{"id":"W4362692092","doi":"10.1002/acn3.51760","title":"Provisional practice recommendation for the management of myopathy in <scp>VCP</scp>‐associated multisystem proteinopathy","year":2023,"lang":"en","type":"review","venue":"Annals of Clinical and Translational Neurology","topic":"Genetic Neurodegenerative Diseases","field":"Neuroscience","cited_by":9,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of Calgary","funders":"National Center for Advancing Translational Sciences; National Institute of Neurological Disorders and Stroke; Grifols; Astellas Pharma; National Institutes of Health; Argenx; Audentes Therapeutics; Myositis Association; Amicus Therapeutics; Valerion Therapeutics; Alexion Pharmaceuticals; Sanofi; National Institute of Arthritis and Musculoskeletal and Skin Diseases; AveXis; Biogen; Spark Therapeutics; Maze Therapeutics; Sarepta Therapeutics; Muscular Dystrophy Association; Acceleron; Ultragenyx Pharmaceutical; AstraZeneca; CSL Behring; Myasthenia Gravis Foundation of America","keywords":"Medicine; Myopathy; Genetic testing; Phenotype; Bioinformatics; Muscular dystrophy; RYR1; Limb-girdle muscular dystrophy; Disease; Pathology; Genetics; Gene; Internal medicine; Biology","routes":{"ca_aff":true,"ca_fund":false,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":false},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.01517353,0.0007104665,0.001086986,0.002214447,0.00225356,0.003017858,0.002740763,0.01115587,0.0243123],"category_scores_gemma":[0.07956465,0.0005497396,0.002291005,0.001195456,0.00115422,0.005104117,0.004173656,0.008123034,0.01503002],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.002905791,"about_ca_system_score_gemma":0.01039516,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.004939215,"about_ca_topic_score_gemma":0.01198289,"domain_scores_codex":[0.9792548,0.01111143,0.003915072,0.0007800946,0.003627938,0.001310636],"domain_scores_gemma":[0.9360837,0.02481594,0.006781701,0.001999866,0.02063583,0.009682973],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"not_applicable","study_design_gemma":"not_applicable","study_design_scores_codex":[0.00008875694,0.0006856116,0.004796897,0.002944054,0.00005636012,0.001602549,0.002455248,0.0003896158,0.0009410786,0.00203382,0.749896,0.23411],"study_design_scores_gemma":[0.0004644771,0.0004518232,0.009462635,0.01977599,0.0001262928,0.002301185,0.005752848,0.001192229,0.000658001,0.009102245,0.9504597,0.000252618],"study_design_candidate":"not_applicable","study_design_consensus":"not_applicable","genre_codex":"commentary","genre_gemma":"review","genre_scores_codex":[0.0151432,0.01442857,0.03184636,0.7917777,0.0148395,0.006765964,0.002244818,0.002367144,0.1205867],"genre_scores_gemma":[0.1083116,0.04319951,0.3434509,0.4138305,0.01172428,0.02046032,0.005997034,0.0009751461,0.0520508],"genre_candidate":"review","genre_consensus":null,"teacher_disagreement_score":0.0243123,"threshold_uncertainty_score":0.0813328,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.3851937776674497,"score_gpt":0.4973177968458501,"score_spread":0.1121240191784004,"validation_status":"score_only:v0-immature-baseline","note":"Baseline scores from an immature model (maturity gate not passed). Scores rank; they never assert a category."}}