The results of follow-up of 76 children with progressive ataxia first reported in 1988 are described with special reference to type of potential underlying metabolic diseases. In 70% of cases the clinical follow-up did not lead to reconsideration of diagnoses. Six of 23 biochemically and morphologically re-examined children got a new and definite diagnosis: 1 myoclonic encephalopathy with ragged red fibres, 2 carbohydrate-deficient glycoprotein syndrome, 1 neuroborreliosis, 1 Hallervorden-Spatz disease and 1 leucodystrophy. Different clinical groups are discussed.
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Ylitalo et al. (2009) studied this question.
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