A Case of Pediatric PainA Case of Pediatric PainEdward C. Jauch, MD MS FACEP

Department of Emergency Medicine

Dawn Kleindorfer, MD Department of Neurology

University of Cincinnati College of Medicineand

Greater Cincinnati / Northern Kentucky Stroke Team

Edward Jauch, MD, MS

CaseCaseHistory of Present IllnessHistory 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

Edward Jauch, MD, MS

CaseCasePast HistoryPast 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

Edward Jauch, MD, MS

CaseCasePhysical ExamPhysical 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

Edward Jauch, MD, MS

CaseCaseLaboratory EvaluationLaboratory Evaluation

• Chest xray unremarkable• CBC and renal profile normal• Hepatic with mildly elevated

transaminases• UA and urine pregnancy negative

Edward Jauch, MD, MS

CaseCaseDispositionDisposition

• 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

Edward Jauch, MD, MS

CaseCaseSecond E.D. VisitSecond 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

Edward Jauch, MD, MS

What is Your Diagnosis?What is Your Diagnosis?

Edward Jauch, MD, MS

Guillain Barre Strohl SyndromeGuillain Barre Strohl Syndrome

Edward Jauch, MD, MS

PathophysiologyPathophysiology

• 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

Edward Jauch, MD, MS

EpidemiologyEpidemiology

• 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

Edward Jauch, MD, MS

PathophysiologPathophysiology

• 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

Edward Jauch, MD, MS

Clinical FindingsClinical 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

Edward Jauch, MD, MS

Common Pain Syndromes in GBSCommon 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

Edward Jauch, MD, MS

Laboratory EvaluationLaboratory Evaluation

• 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

Edward Jauch, MD, MS

Specific Laboratory EvaluationsSpecific Laboratory Evaluations

• Serum –• Antibodies to GM1, Ga1C, or GA1NAc-GD1a

gangliosides• Antibodies to Campylobacter jejuni• Antibodies to CMV• HIV

• Stool cultures for C. jejuni

Edward Jauch, MD, MS

Electromyographic Testing Electromyographic Testing and Neuroimagingand 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

Edward Jauch, MD, MS

Differential DiagnosisDifferential 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

Edward Jauch, MD, MS

Forms of Guillain-Barre SyndromeForms 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)

Edward Jauch, MD, MS

TreatmentTreatment

• 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

Edward Jauch, MD, MS

Signs, Symptoms, and Paraclinical Findings Signs, Symptoms, and Paraclinical Findings in Overt Neuromuscular Respiratory Failurein 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 < 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)

Edward Jauch, MD, MS

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

Edward Jauch, MD, MS

TreatmentTreatmentImmunomodulating TherapiesImmunomodulating 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)

Edward Jauch, MD, MS

TreatmentTreatmentPain SyndromesPain Syndromes

• 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

Edward Jauch, MD, MS

DetailsDetails

• 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

Edward Jauch, MD, MS

Outcome Prediction in GBSOutcome Prediction in GBS

• 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

• 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)

Edward Jauch, MD, MS

PrognosisPrognosis

• 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

Edward Jauch, MD, MS

ConclusionsConclusions

• 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

Edward Jauch, MD, MS

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

“When the end of the world comes, I want to be in Cincinnati

because it's always twenty years behind the times."