GB

Question 1

Guillain–Barré Syndrome (GBS)

Answer 1

• named after the French physicians Georges Guillain and Jean Alexandre Barré, who described it in 1916.
• An acute polyneuropathy affecting the peripheral nervous system
• May be an autoimmune attack on both the Schwann cells and peripheral and cranial nerves by circulating antibodies

Question 2

GBS Onset

Answer 2

• acute – develops rapidly
• often follows the flu or respiratory infection
• may be idiopathic
• Rare (1-2 cases per 100,000 people annually), but is the most common cause of acute non-trauma-related paralysis.

Question 3

Characteristics

Answer 3

• characterized by symmetrical weakness that usually affects the lower limbs first, and rapidly progresses in an ascending fashion.
• Pts generally notice weakness in their legs, manifesting as “rubbery legs” or legs that tend to buckle, with or without numbness or tingling
• As the weakness progresses upward, usually over periods of hours to days, the arms & facial muscles also become affected.

Question 4

Signs and Symptoms

Answer 4

• cranial nerves may be affected
• Sensory loss, if present, usually takes the form of loss of proprioception
• Pain is a common symptom, presenting as deep aching pain, usually in the weakened muscles
• In severe cases loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension, cardiac arrhythmias

Question 5

The cranial nerves may be affected, leading to…

Answer 5

1. Oropharyngeal dysphagia (drooling, or difficulty swallowing and/or maintaining an open airway)
2. Respiratory complications
   - Can cause life-threatening complications, in particular if the respiratory muscles are affected or if the ANS is involved
   - Most pts require hospitalization & about 30% require ventilator assistance
3. Facial weakness is also common

Question 6

Disease Course

Answer 6

• Maximal onset in less than 4 weeks (often in a few days)
• Static phase (plateau of 2-4 weeks)
• Recovery takes months to years [12 months – 1 year]
• Recurs in 10% of cases

Question 7

Age and Gender

Answer 7

• Can occur at any age

- More common in men than women

Question 8

Stats

Answer 8

• 5%: relatively low mortality rate
• At 6 months, 85% are ambulatory
• At 1 year, 20% remain significantly handicapped by weakness
• At 2 years, 8% have not achieved full recovery

Question 9

Poorer prognosis with…

Answer 9

• onset at an older age
• extended time before recovery begins
• need for artificial respiration

Question 10

Diagnosis & Intervention

Answer 10

Diagnosis:

• nerve conduction studies
• Studies of the cerebrospinal fluid

Intervention:

• Supportive medical care
• Plasmaphoresis
• Intravenous immunoglobulins

Question 11

Plasmaphoresis

Answer 11

filtering the blood plasma to remove circulating antibodies responsible for the destruction of the Schwann cells and peripheral and cranial nerves