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Research on frontotemporal
dementia
Professors Halliday and Kril
have started a large collaborative study with Professor John
Hodges from Cambridge in the UK on people with frontotemporal
dementia. This has already resulted in the design of a new
way to assess the progression of the disease and to analyse
its natural history. This work provides patients and their
families and carers with better information to assist in planning
and managing their lives. We are also assessing familial
forms of this disease.

Until recently, very little was known
about frontotemporal dementia, as it was thought to be a rare
form of dementia. We now know it is more common than
previously thought, and our work has developed a scheme to
stage the disease based on the assessment of simple brain
images (see figure). This assists both patients and clinicians
in managing the disease and planning for the future.
The purpose of our ongoing studies is to
investigate the pathological differences and similarities
between the different clinical subtypes of frontotemporal
dementia. Furthermore, we will investigate the changes in
brain atrophy which occur over the course of the disease to
allow us to understand better the initial focus of the disease.
We will also evaluate the role of cellular protein changes
(ubiquitin and tau) in the pathogenesis of neuronal death.
This research will allow us to better diagnose and characterise
frontotemporal dementia. In addition, we will be able to establish
common mechanisms of neurodegeneration in the subtypes of
frontotemporal dementia. This information is required in order
to develop future therapeutic treatments.
How can I help this research? By
participating in our brain
donor program (contact 02 9036 7117).
Key researchers
Glenda
Halliday
Collaborators
Jillian Kril (University of Sydney, Concord Hospital), Cindy
Kersaitis (University of Sydney, Concord Hospital), John Hodges
(University of Cambridge, UK), Helen Creasey (Concord Hospital)
and Diana Caine (University of Sydney).
Key publications
Broe M, Kril J, Halliday GM (2004) Astrocytic degeneration
relates to the severity of disease in frontotemporal dementia,
Brain 127:2214-2220
Hodges JR, Davies R, Xuereb J, Casey B, Broe M, Bak T, Kril
J, Halliday GM (2004) Clinicopathological correlates in
frontotemporal dementia, Annals of Neurology 108:515-523
Kersaitis C, Halliday GM, Kril JJ (2004) Regional and
cellular pathology in frontotemporal dementia: Relationship to
stage of disease, Acta Neuropathologica 108:515-523
Piguet O, Brooks WS, Halliday G, Schofield PR, Stanford PM,
Kwok J, Spillantini MG, Yankopoulou D, Nestor PJ, Broe GA,
Hodges JR (2004) Similar early clinical presentations in
familial and non-familial frontotemporal dementia, Journal of
Neurology, Neurosurgery and Psychiatry 75:1743-1745
Graham A, Davies R, Xuereb J, Halliday G, Kril J, Creasey
H, Graham K, Hodges J (2005) Pathologically proven
frontotemporal dementia presenting with sever amnesia, Brain
128:597-605
Kril JJ, Macdonald V, Patel S, Png F, Halliday GM (2005)
Distribution of brain atrophy in behavioural variant
frontotemporal dementia, Journal of Neurological Sciences
232:83-90
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