NORD gratefully acknowledges Donald L. Gilbert, MD, MS, Co-director, Cincinnati Children’s Hospital Medical Center Movement Disorders Clinic, for assistance in preparation of this report.
Sydenham chorea is a rare neurological disorder characterized by sudden onset chorea, usually in childhood. Chorea is defined as random-appearing, continuous (while awake), involuntary movements which can affect the entire body. This often includes the face and tongue. Symptoms in arms and legs are often worse on one side of the body. Additional symptoms of Sydenham chorea may include slurring of speech and difficulty maintaining steady hand grip. Anxiety, sadness, inattention, and obsessive compulsive thoughts and behaviors may also occur. Sydenham chorea most often affects children over the age of 5 years and adolescents. Sydenham chorea usually develops within weeks to months following group A beta-hemolytic streptococcal infection and may occur as an isolated finding or as a major complication of acute rheumatic fever. It is considered an autoimmune disorder, meaning it occurs when the body's immune system (which normally responds to foreign substances) mistakenly targets part of the body, disrupting normal function.
The severity of chorea and the presence of non-chorea symptoms of Sydenham chorea may vary greatly from one person to another. Most cases follow an identifiable streptococcal infection. Streptococcus is a group of bacteria that can cause several different infections, most commonly “strep throat” – often presenting with a sore throat (pharyngitis) or fever. Symptoms of Sydenham chorea may appear anywhere from 1 week to 6 months following streptococcal infection.
The abnormal movements (chorea) that characterize Sydenham chorea usually emerge over hours, peaking within a few hours or days. Pediatricians and emergency physicians seldom see chorea and may not recognize it. Initially, doctors may misattribute the restless movements and involuntary facial expressions of Sydenham chorea to a child being extremely fidgety, hyperactive, clumsy and/or purposely uncooperative. Parents (and children) generally recognize however that these movements, even in mild cases, are a clear change from the child’s usual status.
The abnormal movements in Sydenham chorea range from subtle symptoms, affecting coordination and tasks such as writing, to severe symptoms, disrupting walking, talking, and performing basic tasks such as dressing, eating, or simply holding objects. Choreic movements may fluctuate through the day. In most cases, chorea disappears during sleep.
In addition to choreic movements, individuals with Sydenham chorea may develop muscle weakness, slurred speech (dysarthria), diminished muscle tone (hypotonia), tics, obsessions, compulsions, inattention, anxiety, labile mood, and decreased verbal output. In some extremely rare cases (less than 2 percent), severe muscle weakness, irritability, or confusion may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea.
Because Sydenham chorea is a complication of rheumatic fever, some individuals will have additional symptoms of joint arthritis or arthralgia, inflammation of the heart valves causing permanent damage to the valves, and ongoing fever.
Sydenham chorea symptoms usually resolve within three weeks to six months. However, symptoms may last longer than one year. Occasionally, the symptoms of Sydenham chorea have recurred later during adult life, particularly in young women during the first trimester of pregnancy (so-called chorea gravidarum, which may represent a recurrence of Sydenham chorea in some cases).
Sydenham chorea is believed to be an autoimmune disorder. Most cases develop following a streptococcal infection or more severe rheumatic fever. An autoimmune disorder occurs when the body’s immune system mistakenly reacts against healthy tissue. In Sydenham chorea, streptococcal infection induces the body’s immune system to produce antibodies to combat the infection. For unknown reasons, these antibodies persist and subsequently target certain cells in the joints, kidneys, heart, and, in the brain, specifically cells of the basal ganglia (a key part of the brain for controlling motor movements). Researchers believe this ultimately leads to the characteristic symptoms of Sydenham chorea.
The exact underlying mechanisms that cause Sydenham chorea are poorly understood. Researchers believe that antigens (substances that are capable of stimulating an immune system response) on streptococcal bacterial cells are similar to antigens found on cells on the human body. When the immune system creates antibodies to combat the streptococcal infection, the antibodies also, in genetically predisposed individuals, mistakenly bind to healthy cells. When they bind to brain cells, they cause disruption in their “signaling” – their ability to control movement.
According to most studies, Sydenham chorea affects girls more often than boys. It usually develops in children between the ages of 5-15. Rarely, the disorder has been reported in children under age 5 years or in adults. Sydenham chorea affects individuals of all races and ethnicities.
Sydenham chorea may occur as a complication of rheumatic fever. Approximately, 25 percent of individuals with rheumatic fever develop Sydenham chorea. The incidence of rheumatic fever in North America declined steadily in the past 50 years, although there have been occasional outbreaks. Sydenham chorea is the most common cause of acute chorea during childhood in the United States. In areas of the world with less access to medical care and antibiotics, rheumatic fever remains a major public health problem due primarily to cases where there is damage to heart valves.
A diagnosis of Sydenham chorea is made based upon identification of new onset choreic movements, a detailed patient history, and a thorough clinical evaluation. In the presence of new onset chorea, which is uncommon in childhood, the documentation of a prior streptococcal infection through throat swabs and/or the current presence of high blood titers of streptococcal antibodies (ASO, anti DNAseB) is useful, as are identification of co-occurring arthritis or cardiac valve abnormalities. In some cases, certain imaging techniques such as magnetic resonance imaging (MRI) may be recommended to exclude other causes. Usually, brain imaging is normal in Sydenham chorea. Of note, because the onset of Sydenham chorea usually occurs weeks after the infection, the characteristic signs of rheumatic fever or streptococcal infection are usually no longer present.
Individuals who are diagnosed with Sydenham chorea should receive an evaluation for inflammation of the heart (carditis).
A confirmed diagnosis of Sydenham chorea is nearly always an indication for long-term antibiotic treatment, until adulthood. The purpose if this treatment is to prevent permanent heart valve damage which could result if the child experiences recurrent streptococcal infections. Most often penicillin is used. Physicians should consult current rheumatic fever guidelines.
Chorea suppressing medications should be considered. Some mild cases may not cause much impairment for the child. These may resolve within weeks. When chorea symptoms are disabling, low doses of potent dopamine receptor blocking agents such as haloperidol, dopamine depleting agents such as tetrabenazine, anti-seizure medications such as valproic acid, or benzodiazepines may help. Because in most cases the treatment will only be needed for weeks to months and at low doses, long term neurological side effects such as tardive dyskinesia are extremely unlikely. Short term side effects such as weight gain may occur. As is the case for any neurological medications, however, a careful discussion of potential benefits as well as risks is advised.
Immune system treatment
Additional short-term immune therapies have been used to treat individuals with impairing Sydenham chorea during the first weeks of symptoms, based on the idea that ongoing acute inflammation is contributing to symptoms. There is some scientific support for using oral steroids and intravenous immunoglobulins from small but rigorous clinical trials.
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