Quantitative assessment of grammar in amyloid-negative logopenic aphasia.

Logopenic primary progressive aphasia (lvPPA) typically results from underlying Alzheimer's disease, but subjects have been reported that do not show beta-amyloid (Aß) deposition. These subjects do not differ on neurological and speech-language testing from Aß-positive lvPPA, but they impressionistically show increased grammatical deficits. We performed a quantitative linguistic analysis of grammatical characteristics in Aß-negative lvPPA compared to Aß-positive lvPPA and agrammatic PPA, which is characterized by increased grammatical difficulties. Aß-negative lvPPA used fewer function words and correct verbs but more syntactic and semantic errors compared to Aß-positive lvPPA. These measures did not differ between Aß-negative lvPPA and agPPA. Both lvPPA cohorts showed a higher mean length of utterance, more complex sentences, and fewer nouns than agPPA. Aß-negative lvPPA subjects appear unique and share linguistic features with both agPPA and Aß-positive lvPPA. Quantitative language analysis in lvPPA may be able to distinguish those with and without Aß deposition.

Longitudinal assessment of language and memory impairments in pathologically confirmed cortico-basal ganglionic degeneration.

We report a longitudinal case study (patient EP) of histologically confirmed cortico-basal ganglionic degeneration (CBD) who presented with non-fluent progressive aphasia (NFPA). While NFPA has been documented in clinical descriptions of other reports of CBD, details are often limited and the majority of studies are cross-sectional in nature. The present study conducted detailed longitudinal assessment with EP over a period of two years that revealed substantial impairments of episodic memory, semantic memory, naming and particular aspects of reading and spelling. Our investigations identify key features of EP's pattern of impairment that warrant further examination with other cases of CBD. In particular, testing of EP's nonword reading and spelling found that both were impaired and declined over time. In addition, verbal recognition deteriorated faster than non-verbal recognition through the course of the disease. Our review of the literature suggests that poor nonword reading and spelling may be consistent features of CBD, but more studies are needed to confirm this suggestion, and to determine whether they warrant inclusion in profiling CBD.