Clinical Pearls & Morning Reports
Published December 13, 2023
Chorea is characterized by involuntary, nonrhythmic movements due to continuous flow of muscle contractions from one muscle group to another, creating a dancelike appearance. Read the NEJM Case Records of the Massachusetts General Hospital here.
Q: What is the most common type of chorea in adults?
A:After levodopa-induced dyskinesias and Huntington’s chorea, autoimmune chorea is the most common type of chorea in adults. It may be associated with systemic autoimmune conditions, such as systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS).
Q: Lesions in what part of the brain give rise to chorea?
A: Chorea usually localizes to extrapyramidal basal ganglia networks. Basal ganglionic diseases can be diffuse or discrete. When they are discrete, they usually also affect adjacent structures, such as the internal capsule, hypothalamus, or cerebral white matter.
A: Medications associated with chorea include neuroleptic agents, anticonvulsant agents, amphetamines, or antiparkinsonian medications. Other causes include an anoxic insult, carbon monoxide poisoning, or an occupational exposure (e.g., exposure to heavy metals). Hyperglycemia can cause chorea, as can hypernatremia and hyponatremia. Hyperthyroidism can cause movement disorders, most commonly an enhanced physiologic tremor but rarely also chorea. Infection with group A beta-hemolytic streptococcus (acute rheumatic fever) can cause Sydenham chorea; this type of chorea is primarily a childhood disease with a decreasing incidence in the United States. Inflammatory disorders that can cause chorea include multiple sclerosis, neurosarcoidosis, and neuro-Behcet’s disease. Chorea is the most common movement disorder among patients with antiphospholipid syndrome (APS), occurring in 1.3% of patients. APS can occur as a primary disorder or can be secondary to other connective-tissue disorders, including SLE.
A: Chorea is the most common movement disorder among patients with SLE, occurring in 2% of patients. When chorea occurs in patients with SLE, it is bilateral in 55% of cases and is limited to a single episode in 66% of cases, although multiple episodes are possible. Other neuropsychiatric symptoms of SLE can include seizures, delirium, brain ischemia, and psychiatric disturbances, ranging from personality changes to psychosis.