Journal
JOURNAL OF NEUROLOGY
Volume 265, Issue 6, Pages 1474-1490Publisher
SPRINGER HEIDELBERG
DOI: 10.1007/s00415-018-8762-6
Keywords
Primary progressive aphasia; Semantic dementia; Logopenic aphasia; Frontotemporal dementia; Alzheimer's disease
Categories
Funding
- Alzheimer's Society [AS-PG-16-007]
- National Institute for Health Research University College London Hospitals Biomedical Research Centre
- UCL Leonard Wolfson Experimental Neurology Centre [PR/ylr/18575]
- ESRC-NIHR [ES/L001810/1]
- EPSRC [EP/M006093/1]
- Wellcome Trust [200783]
- Wellcome Trust Senior Research Fellowship in Clinical Science [091673/Z/10/Z]
- Leonard Wolfson Foundation (Clinical Research Fellowship)
- National Institute for Health Research (NIHR Doctoral Training Fellowship)
- National Brain Appeal-Frontotemporal Dementia Research Fund (CNC)
- Medical Research Council
- Alzheimer's Research UK
- Brain Research Trust
- Wolfson Foundation
- EPSRC [EP/M006093/1] Funding Source: UKRI
- MRC [G0801306, MR/M008525/1, G0601846, G0401247, G116/143, MR/M023664/1, MR/M009106/1, UKDRI-1001, MR/J009482/1] Funding Source: UKRI
- Alzheimers Research UK [ARUK-Network2011-6-ICE] Funding Source: researchfish
- Engineering and Physical Sciences Research Council [EP/M006093/1] Funding Source: researchfish
- Medical Research Council [MR/M009106/1, G0401247, 1332163, G116/143, G0801306, UKDRI-1001, MR/M023664/1, MR/J009482/1, MR/M008525/1, 1332624, G0601846] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0508-10123, NF-SI-0513-10134, CL-2012-18-010, DRF-2015-08-182] Funding Source: researchfish
- Rosetrees Trust [M668-CD1] Funding Source: researchfish
- National Institutes of Health Research (NIHR) [DRF-2015-08-182] Funding Source: National Institutes of Health Research (NIHR)
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The primary progressive aphasias are a heterogeneous group of focal 'language-led' dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including 'clinical pearls' that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic 'roadmap'. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.
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