Our observations suggest that coexisting PSP pathology may be an important factor contributing to the clinical heterogeneity in PD and a potential confounder in diagnosis.

Publication Date

3-1-2015

Publication Title

Movement disorders clinical practice

E-ISSN

2330-1619

Volume

2

Issue

1

First Page

33

Last Page

38

PubMed ID

30363831

Digital Object Identifier (DOI)

10.1002/mdc3.12104

Recommended Citation

Rigby, Heather B.; Dugger, Brittany N.; Hentz, Joseph G.; Adler, Charles H.; Beach, Thomas G.; Shill, Holly A.; Driver-Dunckley, Erika; Sabbagh, Marwan N.; Sue, Lucia I.; and Caviness, John N., "Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology" (2015). Neurology. 812.
https://scholar.barrowneuro.org/neurology/812