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8A03.11 Ataxia due to Cerebrotendinous xanthomatosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Ataxia in the setting of cerebrotendinous xanthomatosis, a rare autosomal recessive disorder of bile acid metabolism caused by a mutation in the CYP27A1 gene encoding mitochondrial enzyme sterol 27-hydroxylase. Accumulation of sterols in multiple tissues leads to premature cataracts and tendon xanthomas in late childhood, followed by progressive neurological dysfunction such as ataxia, dementia, and polyneuropathy.

synonyms

• Ataxia due to Cerebrotendinous xanthomatosis

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