{"id":"W7074201445","doi":"","title":"GAA-FGF14 ataxia (SCA27B): phenotypic profile, natural history progression and 4-aminopyridine treatment response","year":2023,"lang":"en","type":"article","venue":"UCL Discovery (University College London)","topic":"Seismic Imaging and Inversion Techniques","field":"Earth and Planetary Sciences","cited_by":7,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"Canadian Institutes of Health Research; Royal College of Physicians and Surgeons of Canada","keywords":"Natural history; Spinocerebellar ataxia; Ataxia; Natural history study; Dysautonomia; Disease; Cohort; Dystonia; Progressive supranuclear palsy; Cerebellar ataxia","routes":{"ca_aff":false,"ca_fund":true,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":true},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0002719771,0.0002419036,0.0002598392,0.00043965,0.0004214672,0.00003569808,0.0002556334,0.00009580913,0.0005624262],"category_scores_gemma":[0.00003335227,0.0002158305,0.0001038789,0.0005352695,0.0002601803,0.001062108,0.00007195021,0.0001614983,0.0003059358],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001798905,"about_ca_system_score_gemma":0.0002023239,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001517344,"about_ca_topic_score_gemma":0.0001416673,"domain_scores_codex":[0.998464,0.0002193774,0.0001331542,0.0004888285,0.0002889418,0.0004056855],"domain_scores_gemma":[0.9992127,0.0001962351,0.0001036666,0.0002927608,0.0000392361,0.0001554354],"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":[0.012725,0.0002195511,0.07849716,0.000148266,0.00019539,0.002318207,0.002497361,0.00006654618,0.002312445,0.001254868,0.7746235,0.1251417],"study_design_scores_gemma":[0.002774812,0.001150783,0.3127787,0.0001237297,0.00009850289,0.00007195451,0.002735495,0.05711621,0.001336936,0.0001688854,0.6208923,0.0007516239],"study_design_candidate":"not_applicable","study_design_consensus":"not_applicable","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9923683,0.001221464,0.00003305687,0.001478073,0.0005627245,0.0004420701,0.0006258779,0.0004612256,0.00280715],"genre_scores_gemma":[0.8956367,0.0003494156,0.0005418639,0.0003187761,0.0000520231,9.308533e-7,0.0003212448,0.000008515037,0.1027705],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.2342816,"threshold_uncertainty_score":0.8801311,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01225537000242585,"score_gpt":0.2006691382667643,"score_spread":0.1884137682643384,"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."}}