{"id":"W4384818747","doi":"10.1002/mds.29560","title":"Natural History and Phenotypic Spectrum of <scp>GAA‐<i>FGF14</i></scp> Sporadic Late‐Onset Cerebellar Ataxia (<scp>SCA27B</scp>)","year":2023,"lang":"en","type":"article","venue":"Movement Disorders","topic":"Genetic Neurodegenerative Diseases","field":"Neuroscience","cited_by":55,"is_retracted":false,"has_abstract":true,"ca_institutions":"Montreal Neurological Institute and Hospital","funders":"Canadian Institutes of Health Research; Ipsen; EVER Neuro Pharma; International Parkinson and Movement Disorder Society; Teva Pharmaceutical Industries","keywords":"Ataxia; Spinocerebellar ataxia; Gait Ataxia; Cohort; Cerebellar ataxia; Medicine; Movement disorders; Age of onset; Pediatrics; Psychology; Natural history study; Prospective cohort study; Natural history; Internal medicine; Psychiatry","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":[0.0002674389,0.000193928,0.000204374,0.0006200517,0.0003470322,0.0002797334,0.0001860572,0.0002722953,0.000992033],"category_scores_gemma":[0.001211368,0.0001594675,0.0002029614,0.0002548934,0.0002788651,0.0002792282,0.000306679,0.0002394041,0.0002137169],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001945969,"about_ca_system_score_gemma":0.0001799226,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.002015774,"about_ca_topic_score_gemma":0.001806685,"domain_scores_codex":[0.9997955,0.00004332259,0.0000153414,0.00007688525,0.00003026519,0.00003865366],"domain_scores_gemma":[0.9994168,0.00009564088,0.0002281345,0.0000527658,0.00007841654,0.0001282689],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.0001744278,0.00004901006,0.9957699,0.000002877554,0.00001365168,0.0004968818,0.00006748718,0.00003658721,0.001238465,0.00001606599,0.00007764557,0.002056915],"study_design_scores_gemma":[0.000007274293,0.0001908065,0.9962036,0.000003967431,0.00001006334,0.003125141,0.00007192478,0.0001353949,0.0001295056,0.00003341952,0.00008505407,0.000003783836],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9996017,0.00004904388,0.00005073968,0.000009170108,5.75869e-7,0.000004552587,0.0001145331,0.000002979285,0.0001667609],"genre_scores_gemma":[0.9995091,0.00003740103,0.00005135256,0.000008624489,0.000001791873,0.000006093515,0.0003157402,0.000001791904,0.00006819336],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.002015774,"threshold_uncertainty_score":0.004008055,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01481538086020775,"score_gpt":0.2194838956999241,"score_spread":0.2046685148397164,"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."}}