Primary progressive aphasia with parkinsonism

Mov Disord. 2013 Jun;28(6):741-6. doi: 10.1002/mds.25341. Epub 2013 Feb 11.

Abstract

A 65-year-old man presented with word-finding difficulty and gait disturbance. His speech was nonfluent with word retrieval impairment and difficulties with sentence repetition. Other cognitive domains were intact initially. He developed asymmetrical bradykinesia, rigidity and a rest tremor. Over the following 8 years, his speech production impairment slowly deteriorated with the development of a motor speech disorder, anomia, impaired repetition of single words as well as sentences, and impaired comprehension of initially sentences then single words. His parkinsonian syndrome also deteriorated with limited response to levodopa. Serial brain MRI revealed progressive asymmetric perisylvian atrophy. He died after a disease duration of 12 years. The clinical syndrome is discussed by an expert, the pathology is described, and important clinical points from the case are highlighted.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloid beta-Peptides / metabolism
  • Aphasia, Primary Progressive / etiology*
  • Brain / pathology
  • Gait Disorders, Neurologic / etiology
  • Humans
  • Longitudinal Studies
  • Magnetic Resonance Imaging
  • Male
  • Mental Status Schedule
  • Parkinsonian Disorders / complications*
  • Parkinsonian Disorders / pathology
  • Writing
  • alpha-Synuclein / metabolism
  • tau Proteins / metabolism

Substances

  • Amyloid beta-Peptides
  • alpha-Synuclein
  • tau Proteins