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1. Rheumatic Fever
&
Sydenham's Chorea
Dr. Jeetendra Bhandari

2. Rheumatic Fever
• Inflammatory disease occurring in children and young adult
• First attack occur at age of 5-15 years
• Result of Infection with Group A Streptococci
• Affects heart, skin, joints and Central Nervous System
• Use of antibiotics and improved hygiene has reduced streptococcal
infection from 10% in 1910 to 0.01% in 2010

3. Pathophysiology
• An autoimmune reaction triggered by molecular mimicry between
cell wall M proteins of infecting Streptococcus pyogenes and cardiac
myosin and laminin
• Condition is not due to direct infection of heart or to the production
of toxin

4. Modified Jones Criteria(for diagnosis)
• Evidence of antecedent streptococcal infection
• Positive throat culture for group A streptococcus
• Good clinical history (e.g. of scarlet fever)
• Elevated antistreptolysin O titre (or other serological assay for streptococci)
• Major criteria
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules

5. • Minor criteria
• Fever
• Arthralgia (unless arthritis counted as major criterion)
• Previous rheumatic fever
• Raised ESR/C-reactive protein
• Leucocytosis
• Prolonged PR interval on ECG (unless carditis counted as major criterion)
• [Diagnostic if 2 or more major criteria or 1 major and 2 or more minor
criteria; along with evidence of current streptococcal infection]

6. Clinical Feature
• Presents suddenly with fever, joint pain and malaise
• Cardiac Manifestation
• New or changed heart murmur
• Cardiac enlargement or cardiac failure
• Pericardial effusion, ECG changes of pericarditis, myocarditis, AV block or
other cardiac aythmias
• Skin Manifestation
• Erythema Marginatum(transient pink rashes edges, occurs in 20% of cases)
• Erythematous area found mainly on trunk and limbs
• Subcutaneous nodules which are painless, pea-sized, hard nodules

7. • Arthritis
• Fleeting migratory polyarthritis
• Affects large joints i.e. knee, elbows, ankle and wrist
• Once acute inflammation subside arthritis also subside
• Sydenham’s chorea
• Occurs late after Streptococcal infection
• CNS involvement

8. Investigation
• Throat Swab for culture
• Antistreptolysin O titer and anti DNAse B (might be elevated)
• ESR and CRP (usually high)
• Cardiac investigation(ECG and Echocardiogram)

9. Treatment
• Absolute bed rest recommended, but can be mobilized when acute
symptom start to resolve
• For residual Streptococcal infection
• Phenoxymethylpenicilline 500 mg; Oral, Four times a day for 7 days
• Should be given if nasal or pharyngeal swab are negative too
• Arthritis
• NSAIDs can reduce pain
• Has no effect on long term cardiac sequale
• No good evidence on use of steroids(but usual practice of use of
prednisolone if severe carditis)

10. • Recurrence is common when persistent cardiac damage present
• Recurrence prevented by
• Phenoxymethylpenicillin 250 mg, oral, 2 times a day
Or
• Intramuscular Benzathine Penicillin G 1.2 million unit monthly
• Erythromycin or Clarithromycin ir allergic to penicillin
Until the
age of 20
years

11. Prognosis
• >50% of cases of acute Rheumatic fever with carditis develop chronic
rheumatic valvular disease(mitral and aortic valve) after 10-20 years

12. Sydenham's Chorea(St. Vitus
dance)

13. Introduction
• Described by Thomas Sydenham in 1684(as St. Vitus’ dance)
• Relation between Sydenham chorea and rheumatic fever established
on 1780
• Rheumatic syndrome fully described on 1889
• Later after decades etiological role of Streptococcal infection in RF
was established
• Recently has been established that Sydenham chorea is linked to
neuropsychiatric disorder(i.e. OCD, attention deficit hyperactivity
disorder and anxiety)

14. Epidemiology
• Most common cause of acquired chorea in young
• Chorea major manifestation in RF and only RF evidence of RF 
approx. 20%
• Female: male = 2:1
• Age between 5-15 suffer from this
• 3.5% of parents and 2.1% of siblings of children with Sydenham
chorea had also been affected

15. Clinical Feature
• Condition Manifest as
• Involuntary movements
• Hypotonia
• Mild muscular weakness
• Can be generalized or unilateral
• Predominantly involve face, hands and arms
• Movements present at rest, aggravated by stress and usually cease
during sleep
• Children attempts to hide movements

16. • In 20% patient, only one side of body may seems to be involved but
on through examination bilateral movement can be identified
• Choreic movement interfere with usual movement and result in
• Clumpsy gait
• Dropping(fall vertically) or spilling
• Explosive burst of dysarthric speech
• Milkmaid’s grip
• A sign of generalized muscle weakness and inability to maintain tetanic
muscle contraction;
• Subjects, when asked to squeeze the examiner’s fingers, do so by a ‘milking’
motion of contraction and relaxation

17. • Pronator Sign
• Hyperpronation of hand , causing palm to face outward when arms are held
on head
• Choreic hand
• With arm extended, wrist will flex and metacarpophalangeal joint over extend
• Some children have profound weakness that they appear paralysed
• Patient may present with psychiatric symptoms(depression, anxiety,
personality change, emotional liability)

18. Pathophysiology
• Immunology
• Production of immunoglobin G antibodies that cross react with antigens in
membrane of Group A streptococcus and antigen inneuronal cytoplasm of
caudate and subthalamic nuclei ( tubulin and extracellular lysoganglioside)
• Antineuronal antibodies have also been found in CSF
• Immunofluoresent stained has shown in 50% of children have autoantibodies
that react with neuronal cytoplasmic antigen in cardiac and subthalamic
nuclei
• Neurochemistry
• Believed to arise from an imbalance among the dopaminergic system,
intrastriateal cholinergic system and GABA system

19. Neuroimaging
• MRI findings are not consistent and may be normal
• Found abnormality includes
• Areas of increased signal intensity of T2 weighted images that involves basal
ganglia or cerebral white matter

20. Treatment
• Usually self limiting; treatment to those with functional impairment
• Anticonvulsants (valporic acid and Carbamazepine) have shown to be
effective on dose normally used on seizure
• Steroids are used widely but no controlled trial has done till now
• Dopaminergic blockers(haloperidol) are effective and well tolerated
on small dose
• Prednisone plasma exchange and IV immuglubin have shown to be
effective
• Prophylaxis against streptococcus needed until age of 18 years

21. Prognosis
• Disease resolve spontaneously in 3-6 months and rarely lasts longer
than 1 year
• Mild chorea without functional disability may be found on small
proportion of patient up to 10 years
• About 10% patient experience 2-10 recurrence usually within 2 yrs
after initial attack

22. References
• Rheumatic fever. Kumar and Clark textbook of medicine. 8th ed.
Page:127-8.
• Medscape. Search word “chorea on children”
• Up to date. Ver 21.2. Search word “ Sydenham Chorea”

23. Thank you!!