Guillain-Barré Syndrome: What you need to know?

France EllysonKuwait, 2014

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GUILLAIN BARRÉ SYNDROME (GBS)

• Post infectious disorder in which the body’s immune system attacks the myelin sheath that surrounds the axons of many peripheral nerves

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PATHOPHYSIOLOGY

• An immune-mediated response triggers destruction of myelin sheath

• Demyelination process is accompanied by edema and inflammation peripheral nerves

• Demyelination of axons result in loss of conduction

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PATHOPHYSIOLOGY

• The inflammation process may lead to various degrees axonal injury

• This signals poorer recovery

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PATHOLOGY

EPIDEMIOLOGY

• Rare disorder: Average yearly incidence of 1-3 cases /100 000 population

• Occurs world wide / Affects all races • Affects and equally♂ ♀

• Persons of all ages are at risk (peak ages 50-74)• Unknown etiology

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CLINICAL PRESENTATION

• Characterized by motor weakness and areflexia• Usually begins in legs and progresses to trunk and

arms• Resp. failure is due to mechanical failure and

fatigue of intercostals and diaphragm• 85% have cranial nerve involvement. • Facial nerve (VII) most frequently affected along

with CN IX, X, XI and XII.• With sensory changes – paresthesia , tingling, pins

and needles, heightened sensitivity to touch or numbness

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CLINICAL PRESENTATION

• About 25 % patients experience pain • Pain is often worse at night and interferes

with sleep• Many patients experience autonomic

dysfunction• Decreased or absent deep tendon reflexes• Vibration and proprioception are typically

affected

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CLINICAL PRESENTATION

• 3 phases:–Acute (Progressive) stage

lasting 3 –4 weeks–Plateau stage : symptoms

remain unchanged for 2 – 4 weeks–Recovery stage

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DIAGNOSTIC CRITERIA

• Patient history– There are approximately 50 differential diagnosis

• CSF analysis– Increase protein (Albumin-cytological dissociation)– Normal cell count

• Electromyography (EMG)– Shows segmental demyelination– Conduction slowing or block (more apparent in the

motor nerves than in sensory nerves)10

MANAGEMENT OF GBS

• No known preventive treatments

• Therapies aim:– Lessen the severity of the illness – Accelerate the recovery

• There are two components to care– Supportive care– Specific therapies

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SUPPORTIVE CARE

–Respiratory failure–Dysautonomia–Venous

Thromboembolism–Pain 12

RESPIRATORY FAILURE

• Most serious and common complication• Occurs in approximately 40% of cases• Secondary to weakness of respiratory muscles

and diaphragm• Vital capacity needs to be measured regularly• Patients will be intubated electively when vital

capacity falls below 20 ml/kg (ICU admission)• Anxiety and insomnia are classic symptoms

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DYSAUTONOMIA

• Occurs to some extent in most patients with GBS

• More acute in those patients with respiratory problems and severe motor deficits

• Dysfunction in the sympathetic /parasympathetic systems:– Hypertension– Orthostatic hypotension – Cardiac arrhythmia

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VENOUS THROMBOEMBOLISM

• 5% PE in patients immobilized ≥ 2 weeks

• Accounts for 28% of the deaths due to GBS

• Preventative treatments:– Anticoagulation, SCDs TED stockings– Passive ROM /early

mobilization15

PAIN

– Commonly presents as lumbar pain.

– Discomfort is secondary to acute pain in nerve roots

– Treat with analgesia– Due to immobilization– Passive range of motion– Pins and needles or burning

pain– Anticonvulsant drugs /

elavil / neurontin / Lyrica/ Cymbalta

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SPECIFIC THERAPIES

• Immunoglobulin (IVIgG) • Plasma exchange (Plasmapheresis or

PLEX)

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IMMUNOGLOBULIN (IVIG)

• IVIG is usually given on 5 sequential days for overall dose of 2 gms/kg of body weight

• Some patients need second course of treatment

• IVIG is derived from fractionated, purified human plasma collected from a large pool of multiple donors

• Mechanism of action is unclear

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IVIGSIDE EFFECTS

• Most side effects are mild and transient• Most frequent: rashes, chills, fever, mild hypotension,

nausea, malaise, headache, mild arthralgias and short term aseptic meningitis

• More serious and rare: fatal anaphylactic shock, stroke, renal impairment, hemolytic anemia and transmission of blood born pathogens

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PLASMA EXCHANGE

• Removal of immunoglobulins and antibodies from the serum by removing the blood from the body, separating cells from the plasma, and replacing the cells in fresh frozen plasma, albumin, or saline

• Most common regime 5 – 10 days of treatments

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PLASMA EXCHANGE SIDE EFFECTS

• Hypotension /dizziness• Fluctuation in

circulatory volumes• Anemia / Hemorrhage • Infection• Hypocalcemia

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REFERENCES

• Barker, E. (2002). Neuroscience Nursing. A Spectrum of Care (2nd ed.). St. Louis: Mosby.

• Hickey, J. V. (2003). The Clinical Practice of Neurological and Neurosurgical Nursing (5th ed.). Philadelphia: Lippincott Williams & Wilkins, Chapter 32

• Info Neuro: Neuro-Patient Resource Centre: www.infoneuro.mcgill.ca • Kirmse,MSN,RN, CNS., J., (2009). The Nurse’s Role in the Administration of

Intravenous Immunoglobulin Therapy. Home Healthcare Nurse. 27(2):104-111. February 2009.

• Ruts, L., van Koningsveld, R. & van Doorn, P.A. (2005). Distinguishing acute onset CIDP from Guillain-Barré syndrome with treatment related fluctuations. Neurology 2005 ;6:138-140.

• Van Doorm,P.A. Ruts, L. &Jacobs, B. (2008). Clinical features, pathogenesis, and treatment of Guillian- Barré syndrome. Lancet Neurol 2008;7:939-950.

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