Dement Neurocogn Disord.  2016 Jun;15(2):55-58. 10.12779/dnd.2016.15.2.55.

Corticobasal Degeneration Presenting as Non-Fluent/Agrammatic Primary Progressive Aphasia: A Case Report

• Kim JS
• Lee GB
• Hong YJ
• Park KW

• KMID: 2442857
• DOI: http://doi.org/10.12779/dnd.2016.15.2.55

Abstract

BACKGROUND
Non-fluent agrammatic primary progressive aphasia (naPPA) is characterized by progressive non-fluent speech disorder and might be associated with taupathy such as corticobasal degeneration (CBD) and progressive supranuclear palsy. We report a case of overlap syndrome presented with language impairment, and diagnosed as naPPA with possible CBD.
CASE REPORT
A 58-year-old woman visited a memory and dementia clinic, with a 10-month history of progressive language disturbance. She was diagnosed as naPPA and overlapping CBD, based on the clinical features and neuroimaging findings including florbetaben PET.
CONCLUSIONS
naPPA is pathologically caused by taupathy, and might progress to asymmetrical parkinsonism and apraxia, suggestive of CBD. Overlapping clinical features in our case represent various phenotypes of taupathy.

Keyword

non-fluent agrammatic primary progressive aphasia; taupathy; corticobasal degeneration

MeSH Terms

Aphasia, Primary Progressive*
Apraxias
Dementia
Female
Humans
Memory
Middle Aged
Neuroimaging
Parkinsonian Disorders
Phenotype
Supranuclear Palsy, Progressive

Figure

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  Fig. 1 Brain MRI scan and fluorodeoxyglucose positron emission tomography (FDG-PET) scan of the patient. A: Brain MRI images showing diffuse cerebral atrophy, particularly in the left parietal cortex. B: FDG-PET images showing hypometabolism in the diffuse cortical areas, particularly in the left frontal, temporal, and parietal lobes.

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  Fig. 2 Florbetaben positron emission tomography (PET) scan of the patient. Florbetaben PET scans were regarded as brain amyloid plaque load score 1 representing negative for β-amyloid uptake.

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