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Behavioural Variant Frontotemporal Dementia-Defining Genetic and Pathological Subtypes.

Summary of "Behavioural Variant Frontotemporal Dementia-Defining Genetic and Pathological Subtypes."

Behavioural variant frontotemporal dementia (bvFTD) is a clinically, genetically and pathologically heterogeneous neurodegenerative disorder caused by FTLD-tau, FTLD-TDP and FTLD-FUS pathologies. Clinically, patients present with behavioural symptoms that may include one or more of disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative, stereotyped and compulsive/ritualistic behaviour or hyperorality/dietary changes. Cognitive deficits, particularly executive dysfunction, are also seen. Neuroanatomically, patients have frontal and/or temporal lobe atrophy on neuroimaging. However, there is currently no clear correlation between the clinical and neuroanatomical phenotype in life and the underlying pathogenetics. With the advent of clinical trials in bvFTD, establishing the underlying pathology accurately during life will become increasingly important. This review therefore investigates current and future biomarkers that may help make a pathological diagnosis in life, i.e. bvFTD-tau, bvFTD-TDP and bvFTD-FUS, including clinical and neuropsychological data, neuroimaging, blood and CSF markers.

Affiliation

Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK, rohrer@dementia.ion.ucl.ac.uk.

Journal Details

This article was published in the following journal.

Name: Journal of molecular neuroscience : MN
ISSN: 1559-1166
Pages:

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Medical and Biotech [MESH] Definitions

Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA.

The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.

A form of presenile DEMENTIA characterized by cortical dementia, NEUROFIBRILLARY TANGLES without SENILE PLAQUES, Fahr's type CALCINOSIS, and ATROPHY in frontotemporal or TEMPORAL LOBE.

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