Guillain Barré Syndrome
Guillain Barré Syndrome: Excerpt from The 5-Minute Pediatric Consult
James Boyd, MD
Guillain Barré Syndrome - BASICS
Guillain Barré Syndrome - description
Guillain-Barré syndrome (GBS) is an acute disorder of the peripheral nerves–an inflammatory polyradiculoneuropathy. It causes progressive weakness in the limbs, face, and respiratory muscles. Autonomic and sensory disturbance occur with loss of sensation. Neurologic deficits peak by 4 weeks or sooner.
Guillain Barré Syndrome - epidemiology
Guillain Barré Syndrome - incidence
Overall yearly incidence rate of 0.6–1.9 cases per 100,000. Of 95 reported pediatric GBS patients, 45 were age 1–5 years, 36 were age 6–10 years, and 14 were age 11–15 years.
Guillain Barré Syndrome - risk-factors
Guillain Barré Syndrome - genetics
Particular subtypes of GBS are more common among certain human leukocyte antigen (HLA) types. No data indicate an increase in GBS among 1st-order relatives.
Guillain Barré Syndrome - pathophysiology
Inflammatory cell-mediated and humeral-mediated immune mechanisms play a role in segmental demyelination on nerve biopsy; lymphocytes and macrophages participate in myelin destruction. Axonal variants of GBS feature axonal degeneration without demyelination. Circulating anti-ganglioside antibodies (e.g., GM1, GM2, GQ1B) found in particular subtypes suggest a molecular mimicry mechanism stimulated by infection.
Guillain Barré Syndrome - etiology
- Follows viral infection in >50% of cases. Cytomegalovirus, Epstein-Barr virus, varicella-zoster virus, acute HIV infection, others
- Also associated with bacterial infection (especially Campylobacter jejuni), surgery, and vaccination
- Tetanus toxoid is the only vaccination in common use with a clear link to GBS. Often, no precipitating event can be identified.
Guillain Barré Syndrome - associated-conditions
- GBS is seen in a higher-than-expected rate in patients with sarcoidosis, systemic lupus erythematosus, lymphoma, HIV infection, Lyme disease, and solid tumors.
- Muscle atrophy, joint contractures, and pressure ulcers
Guillain Barré Syndrome - DIAGNOSIS
Guillain Barré Syndrome - signs & symptoms
Guillain Barré Syndrome - history
- GBS has a variety of clinical presentations so that index of suspicion is critical. Typical features are progressive motor weakness and areflexia, often following distal sensory changes. Common presentations include decreased ambulation (or crawling in toddlers), facial weakness, back pain, or sensory changes in the extremities.
- After respiratory status has been stabilized, address autonomic dysfunction and pain. Close monitoring required for dysautonomic symptoms: Arrhythmias, BP lability/orthostasis, ileus, urinary retention.
- Most patients 1st note leg weakness or gait instability that progresses over days to weeks.
- Paresthesias and pain typically occur in a stocking/glove distribution frequently early in the course.
- 2/3 of patients will report symptoms of an infection 2–3 weeks earlier. Consider polio if fever is present at symptom onset.
- Weakness may lead to respiratory paralysis in 20–30% of children with GBS.
Guillain Barré Syndrome - physical exam
- Weakness and sensory changes:
- Typical signs of GBS, classically with distal greater than proximal involvement. A proximal predominance of symptoms does not preclude the diagnosis.
- Typically, deep tendon reflexes are lost within 1 week.
- Respiratory difficulty, measured as decreased vital capacity, maximum inspiratory (PiMax) and expiratory (PeMax) pressures:
- Respiratory failure leads to intubation in up to 25% of patients. Bulbar weakness and poor airway protection can also necessitate intubation. Impending respiratory failure can often be unpredictable, and blood gas determination is not a useful indicator of neuromuscular respiratory failure until intubation is imminent. If close monitoring of vital capacity, inspiratory, or expiratory pressures suggest >30% decline in 24 hours, monitor patient in ICU.
- Bilateral facial weakness occurs in ≤50% of cases.
- Neonates and infants may (rarely) present as floppy infants.
Guillain Barré Syndrome - tests
Guillain Barré Syndrome - lab
- In atypical cases, consider heavy metal screen, HIV titer, Lyme titer, porphyria screen, acetylcholine receptor antibodies (myasthenia):
- See “Differential Diagnosis”
- IgA level should be considered if the child has a history of frequent pulmonary infections: IgA deficiency would be a contraindication for intravenous Ig therapy, owing to a risk of anaphylaxis.
Guillain Barré Syndrome - imaging
MRI of the spine (with gadolinium enhancement): Consider MRI for spinal cord compression syndrome in a child presenting with paraparesis. Spinal nerve root enhancement on MRI can support the diagnosis of GBS.
Guillain Barré Syndrome - diag-proced-surgery
- Electrodiagnosis:
- Nerve conduction studies (NCS) and electromyography (EMG) can confirm diagnosis of GBS and are helpful when clinical or cerebrospinal fluid findings are ambiguous. NCS and EMG are abnormal in 50% of patients in the 1st 2 weeks and in 85% of patients afterwards.
- Initially, needle EMG may be normal. Consider serial studies if initially nondiagnostic, high clinical suspicion
- Lumbar puncture: Elevated levels of cerebrospinal fluid protein after the 1st week of symptoms. Minimal pleocytosis (<10 leukocytes/mm3), largely mononuclear leukocytes, may occur.
Guillain Barré Syndrome - differencial diagnosis
- Myasthenia gravis
- Botulism
- Intoxication (e.g., heavy metals, organophosphates)
- Myopathy/myositis
- Poliomyelitis and other acute (i.e., viral) motor neuron disease
- Acute cerebellar ataxia
- Transverse myelitis
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
- Vasculitic neuropathy
- Diphtheric neuropathy (rare)
- Porphyric neuropathy
- Locked-in state
- Conversion, psychogenic weakness, astasia/abasia
- Initially, gait instability may mistakenly be interpreted as having a psychogenic source.
- Reflexes may be preserved in early stages of illness.
- Proximal symptoms may predominate early on.
- Check for reflexes in patients with bilateral Bell palsy. Close observation for further development of symptoms or signs of GBS
Guillain Barré Syndrome - TREATMENT
- Respiratory failure may occur quickly.
- Treat hypertension cautiously; catastrophic refractory hypotension may ensue.
Guillain Barré Syndrome - initial-stabilization
Key elements center around respiratory management and deciding on hospitalization to monitor/treat progressive symptoms including heart block, hypotension, urinary retention, and neuropathic pain.
Guillain Barré Syndrome - general measures
Guillain Barré Syndrome - nursing
Particular attention to preventing skin breakdown, contractures, venous thrombosis, and secondary compressive neuropathies
Guillain Barré Syndrome - special-therapy
A combination of supportive therapy and immunotherapy is the mainstay of treatment for patients with GBS:
- Regular monitoring of vital capacity; strongly consider intubation if vital capacity reaches <50% of normal.
- IV immunoglobulin (IVIg) and plasmapheresis have equivalent efficacy as first-line immunotherapy. Combination of the 2 therapies has not proven more effective than either monotherapy alone. Complications and discontinuation of therapy are less common with IVIg.
- Pediatric studies have shown IVIg and plasmapheresis to be well tolerated and efficacious.
- IVIg at 0.4 g/kg (body weight) for 5 consecutive days and initiated in ambulatory patients within 2–4 weeks of symptom onset
- Plasmapheresis requires placement of a central pheresis catheter. Total plasma exchange volume of 200–250 mL/kg divided in 3–5 treatments over 7–14 days. Therapy initiation is recommended within 4 weeks of symptom onset for patients who cannot walk and 2 weeks for patients who can.
- Corticosteroids have not been shown to be helpful and are not recommended.
- Pain from nerve root inflammation is common in GBS and should be treated aggressively.
Guillain Barré Syndrome - phys-therapy
Avoid contractures with lower extremity splinting and early passive range of motion. Aggressive physical and occupational therapy are essential for good outcomes.
Guillain Barré Syndrome - FOLLOW UP
- Improvement typically begins 2–3 weeks after onset of symptoms, up to 2 months in some patients.
- Improvement continues for up to 2 years.
Guillain Barré Syndrome - disposition
Guillain Barré Syndrome - admission criteria
Admit patients with symptoms progressing over hours to days, with any respiratory or bulbar complaints, or who are nonambulatory.
Guillain Barré Syndrome - discharge-criteria
- Completion of immunotherapy
- Stabilization of symptoms.
- Severity of bulbar, respiratory, and autonomic involvement dictates duration of hospital stay. Consider intensive inpatient rehabilitation.
Guillain Barré Syndrome - prognosis
- ~85% have a good recovery; ultimate functional recovery depends on the degree of axonal injury, which can be predicted from electrodiagnostic studies in adults.
- Early prognosticators include the severity of weakness at the disease nadir and fulminant onset.
- Overall prognosis in children is better than in adults.
Guillain Barré Syndrome - complications
- Complications include respiratory failure, BP dysregulation (hypotension and/or hypertension), urinary retention, aspiration, pain syndromes, deep venous thrombosis, infection
- Death from early respiratory failure, autonomic instability, or other complications occurs in 3–6%.
Guillain Barré Syndrome - bibliography
- Abd-Allah SA, Jansen PW, Ashwal S, et al. Intravenous immunoglobulins as therapy for pediatric Guillain-Barré syndrome. J Child Neurol. 1997;12:376–380.
- Hahn AF. Guillain-Barré syndrome. Lancet. 1998;352:635–641.
- Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: Immunotherapy for Guillain-Barré syndrome: Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003;61:736–740.
- Lawn ND, Fletcher DD, Henderson RD. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001;58:893–898.
- Tekgul H, Serdaroglu G, Tutuncuoglu S. Outcome of axonal and demyelinating forms of Guillain-Barré syndrome in children. Pediatr Neurol. 2003;28:295–299.
Parent Internet Information: Guillain-Barré Syndrome Foundation International: http://www.gbsfi.com
Guillain Barré Syndrome - CODES
Guillain Barré Syndrome - icd9
357.0 Acute infective polyneuritis
Guillain Barré Syndrome - FAQ
- Q: Is GBS contagious?
- A: No.
- Q: Will I get GBS again?
- A: Acute relapses occur in 1–5% of patients in large series. Treatment-related fluctuations (worsening after completion of immunotherapy) can occur in CIDP and can be indistinguishable from GBS.
- Q: Do all cases require hospitalization and immunomodulatory treatment?
- A: Some youngsters with mild, nondisabling symptoms may be observed as outpatients (≤10%).
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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