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  • Updated 03.26.2019
  • Released 11.26.2001
  • Expires For CME 03.26.2022

Tauopathies with dementia and parkinsonism

Introduction

This article includes discussion of tauopathies with dementia and parkinsonism. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The tauopathies are a group of neurodegenerative disorders that manifest as dementias and movement disorders. They have as a common pathological feature, the presence of intracellular accumulation of abnormal filaments of tau protein. The most common tauopathies, Alzheimer disease, progressive supranuclear palsy, corticobasal degeneration, Pick disease, and frontotemporal dementia with parkinsonism, present with either dementia, parkinsonism, or both. Clinical features may overlap, and accurate diagnosis may be challenging. In this article, the author reviews the clinical features, pathogenesis, diagnostic work-up, and potential therapies for this group of disorders.

Key points

• Tauopathies with dementia and parkinsonism represent a group of disorders with shared underlying pathology of intracellular accumulation of tau protein.

• These pathologically defined disorders can manifest with a broad range of phenotypes, in addition to the classically associated syndromes.

• Classical clinical features can help distinguish these disorders, but there is often marked overlap of clinical features.

• There are no confirmatory diagnostic tests in vivo, but ancillary testing such as imaging may be helpful.

• The diagnostic gold standard for these disorders remains postmortem pathologic confirmation.

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