EXTERN C O N F E R E N C E

1. EXTERNCONFERENCE Supervisor Dr . Auranee Sanmaneechai Dr. Worapan Kriengsoontornkit

2. HISTORY TAKING CHIEF COMPLAINT • 9 years old Thai girl • Right hand and leg weakness with unusual movement for 1 month PTA

3. HISTORY TAKING HISTORY OF PRESENT ILLNESS -1 month PTA, she felt her right hand weak and her handwriting was worse. She couldn’t write and eat by herself, so she used her left hand to work. - Her mother told that she couldn’t speak clearly. She often protruded her tongue out . Her right hand always move, but unusual movement disappeared when she slept.

4. HISTORY TAKING HISTORY OF PRESENT ILLNESS • -She has abnormal gait. • -She was moody and got upset easily. • Her symptoms were not better, then her mother brought her to a hospital.

5. HISTORY TAKING HISTORY OF PRESENT ILLNESS • No history of fever, dyspnea, tachypnea, joint pain, rash, sore throat. • No history of head trauma. • No history of headache, nausea, vomiting. • Her voiding and defecation appeared normal • Her ability in calculation and her memory are normal.

6. HISTORY TAKING PAST HISTORY • No underlying disease. • Normal developmental milestone. • Her academic performance was good. • She had no complication and serious illness during the neonatal period. • Her vaccination is updated.

7. HISTORY TAKING FAMILY HISTORY • Her brother and sister are normal in development and have no underlying disease. • Nobody in her family has any symptom like this.

8. PHYSICAL EXAMINATION VITAL SIGN T 37 Co RR 18 /min HR 100 /min BP 110/70 mmHg GENERAL APPEARANCE 9 years old Thai girl, fully conscious, not pale, no jaundice, no dyspnea, no edema. SKIN No rash, no petechiae

9. PHYSICAL EXAMINATION HEENT No cervical lymphadenopathy. Pharynx and tonsils not injected. No tonsilar enlargement. CARDIOVASCULAR SYSTEM No active precardium, no heaving, no thrill, normal S1/S2, Systolic murmur grade I / VI at LLPSB. Radial pulse (or peripheral pulse) 2+ with regular rhythms bilaterally.

10. PHYSICAL EXAMINATION RESPIRATORY SYSTEM Good air entry, no retraction. Normal breath sound, no adventitious sound. ABDOMEN Soft, not tender, Liver and spleen not palpable. Normoactive bowel sound.

11. PHYSICAL EXAMINATION NERVOUS SYSTEM Oriented to time, place, person. Labile mood, restless Cranial nerve : normal Motor power : grade 5 all extremities Motor tone : normal DTR 2+ at all extremities

12. PHYSICAL EXAMINATION NERVOUS SYSTEM Cerebrallar sign : normal Babinski’s sign : negative milkmaid's grip,darting tongue Spoon sign, choreic hand Positive

13. PHYSICAL EXAMINATION

14. PROBLEM LIST • Paroxysmal movement event • Behavioral change • Labile mood

15. Approach to Paroxysmal event

16. APPROACH TO PAROXYSMAL EVENT Paroxysmal event Abnormal movement Seizure

17. APPROACH TO PAROXYSMAL EVENT Paroxysmal event Abnormal movement Seizure Persist during awake & sleep Stereotyped appearance Rhythmatic appearance Epileptiform activity on EEG

18. APPROACH TO PAROXYSMAL EVENT Paroxysmal event Abnormal movement Seizure Pattern recognition Only awake Non – stereotypic Non – Rhythmatic No epileptiform activity on EEG

19. APPROACH TO PAROXYSMAL EVENT • Impaired voluntary movement or/and involuntary movement • Impaired targeting and velocity of intended movements, abnormal postures, or excessive normal – appearing movements at inappropriate or unintended times. • Accompany symptoms : weakness, spasticity, hypotonia, ataxia, apraxia.

20. APPROACH TO PAROXYSMAL EVENT PHENOMENOLOGIC CLASSIFICATION

21. APPROACH TO PAROXYSMAL EVENT FEATURES OF PEDIATRIC MOVEMENT DISORDER • Movement disorders in childhood are primarily symptoms of other disease, rather than diseases in an of themselves • [Clinical Pediatric Neurology, Sanger, 2003b; Sanger et al.. 2003] • Many adult neurologic disorders can be attributed to anatomically localized injury, but childhood disorder frequently result from a global or multifocal injury that may affect particular cell types receptor types or metabolic pathways.

22. APPROACH TO PAROXYSMAL EVENT FEATURES OF PEDIATRIC MOVEMENT DISORDER • The clinical manifestations of a movement disorders will depend on the child’s developmental stage. • No universal “movement disorder workup”

23. Back to the case…

24. DIFFERENTIAL DIAGNOSIS Sydenham’s chorea Systemic Lupus Erythematosus Wilson’s disease PANDAS

25. DIFFERENTIAL DIAGNOSIS Sydenham’s chorea • Pro • The most common cause of acquired chorea in childhood • Age 5-15 yr • Labile mood • Choreiform movement • Con • No history of Streptococcal Infection • No other sign of acute rheumatic fever.

26. DIFFERENTIAL DIAGNOSIS Systemic Lupus Erythematosus • Pro • Sex F:M=4:1 in children • Age > 5 yr • Choreiform movement • Can be only one prodrome symptom of SLE • Con • Rare presentation<5% • No other criteria of SLE

27. DIFFERENTIAL DIAGNOSIS Wilson’s disease • Pro • Choreiform movement • Can be only one prodrome symptom • Con • Too early of age with choreiform presentation. • Neurologic manifestation usually present with Kayser – Fleischer ring.

28. CHOREA PANDAS • Pediatric autoimmune neuropsychiatric disorders associated with streptococcus. • Abrupt or explosive onset of • Tics • Obsessive – compulsive behavior • Chorea • Pro • Labile mood

29. Chorea Thomas Sydenham September 10, 1624 – December 29, 1689

30. CHOREA • Random, nonrhythmic, purposeless • Appears to flow from one muscle or muscle group to another without any evident pattern. • Occurs at rest and with action. • Motor impersistence, loss of fine motor control.

31. CHOREA • - May worsen or improve with voluntary movement. • Many individuals with chorea incorporate the involuntary movements into a voluntary movement to mask the impairment. • Tone is normal or reduced. • Disappears in sleep .

32. CHOREA CHOREA • Sign. • Rapid, irregular and non stereotypic jerks • Milkmaid’s grip • Spooning • Pronator sign

33. CHOREA PRIMARY CHOREA Huntington’s Disease Ataxia – Telangiectasia Familial benign hereditary chorea Neuroacanthocytosis SECONDARY CHOREA Sydenham’s chorea Medication – induced chorea Chorea asso. with brain injury Chorea asso. with systemic illness

34. CHOREA SYDENHAM’S CHOREA • Most common causes of secondary chorea in children. • Onset : weeks or months. • Cardinal symptoms of rheumatic fever • May persist for weeks or month. • Almost always resolved spontaneously within 6 months [Jordan and Singer 2003].

35. CHOREA SYDENHAM’S CHOREA • Acute and convalescent ASO titers must be measured to confirm an acute infection. • Complete evaluation include : thyroid function, toxins, metabolic disorders, or encephalitis. • Triads : Chorea, Mood instability, Hypotonia. • Medication : controversy • Prognosis : complete resolution in most cases. Recurrence is rare.

36. CHOREA SYDENHAM’S CHOREA • Emotional change • Easy crying / inappropriate laughing • Regression in school performance • Psychiatric symptom • Irritability • distractibility • Inappropriate / age-regressed behavior • Obsessive-compulsive symptom

38. ACUTE RHEUMATIC FEVER DIAGNOSIS -By modified Jones Criteria - Evidence of preceding group A streptococcal (GAS) infection - 2 major criterias or - 1 major + 2 minor criterias

39. ACUTE RHEUMATIC FEVER EVIDENCE OF PRECEDING GAS INFECTION • All suspected cases of ARF (except those with chorea or low – grade subacute carditis) should have elevated serum streptococcal serology demonstrated. • If the initial titer is below the upper limit of normal for age, repeat testing after 10 – 14 days.

40. ACUTE RHEUMATIC FEVER EVIDENCE OF PRECEDING GAS INFECTION • Patients with suspected ARF should be offered a single dose of benzathine penicilin G at secondary prophylaxis doses and reviewed in 1 month with a repeat echocardiogram to detect the appearance of new lesions. • If there is evidence of rheumatic valve disease clinically or on echocardiogram, the diagnosis is confirmed, and long – term secondary prophylaxis can be continued.

41. ACUTE RHEUMATIC FEVER CLINICAL MANIFESTATION Major Carditis Arthritis (migratory polyarthritis) Rash (Erythema marginatum) Subcutaneous nodules Sydenham’s chorea Minor Arthralgias Elevated ESR,CRP Fever Prolonged PR interval

42. ACUTE RHEUMATIC FEVER

43. ACUTE RHEUMATIC FEVER CLINICAL MANIFESTATION Exceptions to Jones Criteria - Chorea or indolent carditis may occur as only manifestation - Recurrence may not fulfill Jones Criteria

44. Back to the case…

45. INVESTIGATION Blood for CBC, electrolyte, Ca, ESR,TFT, ASO, anti-DNase B, LFT, lactate Consult ophthalmologist Echocardiography

46. INVESTIGATION

47. INVESTIGATION

48. INVESTIGATION ECHOCARDIOGRAPHY • Note: rheumatic heart disease with Sydenham’s chorea • Mild thickening of ant MVL with mild MR • Mild LV enlargement • No AR • No pericardial effusion

49. INVESTIGATION OPHTHALMOLOGIST NOTE Imp: normal ant segment. noKayser-Fleischerring.

50. INVESTIGATION CXR PA Lateral upright PPD skin test Stool concentration for parasite Urinary examination Consult dentist