Guillain-Barré syndrome in children: Epidemiology, clinical features, and diagnosis
- Monique M Ryan, FRACP
Monique M Ryan, FRACP
- University of Melbourne
- Head, Department of Neurology
- Royal Children's Hospital Melbourne
- Section Editors
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré syndrome (GBS), after the authors of early descriptions of the disease. Historically, GBS was considered a single disorder, but it is now known to be a heterogeneous syndrome with several variant forms. Most often, GBS presents as an acute monophasic paralyzing illness provoked by a preceding infection. In addition to the demyelinating form, which is the most common type, axonal forms of GBS are well-recognized.
This topic will review the epidemiology, clinical features, and diagnosis of GBS in children. Other aspects of GBS are discussed separately. (See "Pathogenesis of Guillain-Barré syndrome" and "Guillain-Barré syndrome in children: Treatment and prognosis".)
The pathogenesis of Guillain-Barré syndrome (GBS) is discussed here briefly and reviewed in detail elsewhere. (See "Pathogenesis of Guillain-Barré syndrome".)
One proposed mechanism for GBS is that an antecedent infection evokes an immune response, which in turn cross-reacts with peripheral nerve components because of the sharing of cross-reactive epitopes (molecular mimicry). The end result is an acute polyneuropathy. This immune response can be directed towards the myelin or the axon of peripheral nerve. The main lesions of GBS are acute inflammatory demyelinating polyradiculoneuropathy and, particularly in patients with Campylobacter-associated disease, acute axonal degeneration. (See "Pathogenesis of Guillain-Barré syndrome", section on 'Molecular mimicry' and "Pathogenesis of Guillain-Barré syndrome", section on 'Pathology'.)
Antecedent infections are common with GBS, and are thought to trigger the immune response that leads to acute polyneuropathy. Approximately two-thirds of patients give a history of an antecedent respiratory tract or gastrointestinal infection. Campylobacter infection is the most commonly identified precipitant of GBS and can be demonstrated in as many as 30 percent of cases. Other precipitants include cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, and influenza-like illnesses. GBS can also occur in association with human immunodeficiency virus (HIV) infection, predominantly in those who are not profoundly immunocompromised. A small percentage of patients develop GBS after another triggering event such as immunization, surgery, trauma, or bone-marrow transplantation. (See "Pathogenesis of Guillain-Barré syndrome", section on 'Antecedent events'.)
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- CLINICAL FEATURES
- Physical examination
- Clinical course
- SUBTYPES OF GUILLAIN-BARRÉ SYNDROME
- Acute inflammatory demyelinating polyneuropathy
- Acute motor axonal neuropathy
- Acute motor-sensory axonal neuropathy
- Miller Fisher syndrome
- Bickerstaff encephalitis
- Polyneuritis cranialis
- Pharyngeal-cervical-brachial weakness
- Other variants
- Cerebrospinal fluid
- Electrodiagnostic studies
- Magnetic resonance imaging
- DIFFERENTIAL DIAGNOSIS
- Chronic inflammatory demyelinating polyneuropathy
- Other polyneuropathies
- Spinal cord disorders
- Neuromuscular junction disorders
- Muscle disorders
- INFORMATION FOR PATIENTS