The nosologic panorama of progressive ataxia in Swedish children.
Described are 76 children with a picture of progressive encephalopathy and ataxia as the principal or joint principal leading signs. The series was hospital-based in Gothenburg between 1973 and 1983, and not representative for epidemiologic analyses. The children were divided in groups by using a combined pathogenetic and clinical grouping system: lysosomal disorders (6 children), non-lysosomal lipid disorders (10), intermediary metabolic disorders (3), heredoataxias (22), phacomatoses including Louis-Bar (5), dysimmune encephalopathies (6), other defined disorders (19) and undefined or incompletely defined conditions (5). Different groups are discussed and, according to this material, a diagnostic pathway is drawn up.
Ylitalo
V
V
Department of Pediatrics, University of Turku, Finland.
Hagberg
B
B
D000293:Adolescent; D001259:Ataxia / Q000145:classification* / Q000209:etiology / Q000378:metabolism; D001927:Brain Diseases / Q000145:classification* / Q000150:complications / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000150:complications / Q000503:physiopathology; D007223:Infant; D008297:Male; D008659:Metabolic Diseases / Q000150:complications / Q000503:physiopathology; D013548:Sweden