Sivasathiaseelan, H;
Marshall, CR;
Agustus, JL;
Benhamou, E;
Bond, RL;
van Leeuwen, JEP;
Hardy, CJD;
... Warren, JD; + view all
(2019)
Frontotemporal Dementia: A Clinical Review.
Seminars in Neurology
, 39
(2)
pp. 251-263.
10.1055/s-0039-1683379.
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Abstract
Frontotemporal dementias are a clinically, neuroanatomically, and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. Clinically, frontotemporal dementias characteristically strike capacities that define us as individuals, presenting broadly as disorders of social behavior or language. Neurobiologically, these diseases can be regarded as “molecular nexopathies,” a paradigm for selective targeting and destruction of brain networks by pathogenic proteins. Mutations in three major genes collectively account for a substantial proportion of behavioral presentations, with far-reaching implications for the lives of families but also potential opportunities for presymptomatic diagnosis and intervention. Predicting molecular pathology from clinical and radiological phenotypes remains challenging; however, certain patterns have been identified, and genetically mediated forms of frontotemporal dementia have spearheaded this enterprise. Here we present a clinical roadmap for diagnosis and assessment of the frontotemporal dementias, motivated by our emerging understanding of the mechanisms by which pathogenic protein effects at the cellular level translate to abnormal neural network physiology and ultimately, complex clinical symptoms. We conclude by outlining principles of management and prospects for disease modification.
| Type: | Article |
|---|---|
| Title: | Frontotemporal Dementia: A Clinical Review |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1055/s-0039-1683379 |
| Publisher version: | https://doi.org/10.1055/s-0039-1683379 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences & Neurology, frontotemporal dementia, primary progressive aphasia, behavioral variant FTD, semantic dementia, PRIMARY PROGRESSIVE APHASIA, BEHAVIORAL-VARIANT, LOBAR DEGENERATION, ALZHEIMERS-DISEASE, PROGRANULIN, SIGNATURES, PATHOLOGY, MUTATIONS, ASSOCIATION, SENSITIVITY |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > IoN Central Administration UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10073844 |
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