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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
⢠Sydenhamâs 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
13. Introduction
⢠Described by Thomas Sydenham in 1684(as St. Vitusâ 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 ď
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
⢠Milkmaidâs gripď
⢠A sign of generalized muscle weakness and inability to maintain tetanic
muscle contraction;
⢠Subjects, when asked to squeeze the examinerâs fingers, do so by a âmilkingâ
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 âchorea on childrenâ
⢠Up to date. Ver 21.2. Search word â Sydenham Choreaâ