GUILLAIN-B ARRE SYNDROME

Epidemiology:

  1. incidence 1-2/100,000 per year
  2. the most common cause of acute weakness with areflexia after cord compression and tick paralysis are ruled out
  3. Peak ages: teens and adults > 55 yo
  4. Male:Female = 1.25:1
PATHOPHYSIOLOGY

GBS is an autoimmune demyelinating process whereby viral infection predisposes a body to make autoAb’s that attack various components of peripheral myelin sheath (gangliosides)

Pathology

There are multifocal areas of demylelination and inflammatory changes in peripheral nerves

Clinical features:

  1. Antecedent illness within previous 4weeks


    2. a. URI

    b. GI infection especially Campylobacter jejuni (more severe disease)

    c. EBV

    d. CMV

  2. Weakness—most often in the legs, symmetric, proximal and distal, progressive in severity, most patients reach a nadir by 3 weeks; about 1/3 patients become bed-bound, 1/3 require ventilatory support secondary to respiratory muscle weakness.
  3. Cranial nerve weakness- symmetric, tongue most common
  4. Tendon reflex loss—common early symptom , progressive loss in 1st week, starts with ankles, progresses proximally
  5. Pain and paresthesias—pain usually in back, hips, legs
  6. Autonomic dysfunction—transient hypertension or hypotension, sinus tachycardia, urinary retention, ileus.
  7. Mild sensory loss including vibratory and propioception
8. Miller-Fisher variant of GBS-Severe form of GBS

a. Clinical features:

i. Acute onset of areflexia, ataxia, ophthalmoplegia,

Evaluation:

  1. CSF: Albumino-cytologic dissociation( i.e. high protein but normal cells)
  2. Nerve conduction test- slowing
Diagnosis

1. Progressive motor weakness involving multiple limbs

2. Areflexia

3. Onset with pain in limb

4. Cranial nerve involvement

5. Autonomic dysfunction

6. Elevated CSF protein

7. Abnormal electrodiagnosis

Management:

1. Supportive care

2. Plasma exchange

3. IVIG

Prognosis

1. 3 % mortality secondary to autonomic disturbances and respiratory failure

2. 80% complete recovery in 12 months

3. Some patients have residual neurologic deficits, but few unable to perform normal daily tasks

References:

  1. Asbury, AK New concepts in Guillain-Barre syndrome. Journal of Child Neurology. 2000 Mar; 15(3):183-91.
  2. Evans, OB, Vedanarayanan V. Guillain-Barre syndrome. Pediatrics in Review. 1997 Jan;18(1):10-6.
  3. Fenichel, G. Clinical Pediatric Neurology, 4th ed. WB Saunders Co., 2001.