Tauopathies with dementia and parkinsonism

Mary Ann Thenganatt MD (Dr. Thenganatt of the University of Pennsylvania has no relevant financial relationships to disclose.)
Joseph Jankovic MD, editor. (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released November 26, 2001; last updated December 30, 2016; expires December 30, 2019

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. The tauopathies may overlap in some clinical features with Parkinson disease dementia (PDD), which is defined as a group of neurodegenerative disorders manifested by cognitive and motor slowing, executive dysfunction, and impaired memory retrieval that result from heterogeneous involvement of several distinct neural dopaminergic and nondopaminergic networks (Gratwicke et al 2015). 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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