Guillain-Barré syndrome (GBS) is a common disease of uncertain cause involving the peripheral nerves and occurring in both sexes and all age groups, although the disease seems milder in children. The illness can follow a nonspecific viral syndrome, like a mild upper respiratory infection or gastroenteritis, which precedes the onset of the neurologic by 1-3 weeks or be associated with HIV infection, Campylobacter jejuni infection, hepatitis, infectious mononucleosis, Mycoplasma pneumoniae, vaccination, surgery, lymphoma, or SLE. Which leads the conclusion that it is almost certainly an immune-mediated disorder.
GBS during many years was synonymous with acute inflammatory demyelinating polyneuropathy, because initially the pathologic substrate of many cases was shown to be lymphocytic infiltration of the spinal roots and peripheral nerves, with macrophage-mediated demyelination and secondary axonal degeneration.
In the other hand, it is known now that a large proportion of cases in the developing world and a small proportion of cases in North America and Europe are Characterized by noninflammatory acute axonal degeneration.
Classically, the disease presents with symmetric leg weakness that spreads to involve other regions of the body in an ascending manner, finally involving the cranial nerves and impeding respiratory function( 25% of the cases ) within 1-3 days from onset of symptoms. The weakness does not ascend in all cases, however.
Relatively minor sensory signs and symptoms occur; however, the patient may complain of painful extremities subjective and objective sensory disturbances are common initially, most commonly occurring in a distal( stocking-glove) distribution.
Cardiac arrhythmias and wide swings in blood pressure can develop and reflect peripheral autonomic nervous system involvement.
Cranial nerve involvement except nerves I e II is common and the most common nerve involved is the seventh nerve ( facial ). Cranial nerves palsies may be the most prominent feature of the illness, as in Miller-Fisher variant which presents with ophthalmoplegia, ataxia and areflexia.
Absence of tendon reflexes almost always occurs and is the most important clue to diagnosis and is even in muscles that cannot yet be shown to be weak by objective testing.
Examination of the
CSF demonstrates elevated protein as high as 100-400 mg/dl and less than 50
mononuclear
cells/microliter
( cytoalbuminologic dissociation ), but it may not be
seen until several
days after the onset of symptoms and are not specific because it may be seen
in any acute or
chronic polyneuritis.An important differential diagnosis is
hypophosphatemia, which may cause
the same clinical manifestations.
Because of the potential for rapid deterioration, patients with presumptive diagnosis of GBS usually require hospitalization for observation. Monitoring should include frequent measurement of the vital capacity and ability to swallow. Intensive-care observation and insertion of an airway should be initiated early, before declining ventilatory strength, autonomic dysregulation, or fatigue due to unproductive coughing erupts into an acute emergency.
Therapy with plasmapheresis can shorten the need for mechanical ventilation. Criteria to initiate plasmapheresis include the inability of a patient to walk or rapid progression of the disease. Intravenous immunoglobulin treatment is also efficacious. Corticosteroids are ineffective and may prolong the disease in the elderly patient.
Acute and chronic inflammatory demyelinating polyradiculoneuropathies. In Wynngaarden
JB, Smith LH, Bennet JC(eds): Cecil Textbook of
Medicine, 20th edition. W B Saunders Company, 1996.
Mckhann GM: Clinical and therapeutic observations. Ann Neurol 27(suppl 1): S13, 1990.
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