Clinical and pathological characterization of progressive aphasia
- 22 December 2005
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 59 (1) , 156-165
- https://doi.org/10.1002/ana.20700
Abstract
Objective The clinical and neuropathological categorization of patients presenting with progressive aphasia is an area of controversy. This study aimed to characterize a large group of progressive aphasic patients from a single center (n = 38), first clinically by case note review, and then pathologically. Methods Hierarchical cluster analysis of the cases according to their clinical language deficits was used to establish an unbiased, data-driven classification. Results This analysis revealed two groups of cases corresponding to the syndromes of progressive nonfluent aphasia (n = 23) and semantic dementia (n = 15). Postmortem analysis showed a majority in both groups of pathologies from the spectrum of frontotemporal lobar degeneration: the most frequent were non–Alzheimer's disease (AD) tauopathy in the nonfluent cases (10 of 23) and frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions in the fluent cases (8 of 15). Despite rigorous exclusion of cases with clinically significant memory deficits or other cognitive impairments, the pathology of AD was present in approximately one third of each group (overall 12 of 38), although often with an atypical neuroanatomical distribution. Interpretation Progressive aphasia is best seen as a composite of two conditions, on both clinical and pathological levels: progressive nonfluent aphasia and semantic dementia. Ann Neurol 2006;59:156–165Keywords
This publication has 64 references indexed in Scilit:
- Clinicopathological correlates in frontotemporal dementiaAnnals of Neurology, 2004
- Cognition and anatomy in three variants of primary progressive aphasiaAnnals of Neurology, 2004
- Primary Progressive Aphasia: A ReviewNeurocase, 2004
- Clinicopathologic Case Report: Progressive Aphasia in a 77-Year-Old ManThe Journal of Neuropsychiatry and Clinical Neurosciences, 2003
- FAMILIAL PROGRESSIVE APHASIA: INSIGHTS INTO THE NATURE AND DETERIORATION OF SINGLE WORD PROCESSINGCognitive Neuropsychology, 1999
- Progressive Dysgraphia: Co-occurrence of Central and Peripheral ImpairmentsCognitive Neuropsychology, 1997
- Charting the progression in semantic dementia: Implications for the organisation of semantic memoryMemory, 1995
- SEMANTIC DEMENTIABrain, 1992
- Neuropathological stageing of Alzheimer-related changesActa Neuropathologica, 1991
- Case 16-1986New England Journal of Medicine, 1986