<unk>  (BHC), also known as<unk>  familial<unk> , is a rare autosomal dominant neurogenetic syndrome. It typically presents itself in childhood with isolated<unk> , with average to below average intelligence. Unlike other neurogenetic causes of<unk>  such as Huntington's disease, BHC is not progressive, and not associated with cognitive decline or psychiatric problems in the vast majority of cases. The first description of BHC was reported in 1967 in an African American family from Mississippi. Two brothers reportedly had delayed motor development in childhood and were diagnosed with<unk> . These findings were reaffirmed by other families reporting similar traits and an autosomal dominant pattern of inheritance was suggested. However, heterogeneity in the presentations of the affected individuals made confirmation of these diagnoses of BHC difficult to prove. Features reported in these families, including dystonia, tremor, and myoclonus, led researchers to question whether BHC actually represents different diagnoses with similar phenotypes inappropriately grouped together. Further research in 2000 confirmed a connection between a Dutch family reporting similar characteristics of BHC and one of the original families. The investigators identified a linkage to a disease locus on the long arm of chromosome 14 from this connection.
Benign hereditary chorea
Non-progressive congenital ataxia
Sydenham's chorea
X-linked dystonia parkinsonism
Autosomal recessive cerebellar ataxia
Autosomal recessive cerebellar ataxia type 1
Spinocerebellar ataxia
Huntington's disease-like syndrome