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. (C) 2007 Elsevier Srl. All rights reserved.