eMedicine Specialties > Neurology > Pediatric Neurology

Guillain-Barre Syndrome in Childhood: Differential Diagnoses & Workup

Author: Brian S Tseng, MD, PhD, Assistant Professor, Department of Neurology, Division of Pediatric Neurology, Harvard Medical School, Massachusetts General Hospital
Coauthor(s): Jennifer A Markowitz, MD, Fellow in Neuromuscular Disease, Massachusetts General Hospital and Brigham and Women's Hospital
Contributor Information and Disclosures

Updated: Sep 18, 2008

Differential Diagnoses

Acute Inflammatory Demyelinating Polyradiculoneuropathy
HIV-1 Associated Progressive Polyradiculopathy
Cauda Equina and Conus Medullaris Syndromes
HIV-1 Associated Vacuolar Myelopathy
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Lyme Disease
HIV-1 Associated Acute/Chronic Inflammatory Demyelinating Polyneuropathy
Myasthenia Gravis
HIV-1 Associated Distal Painful Sensorimotor Polyneuropathy
Organophosphates
HIV-1 Associated Multiple Mononeuropathies
Toxic Neuropathy
HIV-1 Associated Neuromuscular Complications (Overview)

Other Problems to Be Considered

Major categories

Spinal cord lesions - Transverse myelitis, epidural abscess, tumors, poliomyelitis, enteroviral infections of the anterior horn cells, Hopkins syndrome, vascular malformations, cord infarctions, cord compression, lumbosacral disk syndromes, trauma

Peripheral neuropathies - Vincristine, glue sniffing, heavy metals, organophosphate pesticides, HIV, diphtheria, Lyme disease, inborn errors of metabolism, Leigh disease, Tangier disease, porphyria, critical illness polyneuropathy

Neuromuscular junction disorders - Tick paralysis, myasthenia gravis, botulism, hypercalcemia

Myopathies - Periodic paralysis, dermatomyositis, critical illness myopathy

Workup

Laboratory Studies

  • The diagnosis of Guillain-Barré syndrome (GBS) is typically made by the presence of a progressive ascending weakness with areflexia. An LP, electrodiagnostic studies, or occasionally MRI findings can give support for this diagnosis.
  • Typically, the LP is suggestive of demyelination (ie, increased protein >45 mg/dL within 3 wk of onset) without evidence of active infection (lack of CSF pleocytosis), as originally noted by Guillain and Barré.
    • The CSF findings may be normal within the first 48 hours of symptoms, and occasionally the protein may not rise for a week. Serial spinal taps are sometimes often warranted if early studies are normal. 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 casts some doubt on the diagnosis of GBS.

Imaging Studies

  • Spine MRI findings: Nearly 2 weeks after presentation of symptoms, lumbosacral MRI can show enhancement of the cauda equina nerve roots with gadolinium. This imaging study has been described to be 83% sensitive for acute GBS and present in 95% of typical cases.

Other Tests

  • Temperature, blood pressure, heart rate, respiratory capacity (eg, MIFs), blood gases (if necessary), and urine output of the patient should be monitored.
    • Intubation and mechanical ventilation should be considered when vital capacity falls below 15 mL/kg body weight or arterial pressure of oxygen falls below 70 mm Hg (or the patient has significant fatigue). Maximal inspiratory flows (MIFs) or negative inspiratory flows (NIFs) are important measures in older children.
    • During the acute phase of the illness, orthostatic hypotension and urinary retention also may cause significant problems.
  • Electrodiagnostic studies
    • Within the first week of the onset of symptoms, electrodiagnostic studies in at least two limbs reveal 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), and 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 <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.
  • Serum anti-ganglioside antibodies
    • Value as a prognostic marker in children is still under evaluation.
    • Anti-GM1, GM1b, GD1a, and GalNAc-GDIa have been associated in adults with C jejuni infection, acute motor axonal neuropathy, a more severe course, and more residual neurologic deficits. 
    • A recent study of 32 Japanese children diagnosed with Guillain-Barr é syndrome 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.4 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 Guillain-Barr é syndrome.5
    • Other antibodies are associated with specific forms of Guillain-Barr é, such as GQ1b with Miller-Fisher syndrome and GT1a with pharyngeal-cervical-brachial variant, and these may be useful in the diagnostic workup of variant clinical presentations.

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.

Recently, epidemics of GBS were noted to occur annually in the rural areas of North China, particularly during the summer months. This has been associated with C jejuni infection, and many of these patients have antiglycolipid antibodies. In this axonal form of GBS, biopsy specimens reveal Wallerianlike 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.

More on Guillain-Barre Syndrome in Childhood

Overview: Guillain-Barre Syndrome in Childhood
Differential Diagnoses & Workup: Guillain-Barre Syndrome in Childhood
Treatment & Medication: Guillain-Barre Syndrome in Childhood
Follow-up: Guillain-Barre Syndrome in Childhood
References
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Further Reading

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Keywords

Guillain-Barre syndrome in children, Guillain-Barre syndrome, Guillain-Barré-Strohl syndrome, acute inflammatory demyelinating polyneuropathy, acute inflammatory demyelinating polyradiculopathy, AIDP, acute febrile polyneuritis, GBS, acute motor axonal neuropathy, AMAN

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

Jennifer A Markowitz, MD, Fellow in Neuromuscular Disease, Massachusetts General Hospital and Brigham and Women's Hospital
Jennifer A Markowitz, MD is a member of the following medical societies: Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

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; 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Department of Pediatrics, Division of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital
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.

 
 
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