The Guillain-Barre Syndrome

I’ll bet you didn’t know it was called the Landry-Guillain-Barre-Strohl syndrome. Now, we’ve simplified it to a dual eponym, the Guillain-Barre syndrome (GBS)–not unlike the abbreviated title of Lewis-Sumner syndrome. In this week’s episode of continuing medical audiocation on BrainWaves, we discuss the more interesting aspects to this uncommon disease: the variants, the vaccines, and the viciousness.

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Georges Guillain, Parisian professor credited with some of the original descriptions of what is now known as the Guillain-Barre Syndrome. Image under public domain.

You’ve likely been taught that GBS is a monophasic illness that begins with ascending weakness without numbness and can culminate in a catastrophic respiratory failure. I’ve even seen this limited perspective taught to pediatric residents in a published textbook for board preparation. But you can’t limit yourself to these oversimplifications, or you’ll miss a great many cases of GBS. So take a listen to our podcast this week and do this disease some justice.

Most cases of GBS are in fact characterized by ascending weakness. About 80% of cases in the USA actually. But what of the remaining 20%? Variants like the Miller Fisher syndrome and the pharyngeal-cervical-brachial variant may involve only the eyes or the upper extremities and bulbar muscles. Other variants are entirely sensory and can mimic an anti-Hu paraneoplastic syndrome, while yet others can present with encephalopathy and preservation of appendicular strength. The only real symptoms or signs you can hang your hat on are those of areflexia, albuminocytologic dissociation in the CSF, and demyelinating features on nerve conduction studies. But even these are not perfectly sensitive. You’ve got to consider the entire clinical picture.

For instance, was there a preceding illness? Or a vaccine? Many cases of GBS follow a viral prodrome by several weeks and this should always raise your suspicion. Many vaccines have been reported to increase the risk of GBS. But so have their associated pathogens (e.g., influenza, pneumococcus). This does not mean you can justify deferral of a vaccine because you’re afraid of getting GBS. It’s a 1 in a million chance with the latest flu vaccines—your chance of getting the flu, or developing other severe complications are likely much greater.

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Vaccinations. From https://www.flickr.com/photos/93519839@N03/19758454771/ under a Creative Commons license.

Outcomes. Generally, they are pretty good. With treatment, as we discuss on the show, the duration of ventilator-dependence and non-ambulatory time is significantly reduced with therapies like intravenous immune globulin or plasma exchange. Counseling is important because recovery is often delayed (median duration to full recovery is about 200 days), and the risk of recurrence is not trivial (~10%). So know what to tell your patients before going in. There’s more to it than you might have known.

 

[Jim Siegler]


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Tam CC, O’Brien SJ, Petersen I, Islam A, Hayward A and Rodrigues LC. Guillain-Barre syndrome and preceding infection with campylobacter, influenza and Epstein-Barr virus in the general practice research database. PloS one. 2007;2:e344.

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