Neuropsychological, Motor, and Pathological Evaluation of the Primary
Progressive Aphasias
 
   Lead Investigator:    Sarah Kremen
   Institution      :    UCLA
   E-Mail           :    SKremen@mednet.ucla.edu
   Proposal ID      :    117


   Publications:


1  Kremen S, Tassniyom K, Mendez M, Teng E
   Extrapyramidal Signs in Primary Progressive Aphasia: Analyses of the NACC UDS Cohort
   Abstract  Neurology,80:P06.071,2013,

   PUBLISHED





   Proposal Description:
   Extrapyramidal signs (EPS) appear to vary across three major subtypes of          primary progressive aphasia (PPA): progressive non-fluent aphasia (PNFA),         semantic dementia (SD) and progressive logopenic aphasia (PLA). While             clinicians and investigators have noted the presence of extrapyramidal signs      (EPS) in the PPAs, the prevalence of specific EPS features, such as rigidity      and bradykinesia, among the different PPA subtypes has been scarcely assessed.    The literature suggests that PNFA patients demonstrate greater EPS than SD        patients.  Moreover, PNFA is typically associated with tau pathology, while SD    is associated with tau-negative, ubiquitin-positive pathology.  However, these    clinicopathological relationships are not perfect.                                                                                                                  Prior studies that have assessed neuropsychological differences between the       PPAs have been relatively underpowered due to small patient numbers (<20 per      group.)  The pattern and severity of EPS features with the PPAs has not been      extensively studied.  The NACC Uniform Data set (UDS) offers a unique             opportunity to investigate these two issues because it is a large patient data    set (>180 subjects) that includes various neuropsychological tests,               standardized motor examination scores via the Unified Parkinson???s Disease       Rating Scale, and pathology results.  These features make it a potentially        useful data set in the examination of clinical differences in the PPAs that       might correspond with different pathological entities.