Guillain-Barre Syndrome
Definition
Autoimmune disease
Affects peripheral nerve myelin
Causes acute, rapid sergmental demyelination of peripheral nerves and some cranial nerves
Produces ascending weaknesses ' dyskinesia (inability to execute voluntary movements)
Pathophysiology
Myelin sheath covers nerves, insulates, speeds conduction of impulses
Myelin produced by Schwann cell
In Guillain-Barre Syndrome Schwann cell spared - mylination later becomes possible.
Cell mediated immune attack on myelin sheath
Inflammation of the myelin ' destruction of the myelin
Clinical Manifestations
Begins with muscle weakness and diminished reflexes of the lower extremities
May lead to quadriplegia
Respiratory muscles may be affected leading to respiratory failure
About 25% of patients may require mechanical ventilation within 18 days of symptom onset.
Causes
An autoimmune reaction following an acute infection.
Not inherited, although genetic factors may make some people susceptible to develop autoimmune conditions
Clinical Manifestations
Sensory symptoms : paraesthesias of the hands and feet and pain related to the demyelination of sensory fibers
Hyporeflexia
Weakness usually begins in the legs and progresses upwards for about one month.
The paralysis progressive and ascending
Respiratory failure and bulbar weakness may occur
Death may occur due to respiratory failure, autonomic dysfunction, sepsis or pulonary emboli
Blindness - optic nerve demyelination
Bulbar muscle weakness - 9th and 10th nerve demyelination
10th nerve involvement - autonomic dysfunction - instability of the cardiovascular system. Leading to tachycardia, bradycardia, hypertension or orthostatic hypotension
Autonomic dysfunction - rapidly resolves Guillain-Barre Syndrome does not affect cognitive function or level of consciousness.
Complete recovery may take upto 2 years
Diagnosis
Symmetric weakness
Diminished reflexes
Upward progression of motor weakness
H/O viral illness in the previous few weeks
All these suggest the diagnosis of Guillain-Barre Syndrome
proteins ? without any change in the other cells
ed potential sutdies demonstrate a progressive loss of nerve conduction velocity
Medical Management
A medical emergency
Intensive care unit management
Ventilatory support may be needed
Prevent complications of immobility
Plasmapheresis - IVIG
Continuous ECG monitoring
For tachycardia and hypertension - beta blockers
Hypotension - IV fluid
Nursing interventions
Maintain respiratory function : incentive spirometer, chest physiotherapy, monitor vital capacity, early intervention and mechanical ventilation if vital capacity is < 12 to 15 ml/Kg for 4 to 6 hours or if patient is not able to clear secretions
BP and heart rate assessed frequently - if autonomic dysfunction - intervene
Enhance physical mobility : extremities that are paralysed splinted in functional position; physiotherapy, prevent bed sore
.
Symptoms
An initial episode of a headache, vomiting, fever and back and limb pain is followed by paralysis, which starts as tingling and numbness followed by increasing weakness. The paralysis is often progressive and ascending (starting with the feet and moving upwards), but the condition may come on suddenly and affect all four limbs at once. The degree of paralysis varies.
There may also be a loss of sensation, sometimes with pain. In about one in four cases the paralysis spreads to the respiratory nerves controlling breathing and the person needs to be put on a ventilator.
Some patients have a similar but longer-lasting illness called chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), once known as chronic Guillain-Barre but now usually regarded as a related condition.
Guillain-Barre syndrome
Guillain-Barre syndrome - also known as acute post-infective polyneuritis - is a disease of the peripheral nervous system, affecting the nerves to the arms, legs, head and trunk, but not the brain or spinal cord.
Treatment Options
Corticosteriods: Steroids have been used to try to increase muscle usage, but no evidence supports this therapy (Visser et al., 1998).
Plasmapheresis (Plasma Exchange): Approximately 200 to 250mL/kg of plasma is exchanged over 7-14 days to filter out possible antibodies and harmful substances in the blood. This treatment has been shown to provide improvement for only a small population of patients (Kennard, Newland, & Ridley, 1982).
Intravenous immunoglobulin: Injections of high dosages of human immunoglobulin (0.4 g/kg per day for 5 days) have been found to be somewhat effective for a small population of patients.
Antibiotics: Possible early treatment with erythromycin may help produce a shorter duration of illness if the patient has campylobacter associated GBS (Goddard, Lastovica, Argent, 1997).
Rehabilitation: Physical therapy to improve muscle tone and strength as well as counseling is the major form of treatment.
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