Abstract Details

Title Evaluating the Clinical Homogeneity of the Progressive Supranuclear Palsy Richardson Syndrome

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (11:45 AM-12:45 PM)

Poster/Presentation
Number 5-024

Objective To investigate whether specific signs and symptoms observed in PSP-RS cluster and whether PSP-RS is a homogenous syndrome.

Background The Movement Disorders Society (MDS)-Progressive supranuclear palsy (PSP) criteria recognize PSP-Richardson syndrome (PSP-RS) as a distinct homogenous syndrome. A diagnosis of PSP-RS requires core features of early postural instability, falls and vertical supranuclear gaze palsy/slowing. Many patients with PSP-RS, however, have additional features including dysarthria, executive dysfunction, behavioral changes, and parkinsonian features (tremor, axial +/- appendicular rigidity, bradykinesia).

Design/Methods One-hundred eighteen participants meeting MDS-PSP research criteria for probable PSP-RS were recruited by the Neurodegenerative Research Group at Mayo Clinic between 2009 and 2024. We assessed relationships between 16 neurological signs/symptoms, as well as clinical diagnoses made independent of the MDS-PSP criteria. We first performed a Network Analysis and Spearman correlations to identify relationships between the signs and symptoms followed by a k-means cluster analysis.

Results Fifty-three/118 patients were female. Mean age at onset 66 years (range:48-80). The 16 features sorted into five distinct network groups. Sensitivity to bright light and tremor sorted into 2 distinct groups and showed no inter-cluster relationships to other features. Worse neck rigidity and bradykinesia were associated with better ocular motor function, while worse bradykinesia was associated with worse cognitive impairment. Cluster analysis revealed two distinct clusters. Cluster I (n=89) was characterized by patients with more severe dysarthria, parkinsonian features, cognitive and executive dysfunction, and ocular motor impairment (all p<0.05). Cluster II (n=29) consisted of a higher frequency of participants not clinically diagnosed with PSP-RS (33.3% vs 14.8%; p=0.01). There was no difference in demographic features including gender, race, education, age at onset, or disease duration between clusters.

Conclusions Some neurological signs/symptoms occurring in PSP-RS are associated and tend to occur together. Probable PSP-RS, as defined by the MDS-PSP criteria, is not a homogeneous syndrome.

Authors/Disclosures
Mahesh Kumar, MBBS PRESENTER	Dr. Kumar has nothing to disclose.
Benjamin Brewer, PhD	Dr. Brewer has nothing to disclose.
Farwa Ali, MD (Mayo Clinic)	Dr. Ali has nothing to disclose.
Heather Clark	The institution of Heather Clark has received research support from NIH. Heather Clark has received publishing royalties from a publication relating to health care.
Julie Stierwalt (Mayo Clinic)	The institution of Julie Stierwalt has received research support from NIH. Julie Stierwalt has received publishing royalties from a publication relating to health care.
Yehkyoung C. Stephens, PA	Mrs. Stephens has nothing to disclose.
Rodolfo Savica, MD, PhD, FAAN (Mayo Clinic)	The institution of Dr. Savica has received research support from ACADIA Pharmaceuticals, Inc.
J. E. Ahlskog, MD, PhD (Mayo Clinic)	Dr. Ahlskog has received publishing royalties from a publication relating to health care.
Jennifer Whitwell, PhD (Mayo Clinic)	The institution of Dr. Whitwell has received research support from NIH.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic)	Dr. Josephs has nothing to disclose.

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