Pediatric Guillain-Barre Syndrome Workup

  • Author: Marc P DiFazio, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Jan 26, 2012
 

Approach Considerations

The diagnosis of Guillain-Barré syndrome (GBS) is typically based on the presence of a progressive ascending weakness with areflexia.

Findings on lumbar puncture, electrodiagnostic studies, or occasionally MRI can give support for the diagnosis. However, abnormalities on these studies do not develop until days to weeks after onset of symptoms.

Nearly 2 weeks after presentation of symptoms, lumbosacral MRI can show enhancement of the nerve roots with gadolinium. This imaging study has been described to be 83% sensitive for acute GBS, with nerve root enhancement present in 95% of typical cases.[31]

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Lumbar Puncture

Typically, the LP findings are suggestive of demyelination (ie, increased protein >45 mg/dL within 3 weeks of onset) without evidence of active infection (lack of CSF pleocytosis), as originally noted by Guillain, Barré, and Strohl. The CSF findings may be normal within the first 48 hours of symptoms, and occasionally the protein may not rise for a week. Usually by 10 days of symptoms, elevated CSF protein findings will be most prominent.

Most patients have fewer than 10 leukocytes per milliliter, but occasionally a mild elevation (ie, 10-50 cells/mL) is seen. Greater than 50 mononuclear cells/mL of CSF makes the diagnosis of GBS doubtful.

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Electrodiagnostic Studies

Within the first week of the onset of symptoms, electrodiagnostic studies in at least two limbs reveal the following:

  • A dispersed, impersistent, prolonged, or absent F response (88%)
  • Increased distal latencies (75%)
  • Conduction block (58%) or temporal dispersion of compound muscle action potential (CMAP)
  • Reduced conduction velocity (50%) of motor and sensory nerves

Criteria for axonal forms include lack of neurophysiologic evidence of demyelination, with loss of amplitude of CMAP or sensory nerve action potentials to at least less than 80% of lower limit of normal values for age. It is typically prudent to wait at least 7-10 days for electrical studies to be informative. If electrical studies are performed too early, normal results can be falsely reassuring.

By the second week of illness, reduced compound muscle action potential (CMAP, 100%), prolonged distal latencies (92%), and reduced motor conduction velocities (84%) are prominent.

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Serum Anti-Ganglioside Antibodies

In adults with GBS, serum ganglioside antibodies directed against GM1, GM1b, GD1a, and GalNAc-GDIa have been associated with Campylobacter jejuni infection, acute motor axonal neuropathy, a more severe course, and more residual neurologic deficits. The value of these studies as a prognostic marker in children is still under evaluation.

A study of 32 Japanese children diagnosed with GBS identified one or more of these antibodies in 44% and in 64% of those who met the electrodiagnostic criteria for acute motor axonal neuropathy. Those with positive antibodies had a more prolonged recovery with more residual symptoms at the end of the study.[32] However, another study in Western Europe did not find any difference in clinical course or outcome in the 4 patients with positive antibodies out of 63 total children with GBS.[33]

Other antibodies are associated with specific forms of GBS, such as GQ1b with Miller-Fisher syndrome, GD1b with acute sensory neuronopathy, and GT1a with pharyngeal-cervical-brachial variant. Assays for these antibodies may be useful in the diagnostic workup of variant clinical presentations.

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Histologic Findings

Although not typically part of routine GBS diagnostic evaluation in pediatric or adult patients, the following are expected findings in GBS:

  • In the demyelinating form, demyelination and mononuclear infiltration by lymphocytes and macrophages are seen in peripheral nerves
  • Lymphocytes and macrophages surround endoneural vessels and cause an adjacent demyelination
  • These lesions can be discrete and are scattered throughout the peripheral nervous system, although they may have a predilection for inflammation of the nerve roots.
  • The conduction block and demyelination of the motor nerves result in the progressive weakness that is characteristic of this syndrome. Similarly, the involvement of the sensory nerves leads to pain and paresthesias

Many authors believe that the mechanism of the disease involves an abnormal T-cell response precipitated by a preceding infection. This is thought to give rise to an abnormal immune stimulation. A variety of specific endogenous antigens may be involved in this response, including myelin P-2 and ganglioside GM1, GQ1b, and GT1a.

In this axonal form of GBS, biopsy specimens reveal wallerian like degeneration of fibers in the ventral and dorsal nerve roots, with only minimal demyelination or lymphocytic infiltration. These axonal lesions affect both the sensory fibers and the motor fibers.

Although this form of GBS has been associated with Campylobacter infection, it appears to be a rare complication of such infection.

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Contributor Information and Disclosures
Author

Marc P DiFazio, MD  Associate Professor, Department of Neurology, Uniformed Services University of the Health Sciences; Director, Pediatric Subspecialty Services, Shady Grove Adventist Hospital for Children

Marc P DiFazio, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Cerebral Palsy and Developmental Medicine, American Academy of Neurology, Child Neurology Society, and Movement Disorders Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nitin C Patel, MD, MPH  Professor of Clinical Neurology and Child Health, Department of Child Health, Chief for Developmental Pediatrics and Child Neurology, Specialist in Pediatrics/Neurology, University of Missouri Hospital and Clinics at Columbia

Nitin C Patel, MD, MPH is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Mita N Patel  University of Missouri-Columbia School of Medicine

Disclosure: Nothing to disclose.

Sameer Chhibber, MD, FRCPC  Neuromuscular Fellow, Department of Neurology, Brigham and Women's Hospital and Massachusetts General Hospital, Harvard Medical School

Disclosure: Nothing to disclose.

Brian S Tseng, MD, PhD  Assistant Professor, Department of Neurology, Division of Pediatric Neurology, Harvard Medical School, Massachusetts General Hospital

Brian S Tseng, MD, PhD is a member of the following medical societies: Child Neurology Society

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

Neil A Busis, MD Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Jennifer A Markowitz, MD Attending Physician, Department of Neurology, Children's Hospital Boston

Jennifer A Markowitz, MD is a member of the following medical societies: Child Neurology Society

Disclosure: Nothing to disclose.

Robert Stanley Rust Jr, MD, MA Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Sarah Sheikh, MBBCh, MSc, MRCP Neuromuscular Fellow, Department of Neurology, Brigham and Women's Hospital

Sarah Sheikh, MBBCh, MSc, MRCP is a member of the following medical societies: American Academy of Neurology, Massachusetts Medical Society, and Royal College of Physicians of the UK

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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