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Abstract Details

Cross Sectional Analysis of Cognitive and Motor Dysfunction in Four-Repeat Tauopathies
Aging, Dementia, and Behavioral Neurology
Aging and Dementia Posters (7:00 AM-5:00 PM)
027
To compare motor ratings and cognitive scores between clinical presentations of four-repeat (4R) tauopathies.
4R Tauopathies are a subtype of frontotemporal dementia characterized by the aggregation of primarily 4R tau aggregates. 4R tauopathies present with wide-ranging symptoms, including motor and cognitive impairment. Common clinical presentations include progressive supranuclear palsy-Richardson’s syndrome (PSP-RS), corticobasal syndrome (CBS), and the non-fluent variant of primary progressive aphasia (nfvPPA). As these diseases progress, mixed features are common.
The NIH Neurodegenerative Disorders (NDD) Clinic maintains a database of all patients enrolled in the protocol. A retrospective analysis of the motor ratings and cognitive scores was performed in patients with CBS, PSP, and nfvPPA clinical syndromes.
Of 81 patients in the NDD database, 26 had clinical syndromes most predictive of 4R-tau pathology (CBS=13; PSP=7; nfvPPA=5). Patients who had disease duration greater than 10 years were excluded. There were 11 men and 15 women. CBS patients were younger (mean age=70, SD 4.2), compared to PSP (81, SD 2.2) and nfvPPA (81, SD 6.3). In nfvPPA, there was a decline in the lateralized motor ratings among patients who had a longer disease duration; this was not observed in CBS or PSP.  In PSP and nfvPPA, phonoarticulatory diadochokinesis correlated with disease duration. Similar performance was observed in PSP and PPA on phonemic fluency tasks and trails B. Phonemic fluency and function status decline correlated with disease duration in all subgroups.
In our clinic, the average age of each subgroup of patients was older than in reported literature. In the nfvPPA group, the motor ratings over the course of disease showed the greatest decline, suggesting an important inflection in disease course. Phonemic fluency in the PSP subgroup more closely matched that of the nfvPPA than the CBS subgroup. Unlike CBS and PSP, the motor and cognitive measures showed similar trends in nfvPPA. 
Authors/Disclosures
Allison Snyder, MD
PRESENTER 		Dr. Snyder has nothing to disclose.
No disclosure on file
No disclosure on file
Sonja W. Scholz, MD, FÂ鶹´«Ã½Ó³»­ (National Institute of Neurological Disorders and Stroke) Dr. Scholz has received personal compensation for serving as an employee of National Institutes of Health. An immediate family member of Dr. Scholz has received personal compensation for serving as an employee of National Institutes of Health. The institution of Dr. Scholz has received research support from National Institutes of Health. The institution of an immediate family member of Dr. Scholz has received research support from National Institutes of Health. An immediate family member of Dr. Scholz has received intellectual property interests from a discovery or technology relating to health care. Dr. Scholz has a non-compensated relationship as a Scientific Advisory Council Member with Lewy Body Dementia Association that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Scholz has a non-compensated relationship as a Editorial Board Member with JAMA Neurology that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Scholz has a non-compensated relationship as a Editorial Board Member with Journal of Parkinson's Disease that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Scholz has a non-compensated relationship as a Scientific Advisory Board Member with Mission MSA that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Scholz has a non-compensated relationship as a Scientific Advisory Board Member with The GBA1 Canada Initiative (G-Can) that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
Justin Y. Kwan, MD, FÂ鶹´«Ã½Ó³»­ (National Institutes of Health) Dr. Kwan has received research support from National Institutes of Health. Dr. Kwan has received personal compensation in the range of $100,000-$499,999 for serving as a Employee with National Institutes of Health.