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Guillain-Barré
syndrome is an acute, usually rapidly progressive inflammatory polyneuropathy
characterized by muscular weakness and mild distal sensory loss.
Cause is thought to be autoimmune. Diagnosis is clinical. Treatment includes
plasmapheresis, γ-globulin, and,
for severe cases, mechanical ventilation.
Guillain-Barré syndrome is the most common acquired inflammatory neuropathy. Although the cause is not fully understood, it is thought to be autoimmune. There are several variants. In some, demyelination predominates; others affect the axon.
In about 2⁄3 of patients, the syndrome begins 5 days to 3 wk after a banal infectious disorder, surgery, or vaccination. Infection is the trigger in > 50% of patients; common pathogens include Campylobacter
jejuni
, enteric viruses, herpesviruses (including cytomegalovirus and Epstein-Barr virus), and Mycoplasma sp. A cluster of cases followed the swine flu vaccination program in 1975.
Symptoms and Signs
Flaccid weakness predominates in most patients; it is always more prominent than sensory abnormalities and may be most prominent proximally. Relatively symmetric weakness with paresthesias usually begins in the legs and progresses to the arms, but it occasionally begins in the arms or head. In 90% of patients, weakness is maximal at 3 wk. Deep tendon reflexes are lost. Sphincters are usually spared. Facial and oropharyngeal muscles are weak in > 50% of patients with severe disease. Dehydration and undernutrition may result. Respiratory paralysis severe enough to require endotracheal intubation and mechanical ventilation occurs in 5 to 10%.
A few patients (possibly with a variant form) have significant, life-threatening autonomic dysfunction causing BP fluctuations, inappropriate ADH secretion, cardiac arrhythmias, GI stasis, urinary retention, and pupillary changes. An unusual variant (Fisher variant) may cause only ophthalmoparesis, ataxia, and areflexia.
Diagnosis
Diagnosis is primarily clinical. Similar acute weakness can result from myasthenia gravis, botulism, poliomyelitis (mainly outside the US), tick paralysis, West Nile virus infection, and metabolic neuropathies, but these disorders can usually be distinguished as follows:
Tests for infectious disorders and immune dysfunction, including tests for hepatitis and HIV and serum protein electrophoresis, are done.
If Guillain-Barré syndrome is suspected, patients should be admitted to a hospital for electrodiagnostic testing, CSF analysis, and monitoring by measuring forced vital capacity every 6 to 8 h. Initial electrodiagnostic testing detects slow nerve conduction velocities and evidence of segmental demyelination in 2/3 of patients; however, normal results do not exclude the diagnosis and should not delay treatment.
CSF analysis may detect albuminocytologic dissociation (increased protein but normal WBC count), but it may not appear for up to 1 wk and does not develop in 10% of patients.
Prognosis
This syndrome is fatal in < 2%. Most patients improve considerably over a period of months, but about 30% of adults and even more children have some residual weakness at 3 yr. Patients with residual defects may require retraining, orthopedic appliances, or surgery.
After initial improvement, 3 to 10% of patients develop chronic inflammatory demyelinating polyneuropathy (CIDP—see below).
Treatment
Guillain-Barré
syndrome is a medical emergency, requiring constant monitoring and support of vital functions, typically in an ICU. Forced vital capacity should be measured frequently so that respiration can be assisted if necessary; if vital capacity is < 15 mL/kg, endotracheal intubation is indicated. Inability to lift the head off the pillow by flexing the neck is another danger sign; it frequently develops simultaneously with phrenic nerve (diaphragm) weakness.
If oral fluid intake is difficult, IV fluids are given as needed to maintain a urine volume of at least 1 to 1.5 L/day. Extremities should be protected from trauma and from the pressure of bed rest. Heat therapy helps relieve pain, making early physical therapy possible. Immobilization, which may cause ankylosis and contractures, should be avoided. Passive full-range joint movement should be started immediately, and active exercises should be initiated when acute symptoms subside. Heparin 5000 units sc bid helps prevent deep vein thrombosis in bedbound patients.
Given early, immune globulin (γ-globulin) 400 mg/kg IV once/day for 5 consecutive days is the treatment of choice; it has some benefit up to 1 mo from disease onset.
Plasmapheresis (see Transfusion Medicine: Plasmapheresis) helps when done early in the syndrome; it is used if γ-globulin is ineffective. Plasmapheresis is relatively safe, shortens the disease course and hospital stay, and reduces mortality risk and incidence of permanent paralysis. Plasmapheresis removes any previously administered γ-globulin, negating its benefits.
Corticosteroids do not improve the outcome and should not be used.
Chronic
Inflammatory Demyelinating Polyneuropathy
(Chronic Acquired Demyelinating Polyneuropathy; Chronic Relapsing
Polyneuropathy)
Chronic
inflammatory demyelinating polyneuropathy is an immune-mediated
polyneuropathy characterized by symmetric weakness of proximal and
distal muscles and by progression continuing > 2
mo.
Progression for > 2 mo differentiates chronic inflammatory demyelinating polyneuropathy (CIDP) from Guillain-Barré syndrome, which is monophasic and self-limited. CIDP develops in 3 to 10% of patients with Guillain-Barré syndrome.
Diagnosis
Testing includes CSF analysis and electrodiagnostic tests. Results are similar to those in Guillain-Barré syndrome: CSF protein level is increased, and electrodiagnostic testing detects demyelination. Nerve biopsy, which can detect demyelination, is seldom needed.
Treatment
Unlike Guillain-Barré syndrome, CIDP responds to corticosteroids. Azathioprine may be added to decrease corticosteroid dependence. However, in severe or rapidly progressive cases, plasmapheresis or IV immune globulin may be preferred. Immunosuppressants (eg, azathioprine ) may be helpful. Treatment may be needed for a long time.
Last full review/revision February 2008 by Michael Rubin, MD
Content last modified February 2008
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