Dement Neurocogn Disord.  2015 Jun;14(2):87-93. 10.12779/dnd.2015.14.2.87.

Longitudinal Clinical Changes of Non-Fluent/Agrammatic Primary Progressive Aphasia as Tau Spectrum Disorder: A Case Report

• Kim JS
• Jang JW
• Kim SH
• Wang MJ
• Park YH
• Kim S

• KMID: 2443007
• DOI: http://doi.org/10.12779/dnd.2015.14.2.87

Abstract

BACKGROUND
Tauopathies are a group of diseases caused by the accumulation of hyperphosphorylated tau protein in the central nervous system. Previous studies have revealed that there is considerable overlap in clinical, pathological, and genetic features among different taupathies.
CASE REPORT
We report a patient with non-fluent/agrammatic primary progressive aphasia at the initial assessment. Over time, other symptoms belonging to corticobasal degeneration and progressive supranuclear palsy appeared in this patient.
CONCLUSIONS
Clinical overlapping features in these disorders may represent different phenotypes of a single disease process.

Keyword

taupathy; non-fluent/agrammatic primary progressive aphasia; corticobasal degeneration; progressive supranuclear palsy

MeSH Terms

Aphasia, Primary Progressive*
Central Nervous System
Humans
Phenotype
Supranuclear Palsy, Progressive
tau Proteins
Tauopathies
tau Proteins

Figure

• 

  Fig. 1 Fluorodeoxyglucose positron emission tomopraphy scan. A: Initial image demonstrating hypometabolism in the left posterior frontal, temporal cortex, and left basal ganglia in 2009. B: Follow-up image revealing hypometabolism in bilateral frontal, temporal cortex, and basal ganglia in 2012.

• 

  Fig. 2 Brain fluid attenuated inversion recovery image. A: Axial image demonstrating diffuse cerebral atrophy with subtle asymmetric atrophy in the left perisylvian area. B: Midsagittal image demonstrating equivocal midbrain atrophy.

• 

  Fig. 3 Clinical diagnostic spectrum of the patient across time. CBD: corticobasal degeneration, nfvPPA: nonfluent/agrammatic primary progressive aphasia, PSP: progressive supranuclear palsy.

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