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Guillain-Barré Syndrome (GBS) Information

  • Guillain-Barré syndrome (GBS), is an acquired immune-mediated inflammatory disorder of the peripheral nervous system (i.e. not the brain or spinal cord). The pathologic hallmark of the disease is loss of myelin in peripheral nerves due to an acute and progressive inflammation of unknown cause. It is suggested that it is an autoimmune disease, in which the sufferer's immune system is triggered into damaging the nerve covering. There is some support for this in that half of all cases occur soon after a microbial infection or respiratory or gastrointestinal viral infection. Many cases developed in people who received the 1976 swine flu vaccine...
  • GBS is a rare disease affecting about 1 to 2 people in every 100,000 annually. It does not discriminate with regard to the age or sex of sufferers. When diagnosed in young teenagers, it generally does not recur for many years, although when it does, it often does so in the fourth or fifth decade of life, long after the patients may have forgotten the details of the original episode.

    Extensive damage of myelin causes disturbances in peripheral nerve functions, which can be classified as motor (affecting the muscle), sensory (affecting the skin) or autonomic (affecting the internal organs). Therefore, patients usually show two or more of the following symptoms: weakness (often symmetrical, in ascending fashion, leading to respiratory failure in one-third of cases), decreased sensation (numbness, loss of position sense), severe fluctuations in blood pressure, irregularities of heart rate, constipation and incontinence. Additional symptoms may be blurred vision, difficulty moving facial muscles, difficulty swallowing, and drooling.

    The symptoms are ascending weakness with abnormal sensations and then paralysis of the legs, arms, face and possibly breathing muscles. Miller-Fisher Syndrome, however, is a descending weakness, proceeding in the reverse order of the more common form of Guillain-Barré syndrome. Guillain-Barré syndrome is rarely fatal but there is no direct cure and recovery may need care in an intensive care unit and can take years (although people can recover in a few weeks as well).

    Supportive care with monitoring of all vital functions is the cornerstone of successful management. Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early intubation should be considered in any patient with a Vitaly Capacity (VC) <20 cc/kg, a Negative Inspiratory Force (NIF) <-30, a decrease >30% in either VC or NIF within 24 hours, rapid progression of disease, autonomic instability.

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