Tuesday, May 26, 2009

Guillain-Barré Syndrome

  • Post infectious polyneuropathic syndrome mainly affecting motor but may also affect sensory nerves.
  • Affect all ages.
  • It is a demyelinating neuropathy
  • Usually post viral infection (10 days).
  • GI (Campylobacter jejuni) and respiratory (Mycoplasma pneumoniae) symptoms of original infection.
  • Landry ascending paralysis: paralysis beginning from lower extremities and progressing upwards to trunk, upper limbs and bulbar muscles (face, neck, deglutition).
  • 91% symmetric paralysis.
  • Progress of paralysis is gradual (days or weeks)
  • Myalgia in initial stages > weakness > flaccid tetraplegia
  • Sometimes paresthesis
  • Sometimes symptoms of viral meningitis or meniningoencephalitis.
  • Miller-Fisher Syndrome: is a variant of GBS. It is a descending paralysis with paralysis beginning external opthalmoplegia, ataxia, areflexia, sometimes papilledema, without significant motor or sensory deficit in limbs.
  • Tendon reflexes are lost and are usually the last to return.
  • If the autonomic NS is affected, BP, cardiac rate, postural hypotension, bradycardia and occasional asystole can occur. Temporary pacemaker may be required.
  • CSF: *essential; CSF protein is elevated > 2x, glucose is normal, no pleocytosis (leucocytes in CSF) (<10/ul)
  • Motor nerve conduction: greatly reduced conduction velocity in motor nerves and slow in sensory nerves.
  • EMG shows acute denervation of muscles.
  • Creatine Phospokinase (CPK/CK): mildly elevated
  • Antiganglioside antibodies GM! and GD1 are elevated in serum
  • Muscle biopsy (not required): denervation atrophy in chronic GBS.
  • Sural nerve biopsy (nerve below the calf): shows segmental demyelination, focal inflammation, Wallerian degeneration (axon degeneration observed when a nerve is cut)
  • Stool cultures are rarely positive as GBS is post infectious.
  • Immediate admission for observation because of risk of respiratory muscle paralysis.
  • Immunoglobulin I.V.: Octagam 0.4g/kg/day for 3-7 days; caution in renal insufficiency
  • Plasmapheresis (blood serum replacement), steroids (methylprednisolone I.V. 30ug/kg) and/or immunosuppressants if IVIG is ineffective.
  • IVIG + Interferon is effective in some patients
  • Supportive therapy (artificial ventilation and prevention of decibitus)
  • Treatment of bacterial infection
  • Spontaneous full recovery in 2-3 weeks usually
  • Tendon reflexes are last to return
  • Recovery from paralysis in reverse direction of onset
  • Sequelae determined by: cranial nerve involvement, intubation, maximum disability at presentation
  • Relapse present in 7% of children


This is just a place to keep some medical notes I make for myself. If it helps anyone else then that's great!

These are just my own notes. I shall not be held responsible for any subsequent, unfortunate incident. Look stuff up and please correct me if I am wrong!


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