Sydenham's chorea
Encyclopedia
Sydenham's chorea or chorea minor (historically referred to as Saint Vitus Dance) is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea (SC) results from childhood infection with Group A beta-hemolytic Streptococci and is reported to occur in 20-30% of patients with acute rheumatic fever
(ARF). The disease is usually latent, occurring up to 6 months after the acute infection, but may occasionally be the presenting symptom of rheumatic fever. Sydenham's Chorea is more common in females than males and most patients are children, below 18 years of age. Adult onset of Sydenham's Chorea is comparatively rare and most of the adult cases are associated with exacerbation of chorea
following childhood Sydenham's Chorea.
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. Saint Vitus is considered to be the patron saint of dancers, with the eponym given as homage to the manic dancing that historically took place in front of his statue during the feast of Saint Vitus in Germanic and Latvian cultures.
, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache
, slowed cognition, facial grimacing, fidgetiness and hypotonia
. Also, there may be tongue fasciculations ("bag of worms"), and a "milk sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking.
Non-neurologic manifestations of acute rheumatic fever are carditis
, arthritis
, erythema marginatum
, and subcutaneous nodules.
Fifty percent of patients with acute Sydenham's Chorea spontaneously recover after 2 to 6 months whilst mild or moderate chorea or other motor symptoms can persist for up to and over 2 years in some cases (for example a patient in the UK who has suffered the illness since 1999). Sydenham's is also associated with psychiatric symptoms with obsessive compulsive disorder being the most frequent manifestation.
The PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections) syndrome is similar, but is not characterized by Sydenham's motor dysfunction, but presenting with tics and/or with psychological components (OCD) and much sooner, days to week after GABHS infection rather than 6–9 months. It may be confused with other conditions such as lupus
and Tourette syndrome
.
Movements cease during sleep, and the disease usually resolves after several months. It is associated with post-streptococcal
rheumatic fever
, pregnancy
, hyperthyroidism
, and systemic lupus erythematosus
.
. The incidence of Acute Rheumatic Fever and rheumatic heart disease (RHD) is not declining. Recent figures quote the incidence of Acute Rheumatic Fever as 0.6 - 0.7/1 000 population in the USA and Japan compared with 15 - 21/1 000 population in Asia and Africa. The prevalence of Acute Rheumatic Fever and Sydenham's Chorea has declined progressively in developed countries over the last decades. There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism
, Wilson's disease
, Huntington's disease
, and infectious agents.
, included in a number of Holmes' stories published collectively in The Memoirs of Sherlock Holmes
.
American artist Andy Warhol was afflicted with rheumatic fever as a young child, and developed the symptoms of St. Vitus' Dance.
Michael Caine
suffered from St. Vitus' Dance when he was a child, as written in his autobiography What's it All About.
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...
(ARF). The disease is usually latent, occurring up to 6 months after the acute infection, but may occasionally be the presenting symptom of rheumatic fever. Sydenham's Chorea is more common in females than males and most patients are children, below 18 years of age. Adult onset of Sydenham's Chorea is comparatively rare and most of the adult cases are associated with exacerbation of chorea
Chorea (disease)
Choreia is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term choreia is derived from the Greek word χορεία , see choreia , as the quick movements of the feet or hands are vaguely comparable to dancing or piano playing.The term...
following childhood Sydenham's Chorea.
It is named after British physician Thomas Sydenham
Thomas Sydenham
Thomas Sydenham was an English physician. He was born at Wynford Eagle in Dorset, where his father was a gentleman of property. His brother was Colonel William Sydenham. Thomas fought for the Parliament throughout the English Civil War, and, at its end, resumed his medical studies at Oxford...
(1624–1689). The alternate eponym, "Saint Vitus Dance", is in reference to Saint Vitus
Vitus
Saint Vitus was a Christian saint from Sicily. He died as a martyr during the persecution of Christians by co-ruling Roman Emperors Diocletian and Maximian in 303. Vitus is counted as one of the Fourteen Holy Helpers of the Roman Catholic Church....
, a Christian saint who was persecuted by Roman
Roman Empire
The Roman Empire was the post-Republican period of the ancient Roman civilization, characterised by an autocratic form of government and large territorial holdings in Europe and around the Mediterranean....
emperors and died as a martyr in AD 303. Saint Vitus is considered to be the patron saint of dancers, with the eponym given as homage to the manic dancing that historically took place in front of his statue during the feast of Saint Vitus in Germanic and Latvian cultures.
Characteristics
Sydenham's chorea is characterised by the acute onset (sometimes a few hours) of neurologic symptoms, classically chorea, usually affecting all limbs. Other neurologic symptoms include behavior change, dysarthriaDysarthria
Dysarthria is a motor speech disorder resulting from neurological injury of the motor component of the motor-speech system and is characterized by poor articulation of phonemes...
, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
, slowed cognition, facial grimacing, fidgetiness and hypotonia
Hypotonia
Hypotonia is a state of low muscle tone , often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength...
. Also, there may be tongue fasciculations ("bag of worms"), and a "milk sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking.
Non-neurologic manifestations of acute rheumatic fever are carditis
Carditis
Carditis is the inflammation of the heart or its surroundings.It is usually studied and treated by specifying it as:*Pericarditis is the inflammation of the pericardium*Myocarditis is the inflammation of the heart muscle...
, arthritis
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
, erythema marginatum
Erythema marginatum
Erythema marginatum is described as the presence of pink rings on the trunk and inner surfaces of the limbs which come and go for as long as several months. It is found primarily on extensor surfaces....
, and subcutaneous nodules.
Fifty percent of patients with acute Sydenham's Chorea spontaneously recover after 2 to 6 months whilst mild or moderate chorea or other motor symptoms can persist for up to and over 2 years in some cases (for example a patient in the UK who has suffered the illness since 1999). Sydenham's is also associated with psychiatric symptoms with obsessive compulsive disorder being the most frequent manifestation.
The PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections) syndrome is similar, but is not characterized by Sydenham's motor dysfunction, but presenting with tics and/or with psychological components (OCD) and much sooner, days to week after GABHS infection rather than 6–9 months. It may be confused with other conditions such as lupus
Systemic lupus erythematosus
Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
and Tourette syndrome
Tourette syndrome
Tourette syndrome is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical tics and at least one vocal tic; these tics characteristically wax and wane...
.
Movements cease during sleep, and the disease usually resolves after several months. It is associated with post-streptococcal
Streptococcus
Streptococcus is a genus of spherical Gram-positive bacteria belonging to the phylum Firmicutes and the lactic acid bacteria group. Cellular division occurs along a single axis in these bacteria, and thus they grow in chains or pairs, hence the name — from Greek στρεπτος streptos, meaning...
rheumatic fever
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...
, pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
, hyperthyroidism
Hyperthyroidism
Hyperthyroidism is the term for overactive tissue within the thyroid gland causing an overproduction of thyroid hormones . Hyperthyroidism is thus a cause of thyrotoxicosis, the clinical condition of increased thyroid hormones in the blood. Hyperthyroidism and thyrotoxicosis are not synonymous...
, and systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
.
Causes
A major manifestation of acute rheumatic fever, Sydenham's chorea is a result of an autoimmune response that occurs following infection by group A β-hemolytic streptococci that destroys cells in the corpus striatum of the basal gangliaBasal ganglia
The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...
. The incidence of Acute Rheumatic Fever and rheumatic heart disease (RHD) is not declining. Recent figures quote the incidence of Acute Rheumatic Fever as 0.6 - 0.7/1 000 population in the USA and Japan compared with 15 - 21/1 000 population in Asia and Africa. The prevalence of Acute Rheumatic Fever and Sydenham's Chorea has declined progressively in developed countries over the last decades. There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism
Hyperthyroidism
Hyperthyroidism is the term for overactive tissue within the thyroid gland causing an overproduction of thyroid hormones . Hyperthyroidism is thus a cause of thyrotoxicosis, the clinical condition of increased thyroid hormones in the blood. Hyperthyroidism and thyrotoxicosis are not synonymous...
, Wilson's disease
Wilson's disease
Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease...
, Huntington's disease
Huntington's disease
Huntington's disease, chorea, or disorder , is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and dementia. It typically becomes noticeable in middle age. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea...
, and infectious agents.
Treatment and management
Treatment of Sydenham's Chorea is based on the following three principles:- The first tenet of treatment is to eliminate the streptococcus at a primary, secondary and tertiary level. Strategies involve the adequate treatment of throat and skin infections, with a 10-day course of penicillinPenicillinPenicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
when Sydenham's Chorea is newly diagnosed, followed by long-term penicillin prophylaxis. Behavioural and emotional changes may precede the movement disorders in a previously well child. - Treatment of movement disorders. Therapeutic efforts are limited to palliation of the movement disorders. HaloperidolHaloperidolHaloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
is frequently used because of its anti-dopaminergic effect.It has serious potential side-effects, e.g. tardive dyskinesiaTardive dyskinesiaTardive dyskinesia is a difficult-to-treat form of dyskinesia that can be tardive...
. In a study conducted at the RFC, 25 out of 39 patients on haloperidol reported side-effects severe enough to cause the physician or parent to discontinue treatment or reduce the dose. Other medications which have been used to control the movements include pimozidePimozidePimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
, clonidineClonidineClonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
, valproic acidValproic acidValproic acid is a chemical compound that has found clinical use as an anticonvulsant and mood-stabilizing drug, primarily in the treatment of epilepsy, bipolar disorder, and, less commonly, major depression. It is also used to treat migraine headaches and schizophrenia...
, carbamazepineCarbamazepineCarbamazepine is an anticonvulsant and mood-stabilizing drug used primarily in the treatment of epilepsy and bipolar disorder, as well as trigeminal neuralgia...
and phenobarbitone. - Immunomodulatory interventions include steroids, intravenous immunoglobulins, and plasma exchange. Patients may benefit from treatment with steroids; controlled clinical trials are indicated to explore this further.
Society and culture
St. Vitus' dance is noted as the malady suffered by the elderly physician from whom Dr. Watson purchased a medical practice in "The Stock-Broker's Clerk" by Arthur Conan DoyleArthur Conan Doyle
Sir Arthur Ignatius Conan Doyle DL was a Scottish physician and writer, most noted for his stories about the detective Sherlock Holmes, generally considered a milestone in the field of crime fiction, and for the adventures of Professor Challenger...
, included in a number of Holmes' stories published collectively in The Memoirs of Sherlock Holmes
The Memoirs of Sherlock Holmes
The Memoirs of Sherlock Holmes is a collection of Sherlock Holmes stories, originally published in 1894, by Arthur Conan Doyle.-Contents:The twelve stories of the Memoirs are:*"Silver Blaze"...
.
American artist Andy Warhol was afflicted with rheumatic fever as a young child, and developed the symptoms of St. Vitus' Dance.
Michael Caine
Michael Caine
Sir Michael Caine, CBE is an English actor. He won Academy Awards for best supporting actor in both Hannah and Her Sisters and The Cider House Rules ....
suffered from St. Vitus' Dance when he was a child, as written in his autobiography What's it All About.