PROGRESSIVE ATAXIA IN SWEDISH CHILDREN - REEVALUATION STUDY

The results of follow-up of 76 children with progressive ataxia first reported in 1988 are described with special reference to type of poten tial underlying metabolic diseases. In 70% of cases the clinical follo w-up did not lead to reconsideration of diagnoses. Six of 23 biochemic ally and morphologically re-examined children got a new and definite d iagnosis: 1 myoclonic encephalopathy with ragged red fibres, 2 carbohy drate-deficient glycoprotein syndrome, 1 neuroborreliosis, 1 Hallervor den-Spatz disease and 1 leucodystrophy. Different clinical groups are discussed.