{"id":"W2046449217","doi":"10.1017/s001216220600082x","title":"Atypical clinical course in juvenile metachromatic leukodystrophy involving novel arylsulfatase A gene mutations","year":2006,"lang":"en","type":"article","venue":"Developmental Medicine & Child Neurology","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":19,"is_retracted":false,"has_abstract":true,"ca_institutions":"McMaster University Medical Centre; McMaster University","funders":"American Society of Neuroradiology","keywords":"Metachromatic leukodystrophy; Arylsulfatase A; Medicine; Leukodystrophy; Pathology; Pathogenesis; Myelin; Pediatrics; Internal medicine; Disease; Central nervous system","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":[0.0002941671,0.001417679,0.0009143666,0.001284602,0.000928744,0.000644997,0.0004056332,0.001795825,0.001539571],"category_scores_gemma":[0.002639059,0.0005839824,0.0003446355,0.0008292964,0.0008639544,0.0005837156,0.0008589739,0.0008034414,0.0003697614],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004841718,"about_ca_system_score_gemma":0.0003553507,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001075709,"about_ca_topic_score_gemma":0.001930726,"domain_scores_codex":[0.9995919,0.00006241734,0.00005363916,0.00008835485,0.00006235606,0.0001413905],"domain_scores_gemma":[0.998511,0.000393088,0.0003638184,0.00006037613,0.00005998346,0.0006117548],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"case_report","study_design_gemma":"case_report","study_design_scores_codex":[0.0003411668,0.000083744,0.08106422,0.0000549336,0.00003085456,0.9097456,0.0003581342,0.0001381552,0.004666856,0.0002343906,0.000434763,0.002847204],"study_design_scores_gemma":[0.00004591703,0.0001795586,0.05132393,0.000008438797,0.00001314656,0.9475332,0.00009526087,0.0001119216,0.0002759632,0.00009719466,0.0003041002,0.00001138934],"study_design_candidate":"case_report","study_design_consensus":"case_report","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9954848,0.0009982364,0.0004159507,0.0002704331,0.00003580325,0.00004394469,0.0001522317,0.00005186643,0.002546629],"genre_scores_gemma":[0.9988064,0.0002597913,0.0002642017,0.0001690214,0.00009481837,0.00001300985,0.0000766216,0.0000085949,0.0003076246],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001795825,"threshold_uncertainty_score":0.005150318,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03648560156669271,"score_gpt":0.331378009222944,"score_spread":0.2948924076562513,"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."}}