Transcript Slide 1

EXTERN CONFERENCE
Supervisor
Dr . Auranee Sanmaneechai
Dr. Worapan Kriengsoontornkit
HISTORY TAKING
CHIEF COMPLAINT
- 9 years old Thai girl
- Right hand and leg weakness with
unusual movement for 1 month PTA
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.
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.
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.
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.
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.
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
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.
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
Cerebrallar sign : normal
Babinski’s sign : negative
milkmaid's grip,darting tongue
Spoon sign, choreic hand
Positive
PHYSICAL EXAMINATION
PROBLEM LIST
1. Paroxysmal movement event
2. Behavioral change
3. Labile mood
Approach to Paroxysmal event
APPROACH TO PAROXYSMAL EVENT
Paroxysmal event
Seizure
Abnormal movement
APPROACH TO PAROXYSMAL EVENT
Paroxysmal event
Seizure
Abnormal movement
Persist during awake & sleep
Stereotyped appearance
Rhythmatic appearance
Epileptiform activity on EEG
APPROACH TO PAROXYSMAL EVENT
Paroxysmal event
Seizure
Abnormal movement
Pattern recognition
Only awake
Non – stereotypic
Non – Rhythmatic
No epileptiform activity on EEG
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.
APPROACH TO PAROXYSMAL EVENT
PHENOMENOLOGIC CLASSIFICATION
Movement 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
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.
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”
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.
CHOREA
PANDAS
- 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.
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 .
CHOREA
CHOREA
- Sign.
•
•
•
•
Rapid, irregular and non stereotypic jerks
Milkmaid’s grip
Spooning
Pronator sign
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
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
SYDENHAM’S CHOREA
Emotional change
Easy crying / inappropriate laughing
Regression in school performance
Psychiatric symptom
Irritability
distractibility
Inappropriate / age-regressed behavior
Obsessive-compulsive symptom
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
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.
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
CLINICAL MANIFESTATION
Major
Carditis
Arthritis (migratory polyarthritis)
Rash (Erythema marginatum)
Subcutaneous nodules
Sydenham’s chorea
Minor
Arthralgias
Elevated ESR,CRP
Fever
Prolonged PR interval
ACUTE RHEUMATIC FEVER
ACUTE RHEUMATIC FEVER
CLINICAL MANIFESTATION
Exceptions to Jones Criteria
- Chorea or indolent carditis may occur as only
manifestation
- Recurrence may not fulfill Jones Criteria
Back to the case…
INVESTIGATION
Blood for CBC, electrolyte, Ca, ESR,TFT,
ASO, anti-DNase B, LFT, lactate
Consult ophthalmologist
Echocardiography
INVESTIGATION
INVESTIGATION
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
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
PROGRESSION