Neurology Book

Infectious Disease

  • Guillain Barre Syndrome

Miscellaneous

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Guillain Barre SyndromeAka: Guillain-Barre Syndrome, Landry-Guillain-Barre Syndrome, Guillain-Barre-Strohl Syndrome, Acute Inflammatory Demyelinating Polyradiculoneuropathy, Acute Motor Axonal Neuropathy, Acute Motor Sensory Axonal Neuropathy

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  1. Epidemiology
    1. Annual Incidence: 1-3 cases per 100,000
  2. Pathophysiology
    1. Acute inflammatory demyelinating polyradiculopathy
    2. Acute infection precedes Guillain-Barre in 66% of cases
      1. Campylobacter jejuni (most common)
      2. Cytomegalovirus
      3. HIV Infection
      4. Epstein-Barr Virus
      5. Varicella Zoster Virus
      6. Influenza Vaccine (risk 1-2 cases per 1 Million)
  3. Symptoms
    1. Follows acute respiratory or gastrointestinal illness
    2. Progressive and ascending neurologic changes
      1. See signs below for more detailed description
      2. Tingling Paresthesias in distal extremities
      3. Proximal Muscle Weakness
    3. Deep aching pain in weak muscles (50% of cases)
      1. Shoulder
      2. Back
      3. Posterior thighs
  4. Signs
    1. Progressive, symmetric neurologic symptoms
      1. Starts in distal extremities and ascends
      2. Progresses proximally over days to weeks
        1. Paresthesias (tingling) in distal extremities
        2. Proximal Muscle Weakness
        3. Cranial Nerves may be affected
          1. Facial muscles and eye movements may be affected
          2. Swallowing may be difficult
    2. Progressive, symmetric proximal Muscle Weakness
      1. Legs are affected more than arms
    3. Severe weakness occurs in 10-30%
      1. Quadriparesis
      2. Respiratory arrest requiring Ventilator support
      3. Bulbar failure (e.g. Dysphagia)
    4. Loss of Deep Tendon Reflexes within first few days
    5. Variable sensory losses
    6. Autonomic Dysfunction
      1. Hyperhidrosis
      2. Postural Hypotension and Blood Pressure fluctuation
      3. Tachycardia
      4. Urinary Retention
      5. Gastrointestinal pseudo-obstruction
  5. Types
    1. Acute Inflammatory Demyelinating Polyradiculoneuropathy
      1. Abbreviation: AIDP
    2. Acute Motor Axonal Neuropathy (AMAN)
    3. Acute Motor Sensory Axonal Neuropathy (AMSCAN)
    4. Rare variants
      1. Miller Fisher Syndrome
      2. Acute Panautonomic Neuropathy
  6. Differential Diagnosis
    1. See Acute Motor Weakness Causes
  7. Diagnostic Testing
    1. Cerebrospinal fluid (CSF)
      1. Increased CSF Protein >0.55 g/dl
      2. Normal CSF White Blood Cells Count (<10 cells/mm3)
        1. If increased, then consider other diagnosis
        2. Differential diagnosis if CSF WBCs increased
          1. Lyme Disease
          2. Cancer
          3. Human Immunodeficiency Virus (HIV Infection)
          4. Sarcoid Meningitis
    2. Electromyogram (EMG)
      1. Absent H reflex
      2. Low to absent sensory action potential amplitudes
      3. F wave response prolonged
    3. Evaluate respiratory function in all suspected cases
      1. Pulmonary Function Testing
      2. Arterial Blood Gas
  8. Criteria for ICU admission (risk of respiratory failure)
    1. Pulmonary Function Test abnormalities
      1. Vital Capacity < 30 ml/kg
      2. Peak inspiratory pressure <30 cm H2O
    2. Poor swallowing
    3. Ineffective cough
    4. Aspiration Pneumonia
    5. Dysautonomia
    6. Increased Respiratory Rate
    7. Dyspnea
    8. Severe Muscle Weakness
      1. Unable to lift elbows above bed
      2. Unable to lift head above bed
      3. Unable to stand
    9. Other predictors of future intubation
      1. Rapid progression of symptoms over <7 days
      2. Elevated Liver Function Tests
  9. Indications for Intubation
    1. Consider when patient reports Dyspnea
    2. Forced Vital Capacity <20 ml/kg
      1. Some criteria differentiate with bulbar weakness
      2. Vital Capacity 15-18 ml/kg with bulbar weakness
      3. Vital Capacity <15 ml/kg without bulbar weakness
    3. Pressure measurement criteria
      1. Maximal inspiratory pressure <30 cm H2O
      2. Maximal expiratory pressure <40 cm H2O
  10. Course
    1. Respiratory failure within 1-2 weeks of symptoms
  11. Management: Supportive care
    1. Monitor for respiratory failure (25%)
      1. Incentive Spirometry
      2. Control Secretions
    2. Monitor for autonomic failure
      1. Arrhythmias
      2. Blood Pressure abnormalities
    3. Turn patient frequently (prevents Decubitus Ulcers)
    4. Subcutaneous Heparin (prevents Pulmonary Embolism)
    5. Fluid management (Observe for SIADH with low sodium)
    6. Nutrition
    7. Monitor for infections (Urinary Tract Infection in 20%)
    8. Prevent exposure Keratitis
    9. Physical therapy reduces pain
      1. Gentle massage
      2. Range of motion
      3. Position changes
    10. Pain management (significant pain in affected muscles)
      1. See physical therapy above
      2. NSAIDs
      3. Narcotics (use caution due to ileus)
      4. Carbamazepine (Tegretol)
      5. Gabapentin (Neurontin)
      6. Corticosteroids are no longer recommended
        1. Van Der Meche (1992) N Engl J Med 326:1123
    11. Ventilatory management
      1. Indicators to start weaning
        1. Vital Capacity > 15 ml/kg (if no lung disease)
      2. Weaning
        1. First: Change to IMV
        2. Later: lower the Respiratory Rate
  12. Management: Specific Treatment
    1. General Efficacy
      1. Best efficacy if given within first 2 weeks
      2. Both treatments are equally effective
    2. Plasma Exchange (Plasmapheresis)
      1. Protocol
        1. Five exchanges total
        2. Perform one exchange every other day for 10 days
      2. Special Indications
        1. Pregnancy
        2. Renal insufficiency
        3. IgA deficient
    3. IV Immunoglobulin
      1. Dose
        1. IV-Ig 0.4 g/kg daily for 5-7 days
      2. Special Indications
        1. Children
        2. Poor venous access
        3. Autonomic instability
  13. Prognosis
    1. Full recovery within 6-12 months in 85% of cases
    2. Neurologic sequelae in up to 15%
      1. Foot drop
      2. Hand intrinsic Muscle Weakness
      3. Sensory ataxia
    3. Relapse rate <3-5%
    4. Mortality rate <5% overall
      1. Mortality 20% if Mechanical Ventilation required
    5. Predictors of poor prognosis
      1. Age over 60 years
      2. Rapidly progressive, severe disease
      3. Myogram showing axonal loss
      4. Prolonged Mechanical Ventilation >1 month
  14. References
    1. Gallagher in Marx (2002) Rosen's Emergency Med, p. 1510
    2. Shields in Goetz (2003) Neurology, p. 1085-90
    3. Joseph (2002) Adolesc Med 13:487
    4. Newswanger (2004) Am Fam Physician 69(10):2405

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