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Abstract Details

Sensitivity and Specificity of Diagnostic Criteria for Progressive Supranuclear Palsy
Movement Disorders
S4 - Clinical Trials in Movement Disorders (1:55 PM-2:06 PM)
006

Progressive supranuclear palsy (PSP) is a neuropathologically defined entity that is challenging to diagnose antemortem. In 2017, the Movement Disorders Society put forward new clinical criteria for the diagnosis of PSP recognizing diverse PSP phenotypes. 

In this study, we compare the sensitivity and specificity of the MDS-PSP and the NINDS-SPSP and analyze how the criteria perform at different time points in the disease.   

Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP were identified from the Cure PSP brain bank, Mayo Clinic Florida. All patients had neuropathologic diagnoses and detailed clinical examination performed by a specialist in Movement Disorders or Behavioral Neurology at one of the three Mayo Clinic sites located in Florida, Arizona and Minnesota. Clinical data on symptoms and signs were abstracted retrospectively in a blinded fashion and used to determine whether or not patients met NINDS or MDS PSP criteria. Patients

were divided into early and late disease stage groups using a 3-year cut off. Sensitivity and specificity were calculated for the entire cohort and for early versus late time points. 

A total of 129 patients were included of which 66 (51%) had PSP. The remainder had other neurodegenerative diseases including corticobasal degeneration, Parkinson’s disease, multiple system atrophy, frontotemporal lobar degeneration with transactive DNA binding protein 43 and globular glial tauopathy. The overall

sensitivity of the MDS criteria was 87.9% compared to 45.5% for NINDS, whereas the specificity of the MDS probable PSP criteria was 85.7% and 90.5% for NINDS criteria. Patients were noted to have features of multiple PSP phenotypes. 

The MDS criteria for the diagnosis of PSP recognizes several phenotypes of PSP and hence has a higher sensitivity than the NINDS criteria. False positive diagnoses resulted predominantly from other tauopathies. 

Authors/Disclosures
Farwa Ali, MD (Mayo Clinic)
PRESENTER
Dr. Ali has nothing to disclose.
No disclosure on file
Hugo Botha, MD (Mayo School of Graduate Medical 好色先生, Rochester) Dr. Botha has received research support from NIH.
J. E. Ahlskog, MD, PhD (Mayo Clinic) Dr. Ahlskog has received publishing royalties from a publication relating to health care.
James H. Bower, MD, MSc, FAAN (Mayo Clinic) The institution of Dr. Bower has received research support from Abbvie.
Joseph Y. Matsumoto, MD (University of Minnesota) Dr. Matsumoto has nothing to disclose.
Robert D. Fealey, MD (Mayo Clinic/Dept of Neuro) No disclosure on file
Anhar Hassan, MBBCH, FRACP, FRCPI, FAAN (Beaumont Hospital) The institution of Dr. Hassan has received research support from Intrabio . Dr. Hassan has received personal compensation in the range of $500-$4,999 for serving as a Invited speaker with Korean Movement Disorders Society.
Scott D. Eggers, MD Dr. Eggers has received publishing royalties from a publication relating to health care.
Bradley F. Boeve, MD, FAAN (Mayo Clinic) Dr. Boeve has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Rainwater Charitable Foundation. The institution of Dr. Boeve has received research support from Alector. The institution of Dr. Boeve has received research support from EIP Pharma. The institution of Dr. Boeve has received research support from Transposon. The institution of Dr. Boeve has received research support from Cognition Therapeutics. Dr. Boeve has received publishing royalties from a publication relating to health care.
David S. Knopman, MD, FAAN (Mayo Clinic) Dr. Knopman has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for DIAN TU study. The institution of Dr. Knopman has received research support from NIH.
Erika Driver-Dunckley, MD, FAAN Dr. Driver-Dunckley has nothing to disclose.
Jay A. Van Gerpen, MD, FAAN (Neurology Consultants of Huntsville) No disclosure on file
Ryan J. Uitti, MD, FAAN (Mayo Clinic of Jacksonville) Dr. Uitti has nothing to disclose.
Jennifer Whitwell, PhD (Mayo Clinic) Dr. Whitwell has nothing to disclose.
Dennis W. Dickson, MD (Mayo Clinic) Dr. Dickson has nothing to disclose.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic) Dr. Josephs has nothing to disclose.