After writing this recent post about Fyodor Dostoyevsky’s epilepsy, I decided it was time I re-read one of the great author’s novels, and chose The Idiot, because it contains Dostoyevsky’s most vivid descriptions of the epileptic aura. (It is widely believed that Dostoyevsky based the protagonist, Prince Myshkin, on himself.) I’m reading a Penguin Classics edition of the book, which was translated by David McDuff, and was first published in 2004.
In The Idiot, Myshkin’s epilepsy is first mentioned in chapter one. In the online edition of the book, which I quoted in the post about Dostoyevsky’s epilepsy, his condition is described as “some strange nervous malady – a type of epilepsy, with convulsive spasms.” But in McDuff’s translation, the same passage reads slightly differently; Myshkin is said to suffer from “some strange nervous illness akin to epilepsy or St. Vitus’s Dance, with tremors and convulsions.” This aroused my curiosity – I’d never before heard of Saint Vitus’s Dance, so I decided to investigate further.
St. Vitus’s Dance is a disorder of the nervous system that occurs following an A beta-haemolytic streptococcal infection. The condition is usually latent, with the symptoms presenting up to 6 months after the initial infection. It normally occurs between 5-15 years of age, but can also appear later in life, and affects girls about twice as much as it does boys. St. Vitus’s Dance is characterised by involuntary and uncoordinated movements of the face, hands and feet. (References to the facial convulsions can be found in the work of Sir Arthur Conan Doyle. On page 6 of The Greek Interpreter, for example, there is mention of a character whose “lips and eyes were continually twitching like a man with St. Vitus’s Dance”.) The condition is associated with acute rheumatic fever; it is reported in 20-30% of patients with rheumatic fever. It also has various tic disorders and neuropsychiatric symptoms associated with it, the most frequent being Tourette’s Syndrome and obsessive compulsive disorder. It has also been suggested that St. Vitus’s Dance, and Tourette’s and related neuropsychiatric conditions share the same mechanism of pathogenesis.
The convulsions characteristic of the condition are believed to be caused by an autoimmune reaction in neurons in the basal ganglia, a system of subcortical brain structures involved in the control of movement. Accordingly, the streptococci induce antibodies that cross react with basal ganglia antigens. The involuntary movements sometimes resemble a dance; St. Vitus, a fourth century Sicilian martyr, was the patron saint of dancers, hence the name of the condition. St. Vitus’s Dance is also known as Sydenham’s chorea, after the 17th century English physician Thomas Sydenham (1624-1689). The word “chorea” is derived from the Greek word khoreia, meaning “dance”. St. Vitus’s Dance is also sometimes referred to as chorea minor, but should not be confused with Huntingdon’s chorea (now renamed Huntington’s Disease, HD) an inherited neurodegenerative disorder. HD is also characterized by involuntary movements, caused by the death of basal ganglia cells in involuntary movements, and is sometimes referred to as chorea major.
Here is Sydenham’s classic description of the symptoms of St. Vitus’s Dance:
This 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.
In 1779, some 90 years after Sydenham’s death, a paper describing a treatment for St. Vitus’s Dance, by Anthony Fothergill, was published in the Philosophical Transactions. In the paper, entitled An Account of a Cure of the St. Vitus’s Dance by Electricity, Fothergill describes the symptoms presented by the patient:
Ann Agutter, a girl of ten years of age, of a pale, emaciated habit, was admitted an out-patient at the Northampton Hospital on the 6th of June last. From her father’s account it appeared (for she was speechless and with difficulty supported from falling by two assistants) that she had for six weeks laboured under violent convulsive motions, which affected the whole frame, from which she had very short intermissions, except during sleep; that the disease had not only impaired her memory and intellectual faculties, but of late had deprived her of the use of speech…I recommended, as a dernier resort, a trial of electricity, under the management of the Rev. Mr. Underwood, an ingenious electrician.
Fothergill then relates the treatment, which was described to him by Mr. Underwood, who performed it, in a letter dated September 16th, 1779:
July 5th. On the glass-footed stool for thirty minutes: sparks were drawn from the arms, neck and head, which caused a considerable perspiration, and a rash appeared in her forehead. She then received shocks through her hands, arm, breasts and back; and from this time the symptoms abated, her arms beginning to recover their uses.
July 13th. On the glass-footed stool for forty -five minutes: received shocks through her legs and feet, which from that time began to recover their wonted uses; also four strong shocks through the jaws, soon after which her speech returned.
July 23rd. On the glass-footed stool for the space of one hour: sparks were drawn from her arms, legs, head and breast, which for the first time she very sensibly felt; also two shocks through the spine. She could now walk alone, her countenance became more florid, and all her faculties seemed wonderfully strengthened, and from this time she continued mending to a state of perfect health.
This electricity-based treatment sounds extremely barbaric, but it was, in fact, way ahead of its time: it would be another 100 years or so before investigators determined unequivocally that the nervous system generates electrical signals, and it was not until very recently that researchers began using deep brain stimulation to interfere with the aberrant electrical signals underlying movement disorders such as Tourette’s Syndrome and Parkinson’s Disease.
St. Vitus’s Dance is now usually treated with antibiotics. Penicillin, administered orally or by intramuscular injection within one week of the onset of streptococcal pharyngitis (sore throat), and in combination with an immunosuppressant, can prevent onset of acute rheumatic fever. If the condition results in significant impairment of motor function, anticonvulsants such as sodium valproate, can be prescribed. This combination of drugs is preferred over dopamine receptor antagonists, which can cause side effects, particularly tardive dyskinesia (impairment of voluntary movements), with high doses or long-term use. In the most severe cases, treatment with dopamine antagonists can lead to drug-induced Parkinson’s Disease. Approximately 50% of patients with Sydenham’s Chorea recover spontaneously within 2-6 months. And there has, in recent years, been a decrease in the incidence of St. Vitus’s Dance in devloped countries, but its association with the tic disorders and neuropsychiatric symptoms has led to a renewed interest in the condition.