Abstract Details

Title Extrapyramidal Signs in Primary Progressive Aphasia: Analyses of the NACC UDS Cohort

Topic Behavioral Neurology

Presentation(s) P06 - (-)

Poster/Presentation
Number 071

Objective

Background BACKGROUND: Previous studies reported higher frequencies of EPS in non-fluent PPA relative to semantic or logopenic PPA. However, interpretation of these reports has been limited by their reliance on retrospective clinical data and/or small sample sizes.

Design/Methods DESIGN/METHODS: We analyzed the prevalence and severity of EPS in a cohort of 362 PPA subjects enrolled in the National Alzheimer's Coordinating Center (NACC) Uniform Data Set (UDS) between 2005-2010. Subjects were diagnosed with progressive non-fluent aphasia (PNFA; n=148), semantic dementia (SD; n=118), or other PPA (PPA-other; n=96) per NACC UDS criteria. EPS were assessed with the Unified Parkinson's Disease Rating Scale (UPDRS). Subgroup comparisons were performed using total UPDRS and mean UPDRS item scores as well as two UPDRS-derived factor analyses (Louis et al., 2004; Stebbins & Goetz, 1998).

Results RESULTS: Overall, 48% of PPA subjects had any EPS (total UPDRS score > 0), and the proportions of subjects with any EPS were similar across subgroups (PNFA: 31%; SD: 41.3%; PPA-other: 27%). The PNFA group had significantly higher total UPDRS (7.21) and mean UPDRS item (0.27) scores than the SD (total UPDRS=2.42; mean UPDRS item=0.09) and PPA-other (total UPDRS=3.94; mean UPDRS item=0.15) groups. Analyses of UPDRS symptom factors indicated that the PNFA group had significantly more rigidity, axial dysfunction, and bradykinesia than the other two PPA groups. These findings remained robust after adjusting for demographic differences between PPA subgroups.

Conclusions CONCLUSIONS: EPS, particularly rigidity, axial dysfunction, and bradykinesia, are frequently seen in PPA, and are most prominent in PNFA. Our results confirm and extend findings from prior studies. The greater frequency/severity of EPS in PNFA may arise from neurodegeneration of fronto-cortical and basal ganglia networks, and may serve as an extralinguistic diagnostic aid for distinguishing PPA subtypes.

Authors/Disclosures
Sarah Kremen, MD (Cedars-Sinai Medical Center, Department of Neurology) PRESENTER	Dr. Kremen has received publishing royalties from a publication relating to health care.
	No disclosure on file
Mario F. Mendez, MD, PhD, FAAN (VA Greater Los Angeles Healthcare System and UCLA)	Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medical ��ɫ�� Speakers' Bureau. Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UpToDate. The institution of Dr. Mendez has received research support from NIH. Dr. Mendez has received publishing royalties from a publication relating to health care.
Edmond Teng, MD, PhD (Genentech)	Dr. Teng has received personal compensation for serving as an employee of Genentech. An immediate family member of Dr. Teng has received personal compensation for serving as an employee of Evidation Health. Dr. Teng has stock in F. Hoffman-La Roche. An immediate family member of Dr. Teng has stock in Evidation Health. Dr. Teng has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Teng has received intellectual property interests from a discovery or technology relating to health care.
	No disclosure on file

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