Neuropsychology of frontotemporal dementia

Kipps, C.M., Knibb, J.A., Patterson, K. and Hodges, J.R. (2008) Neuropsychology of frontotemporal dementia. In, Aminoff, Michael J., Boller, François, Swaab, Dick F., Goldenberg, Georg and Miller, Bruce L. (eds.) Handbook of Clinical Neurology: Neuropsychology and Behavioral Neurology. (Handbook of Clinical Neurology, 88) Elsevier BV, pp. 527-548. (doi:10.1016/s0072-9752(07)88028-6).

Abstract

Frontotemporal dementia (FTD), also known as frontal variant (fv-FTD) or behavioral variant (bv-FTD), presents with disturbed behavior. The chapter describes two language variants: semantic dementia (SD), known also as temporal variant FTD (tv-FTD) and a progressive nonfluent aphasic syndrome (PNFA). A wide range of neuropsychological tests have been applied to patients with FTD in order to characterize their deficits, and to distinguish them from patients who have other neurodegenerative disorders. Symptoms in FTD are related to the underlying structures involved in the pathological process, and not to the nature of the pathology itself. There are reliable symptom clusters which define syndromes, but there is several language and behavioral features that show significant overlap between the different variants of FTLD. Future work needs to continue to delineate the relevant neural substrates of the disturbed cognition in these patients. As the nature of the disease becomes clearer, it is likely that the disease will be recognized in more patients, particularly those over the age of 65 who have previously been given a diagnosis of Alzheimer's disease. Despite substantial progress in delineating the features of this disease, there remains no effective treatment, either symptomatic or curative.

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Contributors

Author: C.M. Kipps

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