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Article type: Research Article
Authors: Raggi, Alberto; | Marcone, Alessandra | Iannaccone, Sandro | Ginex, Valeria; | Perani, Daniela; | Cappa, Stefano F.;
Affiliations: Department of Neurology and Neurorehabilitation, San Raffaele Turro Hospital, Milan, Italy | Department of Psychology and Neuroscience, Vita-Salute San Raffaele University, Milan, Italy | Department of Nuclear Medicine, San Raffaele Scientific Institute and Milano Bicocca University, Milan, Italy
Note: [] Corresponding author: Alberto Raggi, MD, Department of Neurology and Neurorehabilitation, San Raffaele Turro Hospital, Via Stamira d'Ancona 20, 20127 Milan, Italy. Tel.: +39 02 26433305; Fax: +39 02 26433394; E-mail: [email protected]
Abstract: The corticobasal degeneration syndrome has been suggested to be part of a complex of conditions (including the different subtypes of frontotemporal dementia and progressive supranuclear palsy), which reflect a spectrum of pathological substrates. This concept is supported by the frequent clinical overlap that can be observed among patients diagnosed with these conditions. We report three clinical cases, characterized by the overlap of the clinical features of corticobasal degeneration syndrome with, respectively, nonfluent progressive aphasia, progressive supranuclear palsy and semantic dementia. Current diagnostic criteria emphasize differences in clinical presentation, which probably reflect the preferential location of pathology in the early stages of disease. However, with disease progression, a considerable clinical overlap can be expected among the different syndromes. This concept should be extended not only to the cognitive and behavioural features of the frontotemporal dementia subtypes, but also to the movement disorders of corticobasal degeneration and supranuclear palsy.
Keywords: Corticobasal degeneration, frontotemporal dementia, nonfluent progressive aphasia, progressive supranuclear palsy, semantic dementia
Journal: Behavioural Neurology, vol. 18, no. 3, pp. 159-164, 2007
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