Sydenham’s chorea (Sc), also known as St Vitus Dance or chorea minor, is a disease with a long history. It has been suggested that the condition can be traced to around 400 BC in the writing of the Greek ‘Father of Medicine’, Hippocrates (Quinn 1991). In more recent history, Sc has been a recognised condition since the 17th century, when the pattern of the illness was described by the English physician Thomas Sydenham. During the 19th century, Sc came to be understood to be part of a disease that could affect not just the brain (causing the characteristic abnormal movements) but also potentially , heart, joints and skin, the disease now recognised as rheumatic fever (RF).
RF was a major cause of illness in Europe and North America until the 1950s, and with Sc it has attracted the interest of some of the most well-known physicians in history – this is described further below. In the last century both RF and Sc have become much less common in North America and Europe to the point where they are no longer a focus of medical interest. Sc and RF have increasingly been identified as a significant cause of illness and long-term disability in other parts of the world but until recently this had received relatively little attention from researchers.
Although these diseases have become rare in many countries where it was previously widespread, the reasons for this are not well understood – it could be changing environments, the bacteria responsible (streptococcus) itself changing in some fundamental way, or the widespread use of antibiotics (Carapetis 2016).
In Europe and North America in the nineteenth century, when many of the foundations of modern medicine were laid, mental and neurological disturbance in people was often associated with an underlying infection. Syphilis was a major cause of admissions for insanity in the 19th century asylum (Swayne 2018) and tuberculosis (Tb, also known as phthisis) was common in asylums and similarly was seen as a causal factor (Clouston, 1864). The emotional disturbance (‘mania’) often associated with chorea could also be understood as a mental health problem caused by an infection. In the twentieth century bacterial conditions that might lead to mental as well as physical illness have been better understood and diagnosed, and are now treated (and in some cases also prevented) by antibiotics. Unlike the position with Tb and syphilis, in Sc the precise relationship between infection and symptoms is only gradually being understood.
The first recognised and detailed discussion of a condition that resembles our modern idea of Sc comes six hundred years ago when Paracelsus (Swiss physician, alchemist) investigated the causes of a condition that had become known as St Vitus’ Dance. St Vitus (also known as St Guy, patron saint of dancers, as well as actors, comedians, and epileptics) was a Roman martyr, noted for his powers of healing. Epidemics of uncontrollable dancing had been described in the area around the Rhine for more than three hundred years – sometimes involving hundreds of people, dancing for hours and collapsing (whence, perhaps, there has been confusion with epilepsy). Tthere were various supernatural explanations advanced for this. These ‘plagues’ tended to occur in June, around the time of St Vitus’ feast day on 15th June. Which is also close to 24th June, the feast of St John, the patron saint of epileptics. There is a tradition of dancing at St Vitus’ statue on his saint’s day. Mass healings were reported at the chapel of St Vitus in Alsace in 1278 which may be how the disease became associated with the saint. (Park 1990, Waller 2009)
By the 16th century, there was debate about possible causes of this condition including moral or spiritual causes or a medical cause (from ‘overheated blood’, for example). (Pennant Rea 2018, Park 1990). After reviewing a particularly severe episode of ‘dancing plague’ that broke out on St John’s Day (June 24th) in Strasbourg in 1518 Paracelsus used the medical terminology of ‘chorea’ (meaning dancing in Greek) and recognised anxiety and involuntary laughter as part of the condition. He tried to differentiate forms of chorea caused by disease (chorea naturalis) from that caused by imagination (chorea imaginativa) or sexual desire in 1564, (chorea lasciva). (Park, 1990))
A well-known image from a similar time and a nearby geographical region was made by the artist Pieter Bruegel the Younger and labelled a drawing of the “epileptic women of Molenbeek”. Probably drawn in the 1560s, the subjects are not typically epileptic as almost all are conscious. Many are being supported, with both arms held stiffly by their sides, some also have marked twisting of the neck – both suggest abnormal movements or muscle tone. The inscription below the image says these are pilgrims who “must dance”, which suggests this was an example of a ‘dancing plague’, and who hope to be cured of “St John’s disease”, which is usually thought to be epilepsy. Epilepsy would not be expected to occur in epidemics or outbreaks. It has been suggested that there was an overlap in disease patronage between St Vitus and St John (Park, 1990).
One modern theory of the ‘dancing plagues’ of the late middle-ages has been to attribute this to epidemic ergot poisoning (from poorly stored grain), although it has been suggested that this would make prolonged dancing impossible (Waller, 2009). Another explanation linked to the specific social and cultural conditions of the time suggests that a form of psychic contagion (mass hysteria) occurred (Waller, 2009). Parallels have been drawn with tarantism, which is a similar condition, where there was a compulsion to dance, cry, tremble and laugh for days on end. Originally thought to be triggered by spider bites, it affected individuals or groups in Southern Italy in the 15-17th centuries. Of course, it is not possible to know if any of the people involved in the ‘dancing plagues’ suffered from a form of chorea that was like the Sc that we see today.
Thomas Sydenham (1624-1689) was an English 17th century doctor, from the fabulously named village of Wynford Eagle, in Dorset. He wasn’t particularly famous in his time, despite his recipe for Laudanum (opium and sherry, with spices [Stefano 2017]) and his pioneering treatment of malaria with quinine (in the form of Cinchona bark) (Williams 2006). Besides describing Sydenham’s chorea, his fame lies in his claim to set aside speculative theories in medicine at a time of quackery, and his emphasis on the importance of clinical observation. He has been given the title of the ‘English Hippocrates’ by later historians (Ainstey 2011, Hajar 2016). He was also honest about the value (in some cases) of “doing nothing at all”.
Sydenham certainly gives a great description (Quoted by English 1999)) of a condition that seems to be the Sc that we recognise today, he called it “chorea minor” –
“There is a kind of convulsion, which attacks boys and girls from the tenth year to the time of puberty. It first shows itself by limping or unsteadiness in one of the legs, which the patient drags. The hand cannot be steady for a moment. It passes from one position to another by a convulsive movement, however much the patient may strive to the contrary. Before he can raise a cup to his lips, he does make as many gesticulations as a mountebank; since he does not move it in a straight line, but has his hand drawn aside by the spasms, until by some good fortune he brings it at last to his mouth. He then gulps it off at once, so suddenly and so greedily as to look as if he were trying to amuse the lookers-on.”
Sydenham also provided a classic description of ‘rheumatism’, the acute fever with joint disease that we now recognise as part of RF, but he did not observe chorea and rheumatism occurring in the same patient (English 1999). An association of chorea with ‘rheumatism’ was made in 1810 by Etienne Michel Bouteille, and from 1831 Richard Bright, in London, had linked both chorea and febrile polyarthritis with ‘rheumatism’. This view was promoted by German See who noted in 1850 that ‘for every two rheumatic children there is at least one who is choreic’. (Hajar 2016, Cardoso 2015). The link with ‘rheumatism’ (which included a risk of heart disease) helped clarify chorea as a specific kind of involuntary movement.
The potential for heart problems after chorea was increasingly recognised in the late 19th century. The term rheumatic fever gradually replaced the idea of ‘acute rheumatism’. Nevertheless, in 1898 the link between ‘Rheumatic Heart Disease’ and chorea was still a matter for discussion (Lomax 1996).
Sc is only one cause of chorea and the other causes were gradually elucidated in the later nineteenth century. Published in America in 1872, Huntington’s initial manuscript ‘On chorea’ describing the hereditary chorea he would become associated with, was mostly about other conditions, including Sydenham’s (Cardoso 2015).
The French physician Charcot tried to elaborate on the different causes but seems to have been unaware of the adult hereditary form, described by Huntington (Cardoso, 2005). One of Charcot’s patients with chorea at the Salpetriere Hospital, Jane Avril, became a dancer at the Moulin Rouge. Jane fascinated the artist Henri de Toulouse Lautrec, inspiring several famous images. Her dancing style was unique (“an orchid in a frenzy”) and has been considered to have developed in response to her being affected by St Vitus Dance (Jones, 2011). More recently, Jane Avril’s experience has been described in relation to her abusive childhood and her own records of her experience of two years in an adult female ward in her late teens (Bonduelle, 1999).
In 1884 Charcot gave an assignment to Gilles de la Tourette (who gave his name to Tourette syndrome), who was one of his students, to “sort out the chaos of the choreas” but the task was not completed until William Richard Gowers, a leading neurologist in late nineteenth century London, drew together his long experience of seeing Sc in children with accounts of late onset forms of chorea, chorea in other disease patterns and psychogenic chorea (Cardoso 2015). William Osler in a book on chorea pointed out that the term incorporated a wide range of different movement disorders (Osler 1894, from Cardoso 2015); he described 410 cases of Sydenham’s chorea seen in Philadelphia in the period from 1876 and recognised the infectious basis of the condition and the link to heart valve disease.
Chorea was a major cause of hospitalisation for children in the nineteenth century. Sc was the fourth most frequent cause of admission to Great Ormond St hospital (GOSH) between 1860 and 1900 (and was in the 1880s temporarily the second most frequent cause). Similarly, in Manchester where children with scarlet fever, measles or whooping cough were sent to a separate fever hospital at Monsall, the Children’s Hospital at Pendlebury saw chorea more often than any other condition except TB (Lomax). Delay in diagnosis was also an issue back then, as it is now – “a child with Sydenham’s chorea is punished three times before the diagnosis is made: once for general fidgetiness, once for breaking crockery, and once for making faces at his grandmother”. [text quoted in Wilson, 1969]. In 1887, 45% of cases at GOSH had other affected family members. Having someone else at home who had also been affected was something that helped the diagnosis, in the absence of any useful tests (Martino 2005).
Given the variable course of the condition, hospital admission may not have been strictly necessary for all, but a cautious approach was justified by observations of some children who progressed rapidly from chorea onset to heart symptoms and eventual death (English 1999). Large numbers of children who recovered with persistent signs of heart disease were placed on extended bedrest, for up to one year (English 1999). In 1953 an American paediatrician, Berry Brazelton, published a study of the difficult emotional consequences of this experience, which concluded with an emphasis on the need to give children a factual understanding of their condition to support their capacity to cope with the restrictions it imposed.
Ideas about what causes Sc have changed with the development of medical understanding through history. The approach of Paracelsus to the Strasbourg ‘plague’ shows attempts to separate disease from moral or spiritual problems. In the evolution of medical understanding there followed various concepts about imbalances in the body and a range of theories about the role of the environment (bad air, or “miasma”) or of heredity. A search for infectious causes of Sc began in the nineteenth century but it was only in the 1930s that the bacteria known haemolytic streptococci began to be perceived as a common cause of both RF and Sc. (Lomax 1996). In 1874, Theodor Billroth, a Viennese surgeon studying RF, identified Streptococcus as a type of bacteria causing scarlet fever. Triboulet, Croyon (1897) Wesphal, Wassermann and Walkoff (1899) isolated cocci from the heart and brain of children who died of Sc and ARF (Cardoso 2015). In 1918 the species of streptococci were divided into two groups A and B, later it was found that Group A Streptococcus pyogenes was generally present in a throat infection prior to the onset of ARF and Sc. (Hajar, 2016). How infection led to disease was still not explained, but it suggested treatment could involve using antibiotics. An understanding of autoimmune processes and the development of a robust model for the causation of Sc has been the business of more recent researchers and the story is still not complete.
The dancing plagues of the middle-ages attracted a range of responses from church and state. Possibly the most disastrous was in 1518 in the Strasbourg episode studied by Paracelsus, where professional dancers were employed and a stage set up to encourage the afflicted, which caused a dramatic escalation of the problem (Waller 2009). Other early attempts at treatment included Sydenham’s advice for bleeding and purging but the simple remedy of rest and avoidance of emotional and physical excitement is likely to have been helpful. In 1876, a Dundee physician, Thomas Maclagan, noted that as rheumatism and willow trees both thrived in damp places the illness might respond to salicin, an extract of willow bark (English 1999), which proved effective for fever and joint disease but less so for chorea. From 1875 onwards, salicin and related salicylates (which includes aspirin) were used in the acute treatment of rheumatism and chorea, with this discontinued in favour of other symptomatic treatments (such as opium) if cardiac complications ensued (Lomax 1996).
By the late 1930s sulphonamides were shown to prevent recurrences of rheumatic fever, but these early antibiotics were unable to eradicate the acute infection. Their widespread use in military settings was soon followed by the development of drug resistance (English 1999). In the 1940s, prompt treatment of streptococcal pharyngitis with the new antibiotic penicillin was shown to prevent onset of ARF, and long-term penicillin began to be used to protect people from further risk of infection.
Specific remedies for the movement disorder of chorea were varied and sedatives of various kinds including antihistamines remained central to treatment of more extreme cases until more specific drugs such as antipsychotics and antiepileptic drugs with action on brain neurotransmitters emerged in the second half of the twentieth century. Following early trials of corticosteroids, immunotherapies have now become more widely used for chorea.
The story of Sc is intertwined with wider themes in the history of disease. It may be traced back for many centuries. It has been a major cause of childhood illness and death but its impact on individuals and on populations has varied considerably. The decline in incidence of Sc mirrored the decline in RF in many countries. In the UK, by the 1950’s it was noted that streptococcal throat infections were followed by RF in only 1% of cases, whether or not antibiotics were given. Sc, the chorea associated with RF, has been a more or less prominent feature of the disease over time. This highlights outstanding questions about the immune processes by which infection leads to a particular form of disease and may be a key to understanding the increasing recognition of auto-immune neuropsychiatric conditions, without features of RF, occurring after streptococcal infection (often described as ‘PANDAS’). Following the immune hypothesis, English (1999) suggests that this variation reflects a change in the organism with less exhibition of the components that might trigger cross reactivity with human tissue. Research has so far not established a reason for the changing impact of Sc and RF over time and across the globe.
The authors wish to acknowledge the syntheses of ideas by previous authors cited below, particularly noting the work of Peter English and Thiago and Francesco Cardoso.
Ainstey P. The Creation of the English Hippocrates. Med Hist 2011 Oct. 55(4):457-478. doi: 10.1017/s0025727300004944
Berry Brazelton T, Emotional Aspects of Rheumatic Fever in Children. Journal of Pediatrics (1953) 43: 339-358.
Bonduel M & Gelfand T, Hysteria Behind the Scenes: Jane Avril at the Salpetriere. Journal of the History of the Neurosciences 1999; 8:1, 35, doi: 10.1076/jhin.8.1.35.1778
Carapetis JR, Beaton A, Cunningham MW, Guillherme L, Karthikeyan G, Mayosi BM, Sable C, Steer A, Wilson N, Wyber R Zulker L. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Primers 2, 15084 (2016) doi:10.1038/nrdp.2015.84
Cardoso T & Cardoso F, Chorea: a Journey through History. Tremor Other Hyperkinetic Mov. 2015; 5 doi: 10.7916/D8WM1C98
English P, Rheumatic fever in America and Britain: a biological, epidemiological, and medical history. 1999, New Brunswick, Rutgers University Press.
Hajar R, Rheumatic fever and rheumatic heart disease, a historical perspective. Heart Views 2016; 17:120-6 doi: 10.4103/1995-705X.192572
Jones R, Toulouse-Lautrec at the Courtauld: Just beyond the Moulin Rouge: British journal of General Practice 2011; 61 (589):524 doi:10.3399/bjgp11X588565
Lomax ER, Medical Care. Medical History 1996; 40 (S16): 91-106 doi: 10.1017/S0025727300072744
Martino D, et al, Tracing Sydenham’s chorea: historical documents from a British paediatric hospital. Arch Dis Child 2005; 90:507-511 doi: 10.1136/adc.2004.057679
Osler W. On chorea and choreiform affections. London: HK Lewis & Company, 1894.
Park RHR & Park MP, Saint Vitus’ dance: vital misconceptions by Sydenham and Breughel. Journal of the Royal Society of Medicine 1990; 83, 512-515 https://journals.sagepub.com/doi/pdf/10.1177/014107689008300814
Pennant-Rea N, The Dancing Plague of 1518; The Public Domain Review 2018 https://publicdomainreview.org/essay/the-dancing-plague-of-1518/
Quinn R, Did Scarlet Fever and Rheumatic Fever Exist in Hippocrates Time? Reviews of Infectious Diseases 1991; 13:123-4 https://doi.org/10.1093/clinids/13.6.1243
Stefano GB, Pilonis N, Ptacek R, Kream RM, Reciprocal Evolution of Opiate Science from Medical and Cultural Perspectives. Med Sci Monit., 2017; 23: 2890-2896 https://doi.org/10.12659%2FMSM.905167
Swayne K, ‘Extraordinarily arduous and fraught with danger’; syphilis, Salvarsan and general paresis of the insane. The Lancet Psychiatry2018: https://www.thelancet.com/pdfs/journals/lanpsy/PIIS2215-0366(18)30221-9.pdf
Waller J, A forgotten plague: making sense of dancing mania. The Lancet 2009; 373, 9664, 624-625
Williams AN, Physician, philosopher, and paediatrician: John Locke’s practice of child health care Arch Dis Child, 2006 Jan; 91(1): 85–89. doi: 10.1136/adc.2005.078162
Wilson SK. Neurology, 2nd edn. New York: Hafner 057679Press 1969
English (UK) 
Italiano 
Deutsch 
Español 
Português