Guillain-Barré Syndrome

Owen B. Evans; Vettaikorumakankav Vedanarayanan

doi:10.1542/pir.18-1-10

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Download PDF

Owen B. Evans, MD^*
Vettaikorumakankav Vedanarayanan, MD^†

^*Professor and Chairman.
^†Associate Professor, Department of Pediatrics, University of Mississippi Medical Center, Jackson, MS.

IMPORTANT POINTS

Guillain-Barré syndrome usually presents with a gait disturbance and clinical features of pain, weakness, and areflexia.
Guillain-Barré syndrome is an immune-mediated disease directed against the peripheral nervous system myelin, axon, or both.
Increased cerebrospinal fluid (CSF) protein and abnormal electrodiagnostic studies may be normal early in the course of Guillain-Barré syndrome.
The most serious complications during the acute phase of Guillain-Barré syndrome are respiratory failure and autonomic disturbances.
Plasma exchange or intravenous immune globulin therapy significantly shortens the duration and severity of Guillain-Barré syndrome.

Introduction

Guillain-Barré Syndrome (GBS) is an acquired disease of the peripheral nervous system; the major features are weakness and areflexia. The disease involves both genders and all ages and has been reported worldwide. It is the most common cause of acute generalized paralysis in all ages. There is no distinctive test to confirm the diagnosis of GBS, and there is considerable heterogeneity in the presentation and clinical course. Further, variants of the disease have emerged to make GBS a true syndrome rather than a specific disease.

Epidemiology

The estimated annual incidence of GBS is approximately 0.4 to 1.7 cases per 100,000 population. There is considerable variation in the reported incidence in children. In one large study from a developing country, the overall incidence was 1.1/100,000 population. However, the incidence for children younger than 4 years of age was 1.7/100,000 compared with 0.1/100,000 for children older than 10 years of age. On the other hand, a study from Finland reported an incidence of 0.38/100,000 for children younger than 15 years of age, with no differences between older or younger children. The disease is very rare in children younger than 1 year of age. There is an approximately 1.5:1 male- to-female ratio. Male predominance is more significant in adults, particularly the elderly, than in children.