" /> Benign hereditary chorea - CISMeF





ICD-11 code : 8A01.0;

Preferred Label : Benign hereditary chorea;

ICD-11 definition : Benign hereditary chorea should be considered in people with a relatively stable, nonprogressive chorea, in whom childhood onset and an autosomal dominant family history are present. Benign hereditary chorea can be associated with short stature and developmental delay. Larger deletions of causative gene, TITF-1, can cause a multisystem disorder with congenital hypothyroidism, hypotonia, and pulmonary problems.;

ICD-11 synonym : Benign familial chorea;

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Benign hereditary chorea should be considered in people with a relatively stable, nonprogressive chorea, in whom childhood onset and an autosomal dominant family history are present. Benign hereditary chorea can be associated with short stature and developmental delay. Larger deletions of causative gene, TITF-1, can cause a multisystem disorder with congenital hypothyroidism, hypotonia, and pulmonary problems.

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29/07/2025


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