Benign Hereditary Chorea Diagnosis and Management

Clinical Characteristics

The American Academy of Neurology describes Benign Hereditary Chorea (BHC) as a hyperkinetic movement disorder characterized by irregular, rapid, and non-stereotyped movements that flow randomly, giving an appearance of "dance" or dancing movements during gait 1

BHC is caused by mutations or deletions in the NKX2-1/TITF1 gene, located on chromosome 14q13.3, which is essential for the organogenesis of the basal ganglia, thyroid, and lungs 2

The American Academy of Neurology differentiates tics from chorea, describing tics as very brief movements, typically of shorter duration than attacks of paroxysmal kinesigenic dyskinesia 1
The American Heart Association distinguishes hyperkinesia, conversion reaction, and psychogenic movement disorders from chorea 1

The American Academy of Neurology recommends that patients with chorea be evaluated periodically to detect the development of tardive dyskinesia, especially those receiving long-term neuroleptic treatment 1
The American Academy of Neurology suggests that speech therapy may be beneficial for patients with orofacial chorea that affects speech and swallowing, and occupational therapy may help with activities of daily living affected by choreiform movements 3