Guillain-Barre syndrome (GBS)
What is Guillain Barre Syndrome ?
Guillain-Barre syndrome is an acute, rapidly progressive, and potentially fatal form of polyneuritis that causes segmented demyelination of peripheral nerves. Guillain-Barre syndrome occurs equally in both sexes, usually between the ages of 30 and 50. It affects about 2 of every 100,000 people.The clinical course of Guillain-Barre syndrome has three phases. The acute phase begins when the first definitive symptom develops; it ends 1 to 3 weeks later, when no further deterioration is noted. The plateau phase lasts for several days to 2 weeks and is followed by the recovery phase, which is believed to coincide with remyelination and axonal process regrowth. The recovery phase extends over 4 to 6 months; patients with severe disease may take up to 2 to 3 years to recover, and recovery may not be complete.The disorder is also known as infectious polyneuritis, Landry-Guillain-Barre syndrome, or acute idiopathic polyneuritis.
Causes of Guillain Barre Syndrome
The exact cause of Guillain-Barre syndrome (GBS) is unknown. Theories suggest that GBS is an autoimmune disorder (where the body's immune system fights itself) that can occur after a viral infection, surgery, trauma, or reaction to an immunization.
The incidence of Guillain-Barré syndrome is one to two persons in 100,000 in the general population. GBS can affect children of all ages.
The majority of children diagnosed with GBS experience a full recovery with no further complications. Recovery often begins within a few weeks after the onset of symptoms.
Signs & Symptoms of Guillain Barre Syndrome
The first signs and symptoms of Guillain-Barre syndrome (GBS) are usually numbness or tingling (paresthesia) in the toes and fingers, with progressive weakness in the arms and legs over the next few days. Some patients experience paresthesia only in their toes and legs; others only experience symptoms on one side of the body.
The symptoms may stay in this phase, causing only mild difficulty in walking, requiring crutches or a walking stick. However, sometimes the illness progresses, leading to complete paralysis of the arms and legs. About one quarter of the time, the paralysis continues up the chest and freezes the breathing muscles, leaving the patient dependant on a ventilator. If the swallowing muscles are also affected, a feeding tube may be needed.
In CIDP, the course of illness is longer and respiratory failure is much more unlikely.
Cerebrospinal fluid (CSF) analysis may show a normal white blood cell count, an elevated protein count and, in severe disease, increased CSF pressure.The CSF protein level begins to rise several days after the onset of signs and symptoms, peaking in 4 to 6 weeks, probably resulting from widespread inflammatory disease of the nerve roots.
Electromyography may demonstrate repeated firing of the same motor unit instead of widespread sectional stimulation.Electrophysiologic testing may reveal marked slowing of nerve conduction velocities.
In Guillain-Barre syndrome, treatment is primarily supportive and may require endotracheal intubation or tracheotomy if the patient has difficulty clearing secretions. Mechanical ventilation is necessary if the patient has respiratory difficulties.
Continuous electrocardiogram monitoring is necessary to identify cardiac arrhythmias. Propranolol may be admlnistered to treat tachycardia and hypotension. Atropine may be administered to treat bradycardia. Marked hypotension may require volume replacement.
Plasmapheresis produces a temporary reduction in circulating antibodies. Now an accepted form of therapy, it's most effective when performed during the first few weeks of the disease. The patient may receive three to five plasma exchanges.Immune globulin I.V. can be given if plasmapheresis fails or isn't available.
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