Common Causes
Sydenham Chorea is a neurological disorder that causes the involuntary movement of muscles in various parts of the body. The disorder usually affects children between the ages of five and fifteen.
Related Conditions
Diagnosis is often delayed and attributed to another condition such as tic disorder or conversion disorder. The controversial PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) hypothesis has overlapping clinical features but Sydenham's chorea is one of the exclusion criteria.
What is Sydenham chorea and what causes it?
Children who develop Sydenham chorea after rheumatic fever usually recover without long-term medical problems. What can I do to help relieve symptoms of chorea? Only a doctor can help you relieve the symptoms of chorea.
Is Sydenham's chorea a pandas disorder?
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).
Can a child recover from Sydenham chorea after rheumatic fever?
How long does Sydenham chorea last?
Is Sydenham chorea genetic?
Genetic causes: Benign hereditary chorea starts in childhood and is a non-progressive chorea. Inheritance is usually autosomal dominant, although rare cases of autosomal-recessive and X-linked inheritance have been reported.
How do you get Sydenham chorea?
Causes. 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.
How common is Sydenham chorea?
Sydenham chorea (SD), also referred to as St. Vitus dance is a manifestation of rheumatic fever (RF), occurring in up to 40% of patients with RF. It is uncommon in the United States but occurs at a much higher frequency in developing countries.
What causes sydenhams chorea?
Sydenham chorea (SC) is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever. SC is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles.
What triggers chorea?
Chorea can be caused by a variety of abnormal processes in the body, including metabolic derangements, exposure to certain drugs or toxins, genetic and degenerative diseases of the brain, infections, tumors, and disorders of the immune and inflammatory systems of the body.
Can Sydenham be cured?
Treatment. There is no specific treatment for Sydenham's chorea and symptoms usually resolve themselves in approximately 3 to 6 months. Bed rest, sedatives and medication to control movements may be prescribed. Penicillin prophylaxis may also be prescribed to avoid further streptococcal infection.
How many people have Sydenham's chorea?
Chorea is the most common symptom of Huntington's disease. In the United States, about 4,000 kids a year develop Sydenham chorea after having rheumatic fever.
Can adults get Sydenham chorea?
Sydenham chorea is considered a disease of childhood; however, it also may be seen in adults. Rheumatic chorea is characterized by muscle weakness and the presence of chorea. The patients have the milkmaid grip sign, clumsy gait, and explosive bursts of dysarthric speech.
What medications can cause chorea?
Drug induced chorea Multiple drugs including dopamine agonists, levodopa therapy, oral contraceptives, and anticonvulsants have been implicated in the acute chorea (box 4).
How is Sydenham chorea treated?
Antibiotic treatment and prophylaxis. Despite the fact that patients commonly do not have an active infection at the time of the appearance of chorea, most published treatment recommendations include a 10-day course of oral penicillin or a single intramuscular (IM) dose of penicillin at the time of SC diagnosis.
Can chorea be cured?
Huntington's disease chorea can be treated with antipsychotic drugs, as well as other medications. Chorea due to Parkinson's disease has no cure, but symptoms can be managed.
What is the difference between Sydenham chorea and pandas?
The age of onset is prepubertal in both conditions. Sydenham's chorea usually occurs between the ages of 5 and 15 years whilst neuropsychiatric symptoms in PANDA patients is most often found from 3 years of age till the beginning of puberty.
Can adults get Sydenham chorea?
Sydenham chorea is considered a disease of childhood; however, it also may be seen in adults. Rheumatic chorea is characterized by muscle weakness and the presence of chorea. The patients have the milkmaid grip sign, clumsy gait, and explosive bursts of dysarthric speech.
What medications can cause chorea?
Medications: Levodopa, neuroleptics, amphetamines, antihistamines, tricyclic antidepressants, anticonvulsants, cocaine, oral contraceptives are among the many medications known to cause chorea. Antipsychotic drugs can also cause chorea as part of a condition called tardive dyskinesia.
Why is it called Sydenham chorea?
Etymology. It is named after British physician Thomas Sydenham (1624–1689). The alternate eponym, "Saint Vitus Dance", is in reference to Saint Vitus, a Christian saint who was persecuted by Roman emperors and died as a martyr in AD 303.
How do you treat Sydenham's chorea?
Antibiotic treatment and prophylaxis. Despite the fact that patients commonly do not have an active infection at the time of the appearance of chorea, most published treatment recommendations include a 10-day course of oral penicillin or a single intramuscular (IM) dose of penicillin at the time of SC diagnosis.
Why is Sydenham chorea rare?
SC is rare in North America and Europe because of the widespread use of antibiotics to treat bacterial infections. Lack of antibiotic treatment and unsanitary conditions make the disorder more common in some parts of the developing world.
How long does it take for Sydenham chorea to go away?
In most cases, the symptoms of Sydenham chorea resolve on their own in a few weeks without treatment. If symptoms are severe or persistent, treatment may be recommended.
What is SC in children?
SC usually occurs in pre-pubescent children during or following a bacterial infection. Girls are affected more often than boys. Sometimes symptoms are mild and subtle enough to escape notice, but sometimes they are severe enough to interfere with the child’s daily functioning.
Can a child have chorea?
Chorea is rare in children, so doctors may suspect Sydenham chorea if a child has an onset of choreic symptoms with no other apparent cause. The diagnostic process will typically include tests to look for a bacterial infection, rule out other causes of the symptoms, and look for signs of potential complications.
Where does SC occur?
The immune reaction that causes SC commonly affects cells in the heart, kidneys , and joints. It also affects cells in the area of the brain that controls movement; this is the likely cause of SC.
Is Huntington's disease inherited?
Some types of chorea, such as Huntington’s disease, have a genetic component and may be inherited. However, because it occurs due to a bacterial infection, Sydenham chorea is considered an acquired condition. Researchers believe that some people may have a genetic predisposition that makes them vulnerable to autoimmune conditions such as SC, but a definite genetic link to the disorder has not yet been discovered.
Can a doctor diagnose Sydenham chorea?
Sometimes doctors don’t recognize Sydenham chorea immediately. In some cases, parents don’t know that the child has had a bacterial infection, and the chorea is the only sign that something is wrong. Doctors may misdiagnose the condition as behavioral as a result.
How to diagnose Sydenham chorea?
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.
How long does it take for a child to develop Sydenham chorea?
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.
What percentage of people with rheumatic fever develop Sydenham chorea?
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.
What is the name of the disorder that causes a person to move involuntaryly?
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.
How long does it take for chorea to show?
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.
What are the symptoms of Sydenham chorea?
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 .
What is the condition called when a child is bedridden?
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 ...
What is Sydenham's chorea?
Sydenham's chorea is an autoimmune disease that results from childhood infection with Group A beta- haemolytic Streptococcus. It is reported to occur in 20–30% of people with acute rheumatic fever and is one of the major criteria for it, although it sometimes occurs in isolation.
When did Sydenham's chorea become a disease?
Sydenham's chorea became a well-defined disease entity only during the second half of the nineteenth century. Such progress was promoted by the availability of large series of clinical data provided by newly-founded paediatric hospitals.
How long does Sydenham's chorea last?
The disease occurs typically a few weeks, but up to 6 months, after the acute infection, which may have been a simple sore throat ( pharyngitis ). Sydenham's chorea is more common in females than males and most cases affect children between the ages of 5 and 15 years of age.
Where is Sydenham's chorea most common?
As with rheumatic fever, Sydenham's chorea is seen more often in less affluent communities, whether in the developing world or in aboriginal communities in the global North. High rates of impetigo are a marker for widespread streptococcal transmission.
When is chorea diagnosed?
The diagnosis is then made by the typical acute onset in the weeks following a sore throat or other minor infection , plus evidence of inflammation (raised CRP and/or ESR) and evidence of recent streptococcal infection.
What diseases can cause chorea?
Other disorders that may be accompanied by chorea include benign hereditary chorea, bilateral striatal necrosis, abetalipoproteinemia, ataxia–telangiectasia, biotin-thiamine-responsive basal ganglia disease, Fahr disease, familial dyskinesia–facial myokymia (Bird–Raskind syndrome) due to an ADCY5 gene mutation, glutaric aciduria, Lesch–Nyhan syndrome, mitochondrial disorders, Huntington's disease, Wilson disease, hyperthyroidism, lupus erythematosus, pregnancy ( chorea gravidarum ), drug intoxication and side effects of certain anticonvulsants (e.g. phenytoin) or psychotropic agents. Although some of these can similarly present in an acute way, there will typically be other neurological signs (such as ataxia or cognitive impairment), or other disease manifestations, or positive family history, which will help distinguish between them.
What is the name of the disorder characterized by rapid, uncoordinated jerking movements primarily affecting?
Sydenham's chorea. Other names. Chorea minor, St Vitus' dance. Specialty. Neurology. Sydenham's chorea , also known as chorea minor and historically and occasionally referred to as St Vitus' dance, is a disorder characterized by rapid, uncoordinated jerking movements primarily affecting the face, hands and feet.
How many kids develop Sydenham chorea?
In the United States, about 4,000 kids a year develop Sydenham chorea after having rheumatic fever. Rheumatic fever is a serious complication of untreated strep throat. Girls are more likely than boys to get rheumatic fever. It usually occurs between 5 and 15 years of age.
Where does chorea originate?
Chorea is a neurological symptom that originates in an area of the brain called basal ganglia, which are collections of nerve cells deep inside the brain that control movement. Dozens of genetic conditions, autoimmune diseases, metabolic disorders, infections and medications can affect basal ganglia and lead to the onset of chorea.
What is chorea in medical terms?
Chorea is a movement disorder that occurs in many different diseases and conditions. Dozens of genetic conditions, autoimmune and infectious diseases, endocrine disorders, medications and even pregnancy can have chorea as a symptom. Treatment is based on cause of the chorea.
How do you know if you have chorea?
The most common signs of chorea are: Involuntary muscle movements: Also called fidgety movements or dance-like movements usually appear in the hands, feet, and face. They can affect the way you walk, swallow and talk.
What causes a person to have chorea?
The most common causes of chorea are: Huntington’s disease: People inherit this genetic disorder from their parents.
What causes chorea in the body?
People with other medical conditions: Autoimmune diseases (such as lupus) and hormonal disorders like hyperthyroidism and metabolic disorders such as hypoglycemia can cause chorea. There is a long list of other disorders that can cause chorea. Cleveland Clinic is a non-profit academic medical center.
What is a chorea?
What is chorea? Chorea is a movement disorder that causes involuntary, irregular, unpredictable muscle movements. The disorder can make you look like you’re dancing (the word chorea comes from the Greek word for “dance”) or look restless or fidgety.