‘Challenges and Complexities’ – 22^nd October, Bateman’s Bay  click here for Programme

The FRS has already received a lot of international attention, after it was published in the high-ranking American medical journal Neurology, including a podcast with Dr. Mioshi which can be downloaded from here (May 18 2010 issue).

The FRS can be downloaded for free from our website and is available to all health professionals. Click here to go to the download page

To download the newsletter please click the link below:

AFTD newsletter

Successful grants 2009

Our group had a very successful year (2009) in terms of securing more research money for our frontotemporal dementia research. Here a few of our 2009 grant successes by members of the group:

•  ARC Discovery Project Grant. Prefrontal and medial temporal lobe cortices’ interactions to episodic long-term memory.  2010-2012.
•  NHMRC Project Grant. Progressive aphasia and amyloid deposition: a multidisciplinary approach to improving dementia diagnosis.  Joint with Prof C Rowe and colleagues in Melbourne. 2010-2012.
• NHMRC Project Grant. The impact of circadian disturbances on sleep quality, cognition and psychiatric symptoms in neurodegenerative disease.  Joint with Dr S Lewis at Sydney University. 2010-2012.
•  ARC Discovery Project Grant. Emotion in voice matters: advancing a neural model of auditory emotion perception. Joint with Prof S MacDonald UNSW. 2010-2013. 
• ARC LIEF (Linkage Infrastructure, Equipment and Facilities) Grant. State-of-the-art upgrade to multi-transmit multi-receive technology for research dedicated 3 Tesla MRI scanner. 2010.
• UNSW Early Career Research Grant to Dr Michael Hornberger.  Dissecting behavioural disturbances associated with orbitofrontal cortex dysfunction in frontotemporal dementia and bipolar disorder. 2010-2011

This gives a summary of our groups activities and achievements for 2009.

2009 Newsletter

We have developed a new online survey which investigates the impact of frontotemporal dementia and related disorders on the children of patients.

The survey takes ~15mins to complete and will give us invaluable information of the impact of FTD on the family.

To take the survey just follow the link below. If you do not have the password please contact Eneida: e.mioshi@neura.edu.au

Click Here to take survey

Authors:         Hornberger, M., Piguet, O., Graham, A., Nestor, P.J., Hodges, J.

Journal:         Neurology

Abstract:

Objectives: Studies have shown variable memory performance in behavioral variant frontotemporal dementia (bvFTD) patients. Our study investigated whether this variability is due to the admixture of patients with true bvFTD and phenocopy patients. We also sought to compare bvFTD and Alzheimer disease (AD) patients’ performance.

Methods: We analyzed neuropsychological memory performance in patients with a clinical diagnosis of bvFTD divided into those who progressed (N = 50) and those who remained stable (N = 39), AD patients (N = 64) and healthy controls (N = 64).

Results:Progressive bvFTD patients were impaired on most memory tests to a similar level to that of early AD patients. Findings from a subset of progressive bvFTD patients with confirmed FTLD pathology (N = 10) corroborated these findings. By contrast, phenocopy bvFTD patients performed significantly better than progressors and AD. Logistic regression revealed that bvFTD patients can distinguished to a high degree (85%) on the immediate recall score of a word list learning test (Rey Auditory Verbal Learning Test).

Conclusions: Our results provide evidence for an underlying memory deficit in “real” or progressive bvFTD similar to AD, though the groups differ in orientation scores with bvFTD patients being intact. Exclusion solely based on impaired neuropsychological memory performance can potentially lead to an underdiagnosis of FTD.

Authors:  Eneida Mioshi, John Hodges

Journal:  Dementia and Geriatric Cognitive Disorders

Abstract:

Background: frontotemporal dementia (FTD) causes progressive change in everyday functioning. The rate of decline in activities of daily living (ADLs) has not been investigated for the three major FTD variants. This study aimed to (1) examine changes on the Disability Assessment for Dementia (DAD) in the three FTD variants [behavioural variant FTD (bvFTD), semantic dementia (SemDem) and progressive nonfluent aphasia (PNFA)].; (2) identify if deterioration of ADL abilities would be more prominent in initiation, planning or execution; (3) see if bvFTD phenocopy patients can be identified by these measures and (4) verify if changes in DAD and ACE-R scores are associated.

Methods: A total of 72 DAD assessments were analysed; 36 at baseline and 36 at follow up (bvFTD=16, subdivided into bvFTD pathological and phenocopy on the basis of their presenting MRIs; SemDem=11; PNFA=9).

Results: True bvFTD pathological, SemDem and PNFA groups showed significant decline on the DAD after 12 months, whereas on the ACE-R only the bvFTD pathological group reached significance. In terms of subcomponents of ADLs, SemDem patients had significant decline in planning, whereas the bvFTD pathological group declined significantly in all subcomponents; PNFA was intermediate. The decline in DAD and ACE-R scores were significantly correlated.

Conclusions: FTD variants show differential annual rates of functional decline. Those with the bvFTD phenocopy syndrome can be clearly differentiated within a year of presentation. Patients with PNFA show marked decline that is not easily explained by their language deficits. The rate of decline should be taken into consideration when discussing prognosis.

Authors: John R. Hodges, Jo Mitchell, Kate Dawson, Maria Grazia Spillantini, John H. Xuereb, Paul McMonagle, Peter J. Nestor and Karalyn Patterson

Journal: Brain

Abstract:

A great deal has been written about cognitive aspects of semantic dementia but little is known about the demography orprognosis. We describe these features in a consecutive series of 100 patients seen over a 17-year period; all cases were assessed and followed up in a specialist clinic. The mean age at diagnosis was 64.2 ( +/-7.1) range 40–79 years, but 46 presented after age 65 and 7 after 75; a higher proportion than the existing literature might predict. Fifteen had a first-degree relative with dementia, but in seven this was almost certainly unrelated. Only two had relatives with young-onset dementia. There were no families with more than two affected members. The familial rate was estimated at between 2% and 7% (95% confidence interval 0–12%). Kaplan–Meier analyses indicated a 50% survival of 12.8 years (95% confidence interval 11.9–13.7); a more benign course than suggested by neuropathologically based studies. We were unable to identify any factors influencing survival. Of the 100, 34 have died, with pathological confirmation in 24; 18 had frontotemporal lobar degeneration with ubiquitin-positive inclusions (13 of 13 confirmed TAR DNA binding protein-43 positive), and 3 had classic tau-positive Pick bodies and 3 had Alzheimer’s pathology. The age at diagnosis or death across the pathological subgroups was equivalent. Although semantic dementia has a strong statistical association with ubiquitin-positive pathology, it does not have the signature of familial frontotemporal lobar degeneration with ubiquitin-positive inclusions, notably the presence of intranuclear lentiform TAR DNA binding protein-43 inclusions.
The age of onset is older than predicted and the course more slowly progressive than suggested by earlier studies of small groups of subjects.