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guillain-barre

Guillain-Barre syndrome (GBS)

introduction

  • aka Landry's ascending paralysis
  • Guillain-Barré syndrome is an acute onset autoimmune demyelinating condition which appears to be triggered by viral or bacterial infections, particularly Campylobacter jejuni, in the preceding 1-3 weeks
  • it does not appear to be contagious
  • 10% mortality
  • 75-90% will recover completely but this usually takes 6 months to 2 years

6 sub-types

  • acute inflammatory demyelinating polyneuropathy (AIDP)
    • the most common form of GBS, and the term is often used synonymously with GBS.
    • caused by an autoimmune response directed against Schwann cell membranes.
  • Miller Fisher syndrome (MFS)
    • a rare variant of GBS. Accounting for about 5% of GBS cases, it manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS.
    • usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia.
    • the ataxia predominantly affects the gait and trunk, with the limbs relatively spared.
    • anti-GQ1b antibodies are present in 90% of cases.
  • acute motor axonal neuropathy (AMAN)
    • also known as Chinese paralytic syndrome
    • attacks motor nodes of Ranvier and is prevalent in China and Mexico.
    • probably due to an auto-immune response directed against the axoplasm of peripheral nerves.
    • may be seasonal and recovery can be rapid.
    • anti-GD1a antibodies are present.
    • anti-GD3 antibodies are found more frequently in AMAN.
  • acute motor sensory axonal neuropathy (AMSAN)
    • similar to AMAN, but also affects sensory nerves with severe axonal damage.
    • like AMAN, it is probably due to an autoimmune response directed against the axoplasm of peripheral nerves.
    • recovery is slow and often incomplete
  • acute panautonomic neuropathy
    • the most rare variant of GBS, sometimes accompanied by encephalopathy.
    • is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias.
    • frequently occurring symptoms include impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and constipation unrelieved by laxatives or alternating with diarrhea.
    • initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhoea, dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction.[6] Parasympathetic impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils; loss of accommodation) may also be observed.
  • Bickerstaff's brainstem encephalitis (BBE)
    • a further variant, is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign.
    • course of the disease can be monophasic or remitting-relapsing.
    • large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla, are described in the literature.
    • despite severe initial presentation, BBE usually has a good prognosis.
    • MRI plays a critical role in the diagnosis of BBE.
    • a considerable number of BBE patients have associated axonal Guillain–Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.

clinical features

  • usually starts as a symmetric bilateral ascending weakness
  • may start in upper limbs and descend to legs in patients with the rarer form - Miller Fisher syndrome (MFS)
  • symptoms take days to weeks to progress
  • respiratory failure and immobility cause significant risks such as:

Mx

  • admit all patients to ensure complications can be prevented
  • consider lumbar puncture (LP) - raised CSF protein is usually found
  • Mx respiratory failure and other dysfunction
  • DVT prophylaxis after LP if LP is being considered
  • general nursing care to minimise pressure areas, etc
  • consider:
    • plasmapheresis
    • gammaglobulin (IVIG)
guillain-barre.txt · Last modified: 2013/09/27 05:13 by gary1