Historical Evolution of the Concept of Chorea

The word chorea comes from the ancient Greek choreia which means “dance in choir”. Terms like chorus or choreography have the same etymological origin (8). The first descriptions of chorea originated during the Middle Ages in Europe as a result of the black plague. People all over Europe were infected. Women were found to gather in a circle and dance frantically for hours until they fell to the ground, exhausted. In addition, some authors have stated that during the joint dance people experienced hallucinations and others suffered from abdominal distension, so it was often seen how they tied pieces of cloth around their abdomen (2). It is even attributed to the Belgian painter Pieter Brueghel (1525-1569), also known as the old one, the representation of what could be several people dancing with choreiform features (6).

In Italy, it was believed that the collective hysteria was caused by a venomous spider known as a tarantula. A musical rhythm known as “tarantella” was created to deal with the tarantismo derived from this spider’s bite (7,8). In turn, during this long period of time, an association between saints and different diseases became a common thing. And thus, dance crises started to be referred to as Saint Vitus Dance (chorea Sancti Viti). No doubt the miracle and the death of the saint are linked to this denomination, but also, miraculous healings were attributed to people affected by this ailment, when they had contact with some of Vitus’ relics in different Catholic temples in the old continent (9).

The prestigious Swiss physician Theophrastus Phillippus Aureolus Bombastus von Hohenheim, also known as Paracelsus (1493-1541), coined the term Saint Vitus Dance in medical terminology and classified it into three types: imaginative, lascivious and naturalist. In the year 1686 Thomas Sydenham (1624-1689) made a very precise clinical description of a condition that has been known for a long time as “minor chorea” (9). He also described rheumatic fever but did not associate it with chorea at the time. In 1850 Germain See (18181896) linked these two conditions. Near the end of the 19th century, with the advancement of neurology, Jean Martin Charcot (1825-1893), Silas Weir Mitchell (1829-1914), William Osler (1849-1919) and William Gowers (1845-1915) made significant contributions related to the etiology of chorea, particularly regarding diagnosis and differential diagnosis. In 1841, Charles Oscar Waters gave a very complete description of what later, thanks to the contributions of the American physician George Huntington (1850-1916), would carry his surname as an eponymous to this day and it is known as major chorea or Huntington’s chorea (10).

In 1894 a diplococcus was isolated from brain tissue of a young man affected by Sydenham’s chorea. From that moment several observers including Triboulet and Croyon (1897) Wesphal, Wassermann and Walkoff (1899) isolated different cocci from the pericardium and cerebrospinal fluid of children who had died from rheumatic pericarditis and had presented chorea (11).

Diseases such as erysipelas and puerperal fever have been described since the fourth century by Hippocrates (460-370 B.C.), father of medicine. In spite of these, the cause for these conditions was not found until the eighteenth century, thanks to the work of Anton van Leeuwenhoek (1623-1723), a microbiologist, who first observed microorganisms and proposed them as causal agents for various diseases (12).

One of the first descriptions of Streptococcus was conducted by the Austrian surgeon Theodor Billroth (18291894) who isolated the germ in fluids extracted from wound found in patients with erysipelas, and described it as a chain of four up to twenty links. Sometime after that Louis Pasteur (1822-1895) studied uterine blood from women with puerperal fever and, was able to isolate once again copious amounts of diplococci, rendering it responsible for the clinical presentation of the fever (12).

In the early twentieth century Paine and Holmes were able to induce pericarditis and abnormal movements in rabbits injected with intravenous diplococci found in the bloodstream of patients who had died from rheumatic pericarditis (13).

Around 1930 techniques involving titration of antistreptolysin antibodies were developed in patients suffering from streptococcus-secondary pharyngoamygdalitis, and diagnostic criteria for rheumatic fever were established (14). Today it is well known that there is a crossed-reaction between previously mentioned antibodies and basal ganglia, following a streptococcal infection (15).

In Latin America, the groundbreaking work of Dr. Americo Negrette a Venezuelan Neurologist, alongside Dr. Milton Wexler and the Hereditary Disease Foundation, led to a better understanding of Huntington’s disease including the thorough clinical descriptions of patients in Maracaibo, and the description of the huntingtin gene (16).

Currently, several types of causes for chorea are better understood (genetic, structural, infectious, autoimmune, toxic, metabolic, and drug-induced) and different therapeutic strategies to treat patients are available (10).

PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) have been associated with Sydenham’s chorea, due to its similarity with the clinical background. In both aforementioned conditions, patient gestures are characterized by choreiform movements of limbs particularly fingers and legs, mimicking the action of playing a piano. Aside from the observable characteristics of the movement, there is a molecular correlate. Antibodies produced both in PANDAS and Sydenham’s Chorea act on D2 dopamine receptor (17).

In addition to being the patron saint of chorea, Vitus is the saint of dance, probably because of the manner of his gruesome end.

It is worth noting that several scientific papers written in the past 20 years, continue to use the term “Saint Vitus’ dance” as a synonym of chorea (18–20). The influence of this historical occurrence and its supernatural character is indubitably major.

As it is true about many things in history, religion is a paramount influence over art, and more infrequently over science. The tragedy of Saint Vitus, has been imprinted on medical language for centuries, and it remains today, unknowingly applied in hospitals across the globe.

References

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Recibido: 24 de julio de 2018
Aceptado: 9 de agosto de 2018
Correspondencia:
Leonardo Palacios-Sánchez [email protected]

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