Focal cortical presentations of Alzheimer's disease
1Department of Clinical Neurosciences, 2Department of Histopathology, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ and 3MRC – Cognition and brain Sciences Unit, 15 Chaucer Road, Cambridge CB2 7EF, UK
Correspondence to: Professor John R. Hodges, MRC-Cognition and Brain Sciences Unit, 15 Chaucer Road, Cambridge, CB2 7EF, UK E-mail: john.hodges{at}mrc-cbu.cam.ac.uk
To determine the frequency of Alzheimer's disease (AD) pathology in patients presenting with progressive focal cortical syndromes, notably posterior cortical atrophy (PCA), corticobasal syndrome (CBS), behavioural variant frontotemporal dementia (bvFTD), progressive non-fluent aphasia (PNFA) (or a mixed aphasia) and semantic dementia (SD); and to compare the age of onset, evolution and prognosis in patients with focal cortical presentations of AD versus more typical AD and those with non AD pathology. From a total of 200 patients with comprehensive prospective clinical and pathological data we selected 120 : 100 consecutive cases with focal cortical syndromes and 20 with clinically typical AD. Clinical files were reviewed blind to pathological diagnosis. Of the 100 patients with focal syndromes, 34 had AD as the primary pathological diagnosis with the following distribution across clinical subtypes: all 7 of the PCA (100%); 6 of 12 with CBS (50%); 2 of 28 with bvFTD (7.1%); 12 of 26 with PNFA (44.1%); 5 of 7 with mixed aphasia (71.4%) and 2 of 20 with SD (10%). Of 20 with clinically typical AD, 19 had pathological AD. Age at both onset and death was greater in the atypical AD cases than those with non-AD pathology, although survival was equivalent. AD is a much commoner cause of focal cortical syndromes than previously recognised, particularly in PCA, PNFA and CBS, but rarely causes SD or bvFTD. The focal syndrome may remain pure for many years. Patients with atypical AD tend to be older than those with non-AD pathology.
Key Words: Alzheimer's disease; frontotemporal dementia; posterior cortical atrophy; corticobasal syndrome; progressive aphasia
Abbreviations: PCA, posterior cortical atrophy; CBS, corticobasal syndrome; PNFA, progressive non-fluent aphasia; FTD, frontotemporal dementia; FTLD, frontotemporal lobar degeneration; SD, semantic dementia
Received April 24, 2007. Revised July 3, 2007. Accepted August 10, 2007.
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