Guillain-Barre Syndrome

Posted by Sara Fazio • June 15th, 2012

The latest review in our Medical Progress series considers the current understanding, diagnosis and management of the Guillain-Barré syndrome.

The Guillain-Barre syndrome, which is characterized by acute areflexic paralysis with albuminocytologic dissociation (i.e., high levels of protein in the cerebrospinal fluid and normal cell counts), was described in 1916. Since poliomyelitis has nearly been eliminated, the Guillain-Barre syndrome is currently the most frequent cause of acute flaccid paralysis worldwide and constitutes one of the serious emergencies in neurology.

Clinical Pearls

• What infectious agents are most frequently associated with the development of the Guillain-Barre syndrome?

Two thirds of cases are preceded by symptoms of upper respiratory tract infection or diarrhea. The most frequently identified infectious agent associated with subsequent development of the Guillain-Barre syndrome is Campylobacter jejuni, whereas cytomegalovirus has been identified in up to 10%. The incidence of the Guillain-Barre syndrome is estimated to be 0.25 to 0.65 per 1000 cases of C. jejuni infection, and 0.6 to 2.2 per 1000 cases of primary cytomegalovirus infection. Other infectious agents with a well-defined relationship to the Guillain-Barre syndrome are Epstein-Barr virus, varicella-zoster virus, and Mycoplasma pneumoniae.

• What are the typical clinical manifestations of Guillain-Barre syndrome?

The first symptoms of the Guillain-Barre syndrome are numbness, paresthesia, weakness, and pain in the limbs. The main feature is progressive bilateral and relatively symmetric weakness of the limbs, and the weakness progresses over a period of 12 hours to 28 days before a plateau is reached. Patients typically have generalized hyporeflexia or areflexia. A history of upper respiratory infectious symptoms or diarrhea 3 days to 6 weeks before the onset is not uncommon.

Morning Report Questions

Q: How is the diagnosis of Guillain-Barre syndrome established?

A: A lumbar puncture is usually performed in patients with suspected Guillain-Barre syndrome, primarily to rule out infectious diseases, such as Lyme disease, or malignant conditions, such as lymphoma. A common misconception holds that there should always be albuminocytologic dissociation (i.e., high protein and few cells). Whereas patients with these other conditions generally have a pleocytosis, some patients with human immunodeficiency virus infection and the Guillain-Barre syndrome also have pleocytosis. The protein concentration is normal in more than 50% of patients with the Guillain-Barre syndrome during the first week of illness, and this percentage increases to 75% in the third week.

Q: What is the Miller Fisher variant of Guillain-Barre syndrome?

A: The Miller Fisher syndrome, which is characterized by ophthalmoplegia, ataxia, and areflexia, was reported in 1956 as a likely variant of the Guillain-Barre syndrome, because the cerebrospinal fluid of affected patients showed albuminocytologic dissociation. The Miller Fisher syndrome appears to be more common among patients with the Guillain-Barre syndrome who live in eastern Asia than among those who live in other parts of the world, occurring in up to 20% of patients in Taiwan and 25% of patients in Japan. Most patients with both disorders have evidence of infection 1 to 3 weeks before the development of ophthalmoplegia or ataxia. The presence of distal paresthesia is associated with the Miller Fisher syndrome. Recovery from ataxia and recovery from ophthalmoplegia take a median of 1 and 3 months, respectively.

5 Responses to “Guillain-Barre Syndrome”

  1. dr Abdul Aziz says:

    Informative

  2. Shafi says:

    nice

  3. bog says:

    awesome article.thank you.it is very useful

  4. Bagus Yudha says:

    Nice and informative, thankyou

  5. Mags says:

    Median of one to three months? You’ve neglected to point out that some people never recover or only partially recover. 7 years and counting now…

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