Guillain Barre Syndrome

Guillain - Barre syndrome is an autoimmune condition that leads to ascending symmetric weakness or paralysis. This is a relatively rare disorder with estimates of one or two cases per 100,000 people per year.

Most cases of Guillain Barre Syndrome are precipitated by an infectious illness. Approximately 2/3rds of people afflicted with the condition experience a gastroenteritis or respiratory tract infection in the weeks preceding the illness. Thirty percent of cases have been attributed to a Campylobacter Jejuni infection. It is theorized that the mechanism is related to molecular mimicry. In responding to the Campylobacter Jejuni infection, the body creates antibodies that also respond to antigens on the surface of the body’s own myelin sheath. The antibodies attack the mylein sheath leading to weakness, and if untreated, paralysis.

• Symmetric weakness or paralysis of extremities (usually lower extremity before upper extremity)
• Depressed or absent reflexes
• Paresthesias and occasionally pain in the involved extremities
• Respiratory failure in cases involving the diaphragm
• Cranial nerve involvement
• Autonomic Dysfunction

Physical Examination

• Symmetric weakness of the lower extremities
• Depressed or absent deep tendon reflexes

Diagnostic Studies

• Lumbar Puncture: CSF will show elevated protein and normal WBC count
• Some patients will have a normal lumbar puncture.

Electrodiagnostic Studies

• Nerve conduction studies
• Needle electromyography

MRI

• Enhancement of cauda equina and nerve roots

1. Ensure ABCs. Respiratory failure secondary to diaphragm involvement is the biggest concern with Guillain Barre Syndrome. Ensure continuous oxygen saturation monitoring. Obtain a Forced Vital Capacity (FVC) and Negative Inspiratory Force (NIF) measurement to determine the amount of diaphragm involvement. An FVC value <15mL / kg and NIF <60cmH20 are considered markers of severe involvement. Consider endotracheal intubation if there are signs of respiratory involvement or impending respiratory failure.

2. In consultation with neurology, consider the need for IVIG or plasmapheresis.

3. Admit the patient for continued monitoring. Ideally the patient should be admitted to a Neurology Step Down Unit or Neurologic ICU for further monitoring.