A Case of Pediatric Pain

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A Case of Pediatric Pain

• 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

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CaseHistory of Present Illness

• 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

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CasePast History

• 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

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CasePhysical Exam

• 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

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CaseLaboratory Evaluation

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

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CaseDisposition

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

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CaseSecond E.D. Visit

• 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

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What is Your Diagnosis?
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Guillain Barre Strohl Syndrome
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Pathophysiology

• First described in the 1930s 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

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Epidemiology

• General incidence of GBS in children range from
  0.8-1.5 per 100k annually
• Male to female ratio of 1.51
• No difference based on
• Ethnicity
• Geographic local (although China with outbreaks)
• Socioeconomic status

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Pathophysiology

• 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

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Clinical Findings

• 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

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Common Pain Syndromes in GBS

• 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

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Laboratory Evaluation

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

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Specific Laboratory Evaluations

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

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Electromyographic Testing and Neuroimaging

• 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

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Differential Diagnosis

• Acute Neuropathies
• 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

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Forms of Guillain-Barre Syndrome

• 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)
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Treatment

• ABCs
• 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

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Signs, Symptoms, and Paraclinical Findings in
Overt Neuromuscular Respiratory Failure

• Signs and symptoms
• Air hunger
• Altered mentation
• Accessory muscle use
• Paradoxical respiration
• Inability to count to 20 in one breath
• Staccato speech

• Paraclinical findings
• Vital capacity lt 15 ml/kg
• Negative inspiratory force lt -25 cm H20
• Positive expiratory force lt 40 cm H20
• Vital capacity drop of gt 55 from supine to
  sitting
• Hypoxemia
• Atelectasis

(Chalela, Seminars in Neurology 2001)
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TreatmentAutonomic Dysfunction

• 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

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TreatmentImmunomodulating Therapies

• 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)
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TreatmentPain Syndromes

• Deep muscle ache in low back or large muscles
• Nonsteroidal anti-inflammatory drugs
• Neuropathic pain
• TCAs 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

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Details

• 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

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Outcome Prediction in GBS

• Epidemiologic factors
• Antecedent diarrhea
• Campylobacter jejuni infection
• Cytomegalovirus infection
• Electrophysiologic findings
• Absent or reduced CMAP
• Unexcitable nerves
• Predominantly axonal involvement

• Clinical factors
• 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

(Chalela, Seminars in Neurology 2001)
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Prognosis

• 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

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Conclusions

• 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

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CaseOutcome

• 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

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• When the end of the world comes,
• I want to be in Cincinnati
• because it's always twenty years
• behind the times."