A Case of Pediatric Pain - PowerPoint PPT Presentation

Edward C. Jauch, MD MS FACEP

Department of Emergency Medicine

Dawn Kleindorfer, MD

Department of Neurology

University of Cincinnati College of Medicine

and

Greater Cincinnati / Northern Kentucky Stroke Team

• 12 yo. Caucasian female who presents to the Emergency Department complaining of diffuse body pain
• Symptoms began one week ago originally in distal extremities but now throughout entire body
• Patient has missed school for the past 4 days due to pain with ambulation but she denies any weakness

• Past medical history -
• No significant past medical history
• Mother notes that members of the family had a “stomach flu”, including the patient, 3 weeks ago
• Social history -
• Family recently moved to area, patient is having difficulty adjusting to new school
• No alcohol or drug use
• Review of systems -
• No fever, chills, chest or abdominal pain, rashes
• Patient states it hurts to breath

• VS: BP 98/60 HR 110 T 98.9oF SaO2 99%
• Tearful young female clinging to mom
• HEENT, pulmonary, cardiac, abdominal, extremity, skin exams all unremarkable
• Neuro exam -
• Cranial nerves intact
• Motor largely intact but limited by effort
• Sensory shows extreme sensitivity to touch in all extremities and less so on trunk
• Motor tone normal but reflexes absent
• Patient refused to walk

• Chest xray unremarkable
• CBC and renal profile normal
• Hepatic with mildly elevated transaminases
• UA and urine pregnancy negative

• Discharge diagnosis: Viral syndrome
• Follow-up was scheduled with the patient’s pediatrician the following week
• The physician also discussed with the patient’s parents that her behavior may also reflect her difficulty with her new school

• Patient returns 2 days later now complaining of profound weakness and shortness of breath
• VS: BP 130/78 HR 125 T100.6oF
• General exam unchanged except increased shortness of breath
• Neuro exam now reveals:
• CNI except bilateral VII nerve weakness
• Flaccid lower and weak upper extremities
• Less pain to touch but burning sensation persists
• Deep tendon reflexes remain absent

• First described in the 1930’s GBS is an form of acute polyradicularneuropathy
• Primarily due to demyelination of peripheral nerves
• In severe forms actual axonal damage occurs (associated with worse prognosis)
• Numerous precipitants have been identified

• General incidence of GBS in children range from 0.8-1.5 per 100k annually
• Male to female ratio of 1.5:1
• No difference based on:
• Ethnicity
• Geographic local (although China with outbreaks)
• Socioeconomic status

• Precipitating causes include:
• Prodromal viral or bacterial illness
• Campylobacter jejuni
• Chlamydia, CMV, EBV, HIV, Mycoplasma pneumoniae,
• Vaccinations (influenza, MMR, oral polio, Td)
• Pregnancy
• Malignancy (Hodgkins)
• Surgery
• Other (SLE, drugs)
• Unknown

• Motor
• Ranging from mild weakness to paralysis
• Symmetric and ascending
• Cranial nerves (IV, VI, VII) but rarely bulbar
• Areflexia
• Sensory
• Pain or dysesthesia very common
• Visceral symptoms not infrequent

• Back and leg pain
• Similar in presentation to sciatica
• Affect large muscle groups
• Neuropathic pain
• Burning sensations in the extremities
• Visceral pain
• Bloating, cramping
• Joint pain and myalgias
• Affects primarily large joints

• Basic labs –
• Renal profile (SIADH seen in GBS)
• CBC
• Hepatic (elevations in transaminases common)
• Pregnancy
• ESR (typically < 50 mm/hr)
• CK (elevated in patients with significant pain)
• UA (proteinuria in 25%)
• CSF –
• Usually with normal opening pressure
• Classically with elevated protein (> 400 mg/dL)
• Lack of pleocytosis

• Serum –
• Antibodies to GM1, Ga1C, or GA1NAc-GD1a gangliosides
• Antibodies to Campylobacter jejuni
• Antibodies to CMV
• HIV
• Stool cultures for C. jejuni

• EMG
• Demonstrates
• Demyelination + axonal loss
• Diminished nerve conduction velocities
• Diagnosis more specific if multiple nerves involved
• MRI with gadolinium contrast
• Enhancement of cauda equina and nerve roots suggest areas of inflammation

Critical illness neuropathy

Diphtheria

Porphyrias

Lyme disease

Toxins

Tick paralysis

NMJ Disorders

Botulism

Myasthenia gravis

Myopathies

Critical illness myopathy

Hypocalcemia, hypokalemia

Polymyositis

Rhabdomyolysis

CNS Disorders

Acute spinal cord syndromes

Transverse myelitis

Poliomyelitis

Rabies

• Motor-sensory 75%
• Diagnosis almost exclusively clinical
• Pure motor 20%
• Autonomic dysfunction more common
• Miller Fisher syndrome 3%
• Weakness starts in eye muscles
• Bulbar variant 2%
• Weakness involves muscles of deglutition and or tongue

(Dutch Neuromuscular Research Centre, Eur Neurol 2001)

• ABC’s
• Supportive and expectant care is key.
• Early pulmonary function tests to identify patients at risk of impending respiratory failure
• Recognition and treatment of autonomic instability
• Immunomodulating therapies

Air hunger

Altered mentation

Accessory muscle use

Paradoxical respiration

Inability to count to 20 in one breath

Staccato speech

Paraclinical findings

Vital capacity < 15 ml/kg

Negative inspiratory force < -25 cm H20

Positive expiratory force < 40 cm H20

Vital capacity drop of > 55% from supine to sitting

Hypoxemia

Atelectasis

(Chalela, Seminars in Neurology 2001)

• Paroxysmal hypertension
• Sudden swings make treatment more difficult
• Short acting agents safest (nitroprusside)
• Hypotension and orthostatic hypotension
• Rarely requires therapy (IV fluids)
• Cardiac arrhythmias
• Most life threatening
• Bradycardia treated with atropine
• Tachyarrhythmias may include atrial fibrillation, atrial flutter, and ventricular tachycardia, all respond to standard treatment

• Plasma exchange
• Only therapy with proven benefit
• May require multiple exchanges
• Cautious use in patients with autonomic instability
• Immunoglobulin therapy (IV IgG)
• Relatively easy to administer
• Benefit unclear
• Risk of viral (hepatitis C) transmission
• Steroids without benefit

(Cochrane Review, 2001)

• Deep muscle ache in low back or large muscles
• Nonsteroidal anti-inflammatory drugs
• Neuropathic pain
• TCA’s effective, use with caution in autonomic dysfunction
• Carbamazepine
• Joint pain
• Ice packs, nonsteroidal anti-inflammatory drugs
• Throat pain associated with intubation
• Intermittent cuff deflation, tracheostomy

• Admission to a high acuity area is critical for adequate patient monitoring
• Continuous cardiac monitoring
• Close respiratory observation
• Do not delay intubation until the patient becomes hypoxic!
• Neurology should be involved early on

Advanced age

Rapid symptom progression

Mechanical ventilation

Upper extremity involvement

Inability to walk at 8 weeks

Biochemical markers

Anti-GM1 antibodies

High CSF NSE or S100 levels

Epidemiologic factors

Antecedent diarrhea

Campylobacter jejuni infection

Cytomegalovirus infection

Electrophysiologic findings

Absent or reduced CMAP

Unexcitable nerves

Predominantly axonal involvement

(Chalela, Seminars in Neurology 2001)

• In general the prognosis of GBS is good
• Up to 85% of patients with GBS make a full recovery
• Mortality rates range from 2-12%
• 15% of patients with persistent deficits

• Guillian-Barre Syndrome should no longer have significantly mortality if properly diagnosed and treated
• Guillian-Barre may present with pain as the primary symptom in children
• The key differential is primary spinal cord injury, GBS, and tick paralysis

• Patient required intubation within 24 hours of admission
• Plasma exchange performed 4 times over next 7 days
• Patient was extubated on hospital day 6
• Returned to school 4 weeks from admission
• Patient with minimal residual leg weakness at 6 months follow-up

I want to be in Cincinnati

because it's always twenty years

behind the times."