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Guillain-Barré syndrome

A prospective, population-based incidence and outcome survey

*See the Appendix on page 1149 for a list of members of the Piemonte and Valle d’Aosta Register for GBS (PARGBS).
From the Department of Neuroscience, University of Turin (Drs. Chiò, Cocito, Mutani, and Giordana); and San Giovanni Battista Hospital, Turin (Drs. Chiò, Cocito, and Mutani); the Division of Neurology, "A. Avogadro" University, Novara (Dr. Leone); San Luigi Hospital, Orbassano (Dr. Giordana); and the Department of Neurological Rehabilitation, Fondazione S. Maugeri, Clinical del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Veruno (Dr. Mora), Italy.

Address correspondence and reprint requests to Dr. Adriano Chiò, Department of Neuroscience, Via Cherasco 15, I-10126 Torino, Italy; e-mail: achio{at}usa.net

Objective: The authors evaluated the incidence and long-term prognostic factors of Guillain-Barré syndrome (GBS) in a prospective, population-based study.

Methods: Patients with GBS diagnosed according to National Institute of Neurological and Communicative Disorders and Stroke criteria in the 2-year period 1995 to 1996 in two Italian regions were prospectively followed up for 2 years after onset of GBS.

Results: A total of 120 patients were found, corresponding to a crude annual incidence rate of 1.36/100,000 population (95% CI, 1.13 to 1.63). A total of 7 (5.8%) patients, all but one with axonal or mixed EMG pattern, died acutely within 30 days from the onset of the disease. Acute mortality was due to respiratory involvement and intensive care unit complications. In multivariate analysis, a worse 2-year outcome (Hughes score 2) was related to a higher Hughes grade at nadir, axonal or mixed EMG, age 50 years, and absence of respiratory infections preceding GBS. The persistence of disability 2 years after the acute phase was related to axonal involvement and a worse status at nadir.

Conclusions: After adjustment to US population, the incidence rates for GBS from different countries showed no significant differences. Both acute mortality and long-term disability in GBS seem to be related to an axonal involvement and a Hughes grade 2 at nadir.

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