GUILLAIN-BARRÉ SYNDROME
Guillain-Barré syndrome
is an autoimmune attack of the periph-eral nerve myelin. The result is acute,
rapid segmental demyeli-nation of peripheral nerves and some cranial nerves,
producing ascending weakness with dyskinesia
(inability to execute volun-tary movements), hyporeflexia, and paresthesias (numbness). In 66% of
cases, there is a predisposing event, most often a respira-tory or
gastrointestinal infection, although vaccination, pregnancy, and
surgery have also been identified as antecedent events (Bella Chad, 1998).
Infection with Campylobacter jejuni
(a relatively common gastrointestinal bacterial pathogen) precedes
Guillain-Barreé syndrome in a few cases (Ho & Griffin, 1999; Lindenbaum,
Kissel & Mendel, 2001).
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 in-cludes 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
de-myelination.
The annual incidence of Guillain-Barré is 0.6 to 1.9
cases per 100,000. Eighty-five percent of patients recover with minimal
residual symptoms. Severe residual deficits occur in up to 10% 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 3% to 8% of cases, resulting from respiratory failure,
autonomic dysfunction, sepsis, or pulmonary emboli (Bella & Chad, 1998).
Myelin is a complex substance that covers nerves,
providing in-sulation and speeding the conduction of impulses from the cell
body to the dendrites. The cell that produces myelin in the pe-ripheral nervous
system is the Schwann cell. In Guillain-Barré the Schwann cell is spared,
allowing for remyelination in the recovery phase of the disease.
Guillain-Barré is the
result of a cell-mediated immune attack on peripheral nerve myelin proteins (Ho
& Griffin, 1999). The best-accepted theory is that 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. Studies indicate that an exact location
within the peripheral nervous system, the ganglioside GM1b, is the most likely
target of the immune attack (Yuki, Ang, Koga et al., 2000). With the autoimmune
attack there is an influx of macrophages and other immune-mediated agents that
attack myelin, cause inflammation and destruction, and leave the axon unable to
support nerve conduction.
Classic Guillain-Barré
begins with muscle weakness and dimin-ished reflexes of the lower extremities.
Hyporeflexia and weakness progress and may result in quadriplegia.
Demyelination of the nerves that innervate the diaphragm and intercostal
muscles re-sults in neuromuscular respiratory failure. Twenty-five percent of
patients will require mechanical ventilation within 18 days of symptom onset
(Bella & Chad, 1998). Sensory symptoms include paresthesias of the hands
and feet and pain related to the de-myelination of sensory fibers.
Cranial nerve demyelination can result in a variety of
clinical manifestations. Optic nerve demyelination may result in blind-ness.
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 cardio-vascular system. The presentation is variable and may include
tachycardia, bradycardia, hypertension, or orthostatic hypoten-sion. The
symptoms of autonomic dysfunction occur and resolve rapidly. Guillain-Barré
does not affect cognitive function or level of consciousness.
While the classic
clinical features include areflexia and as-cending 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
(Ho & Griffin, 1999).
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 as-sessed 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).
Because of the
possibility of rapid progression and neuromuscular respiratory failure, Guillain-Barré
is a medical emergency, re-quiring 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. Mechanical ventilation may be required for
an extended period. The patient is weaned from mechanical ventilation when the
res-piratory 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 thigh-high elastic compression stockings or
sequential com-pression boots to prevent thrombosis and pulmonary emboli.
Plasmapheresis and IVIG
are used to directly affect the pe-ripheral nerve myelin antibody level. Both
therapies decrease cir-culating 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-Barré (Bella & Chad, 1999; Winer, 2002).
The cardiovascular risks
posed by autonomic dysfunction re-quire 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.
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