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American Journal of Alzheimer's Disease and Other Dementias logoLink to American Journal of Alzheimer's Disease and Other Dementias
. 2011 Feb;26(1):72–77. doi: 10.1177/1533317510391239

Extrapyramidal Signs in the Primary Progressive Aphasias

Sarah A Kremen 1, Mario F Mendez 2, Po-Heng Tsai 3, Edmond Teng 4
PMCID: PMC3139562  NIHMSID: NIHMS310328  PMID: 21282281

Abstract

Background: Extrapyramidal signs (EPS) may vary across 3 major subtypes of primary progressive aphasia (PPA): progressive nonfluent aphasia (PNFA), semantic dementia (SD), and progressive logopenic aphasia (PLA). Methods: We reviewed initial neurological examinations from a clinical PPA cohort (PNFA = 49, SD = 26, PLA = 28) to determine the prevalence of specific categories of EPS. Results: The presence of any EPS was more common in PNFA (38.8%) and PLA (35.7%) than in SD (3.8%). The PNFA group exhibited the highest prevalence of bradykinesia (PNFA: 22.4%, SD: 3.8%, PLA: 0.0%) and rigidity (PNFA: 30.6%, SD: 0.0%, PLA: 10.7%). Calculated positive likelihood ratios indicated bradykinesia (12.1) or rigidity (5.5) was more strongly associated with PNFA than other PPAs. Conclusion: These findings suggest that on initial presentation, specific EPS may help distinguish PPA subtypes when linguistic and/or neuroimaging profiles are indistinct. Moreover, EPS could represent a marker of underlying tauopathy, linking clinical presentation to neuropathology in PPA.

Keywords: primary progressive aphasia, parkinsonism, progressive nonfluent aphasia, semantic dementia, logopenic aphasia

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Contributor Information

Sarah A. Kremen, Neurobehavior Unit, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA, Department of Neurology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA, skremen@ucla.edu .

Mario F. Mendez, Neurobehavior Unit, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA, Department of Neurology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA.

Po-Heng Tsai, Neurobehavior Unit, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA, Department of Neurology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA.

Edmond Teng, Neurobehavior Unit, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA, Department of Neurology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA, Geriatric Research Education and Clinical Center, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA.

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