Rheumatic fever and syndenham's chorea

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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. 8 th ed. Page:127-8. Medscape. Search word “chorea on children” Up to date. Ver 21.2. Search word “ Sydenham Chorea”

23. Thank you!!