GUILLAIN-BARR SYNDROME

An autoimmune attack of the peripheral nerve myelin. The result is acute, rapid
segmental demyelination of peripheral nerves and cranial nerves, producing
ascending weakness with dyskinesia (inability to execute voluntary movements),
hyporeflexia, and paresthesias (numbness).

Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae,

H. influenza, and HIV are the most common infectious agents that are associated with the
development of Guillain-Barr syndrome
In 66% of cases, there is a predisposing event, most often a respiratory or gastrointestinal
infection, although vaccination, pregnancy and surgery have also been identified as antecedent
events (Bella & Chad, 1998).
Most often a viral infection is also the antecedent event (Palmieri, 2005). The antecedent
event usually occurs 2 weeks before symptoms begin. Weakness usually begins in the legs and
progresses upward for about 1 month. Maximum weakness varies but usually includes
neuromuscular respiratory failure and bulbar weakness. The duration of the symptoms is
variable: complete functional recovery may take up to 2 years (Hickey, 2003). Any residual
symptoms are permanent and reflect axonal damage from demyelination.
The annual incidence of Guillain-Barr is 1 to 2 cases per 100,000, and it is more
frequent in males between 16-25 years of age between 45 and 60 years of age (Palmieri, 2005).
Most indicate that 60-75% of patient recovers completely. Residual deficits of varying degree
occur in 20-25% of patients. Residual deficits are most likely in patients with rapid disease
progression, those who require mechanical ventilation, or those 60 years of age or older. Death
occurs in 5% of cases, resulting from respiratory failure, autonomic dysfunction, sepsis, or
pulmonary emboli (Mazzoni, et al., 2006).
PATHOPHYSIOLOGY
Myelin is a complex substance that covers nerves, providing insulation and speeding the
conduction of impulses from the cell body to the dendrites. The cell that produces myelin in the
peripheral nervous system is the Schwann cell. In Guillain-Barr, the Schwann cell is spared,
allowing for remyelination in the recovery phase of the disease.
Guillain-Barr is the result of cell-mediated and humoral immune attack on peripheral
nerve myelin proteins that causes inflammatory demyelination. The best accepted theory of cause
is molecular mimicry, in which an infectious organism contains an amino acid that mimics the
peripheral nerve myelin protein. The immune system cannot distinguish between the two
proteins and attacks and destroys peripheral nerve myelin. The exact location of the immune
attack within the peripheral nervous system is the ganglioside GM1b. With the autoimmune
attack there is an influx of macrophages and other immune-mediated agents that attack myelin,
cause inflammation and destruction, interruption of nerve conduction and axonal loss. (Ho,
Thakur, Gorson, et.al., 2008; Palmieri, 2005)

CLINICAL MANIFESTATIONS
Guillain-Barr syndrome typically begins with muscle weakness and diminished reflexes
of the lower extremities. Hyporeflexia and weakness progress and may result in terraplegia.
Demyelination of the nerves that innervate the diaphragm and intercostal muscles results in
neuromuscular respiratory failure. Sensory symptoms include paresthesias of the hands and feet
and pain related to the demyelination of sensory fibers.
The antecedent event usually occurs 2 weeks before the symptom begin. Weakness
usually begins in the legs and progresses upward. Maximum weakness, the plateau, varies in
length but usually includes neuromuscular respiratory failure and bulbar weakness. The duration
of the symptoms is variable; complete functional recovery may take up to 2 years. Any residual
symptoms are permanent and reflect axonal damage from demyelination.
Cranial nerve demyelination can result in a variety of clinical manifestations. Optic nerve
demyelination may result in blindness. Bulbar muscle weakness related to demyelination of the
glossopharyngeal and vagus nerves results in an inability to swallow or clear secretions. Vagus
nerve demyelination results in autonomic dysfunction, manifested by instability of the cardiovascular system. The presentation is variable and may include tachycardia, bradycardia,
hypertension, or orthostatic hypotension. The symptoms of autonomic dysfunction occur and
resolve rapidly. Guillain-Barr does not affect cognitive function or level of consciousness.
Although the classic clinical features include areflexia and ascending weakness, variation
in presentation occurs. There may be a sensory presentation, with progressive sensory symptoms,
an atypical axonal destruction, and the Miller-Fisher variant, which includes paralysis of the
ocular muscles, ataxia, and areflexia (Iggulden, 2006).
ASSESSMENT AND DIAGNOSTIC FINDINGS
The patient presents with symmetric weakness, diminished re- flexes, and upward
progression of motor weakness. A history of a viral illness in the previous few weeks suggests
the diagnosis. Changes in vital capacity and negative inspiratory force are assessed to identify
impending neuromuscular respiratory failure. Serum laboratory tests are not useful in the
diagnosis. However, elevated protein levels are detected in CSF evaluation, without an increase
in other cells. Evoked potential studies demonstrate a progressive loss of nerve conduction
velocity (Bella & Chad, 1999).
MEDICAL MANAGEMENT
Because of the possibility of rapid progression and neuromuscular respiratory failure,
Guillain-Barr syndrome is a medical emergency, requiring intensive care unit management.
Careful assessment of changes in motor weakness and respiratory function alert the clinician to
the physical and respiratory needs of the patient. Respiratory therapy or mechanical ventilation
may be necessary to support pulmonary function and adequate oxygenation. Some clinicians
recommend elective intubation before the onset of extreme respiratory muscle fatigue. Emergent
intubation may result in autonomic dysfunction. (Mazzoni et al., 2006). Mechanical ventilation
may be required for an extended period. The patient is weaned from mechanical ventilation when
the respiratory muscles can again support spontaneous respiration and maintain adequate tissue

oxygenation.
Other interventions are aimed at preventing the complications of immobility. These may
include the use of anticoagulant agents and anti-embolism stockings/thigh-high elastic
compression stockings or sequential compression boots to prevent thrombosis and pulmonary
emboli.
Plasmapheresis and IVIG are used to directly affect the peripheral nerve myelin antibody
level (Mazzoni, et. al., 2006). Both therapies decrease circulating antibody levels and reduce the
amount of time the patient is immobilized and dependent on mechanical ventilation. Studies
indicate that IVIG and plasmapheresis are equally effective in treating Guillain-Barr (Bella &
Chad, 1999; Winer, 2002). However, IVIG is the therapy of choice because it is associated with
fewer side effects.
The cardiovascular risks posed by autonomic dysfunction require continuous ECG
monitoring. Tachycardia and hypertension are treated with short-acting medications such as
alpha-adrenergic blocking agents. Hypotension is managed by increasing the amount of IV fluid
administered. The use of short-acting agents is important because autonomic dysfunction is very
labile. Hypotension is managed by increasing the amount of IV fluid administered.
NURSING DIAGNOSES
Ineffective breathing pattern and impaired gas exchange related to rapidly progressive
weakness and impending respiratory failure
Impaired physical mobility related to paralysis
Imbalanced nutrition, less than body requirements, related to inability to swallow
Impaired verbal communication related to cranial nerve dysfunction
Fear and anxiety related to loss of control and paralysis
NURSING INTERVENTIONS
MAINTAINING RESPIRATORY FUNCTION
Respiratory function can be maximized with incentive spirometry and chest physiotherapy.
Monitoring for changes in vital capacity and negative inspiratory force are key to early
intervention for neuromuscular respiratory failure. Mechanical ventilation is required if the vital
capacity falls, making spontaneous breathing impossible and tissue oxygenation inadequate.
Parameters for determining the appropriate time to begin mechanical ventilation include a
vital capacity of 12 to 15 mL/kg, downward vital capacity trend over 4 to 6 hours, and an
inability to clear secretions (Bella & Chad, 1999). The potential need for mechanical ventilation
should be discussed with the patient and family on admission to provide time for psychological
preparation and decision-making. Intubation and mechanical ventilation will result in less
anxiety if it is initiated on a non-emergent basis to a well-informed patient.
Bulbar weakness that impairs the ability to swallow and clear secretions is another factor in
the development of respiratory failure in the patient with Guillain-Barr. Suctioning may be
needed to maintain a clear airway.
The nurse assesses the blood pressure and heart rate frequently to identify autonomic
dysfunction so that interventions can be initiated quickly if needed. Medications are administered
or a temporary pacemaker is placed for clinically significant bradycardia (Winer, 2002).

ENHANCING PHYSICAL MOBILITY

Nursing interventions to enhance physical mobility and prevent the complications of
immobility are key to the function and survival of these patients. The paralyzed extremities are
supported in functional positions, and passive range-of-motion exercises are performed at least
twice daily.
DVT and pulmonary embolism are threats to the paralyzed patient. Nursing interventions
are aimed at preventing DVT. Range-of-motion exercises, altering positioning, anticoagulation,
thigh-high elastic compression stockings or sequential compression boots, and adequate
hydration will decrease the risk for DVT.
Padding may be placed over bony prominences such as the elbows and heels to reduce the
risk for pressure ulcers. The need for consistent position changes every 2 hours cannot be
overemphasized.
PROVIDING ADEQUATE NUTRITION
Paralytic ileus may result from insufficient parasympathetic activity. In this event, the
nurse administers IV fluids and parenteral nutrition as prescribed and monitors for the return of
bowel sounds. If the patient cannot swallow due to bulbar paralysis (immobility of muscles), a
gastrostomy tube may be placed to administer nutrients. The nurse carefully assesses the return
of the gag reflex and bowel sounds before resuming oral nutrition.
IMPROVING COMMUNICATION
Because of paralysis and ventilator management, the patient cannot talk, laugh, or cry and
thus has no method for communicating needs or expressing emotion. Establishing some form of
communication with picture cards or an eye blink system will provide a means of
communication. Collaboration with the speech therapist may be helpful in developing a
communication mechanism that is most effective for a specific patient.
DECREASING FEAR AND ANXIETY
The patient and family are faced with a sudden, potentially life- threatening disease, and
anxiety and fear are constant themes for them. The impact of disease on the family will depend
on the patients age and role within the family. Referral to a support group may provide
information and support to the patient and family.
The family may feel helpless in caring for the patient. Mechanical ventilation and
monitoring devices may frighten and intimidate them. Family members often want to participate
in physical care; with instruction and support by the nurse, they should be allowed to do so.
In addition to fear, the patient may experience isolation, loneliness, and lack of control.
Nursing interventions that increase the patients sense of control include providing information
about the condition, emphasizing a positive appraisal of coping resources, and teaching
relaxation exercises and distraction techniques.
Diversional activities are encouraged to decrease loneliness and isolation. Encouraging
visitors, engaging visitors or volunteers to read to the patient, listening to music or books on

tape, and watching television are ways to alleviate the patients sense of isolation.