Guillain-Barré Syndrome (GBS)

What is it?

GBS is an “acute, rapidly progressing inflammatory demyelination polyneuropathy (AIDP) of the peripheral nerves and spinal nerve roots” (Hamby, 2017). The immune system attacks part of the peripheral nervous system by mistake. Severity can vary from mild with brief weakness to severe paralysis and inability to breathe requiring mechanical ventilation. Many cases are linked to infection, surgery, or vaccination. There is no known cause or cure. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered a chronic form of GBS.

Types

1. Acute Inflammatory Demyelinating Polyneuropathy (AIDP): The most common type (75-80%) in which the immune response damages the myelin coating and interferes with the transmission of nerve signals.

2. Miller-Fisher Syndrome: rare (5-10%). Characterized by abnormal muscle coordination, weakness/paralysis of eye muscles, absence of tendon reflexes, and ataxia.

3. Pharyngeal-cervical-brachial motor variant: rare (3%). Typically presents with muscle weakness involving the neck flexors, facial/pharyngeal muscles, and upper extremities.

Symptoms

• Symptoms generally present as unexplained sensations (i.e., tingling or pain) that come and go or weakness on both sides of the body. Most experience the greatest weakness within the 1st two weeks after symptom onset. By the 3rd week, 90% of individuals are at their weakest point. The cardinal sign is the progression from distal to proximal motor and sensory impairments. A general list of symptoms include:

  • Difficulty with eye muscles

  • Change in vision

  • Difficulty swallowing, talking, or chewing

  • Pins and needles/numbness and tingling in hands and feet

  • Severe pain

  • Impaired coordination

  • Impaired balance

  • Abnormal heart rate or blood pressure

Phases

• Progressive Phase: lasting from days to 4 weeks

• Plateau Phase: lasts from days to months with little clinical change

• Recovery Phase: phase in which the patient starts to recover

How is it diagnosed?

Several different tests can be used to diagnose GBS. A MRI & CT can be ordered to rule out stroke, a lumbar puncture to analyze cerebrospinal fluid, and/or nerve conduction velocity test

Diagnostic Criteria

• Bilateral limb weakness

• Reduced or lost deep tendon reflexes

• Monophasic illness

• 12hr to 28-day syndrome evolution

• Consistent electrophysiology findings

• CSF analysis revealing normal CSF leukocytes but elevated protein

• Absence of an alternate diagnosis

Prognosis

• Most can recover 80-100% of their baseline function within 200 days. A quarter of patients will require mechanical ventilation. Relapse is possible (5% of cases) and may be considered chronic inflammatory demyelinating polyneuropathy (CIDP). Poor prognostic factors: >50 years old, rapid onset (≤ 7 days), vent dependence, and antecedent infection.

Treatment

• Plasmapheresis: blood cleansing procedure in which the plasma antibodies are removed and replaced with albumin or fresh frozen plasma. This appears to reduce both the severity and duration of GBS.

• Intravenous immunoglobulin (IVIG): high-dose plasma protein replacement therapy in which the patient receives intravenous injections of immunoglobulins. This is thought to lower the levels or overall effectiveness of the antibodies that are attacking the nerves.