Guillain-Barre Syndrome

Limb wasting in Guillain-Barre syndrome– A rapidly progressive, acute inflammatory demyelinating polyneuropathy characterized by muscle weakness and paralysis of the extremities and possible respiratory paralysis with abnormal sensation and loss of reflexes.

Causes and Incidence

The cause is unknown, but Guillain-Barre syndrome (GBS) is hypothesized to be an autoimmune disorder involving sensitization of peripheral nerve myelin. It is thought to be connected to a previous nonspecific infection and has been associated with inoculation for the swine flu. The incidence of GBS in the United States is 1.7 per 100,000 individuals, and the disorder occurs across age and gender lines.

Disease Process

Mononuclear cells infiltrate the peripheral nervous system and set up an inflammatory response in the blood vessels of the cranial and spinal nerves. Demyelination of the peripheral nerves results, causing muscle weakness that begins in the lower extremities and ascends through the body in a symmetric fashion. Respiratory paralysis and facial weakness occur in 30% to 40% of cases. In some cases axonal destruction can cause atrophy in distal muscles and permanent neurologic impairment.

Symptoms

The first sign is symmetric muscle weakness in the distal extremities accompanied by paresthesia. This weakness spreads upward to the arms and trunk and then to the face. This ascension usually peaks about 2 weeks after onset. Deep tendon reflexes are absent. Difficulty chewing, swallowing, and speaking may occur, and respiratory paralysis may develop. Bladder atony, postural hypotension, tachycardia, and heart block may be seen. Deep, aching muscle pain is also common.

Potential Complications

About 5% of affected individuals die of respiratory failure. Another 10% have permanent residual neurologic deficits. About 90% of survivors make a full recovery, but the recovery time may be as long as 3 years.

Diagnostic Tests

The diagnosis is based on the clinical presentation and cerebrospinal fluid samples, which show an increase in protein without an increase in lymphocyte count. Electromyography produces abnormal nerve conductionresults.

Treatments

Surgery
Tracheostomy to provide ventilation in the event of respiratory failure.

Drugs
Immunoglobulin given IV to counteract neurologic defect; narcotic analgesics for pain; prophylactic antiinfectives

Corticosteroids are contraindicated because they worsen the ultimate outcome.

General
Plasma exchange to speed recovery of neurologic deficit; respiratory monitoring and mechanical ventilation for respiratory paralysis; cardiac monitoring for sinus tachycardia, bradyarrhythmia; communication systems if ventilator is used or with facial paralysis; passive range-of-motion exercises; turning to prevent contracture and skin breakdown; rehabilitation to aid neurologic recovery; counseling and support of individual and family for long-term adaptation.