{"id":"W4365449093","doi":"10.1101/2023.04.12.536631","title":"Severe Central Nervous System Demyelination in Sanfilippo Disease","year":2023,"lang":"en","type":"preprint","venue":"bioRxiv (Cold Spring Harbor Laboratory)","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":1,"is_retracted":false,"has_abstract":true,"ca_institutions":"Montreal Neurological Institute and Hospital; Université de Montréal; McGill University; Centre Hospitalier Universitaire Sainte-Justine","funders":"","keywords":"Myelin; White matter; Neuroinflammation; Corpus callosum; Pathology; Oligodendrocyte; Central nervous system; Neurodegeneration; Biology; Multiple sclerosis; Corticospinal tract; Luxol fast blue stain; Neuroscience; Medicine; Diffusion MRI; Immunology; Disease; Magnetic resonance imaging","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":[0.0002017486,0.0009947034,0.0004132017,0.0007153221,0.0004149697,0.0003045004,0.0001349285,0.0004994574,0.001353238],"category_scores_gemma":[0.000198848,0.000204767,0.0001725199,0.0002606187,0.0005398309,0.000185242,0.0003382296,0.0003653601,0.0002824503],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002357396,"about_ca_system_score_gemma":0.0001739748,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001027919,"about_ca_topic_score_gemma":0.001174386,"domain_scores_codex":[0.9999028,0.00001370578,0.000013234,0.00003859125,0.00001672754,0.00001495477],"domain_scores_gemma":[0.9998645,0.00001565276,0.00004982109,0.000008124463,0.00001361908,0.00004834041],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"bench_or_experimental","study_design_gemma":"case_report","study_design_scores_codex":[0.003300467,0.0003660288,0.1176523,0.0004471244,0.0001856121,0.06236093,0.0008469723,0.000334052,0.8020064,0.0002620762,0.0005739206,0.01166424],"study_design_scores_gemma":[0.00009832717,0.001446604,0.8047994,0.0000729149,0.0002385774,0.1361791,0.0004847838,0.001272875,0.05166316,0.0004245119,0.003271678,0.00004808046],"study_design_candidate":"case_report","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9984413,0.0003541294,0.0002109724,0.00003069492,0.000004867821,0.00001388465,0.0001053148,0.0000169079,0.0008219237],"genre_scores_gemma":[0.9989744,0.0002789575,0.0002501479,0.00003033588,0.000009011533,0.000008230494,0.0001824324,0.000006579004,0.0002598521],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001353238,"threshold_uncertainty_score":0.004527032,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02369417072747304,"score_gpt":0.2592658320693116,"score_spread":0.2355716613418386,"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."}}