10th International Conference on Frontotemporal Dementias

August 31-September 2, 2016 Munich/Germany

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Scientific Program

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12.15-12.30 Opening
  Janine Diehl-Schmid (Munich/DE), Manuela Neumann (Tübingen/DE)
Session 1 “FTLD: current perspectives - future directions”

Julie Snowden (Manchester/GB), Mario Mendez (Los Angeles, CA/US)
12.30 – 12.55 S1 Julie Snowden (Manchester/GB):
Changing perspectives on frontotemporal dementia
12.55 - 13.20 S2William Seeley (San Francisco, CA/US):
Frontotemporal dementia neuroanatomy: onset, spread, and implications for treatment
13.20 - 13.35 O1 James Galvin (Boca Raton, FL/US):
Social and Economic Burden of Frontotemporal Degeneration
13.35 – 13.50 O2 Brad Dickerson (Charlestown, MA/US):
Toward tau PET imaging in FTLD: An update
13.50 - 14.20 Break
Session 2 Social cognition and behavior: challenges and interventions
Chairs: Adrian Danek (Munich/DE), Mario Mendez (Los Angeles, CA/US)
14.20 - 14.45 S3 Bruno Dubois (Paris/FR):
Current concepts of social cognition and assessment in FTD
14.45 - 15.10 S4 Katherine Rankin (San Francisco, CA/US):
Empathy and emotional processing in bvFTD
15.10 - 15.25 O3 Chiadi Onyike (Baltimore, MD/US):
An analysis of the trajectories of illness severity measurements in behavioral variant frontotemporal dementia
15.25 - 15.40 O4 Janine Diehl-Schmid (Munich/DE):
Phenotype, neuropsychology and psychopathology of C9orf72 mutation carriers from the German FTLD-Consortium
15.40 - 15.42 DB1 David Perry (San Francisco, CA/US):
Reward deficits in behavioral variant frontotemporal dementia include insensitivity to negative stimuli
15.42 - 15.44 DB2 Charles Marshall (London/GB):
Embodied Cognition of Dynamic Emotion in Frontotemporal Lobar Degeneration
15.44 – 15.46 DB3 Janne Papma (Rotterdam/NL):
Longitudinal decline in cognitive functioning in presymptomatic frontotemporal dementia
15.46 -15.48 DB4 Fiona Kumfor (Syndey/AU):
The impact of context on emotion decoding in frontotemporal dementia
15.48 - 15:50 DB5 Everard Vijverberg (Amsterdam/NL):
Neuropsychological profiles in behavioural variant Frontotemporal Dementia and Psychiatric disorders
15.50 – 15.52 DB6 Chiadi Onyike (Baltimore, MD/US):
Frontotemporal Dementia in Patients of African Descent
15.55 - 17.30 Break, Poster Session (Caregiver, Social Cognition) and Exhibits
Session 3 Genetics of FTLD
Chairs: Peter Heutink (Tuebingen/DE), Christine van Broeckhoven (Antwerp/BE)
17.30 - 17.55 S5 Stefan Züchner (Miami, FL/US):
Next generation sequencing approaches in ALS and related disorders
17.55 - 18.10 O5 Carol Dobson-Stone (Sydney/AU):
Identification Of A Novel FTD-MND Gene On Chromosome 16
18.10 – 18.25 O6 Cyril Pottier (Jacksonville, FL/US):
Global initiative to identify genetic modifiers of disease onset and presentation in patients with progranulin mutations
18.25 - 18.27 DB7 Rita Cacace (Antwerp/NL):
Loss of function mutations in dipeptidyl peptidase 6 in Alzheimer disease and Frontotemporal dementia: a novel pathway in neurodegeneration
18.27 - 18.29 DB8 Katharina Dick (London/GB):
Symptom Onset in Genetic Frontotemporal Dementia
18.29 - 18.31 DB9 Elisa Rubino (Turin/IT):
ATXN2 polyQ intermediate repeats influence Frontotemporal Lobar Degeneration phenotype
18.31 - 18.33 DB10 Aniket Mishra (Amsterdam/NL):
Gene-based analysis detects novel loci associated with frontotemporal dementia and its clinical subtypes
18.35 - 19.00 S6 Peter Heutink (Tuebingen/DE):
Panel discussion: How do we move from large scale sequencing efforts in genetics to biology?
Stefan Züchner (Miami, FL/US), Christian Haass (Munich/DE), Aaron Gitler (Standford, CA/US)
19:30 Welcome reception
Session 4 Molecular neuropathology of FTLD
Chairs: Manuela Neumann (Tuebingen/DE), Ian Mackenzie (Vancouver/CA)
8.30 - 8.55 S7 Ian Mackenzie (Vancouver/CA):
Molecular neuropathology of FTLD with focus on C9orf72 mutations
8.55 - 9.20 S8 Glenda Halliday (Sydney/AU):
Evidence from human studies for spreading/staging of TDP-43 pathology in FTLD and ALS
9.20 - 9.35 O7 John Trojanowski (Philadelphia, PA/US):
New Models For Alzheimer's And other Related Tauopathies By Transmission Of Neuronal And Glial Tau Pathology
9.35 - 9.50 O8 Sang Wong Seo (San Francisco, CA/US):
Clinicopathological correlations in early vs. late age-of-onset frontotemporal dementia and frontotemporal lobar degeneration
9.50 - 9.52 DB11 Laura Taylor (Seattle/US):
TTBK1/2 hyper-activation drives pTau and pTDP accumulation
9.52 - 9.54 DB12 David Irwin (Philadelphia, PA/US):
Cerebrospinal fluid phosphorylated tau levels are associated with post-mortem tau inclusion burden in frontotemporal lobar degeneration
9.54 - 9.56 DB14 Stephanie E. Gaus (San Francisco, CA/US):
Abundant lower motor neuron RNA foci with perinucleolar studding in C9orf72 FTD-ALS
10.00 - 10.30 Break
Session 5 Progressive aphasias
Chairs: Marilu Gorno-Tempini (San Francisco, CA/US),  Peter Nestor (Magdeburg/DE)
10.30 - 10.55 S9 Maya Henry (Austin,TX/US):
Apraxia of speech and its rehabilitation
10.55 - 11.20 S10 Peter Garrard (London/GB):
The right hemisphere and its semantics
11.20 – 11.35 O9 Edoardo Spinelli (Milan/IT and San Francisco, CA/US):
In vivo correlates of pathological diagnosis in clinical variants of primary progressive aphasia
11.35 – 11.50 O10 Kyrana Tsapkini (Baltimore, MD/US):
Imaging tDCS effects in primary progressive aphasia
11.50 – 11.52 DB15 Ana Sofia Costa (Braga/PT):
Bilingualism in primary progressive aphasia: systematic review and case reports from two European centers
11.52 – 11.54 DB16 Jennifer Whitwell (Rochester, NY/US):
Differential binding of AV-1451 tau-PET across variants of primary progressive aphasia
 11.54 – 11.56 DB17

Jolien Schaeverbeke (Leuven/BE):
Deficits in Rhythm Processing in PPA are linked to SMA atrophy

11.56 – 11.58 DB18 Clara Sanches (Paris/FR):
Anatomo-functional segregation of the mental lexicon: the model of Primary Progressive Aphasia
12.00 - 14.00 Break, Poster Session (Genetics, Neuropathology, Progressive aphasias) and Exhibits
Session 6 CBS, PSP, and MND
Chairs: Günter Höglinger (Munich/DE), Albert Ludolph (Ulm/DE)
14.00 - 14.25 S11 Günter Höglinger (Munich/DE):
PSP & CBD: overlaps and distinctions within the FTLD spectrum
14.25 - 14.50 S12 Albert Ludolph (Ulm/DE):
ALS - Pathology, genotype and phenoptype
14.50 - 15.05 O11 Julio Rojas (San Francisco, CA/US):
Plasma neurofilament light chain predicts disease progression in progressive supranuclear palsy
15.05 – 15.20 O12 Corey McMillan (Philadelphia, PA/US):
Converging Multimodal Evidence of Myelin Associated Proteins As Biomarkers in Four-Repeat Tauopathy
15.20 – 15.22 DB19 Zhangyu Zou (Fuzhou/CN):
Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis
15.22 – 15.24 DB20 Patricia Vazquez Rodriguez (Cambridge/GB):
[18F]AV-1451 PET imaging differentiates the tauopathies of Progressive Supranuclear Palsy (PSP) and Alzheimer’s disease (AD)
15.24 – 15.26 DB21 James Rowe (Cambridge/GB):
On The Clinical Overlap Between Progressive Supranuclear Palsy and Corticobasal Syndrome: evidence from the PiPPIN epidemiology study
15.26 -15.28 DB22 Angela Rosenbohm (Ulm/DE):
Clinical comparison of FTLD and ALS-FTD patients – is there a difference?
15.30 - 16.00 Break
Session 7 Biomarkers for FTLD
Chairs: Markus Otto (Ulm/DE), Alessandro Padovani (Brescia/IT)
16.00 - 16.25 S13 Jason Warren (London/GB):
New physiological biomarkers of FTLD
16.25 - 16.50 S14 John van Swieten (Rotterdam/NL):
Blood and CSF biomarkers in the presymptomatic stage of hereditary FTD
16.50 - 17.05 O13 Howard Rosen (San Francisco, CA/US):
Data-driven Regions of Interest for Longitudinal Change in Three Variants of Frontotemporal Lobar Degeneration
17.05 – 17.20 O14 Martina Bocchetta (London/GB): Multimodal imaging analysis of C9orf72-associated FTD in the Genetic Frontotemporal dementia Initiative (GENFI) study
17.20 – 17.22 DB23 Joery Goossens (Antwerp/BE):
Increased CSF levels of biomarkers for neurodegeneration in FTLD-GRN mutation carriers
17.22 – 17.24 DB24 Liza Jiskoot (Rotterdam/NL):
Presymptomatic structural connectivity loss in familial frontotemporal dementia – a four year follow-up study of Dutch FTD pedigrees
17.24 – 17.26 DB25 Raphael Schneider (Toronto/CA):
Exosomal microRNAs in the cerebrospinal fluid as biomarkers in familial FTD
17.26 – 17.28 DB26 Claudia Jacova (Hillsboro, OR/US):
Brain glucose metabolic abnormalities differ between predementia carriers of GRN and C9ORF72 mutations
19.00 Social Evening
Session 8 Hot topics
Chairs: Glenda Halliday (Sydney/AU), Masato Hasegawa (Tokyo/JP)
8.30 - 8.45

Francisco Baralle (Trieste/IT)
New insights into the role of TDP 43 expression, structural features and aggregation in disease pathogenesis      

8.45 – 9.00

Tom Kukar (Atlanta, GA/US)
Granulins (GRNs) are endogenously produced in the lysosome: Implications for the pathogenesis and treatment of FTD-GRN

9.00 – 9.15 O15 Gernot Kleinberger (Munich/DE):
A mouse model for FTD-like syndrome due to TREM2 mutations
9.15 – 9.17 DB27 Benjamin Schwenk (Munich/DE):
TDP-43 loss of function inhibits endosomal trafficking and alters signaling in neurons
9.17 – 9.19 DB28 Erik Roberson (Birmingham, AL/US):
Neuronal Progranulin Overexpression Reverses Social Deficits in Progranulin-insufficient Mice
9.19 – 9.21 DB29 Christopher Holler (Atlanta, GA/US):
Trehalose enhances progranulin expression in progranulin-deficient patient cells and mouse brain
9.21 – 9.23 DB30 Daniel Bittner (Magdeburg/DE):
Chloroquine rescues Cerebrospinal fluid progranulin deficiency in FTD
09:30 - 10.00 Break
Session 9 Mechanisms of cell death
Chairs: Dieter Edbauer (Munich/DE),  Adrian Isaacs (London/GB)
10.00 – 10.25 S15 Virginia Lee (Philadelphia, PA/US):
Insights on motor neuron degeneration in a mouse model of TDP-43 proteinopathy
10.25 – 10.40 O16 Fen-Biao Gao (Worcester, MA/US):
Insights from iPSC and Drosophila Models of C9ORF72‐Related ALS/FTD
10.40 – 10.55 O17 Elaine Liu (Philadelphia, PA/US):
Loss of TDP-43 Contributes to Non-coding RNA Mediated Toxicity
10.55 – 10.57 DB31 Chiara Valori (Tuebingen/DE):
Fus regulates the transcriptome of the developing mouse brain through several mechanisms
10.57 – 10.59 DB32 Sander Beel (Leuven/BE):
Progranulin is neurotrophic in vivo, stimulating functional recovery after nerve crush injury
10.59 – 11.01 DB33 Patrizia Rizzu (Tuebingen/DE):
C9orf72 differential expression in brain and monocytes suggests a possible role for C9orf72 in monocyte function
11.01 – 11.03 DB34 Sami Baramada (Ann Arbor, MI/US):
RNA destabilization in frontotemporal lobar dementia and amyotrophic lateral sclerosis
11.05 -11.30

S16 S17

Dieter Edbauer (Munich/DE), Adrian Isaacs (London/GB):
Panel discussion: What is the toxic species for C9FTD/ALS?
Christine von Broeckhoven (Antwerp/BE), Don Cleveland (San Diego, CA/US), Ian Mackenzie (Vancouver/CA)
11:30 – 13:30 Break, Poster Session (CBS, PSP, MND, Blood and Imaging biomarkers,
Mechanisms of cell death, Disease modulation, Treatment) and Exhibits
Session 10 Disease modulation
Chairs: Christian Haass (Munich/DE), Don Cleveland (San Diego, CA/US)
13.30 - 13.55 S18 Don Cleveland (San Diego, CA/US):
Gene silencing therapy for human neurodegenerative disease
13.55 - 14.20 S19 Aaron Gitler (Stanford, CA/US):
Therapeutic reduction of ataxin 2 improves motor performance and extends lifespan in a mouse model of TDP-43 proteinopathy
14.20 – 14.35 O18 Emanuele Buratti (Triest/IT):
hnRNP proteins as general enhancers or suppressors of TDP-43 gain- and loss-of-function pathological properties
14.35 – 14.50 O19 Alexandra Nicholson (Rochester, NY/US):
Reducing levels of Tmem106b as a therapeutic strategy in an FTLD-TDP mouse model
14:50- 15.10 Break
Session 11 Treatment
Chairs: Adam Boxer (San Francisco, CA/US), Jon Rohrer (London/GB)
15.10 – 15.20 S20 Jon Rohrer (London/GB), Adam Boxer (San Francisco, CA/US): Introduction
15.20 - 15.35 O20 Howard Feldman (San Diego, CA/US):
A phase 3 trial of the tau and TDP-43 aggregation inhibitor, leuco-methylthioninium-bis(hydromethanesulfonate) (LMTM), for behavioural variant frontotemporal dementia (bvFTD)
15.35 – 15.50 O21 Adam Boxer (San Francisco, CA/US):
An 8-week, Open-label, Dose-Finding Study of Nimodipine for the Treatment of Progranulin Insufficiency from GRN Gene Mutations
15.50 – 16.05 O22 Edward Huey (New York, NY/US):
Clinical trial of COMT-inhibition in 28 patients with FTD
16.05 – 16.40 Panel discussion: Update and future directions on clinical trials in FTD and associated disorder
Philipp von Rosenstiel, (Cambridge, MA/US), Christoph Wiessner (Lausanne/CH), Norbert Zilka (Bratislava/SK)
Hans Moebius (Baar/CH), Howard Feldman (La Jolla, CA/US), Edward Huey (New York, NY/US)
16.30 - 17.00 Closing Remarks