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Abstract Details

Essential Tremor Followed by Progressive Supranuclear Palsy: A Postmortem Series of Seven Additional Cases
Movement Disorders
Movement Disorders Posters (7:00 AM-5:00 PM)
183

We report the prevalence of progressive supranuclear palsy (PSP) pathology in essential tremor (ET) cases in a prospective brain repository. We also report the prevalence of incidental PSP in clinically normal controls in that repository.
The Essential Tremor Centralized Brain Repository (ETCBR), in the New York Brain Bank (NYBB), is the largest collection of ET brains in the world. We previously reported that 11/89 (12.4%) ET donors (2003 – 2012) had developed PSP, a proportion that far exceeds the population prevalence of PSP. We now report our experience from 2012 – 2020.
ET patients were followed prospectively and phenotyped by a movement disorders neurologist. No controls had diagnoses of mild cognitive impairment or dementia. Neuropathologic diagnosis of PSP was based on the presence of tau-positive tufted astrocytes, globose neuronal tangles and tau-positive glial cytoplasmic inclusions. 
Seven of 120 (5.8%) ET donors carried a postmortem diagnosis of PSP. By comparison, 0 of 158 (0.0%) controls did.  All seven had long-standing ET (25 – 90 years; median = 40 years). Tremor was familial in six. None was diagnosed with PSP during life although three had dementia and four had soft signs of parkinsonism aside from rest tremor. 

The prevalence of PSP in this cohort of ET cases was 5.8%. This is clearly larger than the population prevalence of PSP (0.001%– 0.0065%) and higher than the prevalence of incidental PSP in our brain bank and others. These and other studies indicate that ET patients are at increased risk of developing tauopathies.
Authors/Disclosures
Elan D. Louis, MD, MS, FÂ鶹´«Ã½Ó³»­ (University of Texas Southwestern Medical Center)
PRESENTER 		Dr. Louis has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wolters Kluwer - Merritt's Textbook of Neurology. Dr. Louis has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Legal Firm. The institution of Dr. Louis has received research support from National Institutes of Health. Dr. Louis has received publishing royalties from a publication relating to health care. Dr. Louis has a non-compensated relationship as a Board of Directors with International Essential Tremor Foundation that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Louis has a non-compensated relationship as a Medical Advisory Board with HopeNET that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
No disclosure on file
Nora Hernandez No disclosure on file
No disclosure on file
Phyllis Faust Phyllis Faust has nothing to disclose.