Table of Contents
Primary Category
Movement Disorder
P-Category
Secondary Category
S-Category
Introduction
- A rare hyperkinetic autoimmune movement disorder affecting children
- Phenomenology: brief, involuntary, jerky, uncontrollable movements of the face, arms, legs, and trunk muscles
Affected Population
- More prevalent in girls more than boys
- More common between the ages of 5 and 15
- It affects all races and ethnicities
- 25% of patients with rheumatic fever develop Sydenham chorea
Clinical Features
- Acute to subacute onset following streptococcal infection
- In addition to chorea, symptoms also include behavioral changes, including anxiety, obsessive-compulsive disorder, and attention
- In rare cases, affected children may become bedridden due to severe muscle weakness, and this condition is called paralytic chorea
- The symptoms may vary and are presented in Table 1
Signs & Symptoms
- Choreic movements
- Muscle weakness
- Behavioural disturbances such as inattention/obsessions/compulsion/anxiety/ labile mood
- Facial grimacing
- Restlessness
- Slurred speech
- Diminished muscle tone
Causes
- The choreiform movement is caused by the autoimmune reaction to the streptococcal infection
- The antibodies produced by the immune system persist and attack certain cells in the joints, kidneys, heart and in the brain specifically basal ganglia which plays a key role in controlling motor movements
Differential Diagnosis
- Autoimmune or paraneoplastic: Anti-Hu(ANNA-1); Anti-NMDA; anti-CRMP5; anti-IgLON5; anti-LGI-1
- Polycythemia vera
- Endocrinological disorders-hyperparathyroidism, pseudohypoparathyroidism, both hypo or hyperthyroidism, and nonketotic hyperglycemia
- Neurodegenerative disorders: Huntington's disease, Dentatorubropallidolysian atrophy, C9orf72 disease, and Spinocerebellar ataxias
Related Disorders
- Abrupt withdrawal of Psychiatric Medications
- Diseases related to metabolism or mitochondrial function
- Neuroacanthocytosis, Ataxia-telangiectasia, Anti-NMDA receptor encephalitis
Diagnosis
- Clinically - acute onset of choreic movements associated with behavioral changes
- A confirmation of streptococcal infection via swab
- High blood titres of streptococcal antibodies such as ASO is also useful in making the diagnosis
- Brain imaging is usually normal in Sydenham Chorea
- Often during the acute phase SPECT shows striatal hyperperfusion which evolves to hypoperfusion during the illness
Treatment
Symptomatic Medications
- Bed rest
- During the period of active movements is recommended for the mildest form
- When chorea is disabling and interferes with rest
- Low Dose Anticonvulsants
- Valporate (20mg/kg/day)
- Carbamezatpine (15 mg/kg/day)
- Neuroleptics
- Symptomatic treatment with neuroleptics can be considered while treating the underlying illness
- Of note neuroleptics should be used for patients who fail valproic acid or carbamazepine therapy due to negative side effects
- The most common neuroleptics used are pimozide and haloperidol
- Atypical antipsychotics, such as risperidone and olanzapine can also be considered
Acute and Prophylactic Penicillin Therapy
- Treatment with Penicillin at the time of diagnosis of SC, 10 days
- Prevents additional complications of rheumatic fever
- The WHO recommends Penicillin G (1.2 million units) IM QDaily for 21 Days
- Duration depends upon the severity of carditis
- It is recommended to stop the prophylaxis after 5 years or age 18 (whichever is longer) in patients with no carditis
- Patients with mild carditis should continue for 10 years or age 21
- Patients with moderate to severe carditis should receive lifelong prophylaxis
Immunomodulatory therapy
- Short term immunotherapy such as oral steroids and IV immunoglobulins have been used to treat patients with disabling symptoms and in cases of uncontrolled or persisting chorea,
- The two most common regimens are:
- Oral prednisone (2 mg/kg/day) for ~ 21 - 30 days prolonged taper
- Methylprednisolone (25 mg/kg/day) ~ 21 - 30 days prolonged taper
Prognosis
- Good with complete recovery in most cases
- Duration of symptoms usually vary between 3-6 weeks; some children can have symptoms for several months
- Cardiac complications such as endocarditis may occur in some children
- 1/3rd of patients will experience recurrence of SC, usually after 1 and a half to 2 years after the initial attack
- Researchers have noticed an association between Recurrent SC and Rapid onset of PANDAS
- PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus infection)
- PANDAS is characterized by Obsessive-compulsive disorder, attention deficit/hyperactivity-disorder, tic disorders, and autism
Further Reading
- Ben-Pazi, H., Stoner, J. A., & Cunningham, M. W. (2013). Dopamine receptor autoantibodies correlate with symptoms in Sydenham's chorea. PloS one, 8(9), e73516. https://doi.org/10.1371/journal.pone.0073516
- Sehar, A., Nasir, S., & Seja, A. (2019). Rheumatic Chorea as the First Presenting Sign in a 13-year-old Female Child. Cureus, 11(8), e5447. https://doi.org/10.7759/cureus.5447
- https://bmcinfectdis.biomedcentral.com/articles/10.1186/s12879-021-06005-x
Bibliography
- Dean, S. L., & Singer, H. S. (2017). Treatment of Sydenham's Chorea: A Review of the Current Evidence. Tremor and other hyperkinetic movements (New York, N.Y.), 7, 456. https://doi.org/10.7916/D8W95GJ2
- Sydenham chorea. NORD (National Organization for Rare Disorders). (2020, September 1). https://rarediseases.org/rare-diseases/sydenham-chorea/.
- Sydenham's chorea. Brain Foundation. (n.d.). https://brainfoundation.org.au/disorders/sydenhams-chorea/.
- U.S. Department of Health and Human Services. (n.d.). Sydenham chorea information page. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Sydenham-Chorea-Information-Page.
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