{"id":"W2004548675","doi":"10.1055/s-2004-821172","title":"Late Infantile Onset Krabbe Disease in Siblings with Cortical Degeneration and Absence of Cerebral Globoid Cells","year":2004,"lang":"en","type":"article","venue":"Neuropediatrics","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":3,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of Manitoba; Children's Hospital Research Institute of Manitoba; Health Sciences Centre; Manitoba Health","funders":"Canada Research Chairs","keywords":"White matter; Cerebral cortex; Pathology; Medicine; Atrophy; Degeneration (medical); Krabbe disease; Anatomy; Leukodystrophy; Disease; Magnetic resonance imaging; Internal medicine","routes":{"ca_aff":true,"ca_fund":true,"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":[0.0006399674,0.002591794,0.0009087134,0.00161679,0.00184777,0.0007995783,0.000919555,0.002717338,0.00322935],"category_scores_gemma":[0.004757326,0.001252917,0.0005343141,0.0009638223,0.001411244,0.0008934839,0.001343081,0.001870779,0.0008048518],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0008933708,"about_ca_system_score_gemma":0.0008447788,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.004072512,"about_ca_topic_score_gemma":0.004271653,"domain_scores_codex":[0.9992305,0.0001396927,0.00008098112,0.000170943,0.0001746227,0.0002031961],"domain_scores_gemma":[0.9986252,0.0003274012,0.0003833979,0.00009940982,0.0001305596,0.0004340316],"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":[0.0001295014,0.0001062385,0.02961212,0.00005411122,0.00002761599,0.9599274,0.001423716,0.00009872301,0.005521614,0.0004094641,0.0004125909,0.00227682],"study_design_scores_gemma":[0.00004194388,0.0002549056,0.03062858,0.00003711245,0.00003834863,0.9657999,0.0004034985,0.0002014883,0.001510055,0.0002752231,0.0007815883,0.0000272447],"study_design_candidate":"case_report","study_design_consensus":"case_report","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.994608,0.0005965598,0.0009561179,0.0005468789,0.00006140739,0.00006125854,0.0001777099,0.0001052931,0.002886565],"genre_scores_gemma":[0.9978808,0.0003854587,0.0005270677,0.000153922,0.00005992628,0.00002334265,0.00008729089,0.00002681585,0.000855346],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.004072512,"threshold_uncertainty_score":0.01080322,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01211339546531345,"score_gpt":0.2567444452702973,"score_spread":0.2446310498049839,"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."}}