Sydenham’s Chorea

Ayesha Hameed (1) Tasneem Kousar (2),  Adeel Memon (3)
1 Fatima Jinnah Medical College, Pakistan
2 Foundation University Medical College, Pakistan
3 Dow Medical College, Pakistan


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
 
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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

  • 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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