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.