Sydenham's Chorea
Top Contributors - Mahnoor Zubair, Kim Jackson, Lucinda hampton, Khloud Shreif and Ewa Jaraczewska
Introduction
SC, or Sydenham's Chorea, is one of the neuropsychiatric disorders and a dominant form of acute rheumatic fever[1], accounting for the commonest form of chorea seen in children.[2] The movement disorder is connected to streptococcal infection and is quite rare. [3] Being generally benign and self-limiting, SC can, however, manifest as recurrent or persistent symptoms at times.[4]
SC occurs much more frequently in developing countries and in first nations people in certain countries eg there is a significant burden of Sydenham chorea among First Nations children living in Northern Australia.[5] [6]
Pathologenesis
The autoimmune response in SC is caused by molecular mimicry, where the antibodies generated against the causative GABHS cross-react with neuronal antigens of the basal ganglia and give rise to cross-reactive antibodies. Such antibodies are largely antineuronal antibodies that interfere with the fundamental functioning of the basal ganglia, and therefore the presentation of combined motor and neuropsychiatric manifestations.
Recommend both humoral (Th2) and cellular (Th1) immunity could participate in the pathogenesis, because indeed levels of Th2 cytokines-IL-4 and IL-10-were higher during the acute SC, but Th1 markers were sometime described in SC patients and one group found increased amounts of IL-12 chemokines CXCL9 and CXCL10 within the sera of SC patients: while vascular mechanisms will apparently play a role; still, data now mostly emphasis the role of cross-reactive antibodies in the development of the disease.[7]
Clinical Presentation
Sydenham's Chorea is essentially a combination disorder of both motor and non-motor features.
For example, it is associated by chorea, although actually more typically seen with diminution of tone and even tics. Nonmotor features are obsessions, compulsions, attention deficiency, emotional lability, decreased verbal fluency, and executive impairment. Most all have accompanying acute rheumatic fever features such as carditis and arthritis. [7]
Diagnostic Procedures
The diagnosis is made after proper clinical assessment, as it solely serves as the basis of diagnosis. Because, no specific biological markers for the illness are currently available. [7]
Management
Pharmacological Management:
In SC, antibiotic prophylaxis is important, aimed at lowering the opportunity of reinfections by Group A beta-hemolytic streptococcal (GABHS) and consequently the cardiac complications and recurrence of chorea. Anti-chorea drugs of symptomatic treatment include neuroleptics and anti-epileptic drugs; immunomodulatory therapies might be used for cases in which those treatments have failed. Emerging evidence shows that early immunomodulatory treatment could become helpful to prevent recurrence or persistence of symptoms in SC.[8]
Although its role in recurrent chorea and further cardiac complications can be reduced through penicillin prophylaxis, little data is available on the symptomatic therapy of chorea. Much of what is known today is in the form of individual case reports, small case series, and few comparison studies. For example, steroid use has been successful in some instances, although this is based mainly upon a single placebo-controlled study besides several smaller case series. A few reports and a comparison study have examined other immunomodulatory treatments, including IVIG and plasmapheresis, so further research would be necessary to determine their effectiveness in managing Sydenham's Chorea. [9]
Role of Physiotherapy:
The major areas involved in the motor disturbances and functional impairments in Sydenham's Chorea are therefore handled by physiotherapy. As coordination of muscles, muscle tone, and fine motor disturbances are central to SC, physiotherapy intervention becomes important to further enhance the control over movements and further reduce chorea.
Differential Diagnosis
Differential diagnosis of SC involves the process of elimination of all conditions that may cause similar involuntary movements, neuropsychiatric symptoms, or both.
Key differentials include:
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, or PANDAS, is identical in its manifestation of having chorea and neuropsychiatric symptoms, but other criteria include obsessive-compulsive behaviors and tics. [10]
Systemic Lupus Erythematosus: SLE-associated chorea can be distinguished by specific laboratory findings and other systemic signs.[11]
Huntington's Disease: SC is also unlike this other inherited disease that is of a progressive nature and genetic basis. [12]
Drug-induced Chorea: There are certain drugs known to cause chorea as a side effect.
Chorea Gravidarum: Specific to pregnancy, this condition typically has a self-limiting course.[13]
References
- ↑ Orsini A, Foiadelli T, Magistrali M, Carli N, Bagnasco I, Dassi P, Verrotti A, Marcotulli D, Canavese C, Nicita F, Capuano A. A nationwide study on Sydenham's chorea: Clinical features, treatment and prognostic factors. European Journal of Paediatric Neurology. 2022 Jan 1;36:1-6.
- ↑ Dean SL, Singer HS. Treatment of Sydenham’s chorea: a review of the current evidence. Tremor and Other Hyperkinetic Movements. 2017;7.
- ↑ Ridel KR, Lipps TD, Gilbert DL. The prevalence of neuropsychiatric disorders in Sydenham's chorea. Pediatric neurology. 2010 Apr 1;42(4):243-8.
- ↑ Gurkas E, Karalok ZS, Taskin BD, Aydogmus U, Guven A, Degerliyurt A, Bektas O, Yilmaz C. Predictors of recurrence in Sydenham’s chorea: clinical observation from a single center. Brain and Development. 2016 Oct 1;38(9):827-34.
- ↑ Beier K, Pratt DP. Sydenham chorea.Available:https://www.ncbi.nlm.nih.gov/books/NBK430838/ (accessed 1.11.2024)
- ↑ Soller T, Roberts KV, Middleton BF, Ralph AP. Sydenham chorea in the top end of Australia's Northern Territory: A 20‐year retrospective case series. Journal of Paediatrics and Child Health. 2023 Nov;59(11):1210-6. Available:https://pubmed.ncbi.nlm.nih.gov/37589435/ (accessed 1.11.2024)
- ↑ 7.0 7.1 7.2 Cardoso F. Sydenham's chorea. Handbook of clinical neurology. 2011 Jan 1;100:221-9.
- ↑ Vasconcelos LP, Vasconcelos MC, Nunes MD, Teixeira AL. Sydenham’s chorea: an update on pathophysiology, clinical features and management. Expert Opinion on Orphan Drugs. 2019 Nov 2;7(11):501-11.
- ↑ Dean SL, Singer HS. Treatment of Sydenham’s chorea: a review of the current evidence. Tremor and Other Hyperkinetic Movements. 2017;7.
- ↑ Swedo SE, Leonard HL, Rapoport JL. The pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) subgroup: separating fact from fiction. Pediatrics. 2004 Apr 1;113(4):907-11.
- ↑ Bernatsky S, Boivin JF, Joseph L, Manzi S, Ginzler E, Gladman DD, Urowitz M, Fortin PR, Petri M, Barr S, Gordon C. Mortality in systemic lupus erythematosus. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology. 2006 Aug;54(8):2550-7.
- ↑ Walker FO. Huntington's disease. The Lancet. 2007 Jan 20;369(9557):218-28.
- ↑ Karageyim AY, Kars B, Dansuk R, Aygun E, Unal O, Turan MC. Chorea gravidarum: a case report. The Journal of Maternal-Fetal & Neonatal Medicine. 2002 Jan 1;12(5):353-4.