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Pediatric Guillain-Barre Syndrome Clinical Presentation

  • Author: Marc P DiFazio, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: Dec 11, 2014
 

History

Patients with Guillain-Barré syndrome (GBS) present with complaints of weakness and/or unsteadiness (ataxia). Weakness is a hallmark of GBS. The weakness typically starts in the legs and ascends to the arms (hence, the description progressive ascending flaccid paralysis). This progression may occur over hours to days to weeks. The weakness is usually symmetric.

Pain and dysesthesias also are noted, particularly in children. Pain may be the initial manifestation in almost half of affected children. The nonspecific nature of the symptoms may distract from the actual diagnosis, for exampe, a pediatric patient presenting with prolonged and unexplained abdominal pain.

Often, onset of these symptoms is within 2-4 weeks of an illness or immunization. The preceding illness often involves fever, muscle pains, diarrhea or upper respiratory infection.

Urinary retention is also noted early in the course of 10-15% of children with GBS. At the peak of illness, about half the pediatric patients with GBS may have associated autonomic dysfunction and cranial nerve (CN) involvement, and about 10-12% require a mechanical ventilator. In those with CN involvement, the facial nerve is most commonly affected, resulting in bilateral facial weakness.

Subtypes of GBS

GBS peripheral nerve damage can be classified histopathologically into 2 main types: demyelinating forms and axonal-degenerating forms. Motor nerves are more susceptible to disease than sensory ones. In 1995, GBS was subdivided into 4 distinct forms based on histopathological and neurophysiological basis: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and Miller-Fisher syndrome (MFS).[25, 26]

The clinical spectrum of GBS, which includes individual variation and variable severity of presentation, comprises the following:

  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) - This accounts for 80-90% of GBS cases in Europe and North America. It is characterized by an immune-mediated attack on myelin with infiltration of lymphocytes and macrophages with segmental stripping of myelin. Motor and sensory fibers are usually affected simultaneously, producing corresponding deficits. Electrophysiology shows slow nerve conduction velocity and prolonged F waves.
  • Acute motor axonal neuropathy (AMAN) - This form of neuropathy is most commonly seen in China and Japan (50-60% of cases), as apposed to Western countries (10-20% of cases). In this form, axonal degeneration occurs by immune attack within 1-2 weeks after infection. Specific antibodies to axonal membranes of motor fibers attack the nodes of Ranvier. This, in turn, activates complement and intrusion of macrophages into periaxonal space, resulting in destruction of axons. C jejuni is the most common preceding infection, and antiganglioside antibodies are usually found in this type. Electrophysiology shows reduction in muscle action potentials with relatively preserved motor nerve conduction velocity and normal sensory nerve action potentials and F waves. [25, 27]
  • Acute motor and sensory axonal neuropathy (AMSAN) - This type is rare and resembles AMAN except sensory nerves are also affected. This type is associated with a severe course and poor prognosis.
  • Miller-Fisher syndrome (MFS) - The involvement of CNs is very distinct in this form of GBS. Ocular motor nerves (oculomotor, trochlear, and abducens) are affected and produce a triad of ophthalmoplegia, ataxia, and areflexia. Electrophysiology is normal. The characteristic autoantibodies are against gangliosides GQ1b and GT1a. GQ1b plays a key role in the pathogenesis of MFS. [27]
  • Polyneuritis cranialis - This is an acute onset of multiple CN palsies (usually bilateral CN VII with sparing of CNs I and II), elevated cerebrospinal fluid protein, and slowed nerve conduction velocity with uncomplicated recovery.
  • Pharyngo-cervical-brachial syndrome - This variant form of GBS is characterized by localized and regional involvement of autonomic and motor nerves in the pharyngeal-cervical-brachial distribution. The diagnosis of this condition is based on clinical, laboratory, and neurophysiological findings and the exclusion of other conditions mimicking this disorder.
  • Acute sensory neuropathy of childhood
  • Acute pandysautonomia - Besides the above main forms of GBS, acute pandysautonomia is also a common subtype with which the autonomic nervous system is involved. Parasympathetic and sympathetic involvement is seen along with sensory or motor nerve involvement.
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Physical Examination

On physical examination, an ascending motor weakness is noted along with areflexia in the classic form. Areflexia is a hallmark of GBS. Occasionally, some of the more proximal reflexes still may be elicited during the early phase of the disease. Of clinical value is documenting reflexes in serial exams; the progression from normoreflexia/hyporeflexia to areflexia is consistent with acute features of GBS.

Occasionally, autonomic instability (26%), ataxia (23%), dysesthesias (20%), and cranial nerve findings (35-50%), predominantly facial palsy, are noted.[28] These latter findings are probably more frequent in children than in adults with this syndrome.

Leg weakness (ie, foot drop) is usually noticed first and weakness eventually involves the calves and thighs. Later, respiratory muscles and upper extremities show involvement. Some children may become non-ambulatory. Weakness also may involve the respiratory muscles, and some children need respiratory support during the course of the disease. Mechanical ventilation is used until respiratory muscle function returns.

The autonomic neuropathy involves both the sympathetic and parasympathetic systems. Manifestations include orthostatic hypotension, hypertension, pupillary dysfunction, sweating abnormalities, and sinus tachycardia.

Complications of GBS

The most common serious complications are weakness of the respiratory muscles and autonomic instability. Pneumonia, adult respiratory distress syndrome, septicemia, pressure sores, pulmonary embolus, ileus, constipation, gastritis and dysesthesias are also important potential complications. Nephropathy has been reported in pediatric patients.[29]

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Clinical Summary

Features that would put the diagnosis in doubt include (1) marked persistent weakness, (2) bowel and bladder dysfunction at onset, (3) persistent bladder or bowel dysfunction, (4) mononuclear leukocytosis in the cerebrospinal fluid (>50 cells/µL), and (5) a sharp sensory level

Features that rule out the diagnosis include (1) a current history of hexacarbon abuse; (2) abnormal porphyria metabolism; (3) recent diphtheria infection; and (4) evidence of polio, botulism, toxic neuropathy, tic paralysis, or organophosphate poisoning.

Features required for diagnosis are (1) progressive weakness of more than one extremity, (2) hyporeflexia or areflexia, (3) elevated cerebrospinal fluid protein (>45 mg/dL) after 1 week following onset of symptoms, and (4) slow conduction velocity or prolonged F wave on electrophysiology testing.

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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, International Parkinson and Movement Disorder Society, American Academy of Cerebral Palsy and Developmental Medicine, American Academy of Neurology, Child Neurology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nitin C Patel, MD, MPH, FAAN Professor of Clinical Pediatrics and Neurology, Southern Illinois University School of Medicine; Private Practice, Columbia Center for Child Neurology

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

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.

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.

Mita N Patel University of Missouri-Columbia School of Medicine

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 Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Acknowledgements

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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