Table of Content – Abstracts ENCALS Meeting 2021

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Table of Content – Abstracts ENCALS Meeting 2021 Table of Content – Abstracts ENCALS Meeting 2021 Abstract Title & Author(s) Page number A Blinded Interim Update on RESCUE-ALS: A Randomized, Placebo-Controlled, Phase 2 Study to 21 Determine the Effects of CNM-Au8 to Slow Disease Progression in Amyotrophic Lateral Sclerosis Glanzman R1, Steve Vucic, PhD, MBBS (Hons I), DSc, FRACP, FAHMS2 S2, Mahoney, PhD, MB, MRCPI C3,5, Rynders A1, Ho, PhD, MSc K1, Evan J1, Hotchkin M1, Kiernan, PhD, MBBS, BSc (Hons), FRACP M3,4 A Knowledge-Based Machine Learning Approach to Gene Prioritisation in Amyotrophic Lateral 23 Sclerosis Hu J1,2, Lepore R3, Dobson R1,4,5, Al-Chalabi A2,6, Bean D1,4, Iacoangeli A1,2,7 A national survey exploring the nutritional management of people with ALS delivered by 25 Dietitians in the UK White S1, Zarotti N2, Beever D3, Bradburn M3, Norman P4, Coates E3, Stavroulakis T2, White D3, McGeachan A2, Williams I4, Hackney G3, Halliday V3, McDermott C2 A Phase 1, Multicenter, Open Label, Single-Ascending Dose Study to Evaluate Safety, Tolerability, 26 and Pharmacokinetics of AP-101 in Familial and Sporadic Amyotrophic Lateral Sclerosis (ALS) Genge A, Bowden E, Berube M, Zinman L, Showsmith C, Salzmann M, Schott R, Boggs B, Clermont P, Bingham J, Bowden E A preliminary comparison between ECAS and ALS-CBS in classifying Amyotrophic Lateral Sclerosis 27 cognitive-behavioural changes in a cohort of non-demented patients. Lizio A1, Lizio A1, Casiraghi J1, Sansone V2, Tremolizzo L3, Riva N4, Filippi M6, Solca F8, Torre S9, Ticozzi N8, Silani V8, Poletti B9, Lunetta C1 A systematic review of the utility of wearable devices for monitoring motor progression in 28 amyotrophic lateral sclerosis. Beswick E1, Fawcett T1, Hassaan Z1, Forbes D1, Newton J1, Dakin R1, Weaver C1, Chandran S1, Pal S1 Abnormal microstate resting-state EEG characteristics in ALS disease 29 Metzger M1, Dukic S1,2, McMackin R1, Giglia E1, Mitchell M1, Bista S1, Peelo C1, Tadjine Y1, Sirenko V1, Coffey A1, McManus L1, Heverin M1, Bede P1, Muthuraman M3, Pender N1,4, Hardiman O1,4, Nasseroleslami B1 1 Adoption and Implementation of the Sheffield Telemedicine in MND (TiM) System in the Irish 30 MND Service Murray D1,2, Galvin M1, Heverin M1, Kennedy P1, Peelo C1,2, Grogan O2, Knox L4, Hobson E3,4, McDermott C3,4, Hardiman O1,2 Age-dependent cytoskeletal shifts in human sensory nerve fibers 31 Metzner K1,2, Gaur N1, Wang M2, Rödiger A1, Steinbach R1, Stubendorff B1, Axer H1, Brill M2, Grosskreutz J1 ALS in Emilia Romagna Region: epidemiological, clinical and genetic features in a prospective 32 population-based study in the last decade. Gianferrari G1, Martinelli I1,2, Zucchi E1, Simonini C1,2, Fini N2, Canali E3, Sette E4, Pugliatti M4, Salvi F5, Liguori R6, Vacchiano V6, Zinno L7, Santangelo M8, De Pasqua S6, Casmiro M9, Terlizzi E10, Medici D11, CurròDossi M12, Patuelli A13, Morresi S14, Vinceti M15, Mandrioli J1,2 ALS subgroups revealed by clustering neuroimaging patterns of brain degeneration 33 Tan H1, Westeneng H1, Meier J1, Van der Burgh H1, Nitert A1, Van Veenhuijzen K1, Van Es M1, Veldink J1, Van den Berg L1 ALSFRS-r scale increases arterial blood gas analysis’ sensitivity in assessing pulmonary function in 34 Amyotrophic Lateral Sclerosis. Torrieri M1, Manera U1, Mora G2, Canosa A1,3, Vasta R1, Mattei A4, Moglia C1,3, Calvo A1,3, Chiò A1,3 Amyotrophic lateral sclerosis – Riga East University Hospital patients data characteristics. 35 Tihonovs J1, Karelis G1,2, Pastare D1,3, Polunosika E1,3, Kande L1 Antibodies-based therapy to overcome TDP-43 proteinopathy. 36 Pozzi S1,3, Thammisetty S1, Codron P2, Soucy G1, Rahimian R1, Plourde K1, Renaud L1, Cordeau P1, Dutta K1, Bareil C1, Phaneuf D1, Kriz J1,3, Gravel C1,3, Julien J1,3 Anxiety predicts survival more than depression in Amyotrophic Lateral Sclerosis 37 Matteoni E1, Manera U1, Iazzolino B1, Peotta L1, Canosa A1,2, Vasta R1, Palumbo F1, Torrieri M1, Grassano M1, Moglia C1,2, Calvo A1,2, Chiò A1,2 APPLICATIONS OF SURFACE EMG ARRAYS FOR IDENTIFICATION AND TRACKING OF MOTOR UNITS 38 IN AMYOTROPHIC LATERAL SCLEROSIS McManus L1, Liegey J2, Senneff S2, Lowery M2, Hardiman O1, Nasseroleslami B1 2 Are psychiatric symptoms in people with MND and their kindreds associated with cognition and 39 behaviour in people with MND? McHutchison C1,2, McIntosh A3,4, Ryan M5, Costello E5, Heverin M5, Newton J2, Pal S2,4, Chandran S2,4, Haridman O5,6, Abrahams S1,2 ATXN2 intermediate CAG expansion in Catalonian ALS population 40 Domínguez-Rubio R1, Padró-Miquel A2, Marco-Cazcarra C1, Alia-Ramos P2, Povedano-Panadés M1 ATXN2 phenotype-genotype study in ALS Spaniard patients 41 Borrego Hernández D1, Palma-Guzmán P1, Expósito-Blázquez L1, Herrero-Manso M2, Cordero- Vázquez P1, Villarejo-Galende A3, Martín-Casanueva M4, Esteban-Pérez J1, García-Redondo A1 Benchmarking and using bioinformatics tools for the detection of HERVK insertions in ALS whole- 43 genome sequencing data. Bowles H1,2, Al Khleifat A1, Jones A1, Swanson C3, Al-Chalabi A1, Iaocoangeli A1,2 Blood lipid levels and the risk of amyotrophic lateral sclerosis: a prospective population-based 44 cohort analysis Thompson A1, Turner M1, Talbot K1 Brain 18F-FDG-PET signature of SOD1 and TARDBP ALS patients 45 Canosa A1,2, Manera U1, Vasta R1, Di Pede F1, Grassano M1, Brunetti M1, Moglia C1,2, Calvo A1,2, Pagani M3,4, Chiò A1,2 Brain architecture changes across the FTLD spectrum 46 Cividini C1,6, Basaia S1, Spinelli E1,6, Canu E1, Castelnovo V1,6, Riva N4, Cecchetti G1,2,6, Caso F2, Magnani G2, Falini A5,6, Filippi M1,2,3,4,6, Agosta F1,2,6 Calculating variant penetrance using family history of disease and population data 47 Spargo T1, Opie-Martin S1, Lewis C2,3, Iacoangeli A1,4,5, Al-Chalabi A1,6 Cerebrospinal Fluid Heavy Neurofilaments Discriminate Motor Neuron Diseases with Upper 48 Motor Neuron involvement Simonini C1, Zucchi E1, Bedin R1, Martinelli I1, Gianferrari G1, Fini N2, Sorarù G3, Liguori R4, Vacchiano V4, Mandrioli J1 Chitinase Dysregulation in ALS: Novel insights on Expression Dynamics from a Clinical Cohort and 49 Murine Models Gaur N1, Wang M3, Steinbach R1, Riemenschneider H5, Dreger M1, Vlad B1, Stubendorff B1, Edbauer D4,5,6, Witte O1,2, Leischner-Brill M3,4, Grosskreutz J1,2 3 Circulating miR-181 is a prognostic biomarker for amyotrophic lateral sclerosis 50 Magen I1,2, Yacovzada N1,2, Yanowski E1,2, Coenen-Stass A3, Grosskreutz J4,5, Lu C6,7, Greensmith L3, Malaspina A3,6, Fratta P3, Hornstein E1,2 Clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat 51 expansion Marjanović A1, Palibrk A2, Branković M1, Janković M2, Dobričić V2, Novaković I1,2, Stević Z1,2 Clinical features at onset and longitudinal trajectories of decline in MND patients with cognitive- 52 behavioral impairment. Ferraro P1, Meo G2, De Maria E1, Pardini M2,3, Schenone A2,3, Roccatagliata L1,4, Caponnetto C2 Clinical features of speech and respiration accompanying decision-making for PEG insertion in 53 bulbar ALS: Hellenic experience from ENCLAS center - Patras University Hospital Kotsia E3, Michou E1, Katri M1, Veltsista D2, Chroni E2 Clinical Utility of Whole-Genome Sequencing in a large ALS cohort 54 Grassano M1,2, Calvo A1,3, Moglia C1,3, Canosa A1,3, Vasta R1, Manera U1, Brunetti M4, Chia R2, Ding J5, Gibbs R5, Dalgard C6, Scholz S7, Mazzini L8, De Marchi F8, Corrado L9, D'Alfonso S9, Traynor B2, Chio A1,3 Clustering for clinical stratification of ALS patients: 55 Unearthing the potential of an understudied set of techniques and tools. Karray M1, Jmaiel M1 Cognitive deficits reported in the M323K mouse, a TDP-43 ALS model 57 Ali Z1, Raghavan-Nair R1, Godoy Corchuelo J4, Banks G1, Fratta P2, Acevedo-Arozena A3, Fisher E2, Corrochano S4, Cunningham T1 Cognitive Reserve in ALS: A longitudinal study 58 Temp A1, Kasper E1,2, Machts J3,4, Vielhaber S3,4, Teipel S1,5, Hermann A1,2, Prudlo J1,2 Common and rare variant association analyses in Amyotrophic Lateral Sclerosis identify 15 risk 59 loci with distinct genetic architectures and neuron-specific biology Van Rheenen W1, on behalve of the project MinE GWAS consortium2 Comparative study of CSF chitinases in ALS: correlation with clinical features 60 Costa J1, Gromicho M2, Pronto-Laborinho A2, Almeida C3,4, A. Gomes R3,4, C.L. Guerreiro A3,4, Oliva A1, Pinto S2, de Carvalho M2,5 4 Comparison between Amyotrophic Lateral Sclerosis Electromyography abnormalities SCORE and 61 diagnostic certainty of ALS Peña Preciado M1,6, Salcedo-Maldonado M1, Suárez-Obando F3,7, Peña-Preciado M2, Quiroga- Carrillo M5, Castellar-Leones S1,6, Ruiz-Ospina E1,6, Correa-Arrieta C2, Soto-Peña D4, Lemus-Parra A4 Conditioned Medium from Cells Overexpressing TDP-43 Alters the Metabolome of Recipient Cells 62 Hergesheimer R1, Lanznaster D1, Bourgeais J2, Hérault O2, Vourc’h P1,3, Andres C1,3, Corcia P1,4, Blasco H1,3 Continued Intravenous (IV) Edaravone Treatment of ALS Patients Increases Overall Survival 63 Compared With No IV Edaravone Treatment in a US Administrative Claims Database Brooks B1, Ciepielewska M2, Zhang J3, Liu Y3, Zambrano G2, Hagan M2 Cortical and subcortical damage in C9orf72 ALS patients compared to matched wild-type ALS 64 patients. Moglia C1,5, Manera U1,5, Stanziano M1,2, Ferraro S2, Agosta F3, Valentini M4, Canosa A1,5, Calvo A1,5, Chio' A1,5, Bruzzone M2, Nigri A2 Corticomuscular Coherence Patterns in Primary Lateral Sclerosis 65 Coffey A1, Saroj B1, Mitchell M1, Fasano A1, Dukic S1,3, Carson R1, Lowery M2, Heverin M1, Nasserolislami B1, Hardiman O1 COVID-19 pandemic impact on the respiratory function of ALS patients at first visit 66 Pinto S1, Oliveira Santos M1,2, Gromicho M1, de Carvalho M1,2 Creatinine Kinase-MB as a complementary biomarker in the evaluation of patients with 67 Amyotrophic Lateral Sclerosis.
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