[Benign non-progressive familial chorea].
Benign non-progressive familiar chorea is a chronic childhood (less than 5 years) choreatic disorder inherited as an autosomal dominant trait but not associated with dementia or progressive motor dysfunction. The major feature of this disease is chorea with early insidious onset and an arrested progression. The lack of mental retardation, mental deterioration or convulsion is the other significant aspects of this disorder. The chorea usually involves the distal limbs, face and trunk. This does not progress to a profound degree throughout the life. There is a controversy regarding whether this benign disorder might be a subtype variant of Huntington's disease. This should be clarified by more extensive molecular genetic studies of this disease.
Hirose
G
G
Department of Neurology, Kanazawa Medical University.
D017668:Age of Onset; D002819:Chorea / Q000175:diagnosis / Q000235:genetics*; D002908:Chronic Disease; D003937:Diagnosis, Differential; D005799:Genes, Dominant; D006801:Humans; D006816:Huntington Disease