Abstract Details

Title Analysis of a Large Progressive Supranuclear Palsy Cohort Reporting to a Single Clinical Center

Topic Movement Disorders

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 10-024

Objective To identify and report the various subtypes of Progressive Supranuclear Palsy (PSP) cases that report to Mayo Clinic Florida.

Background PSP was first described by Steele Richardson in 1964 (Steele JC et al., Arch Neurol 1964;10:333-359). Diagnosing PSP can be rather challenging; there is an overlap in symptoms of PSP and other neurological conditions such as Parkinson’s disease. Nine different subtypes have been identified since its initial discovery. A set proposed criteria have been developed by the National Institute of Neurological Disorders and Stroke and Society for PSP and are widely used when diagnosing PSP (Litvan et al., Neurology 1996;47:1-9).

Design/Methods 187 patients underwent chart analysis; all cases were clinically evaluated and managed at our facility between the years 1998 and 2017. They were evaluated by four different clinicians. Extracted data was based on initial and last visit. Data points were tabulated, some of which include: age, sex, ethnicity, age of onset, symptom of onset, family history, initial clinical suspicion, parkinsonian features, as well as response to Cabidopa-levodopa. We then compared initial to final diagnoses. Magnetic resonance imaging (MRI) scans were also reviewed. Blood samples were obtained form 140 cases.

Results Two patients were excluded due to inconclusive evidence. Of the 185 patients, our classification yielded the following: one PSP progressive gait freezing, 124 (66.7%) PSP-Richardson Steele, 15 (8.1%) PSP-Parkinson’s disease, 45 (24.2%) were identified as mixed PSP-RS and PSP-P. The cohort included 107 males and 77 females, with an average age of onset of 67.4 ± 7.6; they ranged between 41 and 82 years of age. 57 cases report positive family history of neurological disease.

Conclusions

Given the rarity of PSP, our cohort proves to be very substantial. The study findings demonstrate that three subtypes are commonly seen here at our clinic: PSP-RS, PSP-P and mixed.

Authors/Disclosures
Rana Hanna AL-Shaikh, MD (Mayo Clinic Florida) PRESENTER	Dr. Hanna AL-Shaikh has nothing to disclose.
	No disclosure on file
	No disclosure on file
Audrey Strongosky	No disclosure on file
Owen A. Ross, PhD (Mayo Clinic Jacksonville)	Dr. Ross has nothing to disclose.
Jay A. Van Gerpen, MD, FAAN (Neurology Consultants of Huntsville)	No disclosure on file
Neill R. Graff-Radford, MD, FAAN (Mayo Clinic Jacksonville)	The institution of Dr. Graff-Radford has received research support from Biogen. The institution of Dr. Graff-Radford has received research support from Lilly. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Biogen. Dr. Graff-Radford has received publishing royalties from a publication relating to health care.
Ryan J. Uitti, MD, FAAN (Mayo Clinic of Jacksonville)	Dr. Uitti has nothing to disclose.
Zbigniew K. Wszolek, MD, FAAN (Mayo Clinic- Jacksonville)	Dr. Wszolek has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Polish Neurological Society/Via Medica. Dr. Wszolek has received intellectual property interests from a discovery or technology relating to health care.

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