Sunday, June 16, 2013

Guillain-Barré review

Once an open access journal, American Family Physician for the past few years has been behind heavy access controls. Every once in a while a really good review shows up there. Recently they published this article on Guillain-Barré syndrome. It is worth reading in the full text if you can get it.

Some points of interest:

Though the varied spectrum of GBS has enough important features in common to discuss for practical purposes as a single entity it is probably best thought of as a group of diseases reflecting the different immune responses, different targets of immune injury (myelin vs axon), differences in epidemiology and the clinical variants.

Pain and autonomic instability are very common features, I think more than generally appreciated. So maybe you've got a patient presenting with funny unexplained pains and vital signs all over the map. Maybe with multiple preceding ER visits. Think of it!

Symptoms are bilateral and tend to be symmetric but may not be perfectly so at all points in the clinical course.

Reflexes have to be decreased but not necessarily absent at all points in the clinical course.

The epidemiology is complex. While often discussed in the context of Campylobacter jejuni, that infection is associated with a variant of GBS (axonal) not typical in the U.S. A variety of illnesses may precede GBS.

There are two arms of treatment: supportive and disease specific.

Supportive care addresses DVT prophylaxis, pain control, skin care, bowel and bladder issues, physical therapy, cardiac and vital sign monitoring and respiratory care. Multiple respiratory parameters can be monitored. The article lists, among other signs, forced vital capacity less than 20 mL/kg, maximum inspiratory pressure less than 30 cmH2O, and maximum expiratory pressure less than 40 cmH2O as risk factors for respiratory failure. The presence of any of the three signs, according to Up to Date, is an indication for urgent intubation and mechanical ventilation.

Disease specific modalities are plasma exchange and IVIG. One or the other is used, not both. Is one preferred over the other? The review article says plasma exchange is “first line.” The Up to Date section on treatment of GBS says the treatments are equivalent, and that IVIG tends to be favored due to availability and ease of use.

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