Abstract
The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer’s disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.
Highlights
The nonfluent/agrammatic variant of primary progressive aphasia is one of the three subtypes of PPA [1,2] for which consensus clinical diagnostic criteria have recently been updated [3]
It has been proposed that, in the presence of a nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) syndrome, motor speech impairment could predict corticobasal degeneration (CBD) or progressive supranuclear palsy (PSP) pathology [17], while a greater deficit in grammatical function would be associated with FTLD-TDP [18]
We argue that Pick’s disease was the main cause of her clinical syndrome, while Alzheimer’s disease (AD) might have contributed to the development of mild late-emerging memory deficits
Summary
The nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) is one of the three subtypes of PPA [1,2] for which consensus clinical diagnostic criteria have recently been updated [3]. It has been proposed that, in the presence of a nfvPPA syndrome, motor speech impairment could predict CBD or PSP pathology [17], while a greater deficit in grammatical function would be associated with FTLD-TDP [18]. The two studies that have applied this technique to PPA [20,21] reported results consistent with previous pathological data in showing that cortical amyloidosis is uncommon in nfvPPA as currently defined, while common in the logopenic variant (lvPPA). We present a detailed, prospective, longitudinal clinical and neuroimaging study of FC (fictitious name), a 66 year old, right-handed woman with the typical features of a mainly speech-impaired nfvPPA who unexpectedly showed a PET-PIB positive scan and was found to have Pick’s disease and AD pathological changes post-mortem. We argue that Pick’s disease was the main cause of her clinical syndrome, while AD might have contributed to the development of mild late-emerging memory deficits
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