Programme for the 19th international symposium on ALS/MND

SESSION 1 JOINT OPENING SESSION

08.30-08.40 Welcome - P Shaw (UK)
08.40-09.20 Pathways to Phase 1 clinical trials – W Robberecht (Belgium)
09.20-10.00 Communicating science to politicians and public – C Blakemore(UK)  


SESSION 2A CELL BIOLOGY & PATHOLOGY

10.30-11.00 Roles of the blood-brain barrier in neurodegenerative disease – NJ Abbott (UK)
11.00-11.15 A new role for angiogenin in neurite pathfinding and survival: implications for ALS – V Subramanian (UK)
11.15-11.30 Retinoid signalling alterations in ALS and the consequences of these alterations in motor neuron-enriched cultures – C Kolarcik (USA)
11.30-11.45 Metabolic differences between brain and spinal cord mitochondria of wild type and human familial ALS mutant SOD1 transgenic rats – A Panov (USA)
11.45-12.00 Differentially expressed biological processes in relevant spinal compartments isolated by microdissection in SOD1 transgenic mice – J Ravits (USA)
12.00-12.15 Gene expression profiling to investigate the stress effects of physical exercise on the motor neuron transcriptome – L Ferraiuolo (UK)
12.15-12.30 An ALS-associated mutation in VAPB impairs axonal transport of mitochondria - K de Vos (UK)
12.30-12.45 Spastin mutations disrupt axonal transport in hereditary spastic paraplegia - P Kasher (UK)

SESSION 2B MND PHENOTYPES

10.30-11.00 ALS mimic syndromes - K Talbot (UK)
11.00-11.15 Natural history and prognosis of the flail arm and flail leg syndromes – L Wijesekera (UK)
11.15-11.30 The clinical features that distinguish primary lateral sclerosis (PLS) from ALS – P Gordon (USA)
11.30-11.45 Progressive muscular atrophy (PMA) is slow ALS – W-K Kim (Korea)
11.45-12.00 Differentiation of hereditary spastic paraparesis from PLS in sporadic adult-onset upper motor neuron syndromes – F Brugman (Netherlands)
12.00-12.15 Defining survival as an outcome measure in ALS – P Gordon (USA)

12.15-12.30 How well can we predict one and two year survival following diagnosis of ALS? – R Miller (USA)

SESSION 3A TRANSLATIONAL STRATEGIES

14.00-14.30 How can academic labs contribute to therapy development? – C Lipinski (USA)
14.30-15.00 Small molecule screening for MND protection – T Bordet (France)
15.00-15.15 Small molecule activators of the NF2-ARE pathway for treatment of ALS – A Higginbottom (UK)
15.15-15.30 Protein biomarkers for ALS disease progression – R Bowser (USA)

SESSION 3B QUALITY OF LIFE & DECISION MAKING

14.00-14.30 Measuring quality of life in health care - A Tennant (UK)
14.30-14.45 A national survey of quality of life for ALS patients in the United States – Z Simmons (USA)
14.45-15.00 Validation of the shorter ALS-specific quality of life instrument: the ALSSQOL-R – Z Simmons (USA)
15.00-15.15 Giving the diagnosis of ALS and patient choice in Japan – M Ogino (Japan)
15.15-15.30 End of life practices in ALS in the Netherlands – M Maessen (Netherlands)

SESSION 4A TDP-43

16.00-16.30 Functional characterization of TDP-43 mutations found in ALS patients - M Strong (Canada)
16.30-16.45 Distribution of TDP-43 and ubiquitinated intracytoplasmic inclusions in the neuropathic gradiant of neurodegeneration in sporadic ALS – J Ravits (USA)
16.45-17.00 Distinct patterns of TDP-43 and progranulin expression following neuronal injury - K Moisse (Canada)
17.00-17.15 TDP-43 pathology in ALS-linked mutant VAPB transgenic mice – E Tudor (UK)
17.15-17.30 TDP-43 mutations as a cause of ALS - J Sreedharan (UK)

SESSION 4B COMMUNICATION

16.00-16.30 Social aspects of communication - S Bloch (UK)
16.30-17.00 Advances in communication technology - E Horvitz (USA)
17.00-17.30 The Internet, social networking and patient empowerment - P Wicks (UK)

SESSION 5 POSTER SESSION

08.30-11.00 Presenters will attend their posters, in allocated 20-minute slots

POSTER THEMES:

• Therapeutic Strategies
• In Vivo Experimental Models
• In Vitro Experimental Models
• Epidemiology & Genetics
• Human Cell Biology & Pathology
• Improving Diagnosis, Prognosis & Disease Progression
• Imaging, Electrophysiology & Markers of Disease Progression
• Cognitive and Psychological Assessment and Support
• Respiratory & Nutritional Management
• Multidisciplinary Care and Quality of Life

SESSION 6A USE OF THE G93A SOD1 MOUSE IN THERAPEUTIC TESTING

11.30-11.40 Introduction - L Greensmith (UK)
11.40-11.50 Design, power and interpretation of studies in the standard murine model of ALS - S Scott (USA)
11.50-12.00 Refinement of a mouse model of MND and generation of novel readouts for therapeutic assessment - R Mead (UK)
12.00-12.10 Identification of therapeutic cocktails for ALS based on combinations of FDA-approved drugs, nutraceuticals and metabolic precusors - J Crow (USA)
12.10-12.40 PANEL DISCUSSION

SESSION 6B MANAGING ALS/MND IN CLINICAL PRACTICE

11.30-12.00 New approaches to symptom management - J Rosenfeld (USA)
12.00-12.15 Randomized placebo-controlled crossover trial with THC (delta 9-tetrahydrocannabinol) for the treatment of cramps in ALS – M Weber (Switzerland)
12.15-12.30 Evaluation of a ‘fast track’ process for the evaluation and investigation of people with suspected ALS - D Mitchell (UK)

SESSION 7A ROLE OF NON-NEURONAL CELLS

14.00-14.30 Glial cells and neuronal repair: lessons from spinal cord injury – G Raisman (UK)
14.30-14.45 Non-neuronal neuroprotection in ALS using glial restricted precursor transplantation; a novel approach for respiratory neuroprotection – N Maragakis (USA)
14.45-15.00 Focal degeneration of glutamate-vulnerable astrocytes in ALS – D Rossi (Italy)
15.00-15.15 Motor neuron ROS may contribute to astrocyte pathology in a mutant SOD1 rat model of ALS – J Weiss (USA)
15.15-15.30 Riluzole and dexamethasone but not ceftriaxone upregulate glutamate transport activity and expression in striatal astrocytes – M Rattray (UK)

SESSION 7B MULTIDISCIPLINARY CARE MANAGEMENT

14.00-14.30 Use of prospective registers in defining clinical characteristics and improving care provision - A Chio (Italy)
14.30-14.45 Diagnostic process and neurological care in patients with ALS in Spain – J Mora (Spain)
14.45-15.00 Laparoscopic placement of gastrostomy feeding tube (LSCG tube): initial experience – P Nuttleman (USA)
15.00-15.15 Measuring disease progression in advanced ALS: the ALSFRS-R Extension (ALSFRS-EX) – P Wicks (UK)
15.15-15.30 An investigation of emotional stresses experienced by multidisciplinary ALS clinic staff - M Bromberg (USA)

SESSION 7C CLINICAL ELECTROPHYSIOLOGY & IMAGING

14.00-14.30 Concepts and techniques in clinical electrophysiology - M de Carvahlo (Portugal)
14.30-14.45 Motor Unit Number Index (MUNIX): a novel neurophysiological technique to follow disease progression in ALS – C Neuwirth (Switzerland)
14.45-15.00 Facilitation of the jaw-jerk reflex in bulbar onset ALS patients – J Gutierrez (Cuba)
15.00-15.15 Quantitative muscle ultrasonography in ALS – I Arts (Netherlands)
15.15-15.30 Using the clinical phenotype to predict MRI changes in ALS: a 4-Tesla study using fractional anisotropy – J Katz (USA)

SESSION 8A GENETICS

16.00-16.30 Genome-wide association studies in complex diseases: lessons for ALS – J Hardy (UK)
16.30-16.45 Whole genome association study reveals genetic variants that modify survival in sporadic ALS – J Landers (USA)
16.45-17.00 Screening for replication of genome-wide association signals in the Irish and Polish ALS populations – S Cronin (Ireland)
17.00-17.15 Clinical and genetic phenotype of 283 ALS families – P Corcia (France)
17.15-17.30 Ang K17I mutation segregating with autosomal dominant familial ALS in a large Dutch pedigree – M van Es (Netherlands)

SESSION 8B COGNITIVE & PSYCHOLOGICAL CHANGE

16.00-16.15 Rapid screen exam for the detection of frontal and temporal dysfunction syndromes; application to ALS – C Flaherty-Craig (USA)
16.15-16.30 Neuroanatomical correlates of apathy in ALS: a 4 Tesla study using fractional anisotropy – S Woolley (USA)
16.30-16.45 Social and emotional cognition and behaviour: evidence of subclinical frontotemporal dementia in ALS - S Abrahams (UK)
16.45-17.00 Influence of frontotemporal dementia on ALS patients and caregivers on quality of life and depression - A Vignola (Italy)
17.00-17.30 Research into cognitive change in ALS/MND: implications for clinical/care management - L Goldstein (UK)

SESSION 8C BEYOND GUAM: NEW ASPECTS OF THE CYANOBACTERIA/BMAA HYPOTHESIS

16.00-16.15 BMAA as a possible trigger for sporadic ALS/MND: insights from the Chamorro – P Cox (USA)
16.15-16.30 Cyanobacteria, neurotoxicity and water resources – J Metcalf (UK)
16.30-16.45 Production of the neurotoxin BMAA by cyanobacteria throughout the world: implications for human health – H Johnson (USA)
16.45-17.00 In vitro neurotoxicity of the cycad neurotoxin BMAA – J Weiss (USA)
17.00-17.30 New aspects of the cyanobacteria hypothesis and future directions - W Bradley (USA)

SESSION 9A IN VIVO MODELS

08.30-08.45 SMN deficiency accelerates progression in a mouse model of ALS – B Turner (Australia)
08.45-09.00 A novel mutation in glycine tRNA synthetase ameliorates SOD1G93A motor neuron degeneration – E Fisher (UK)
09.00-09.15 Loss of the HSJ1 molecular chaperone exacerbates disease phenotype in SOD1G93A mice – W Mustill (UK)
09.15-09.30 A copper-bis(thiosemicarbazonato) complex delays disease progression and increases survival in a transgenic SOD1G93A mouse model of ALS – C Soon (Australia)
09.30-09.45 Drosophila as a model system to elucidate the molecular mechanisms underlying motor neuron diseases – G Pennetta (UK)
09.45-10.00 A SOD1 missense mutation in dogs with degenerative myelopathy: a spontaneous animal model for ALS – J Coates (USA)

SESSION 9B CLINICAL TRIALS & TRIAL DESIGN

08.30-08.45 Results of a randomized controlled Phase II trial of coenzyme Q10 (coQ10) for ALS – P Kauffman (USA)
08.45-09.00 Subcutaneous insulin-like growth factor type 1 (IGF-1) is not beneficial for patients with ALS in a two-year trial - E Sorenson (USA)
09.00-09.15 Two-year placebo-controlled randomized trial of gene therapy in patients with ALS – M Zakharova (Russia)
09.15-09.30 Pyrimethamine as a therapy for SOD1 associated FALS: early findings - D Lange (USA)
09.30-09.45 A Bayesian model to design two-stage clinical trials in ALS – D Moore (USA)
09.45-10.00 Time to failure clinical trial for promising therapeutics in ALS: a novel design for the best of both worlds – D Schoenfeld (USA)

SESSION 10A SOD1 PATHOGENESIS

10.30-11.00 The potential role of SOD in the pathogenesis of sporadic ALS - J Liu (USA)
11.00-11.15 Modulation of endoplasmic reticulum stress as a potential treatment for ALS – A Walker (Australia)
11.15-11.30 Trafficking from endoplasmic reticulum to Golgi is disrupted in mutant SOD1 expressing cells – J Atkin (Australia)
11.30-11.45 Zinc-deficient monomers are well-populated SOD1 unfolding intermediates : implications for ALS pathogenesis – V Mulligan (Canada)
11.45-12.00 Isolation and proteomic characterization of mutant SOD1-containing inclusion bodies – D Bergemalm (Sweden)
12.00-12.15 ALS2/alsin-deficient SOD1H46R transgenic mice exhibit increased accumulation of insoluble proteins in the spinal cord – S Hadano (Japan)
12.15-12.30 Analysis of the role of dynein mutations in attenuating the phenotype of SOD1G93A transgenic mice – M Hafezparast (UK)

SESSION 10B RESPIRATORY MANAGEMENT

10.30-11.00 The ALS nutrition/NIPPV study - E Kasarskis (USA)
11.00-11.15 Predictive value of respiratory tests for respiratory insufficiency in ALS – S Pinto (Portugal)
11.15-11.30 Factors associated with NIPPV compliance in patients with ALS/MND – C Jackson (USA)
11.30-11.45 Wireless patient monitoring for the control of compliance to NIV in ALS patients: functional and survival outcome – A Pinto (Portugal)
11.45-12.00 Elective termination of non-invasive and mechanical ventilation in ALS – T Meyer (Germany)
12.00-12.30 Results of a prospective multi-center trial of diaphragm pacing in ALS: maintaining diaphragm function and improving survival - R Onders (USA)

SESSION 11 JOINT CLOSING SESSION

14.00-14.20 Symposium Highlights (Basic research)
14.20-14.40 Symposium Highlights (Clinical research and care management)
14.40-15.10 Emerging therapeutic approaches: stem cell strategies - C Svendsen (USA)
15.10-15.40 Vaccination strategies for ALS – J-P Julien (Canada)