Sydenham's Chorea, a rare neurological disorder, originates from an infection caused by a bacterium known as Group A beta-hemolytic streptococcus (Ruiz, n.d.). Among its most notable symptoms is the alteration of movements, which significantly affects motor coordination (Ruiz, n.d.). According to Gonzáles et al. (2007), this disease was first recorded in the year 1416, although it was described in detail several centuries later, in 1686, by the English physician Thomas Sydenham.
Definition and General Characteristics
Sydenham's Chorea, also known as Saint Vitus' dance, Minor Chorea, and Rheumatic Chorea, is an inflammatory, degenerative, and non-progressive disease that affects the central nervous system (Ruiz, n.d.; MedlinePlus, 2020). This movement disorder is caused by the infection of bacteria belonging to Group A, specifically Group A beta-hemolytic streptococcus (Ruiz, n.d.). In addition to causing Sydenham's Chorea, streptococcus is responsible for other diseases, such as rheumatic fever and streptococcal pharyngitis. The pathophysiological mechanism of this disorder is related to the production of autoantibodies directed against the brain's basal ganglia. According to Gonzáles et al. (2007), these autoantibodies alter dopaminergic presynaptic activity following a streptococcal infection, which explains the characteristic motor symptoms of the disease.
Epidemiology
It is estimated that between 10% and 30% of patients affected by rheumatic fever develop Sydenham's Chorea, with an incubation period that generally varies between 8 and 12 months, followed by a progressive disappearance of the disease (Vásquez et al., 2018; Ruiz, n.d.). This is reflected in an annual incidence of between 300,000 and 350,000 cases (Vásquez et al., 2018). The most common age for the onset of Sydenham's Chorea ranges from 5 to 14 years. Before adolescence, it affects both genders similarly, while after puberty, there is a 2:1 ratio of girls to boys, indicating a possible influence of hormonal factors on its development, in addition to genetic and environmental components. In developed countries, the incidence of rheumatic fever and Sydenham's Chorea has decreased significantly in recent decades. However, according to Vásquez et al. (2018), in developing countries, both conditions continue to represent a significant public health problem.
Symptoms
Movement Disorders
Sydenham's Chorea is primarily characterized as a movement disorder, affecting various parts of the body (Ruiz, n.d.). The main change associated with this condition lies in the presence of uncoordinated, uncontrollable, purposeless movements that involve the hands, arms, shoulders, face, legs, and trunk (MedlinePlus, 2020). These movements, although markedly altered, have a limited duration and do not leave permanent neurological sequelae (Ruiz, n.d.). It is important to note that during sleep, the movements disappear completely, and while they can involve almost any muscle group, the ocular muscles are unaffected. Moreover, according to Ruiz (n.d.), in mild cases of Sydenham's Chorea, patients may experience difficulties in everyday tasks such as dressing or eating, which gives them a clumsy and uncoordinated appearance.
Psychic Disturbances
Temporary psychiatric alterations constitute a relevant symptom of Sydenham's Chorea (Ruiz, n.d.). In this context, Ruiz (n.d.) highlights three predominant symptoms: emotional instability, irritability, and restlessness.
Rheumatic Fever
Rheumatic fever is considered a symptom associated with Sydenham's Chorea, resulting from an infection caused by Group A streptococci (Ruiz, n.d.). This disease can manifest through various symptoms, including high fever, cardiac problems, and pain or swelling in the joints (MedlinePlus, 2020). Additionally, according to MedlinePlus (2020), lumps or rashes on the skin and nosebleeds may also occur.
Rheumatic Carditis
One-third of cases of Sydenham's Chorea are associated with another significant symptom, which is rheumatic carditis (Ruiz, n.d.). This condition, according to Ruiz (n.d.), causes irreversible damage to the heart valves, which can lead to serious complications for the patient's cardiovascular health.
Treatment
Regarding the treatment of Sydenham's Chorea, there is currently no medication that guarantees 100% effectiveness in all cases (Ruiz, n.d.). However, Ruiz (n.d.) notes that there are medications available that can alleviate symptoms, depending on their severity.
Phenobarbital
Phenobarbital is an antiepileptic drug that belongs to the class of barbiturates (Pinchón, 2021). This medication is considered the most effective treatment option for Sydenham's Chorea (Ruiz, n.d.). It is administered orally, and the duration of the prescription may extend as long as necessary to eliminate abnormal movements. According to Ruiz (n.d.), the effectiveness of this drug reaches approximately 80%.
Diazepam
Diazepam, commercially known as Valium, is a benzodiazepine (Cigna, 2021). This medication is often considered the second option for the treatment of Sydenham's Chorea (Ruiz, n.d.). In brief, according to Ruiz (n.d.), it is prescribed in cases where phenobarbital has not achieved the expected results.
Haloperidol
Haloperidol is a potent antipsychotic and a dopamine receptor antagonist in the brain (Spanish Pediatric Association, 2020). This medication is used as a third option to treat Sydenham's Chorea (Ruiz, n.d.). It is utilized when previous treatments have failed. However, according to Ruiz (n.d.), this drug is highly toxic in children.
Corticosteroids
Corticosteroids, or glucocorticosteroids, encompass a group of steroid hormones produced naturally in the adrenal cortex, as well as synthetic derivatives obtained by modifying their basic chemical structure (Muñoz, 2021). In this regard, according to Ruiz (n.d.), this type of medication is also used in some cases, although its effectiveness in improving the symptoms of Sydenham's Chorea has not been conclusively demonstrated.
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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