Scientific Programme 

Wednesday, 5 September 2012

TIME

HALL A

07:30

Registration opens

08:30-08:45

Opening Address
David Neary
Professor of Neurology
University of Manchester, UK

Genetics
Session Lead: Peter Heutink

08:45-09:15

Plenary lecture
New and old genes for Frontal Temporal Dementia
Peter Heutink
VU University Medical Centre, Netherlands

09:15-09:30

International Genome Wide Association Study on Frontotemporal Dementia: an Update
Raffaele Ferrari, USA

09:30-09:45

Altered epigenetic regulation of progranulin (GRN) expression in Frontotemporal Lobar Degeneration
Julia Strathmann, Germany

09:45-10:00

SQSTM1 Gene Sequencing in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Innocenzo Rainero, Italy

10:00-10:15

Genetic analysis of inherited leukodystrophies: genotype phenotype correlations in the CSF1R gene
Rita Guerreiro, UK

10:15-10:30

Identification of novel causal genes for frontotemporal lobar degeneration using whole genome sequencing
Stéphanie Philtjens, Belgium

10:30-11:00

Coffee Break, Visit Exhibition and Poster Viewing

Clinical Aspects
Session Lead: Keith Josephs

11:00-11.30

Plenary lecture
Reassessing and refining clinical associations within frontotemporal lobar degeneration
Keith Josephs
Mayo Clinic, USA

11:30-11:45

The frontotemporal lobar degenerations as ‘molecular nexopathies’
Jason Warren, UK

11:45-12:00

Sensitivity and Specificity of Behavioural Variant Frontotemporal Dementia Criteria
Claire Gall, UK

12:00-12:15

Physiological phenotyping of auditory emotion processing in canonical syndromes of frontotemporal lobar degeneration
Phillip Fletcher, UK

12:15-12:30

Clinicopathological differences among patients meeting clinical criteria for behavioral variant frontotemporal dementia
Mario Mendez, USA

12:30-14:00

Lunch break, Visit Exhibition and Poster Viewing

Neuropsychology
Session Chair: John Hodges

14:00-14:15

Social Coordination Limitations in Behavioral Variant Frontotemporal Dementia Impacts Communication
Corey McMillan, USA

14:15-14:30

Discrete Neural Correlates for the Recognition of Basic Emotions in Frontotemporal Dementia
Fiona Kumfor, Australia

14:30-14:45

Memories and feelings of music in Semantic Dementia
Sharpley Hsieh, Australia

14:45-15:00

Processing of Pitch, Rhythm and Timbre in Primary Progressive Aphasia
Rik Vandenberghe, Belgium

15:00-15:30

Coffee Break, Visit Exhibition and Poster Viewing

15:30-16:30

Poster Session

Round table session
Mechanisms of Disease in FTLD – a gain, or a loss, of function effect?
Convenor: Glenda Halliday

Supported by Acta Neuropathologica, Springer

16:30-16:40

FUS loss of function 
Ian Mackenzie
University of British Columbia, Canada

16:40-16:50

FUS gain of function
Manuela Neumann
University of Zurich, Switzerland

16:50-17:00

TDP gain of function
Eileen Bigio
Northwestern University, USA

17:00-17:10

TDP loss of function
Nigel Cairns
Washington University School of Medicine, USA

17:20-17:30

tau gain/loss of function
Glenda Halliday
Neuroscience Research Australia, Australia

17.30-18.00

Discussion

19:00-20:30

Welcome reception, Manchester Town Hall sponsored by

Thursday, 6 September 2012

TIME

HALL A

07:30

Registration opens

Cell biology
Session Lead: Christian Haass

08:30-09:00

Plenary lecture
Function and dysfunction of TDP-43 and FUS
Christian Haass
Ludwig-Maximilians-University München (LMU), Germany

09:00-09:15

Investigating changes in alternative splicing and gene expression in stably transfected cells following TDP-43 overexpression and downregulation.
Emanuele Buratti, Italy

09:15-09:30

Uptake and transport of tau conformers by neurons - implications for propagation of tauopathy
Karen Duff, USA

09:30-09:45

Arginine Methylation Modulates Transportin Binding and Nuclear Import of FUS
Dorothee Dormann, Germany

09:45-10:00

Regulation of tau exon 10 splicing: a shRNA screen of the human spliceosome
Sasja Heetveld, Netherlands

10:00-10:30

Coffee Break, Visit Exhibition and Poster Viewing

Management
Session Lead: Martin Rossor

10.30-11.00

Plenary lecture
Treatment and Management – a Major Challenge
Martin Rossor
UCL Institute of Cognitive Neuroscience, UK

11:00-11:15

Problems, burden and needs of 90 German caregivers of patients with frontotemporal lobar degeneration
Janine Diehl-Schmid, Germany

11:15-11:30

Specific FTD carer interventions can reduce carers’ perception of behaviours of concern
Colleen McKinnon, Australia

11:30-11:45

Augmented (8-item) Clinical Dementia Rating Scale is an effective instrument for longitudinal studies of bvFTD and PPA
David Knopman, USA

11:45-12:00

Successful treatment for anomia associated with fronto-temporal dementia
Regina Jokel, Canada

Therapeutics
Session Chair: Martin Rossor

12:00-12:15

Neurofeedback in FTD and PSP Patients: a New Therapeutic Tool in the Treatment of Emotion Recognition? A Proof-of-Concept Study
Hergueta Thierry, France

12:15-12:30

Modulating tau aggregation through inhibition of OGA – a new treatment paradigm for FTD and related tauopathies 
Jon Tinsley, UK

12:30-12:45

Small Molecules Can Rescue Progranulin Haploinsufficiency Underlying Frontotemporal Lobar Degeneration Caused by GRN mutations
Louis De Muynck, Belgium

12:45-13:00

Davunetide (NAP) Influences Growth Cone Microtubules and Protects Microtubule-Dependent Axonal Transport
Illana Gozes, Israel

13:00-4:30

Lunch break, Visit Exhibition and Poster Viewing

Imaging
Session Chair: Nick Fox

14:30-14:45

Alterations in Structural and Functional Brain Connectivity in Presymptomatic Familial Frontotemporal Dementia
Elise G.P Dopper, Netherlands

14:45-15:00

White Matter Helps In Vivo Dissociation Between Tau from TDP-43 in Frontotemporal Degeneration
Corey McMillan, USA

15:00-15:15

Functional Connectivity During Resting State Functional MR Imaging in the Semantic Variant of Primary Progressive Aphasia
Federica Agosta, Italy

15:15-15:30

Primary Progressive Apraxia of Speech versus PPA-Agrammatic: an MRI, FDG-PET and PiB-PET comparison
Jennifer Whitwell, USA

15:30-16:00

Coffee Break, Visit Exhibition and Poster Viewing

16:00-17:00

Poster Session

17:00-18:00

Debate
Frontotemporal Lobar Degeneration and Motor Neurone Disease – a spectrum of underlying pathologies or separate diseases linked by a common protein?

John Hardy
University College London, UK

Chris Shaw
King's College London, UK

Supported by Acta Neuropathologica, Springer

19:00-Late

Invited Faculty Dinner supported by

Carers Dinner sponsored by

Friday, 7 September 2012

TIME

HALL A

07:30

Registration opens

Disease models
Session Lead: Jada Lewis

08:30-09:00

Plenary lecture
Achievements and Challenges in Creating Animal Models of Frontotemporal Dementias
Jada Lewis
University of Florida, USA

09:00-09:15

Progressive Neuronal Inclusion Formation and Neurodegenerative Changes in CHMP2B mutant transgenic mice
Adrian Isaacs, UK

09:15-09:30

Identification of TDP-43 modifying kinases in a C. elegans model of TDP-43 proteinopathy.
Nicole Liachko, USA

09:30-09:45

Disruption of microtubule-dependent transport triggers cytoplasmic aggregation of TDP-43, leading to neurodegeneration in Drosophila 
Nobuhiro Fujikake, Japan

09:45-10:00

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS and FTD disease models
Aaron Gitler, USA

10:00-10:30

Coffee Break, Visit Exhibition and Poster Viewing

Frontotemporal dementia and motor neuron disease
Session Lead: Bryan Traynor

10:30-11:00

Plenary lecture
Frontotemporal dementia and motor neuron disease
Bryan Traynor
National Institute of Aging, USA

11:00-11:15

Characterization of Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis Associated with the GGGGCC Repeat Expansion in C9ORF72
Brad Boeve, USA

11:15-11:30

The C9ORF72 expansion mutation has a single European founder that arose from a backgrounf haplotype associated with repeat instability
Christopher Shaw, UK

11:30-11:45

Comparisons of clinical features in FTD due to GRN and C9ORF72 mutations
Ging-Yuek Hsiung, Canada

11:45-12:00

Longitudinal neuroimaging and neuropsychological profiles of frontotemporal dementia with the C9ORF72 mutation
Colin Mahoney

12:00-13:00

Lunch break, Visit Exhibition and Poster Viewing

Motor neuron disorders
Session Chair: Pam Shaw

13:00-13:15

Age-dependent penetrance and anticipation in ALS+/-FTLD kindreds due to C9ORF72 hexanucleotide intronic repeat expansion mutations (C9HIREM)
Christopher Shaw, UK

13:15-13:30

Semantic Dementia with Primary Lateral Sclerosis (SD-PLS): A Variant of FTLD-TDP Type C Pathology
Keith Josephs, USA

13:30-13:45

Subphenotypes on the ALS-FTD Spectrum: The Contribution of FTD Subtype Features to the Cognitive Impairment in ALS
Egberdina-Józefa van der Hulst, UK

13:45-14:00

VALUES: A National Multicenter Study of Gender Differences in the Behavioral Variant of Frontotemporal Disease in Amyotrophic Lateral Sclerosis.
Claire Flaherty-Craig, USA

Hot Topics
Session Chair: John Hardy

14:00-14:15

Large C9ORF72 hexanucleotide expansions arise spontaneously in the healthy population but can be distinguished from pathogenic mutations by Sothern Blotting
Simon Mead

14:15-14:30

A deletion mutant in the C. elegans orthologue of C9orf72 provides an animal model for studies on FTLD/ALS
David Sattelle

14:30-14:45

Phosphorylated TDP-43 protein levels are elevated in plasma of frontotemporal dementia patients with a C9ORF72 expansion or PGRN mutations.
Marc Suárez-Calvet

14:45-15:00

RNA-binding proteins with prion-like domains in FTD and beyond.
James Shorter

15:00-15:15

The roles of SORT1 and TDP-43 in regulating PGRN levels in the brain
Leonard Petrucelli

15:15

Closing Ceremony and Prizes

Supported by Acta Neuropathologica, Springer