Sydenham's chorea is a rare neurological disorder caused by infection with a type of bacteria called group A b-hemolytic streptococcus (Ruiz, n.d.). Its main symptom is an alteration of movements (Ruiz, n.d.). According to Gonzáles et al. (2007), this disease was first described in 1416 and in detail by Thomas Sydenham in 1686.
Definition and General Characteristics
Sydenham's chorea is a degenerative, non-progressive, inflammatory disease of the central nervous system (Ruiz, n.d.). Other names it has received are: St. Vitus' disease, Chorea Minor, and Rheumatic chorea (MedlinePlus, 2020). It is a movement disorder caused by infection with bacteria belonging to group A (Ruiz, n.d.). Specifically, according to Ruiz (n.d.), the bacterium that causes Sydenham's chorea is called group A b-hemolytic streptococcus.
Streptococcus is a bacterium that causes other diseases, such as rheumatic fever and streptococcal pharyngitis (Ruiz, n.d.). According to Gonzales et al (2007), the pathophysiological mechanism of Sydenham's chorea is due to the production of autoantibodies against the basal ganglia of the brain that alter dopaminergic presynaptic activity and are produced after streptococcal infection.
Epidemiology
It is estimated that between 10 and 30 percent of patients affected by rheumatic fever develop Sydenham's chorea, with an incubation period that is usually 8 to 12 months, with a gradual disappearance of the disease (Vásquez et al., 2018; Ruiz, n.d.). Vasquez, et al. mention that, the above is reflected in an annual incidence of between 300,000 and 350,000 cases per year.
The most frequent age of presentation of Sydenham's chorea is between 5 and 14 years old (Vásquez et al., 2018). According to Vasquez et al. (2018), it similarly affects both genders before adolescence and in a 2:1 girl/boy ratio after puberty, suggesting hormonal factors in its pathogenesis, as well as genetic and environmental factors.
Corresponding with Vasquez et al. (2018), in developed countries, the incidence of rheumatic fever and Sydenham's chorea has decreased dramatically in recent decades, however, in developing countries both diseases remain a public health problem.
Symptoms
Movement Disorders
The main change in Sydenham's chorea is a movement disorder (Ruiz, n.d.). Sydenham's chorea mainly involves disjointed, uncontrollable, and purposeless movements of the hands, arms, shoulders, face, legs, and trunk (MedlinePlus, 2020). According to Ruiz (n.d.), its duration is limited and does not leave neurological sequelae.
These movements disappear during sleep and affect any muscle group, except the ocular muscles (Ruiz, n.d.). Likewise, in correspondence with Ruiz (n.d.), it is necessary to point out that in mild cases of Sydenham's chorea, patients simply present difficulties in dressing and feeding themselves, in addition to a clumsy appearance.
Psychic Disturbances
Another symptom of Sydenham's chorea is temporary psychic disturbances (Ruiz, n.d.). According to Ruiz (n.d.), the three most common symptoms are emotional instability, irritability and restlessness.
Rheumatic Fever
Rheumatic fever is another symptom of Sydenham's chorea and, likewise, is the consequence of a group A streptococcal infection (Ruiz, n.d.). Corresponding to MedlinePlus (2020), these can include high fever, heart problems, joint pain or swelling, skin lumps or rashes, and nosebleeds.
Rheumatic Carditis
One third of Sydenham's chorea cases are related to another symptom: rheumatic carditis (Ruiz, n.d.). According to Ruiz (n.d.), this is a condition that causes permanent damage to the heart valves.
Treatment
Regarding the treatment of Sydenham's chorea, Ruiz (n.d.) mentions that there is currently no drug that is 100% effective in all cases, however, there are medications that can alleviate symptoms depending on their severity.
Phenobarbital
Phenobarbital is an antiepileptic drug that belongs to the barbiturate class (Pinchón, 2021). This is the drug of choice to treat Sydenham's chorea (Ruiz, n.d.). It is administered orally and the prescription can be maintained as long as necessary to eliminate abnormal movements. According to Ruiz (n.d.), its efficiency is about 80%.
Diazepam
Diazepam or valuim is a benzodiazepine (Cigna, 2021) This drug is usually the second choice for treating Sydenham's chorea (Ruiz, n.d.). In other words, in correspondence with Ruiz (n.d.), it is administered in cases where phenobarbital has failed.
Haloperidol
Haloperidol is a potent neuroleptic and a brain dopaminergic receptor antagonist (Asociación Española de Pediatría, 2020). This drug is the third option for treating Sydenham's chorea (Ruiz, n.d.). It is used when previous medications have not worked (Ruiz, n.d.). However, according to Ruiz (n.d.), in children it is very toxic.
Corticosteroids
Corticosteroids, or more technically glucocorticosteroids, include, on the one hand, a range of steroid hormones produced naturally in the adrenal cortex and, on the other hand, synthetic derivatives obtained by altering their basic chemical structure (Muñoz, 2021). In correspondence with Ruiz (n.d.), this type of medication is also used in some cases, although it has not been 100% proven to improve the symptoms of Sydenham's chorea.
References
Amadi, M. A., Gareis, M. T., Orlandi, M., Pérez, F. E., & Goldaracena, P. X. (2019). ¡Alerta fiebre reumática!: a propósito de tres casos de Corea de Sydenham. CIC Digital. Recuperado 4 de marzo de 2022, de https://digital.cic.gba.gob.ar/handle/11746/11055
Asociación Española de Pediatría. (2020). Haloperidol. Recuperado 6 de abril de 2022, de https://www.aeped.es/comite-medicamentos/pediamecum/haloperidol
Cigna. (2021). Diazepam. Recuperado 6 de abril de 2022, de https://www.cigna.com/es-us/individuals-families/health-wellness/hw/medicamentos/diazepam-d00148a3
González, G. M., Mayol, L., Villalobos, P., Vásquez, M., & Cabacas, A. (2007). Corea de Sydenham: presentación de un caso tratado con carbamazepina con excelente respuesta clínica | Anales de Pediatría. Anales de Pediatría. Recuperado 4 de marzo de 2022, de https://www.analesdepediatria.org/es-corea-sydenham-presentacion-un-caso-articulo-13097365
MedlinePlus. (2020). Corea de Sydenham. Recuperado 6 de abril de 2022, de https://medlineplus.gov/spanish/ency/article/001358.htm
Muñoz, F. J. (2021). Los corticoides. Fundación BBVA. Recuperado 6 de abril de 2022, de https://www.fbbva.es/alergia/el-tratamiento-de-las-enfermedades-alergicas/los-corticoides/
Pinchón, D. (2021). Fenobarbital: ¿Qué es y para qué sirve? Prixz. Recuperado 6 de abril de 2022, de https://prixz.com/salud/fenobarbital-que-es-y-para-que-sirve/
Ruiz, L. (s.f.). Corea de Sydenham: causas, síntomas y tratamiento. Psicología y Mente. Recuperado 4 de marzo de 2022, de https://psicologiaymente.com/clinica/corea-sydenham
Vásquez, S., Correa, M. C., Rojas, I. C., Tieck, M. P., & Díaz, A. (2018). Corea de sydenham: revisión práctica de la literatura actual. Revista Mexicana de Neurociencia. Recuperado 4 de marzo de 2022, de http://previous.revmexneurociencia.com/articulo/corea-de-sydenham-revision-practica-de-la-literatura-actual/
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