Queston #22289Note

This patient’s non-progressive mild chorea is consistent with benign hereditary chorea. This is an autosomal dominant disorder caused by a mutation in a thyroid transcription factor (NKX201, TITF1). The disorder typically arises in childhood and patients have normal intelligence. Chorea symptoms can range from barely noticeable to interfering with daily life. Manifestations of the disease may become milder with time, and in some patients, the symptoms may be barely present by adulthood.