• Open Access

Primary progressive aphasia with parkinsonism

Authors

  • Karen M. Doherty MRCP,

    1. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, UK
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, London, UK
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  • Jonathan D. Rohrer MRCP, PhD,

    1. Dementia Research Center, UCL Institute of Neurology, London, UK
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  • Andrew J. Lees FRCP, MD,

    1. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, UK
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, London, UK
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  • Janice L. Holton FRCPath, PhD,

    1. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, UK
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, London, UK
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  • Jason Warren FRACP, PhD

    Corresponding author
    1. Dementia Research Center, UCL Institute of Neurology, London, UK
    • Correspondence to: Dr. Jason Warren, Dementia Research Center – UCL Institute of Neurology, 8-11 Queen Square, London, WC1N 3AR, UK; jason.warren@ucl.ac.uk

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  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

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. © 2013 Movement Disorder Society

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