Benign Hereditary Chorea
Benign hereditary chorea (BHC) is a relatively rare autosomal dominant disorder which produces childhood onset chorea, usually under age 5, with a stable or only slightly progressive course. In some cases, chorea decreases in adolescence or early adulthood. The clinical picture is apparently not identical in all families and myoclonus, dystonia, gait disturbance, and tremor have also occasionally been present. Linkage to a region on chromosome 14q13.1-q21.1 has been reported in several families. It is possible that the phenotype may also be due to other yet unidentified genetic causes. Unlike Huntington’s disease (HD), BHC is not associated with behavioral changes or dementia. Brain MRI is normal.
KeywordsBehavioral Change Primary Care Physician Clinical Picture Early Adulthood SPECT Imaging
Benign Hereditary Chorea.mp4 (MP4 16,584KB)
The patient displays continuous low-amplitude and largely distal choreiform movement of her arms, hands, fingers, ankles, and toes.
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