Extrapyramidal signs in the primary progressive aphasias

Am J Alzheimers Dis Other Demen. 2011 Feb;26(1):72-7. doi: 10.1177/1533317510391239.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aphasia, Primary Progressive / complications*
  • Aphasia, Primary Progressive / physiopathology
  • Basal Ganglia Diseases / etiology*
  • Basal Ganglia Diseases / physiopathology
  • Disease Progression
  • Female
  • Gait / physiology
  • Humans
  • Hypokinesia / etiology
  • Hypokinesia / physiopathology
  • Male
  • Middle Aged
  • Muscle Rigidity / etiology
  • Muscle Rigidity / physiopathology