Frontotemporal dementia and related disorders

2011 Research Projects:

Memory dysfunction within limbic cortices - It is apparent that the complexities of the memory dysfunction seen in different neurodegenerative diseases cannot solely be due to pathology in the hippocampus. In this study we will examine regions within known memory circuits to determine which cell populations are affected in people with impairment of episodic memory. This will allow better targeting of future mechanistic therapies.

Blood protein biomarkers for frontotemporal lobar degeneration - This project will assess blood proteins as biomarkers for different pathogenic forms of frontotemporal dementia (FTD), one of the major neurodegenerative dementias with a very rapid disease progression (mean survival 3 years). At present, it is not possible to predict which pathological variant is present in any given patient. We plan to develop blood protein biomarker assays capable of diagnosing the pathology in vivo.

Finding clinical predictors for the underlying pathology in different frontotemporal dementia (FTD) syndromes - Due to the ageing population in Australia it is predicted that the prevalence of dementia will increase four-fold by 2050. Developing disease-modifying therapies for dementia is therefore a priority, however we also need to be able to accurately identify patients for whom these therapies will be beneficial. Here we will develop strategies for identifying patients with particular protein abnormalities in their brain, the substrate of most therapeutic interventions.

Academic Staff

To be appointed, PhD, NeuRA Research Officer

Support Staff

Heidi Cartwright, BSc, P/T Senior Research Assistant Amanda Gysbers, BBTEC with honours, Stephanie Wong BPsych with honours, Research Assistant

Students

Cosupervisor Prof Glenda Halliday Bonnie Lam, BScHons, PhD Student - White matter changes in frontotemporal dementia Jonathon Kam, MBBS Student, BScMedHons Student - Disturbances in eating and appetite-regulating hormones in frontotemporal dementia Joseph Sofaer, MBBS Student, BScMedHons Student – To determine how pathology in the putamen contributes to frontotemporal dementia

Collaborators

Jillian Kril, Professor of Medicine and Pathology, University of Sydney, Australia Juergen Goetz, Professor of Physiology, Brain and Mind Research Institute, Sydney, Australia Lars Ittner, Senior Research Fellow, Brain and Mind Research Institute, Sydney, Australia John Hodges, ARC Federation Fellow, University of New South Wales, Sydney, Australia Olivier Piguet, NHMRC Career Development Fellow, NeuRA, Sydney, Australia Michael Hornberger, ARC Fellow, NeuRA, Sydney, Australia John Kwok, NeuRA Research Fellow, NeuRA, Sydney, Australia Peter Schofield, Executive Director, NeuRA, Sydney, Australia

Recent Publications:

Loy CT, Kril JJ, Trollor J, Kiernan MC, Kwok JBJ, Halliday GM, Hodges JR (2010) A 48 year- old woman with bizarre and complex delusions. Case report. Nat. Rev. Neurol. 6:175-179.
Piguet O, Petersén Å, Lam BYK, Gabery S, Murphy K, Hodges JR, Halliday GM (2011) Eating disturbances and hypothalamus degeneration in behavioural-variant frontotemporal dementia. Ann. Neurol. 76:253-259.
Piguet O, Halliday GM, Reid WGJ, Casey BJ, Carmen R, Huang Y, Hodges JR, Kril JJ (2011) Clinical phenotypes of autopsy-confirmed Pick’s disease. Neurology 76:253-259.
Schofield EC, Halliday GM, Kwok J, Loy C, Hodges JR (2010) Low serum progranulin predicts the presence of mutations: a prospective study. J. Alz. Dis. 22:981-984.

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