Chorea is a movement disorder that causes sudden jerky, random, and purposeless movements. It comes from the Greek word for "dance”.
Athetosis means “without position or place". The most common underlying cause of athetosis is Huntington's disease. In children, athetosis is commonly associated with dyskinetic cerebral palsy caused by kernicterus, hypoxic-ischemic injury at term birth or prematurity with associated brain injury.
Athetosis most commonly involves the distal extremities: fingers (hand or feet). It can occur because of damage to the corpus striatum (caudate and putamen) in the basal ganglia.
Lesions in basal ganglia motor loops are responsible for loss of coordination of movements. These may include structures such as the caudate nucleus, putamen, and globus pallidus.
Huntington’s disease is the most common cause of chorea in adults. Activation of the nigrostriatal dopamine pathway is believed to induce chorea.
Sydenham's chorea is a milder form of chorea and is listed as one of the five major manifestations of rheumatic fever (Jones criteria). Sydenham's chorea is believed to occur as a result of an autoimmune reaction towards basal ganglia circuitry.
The treatment of chorea depends on the underlying disease. If Huntington is the cause, tetrabenazine is effective. Patients with Sydenham chorea can be treated with antibiotics to handle the infection. If chorea is occurring as a side effect of a medication, stopping the agent will help to reduce the symptoms.
Treatment of chorea depends on the etiology. A common medication is tetrabenazine, which works by inhibiting vesicular monoamine transporter (VMAT) thereby reducing dopamine exocytosis from presynaptic neurons.
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