EXTERN CONFERENCE

Supervisor

Dr . Auranee Sanmaneechai

Dr. Worapan Kriengsoontornkit

HISTORY TAKINGCHIEF COMPLAINT

- 9 years old Thai girl

- Right hand and leg weakness with unusual movement for 1 month PTA

-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.

HISTORY OF PRESENT ILLNESS

HISTORY TAKING

-She has abnormal gait.

-She was moody and got upset easily.

-Her symptoms were not better, then her mother brought her to a hospital.

HISTORY OF PRESENT ILLNESS

HISTORY TAKING

-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.

HISTORY OF PRESENT ILLNESS

HISTORY TAKING

-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.

PAST HISTORY

HISTORY TAKING

-Her brother and sister are normal in development and have no underlying disease.

-Nobody in her family has any symptom like this.

FAMILY HISTORY

HISTORY TAKING

PHYSICAL EXAMINATIONVITAL 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

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.

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.

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

PHYSICAL EXAMINATION

NERVOUS SYSTEM

milkmaid's grip,darting tongue

Spoon sign, choreic hand Positive

Cerebrallar sign : normal

Babinski’s sign : negative

PHYSICAL EXAMINATION

PHYSICAL EXAMINATION

PROBLEM LIST

1. Paroxysmal movement event

2. Behavioral change

3. Labile mood

Approach to Paroxysmal event

Seizure

Paroxysmal event

Abnormal movement

APPROACH TO PAROXYSMAL EVENT

Paroxysmal event

Abnormal movement

Persist during awake & sleep

Stereotyped appearance

Rhythmatic appearance

Epileptiform activity on EEG

APPROACH TO PAROXYSMAL EVENT

Seizure

Seizure

Paroxysmal event

Abnormal movement

Pattern recognition

APPROACH TO PAROXYSMAL EVENT

Only awake

Non – stereotypic

Non – Rhythmatic

No epileptiform activity on EEG

- 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.

APPROACH TO PAROXYSMAL EVENT

PHENOMENOLOGIC CLASSIFICATIONMovement disorder Brief Description

Athetosis Slow, continuous writhing movement of distal body parts, expecially the fingers and hands.

Chorea, ballism Chaotic, random, repetitive, brief, purposeless movement. Large amplitude affecting proximal joint are often called ballism.

Dystonia Repetitive, sustained, abnormal postures or movements. Abnormal postures typically have a twisting quality.

Myoclonus Sudden, brief, shocklike movements that may be repetitive or rhythmic.

Stereotypy Patterned, episodic, repetitive, purposeless, rhythmic movement

Tics Sterotyped intermittent, sudden, discrete, repetitive, nonrhythmic movements, most frequently involving head and upper body.

Tremor Rhytmic oscillation around a central pint or position involving any one body part or more than one

APPROACH TO PAROXYSMAL EVENT

- 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.

FEATURES OF PEDIATRIC MOVEMENT DISORDER

APPROACH TO PAROXYSMAL EVENT

- The clinical manifestations of a movement disorders will depend on the child’s developmental stage.

- No universal “movement disorder workup”

FEATURES OF PEDIATRIC MOVEMENT DISORDER

APPROACH TO PAROXYSMAL EVENT

Back to the case…

DIFFERENTIAL DIAGNOSIS

Sydenham’s chorea

Systemic Lupus Erythematosus

Wilson’s disease

PANDAS

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.

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

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.

PANDAS

CHOREA

- Pediatric autoimmune neuropsychiatric disorders associated with streptococcus.

- Abrupt or explosive onset of

- Tics

- Obsessive – compulsive behavior

- Chorea

- Pro

-Labile mood

Chorea

Thomas Sydenham

September 10, 1624 – December 29, 1689

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.

- 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 .

CHOREA

CHOREA

- Sign.

• Rapid, irregular and non stereotypic jerks• Milkmaid’s grip• Spooning• Pronator sign

CHOREA

PRIMARY CHOREA

Huntington’s DiseaseAtaxia – TelangiectasiaFamilial benign hereditary choreaNeuroacanthocytosis

SECONDARY CHOREA

Sydenham’s choreaMedication – induced choreaChorea asso. with brain injuryChorea asso. with systemic illness

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].

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.

CHOREA

Emotional change

Easy crying / inappropriate laughingRegression in school performance

Psychiatric symptom

IrritabilitydistractibilityInappropriate / age-regressed behaviorObsessive-compulsive symptom

CHOREASYDENHAM’S CHOREA

ACUTE RHEUMATIC FEVERDIAGNOSIS

-By modified Jones Criteria

- Evidence of preceding group A streptococcal (GAS) infection

- 2 major criterias or

- 1 major + 2 minor criterias

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.

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.

ACUTE RHEUMATIC FEVER

Major Carditis

Arthritis (migratory polyarthritis)

Rash (Erythema marginatum)

Subcutaneous nodules

Sydenham’s chorea

Minor Arthralgias Elevated ESR,CRP

Fever Prolonged PR interval

ACUTE RHEUMATIC FEVERCLINICAL MANIFESTATION

ACUTE RHEUMATIC FEVER

CLINICAL MANIFESTATION

Exceptions to Jones Criteria

- Chorea or indolent carditis may occur as only manifestation

- Recurrence may not fulfill Jones Criteria

ACUTE RHEUMATIC FEVER

Back to the case…

INVESTIGATION

Blood for CBC, electrolyte, Ca, ESR,TFT,

ASO, anti-DNase B, LFT, lactate

Consult ophthalmologist

Echocardiography

INVESTIGATION

INVESTIGATION

Note: rheumatic heart disease with Sydenham’s chorea

Mild thickening of ant MVL with mild MR

Mild LV enlargement

No AR

No pericardial effusion

ECHOCARDIOGRAPHY

INVESTIGATION

OPHTHALMOLOGIST NOTE

Imp: normal ant segment.

no Kayser-Fleischer ring.

INVESTIGATION

CXR PA Lateral upright

PPD skin test

Stool concentration for parasite

Urinary examination

Consult dentist

INVESTIGATION

PROGRESSION