What is Guillain-Barre Syndrome (GBS)?
Guillain-Barre Syndrome is a rare condition where the body’s immune system attacks the nerve tissue. It is an acute, progressive, autoimmune, inflammatory demyelination of peripheral sensory and motor nerves and nerve roots. GBS is the most common cause of acute non-trauma-related paralysis in the world. It is also the most common cause of acute motor paralysis in children.
What is its prevalence?
The annual incidence in the United States is 1.65 to 1.79 per 100,000 persons.1 Males are one and a half times more likely to be affected than females. No evidence exists for any racial predilection.
What are the triggering factors?
The exact cause is unknown. Two-thirds of patients report symptoms of viral or bacterial infections like Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumonia in the preceding 6 weeks. Vaccination against the flu, Rabies, meningitis is also the documented precipitating factors. It can also be seen in patients with lymphoma, HIV, SLE.
What are its subtypes?
There are 3 subtypes of Guillain-Barre syndrome:
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): It is the most common form in the U.S. the most common sign of AIDP is muscle weakness that starts in the lower part of the body and spreads upwards.
- Miller Fisher syndrome (MFS) in which the paralysis starts in the eyes. It is also associated with an unsteady gait. It occurs in about 5 percent of people with GBS in the U.S. but is more common in Asia.
- Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) which is less common in the U.S.
When to see a doctor?
The symptoms of Guillain-Barre syndrome are pain, progressive muscle weakness in the legs spreading to the upper body, tingling sensations, blood pressure instability, paresthesia (abnormal sensations in the skin), and muscle pain. There are also symptoms of muscle spasms, difficulty in eye movement, facial movement, speaking, chewing, or swallowing.
Weakness and tingling sensation in the fingers, toes, ankles, or wrists are usually the first symptoms. These sensations can quickly spread, eventually paralyzing the whole body. There is no history of fever at the onset of weakness.
What does a doctor see on physical examination?
Ascending motor weakness is noted along with areflexia is the classic form. Areflexia is a hallmark of GBS. Some of the proximal reflexes may still be elicited during the early phase of the disease. Documentation of the reflexes in serial examinations is of clinical value. Occasionally there may be autonomic instability (26%), ataxia (23%), dysesthesias (20%), cranial nerve findings (35-50%) predominantly facial nerve palsy are noted. Later, respiratory muscles and upper extremities show involvement.
Autonomic neuropathy manifests as orthostatic hypotension or hypertension, pupillary dysfunction, sweating abnormalities, and sinus tachycardia.
How does the doctor confirm the diagnosis?
After taking the history of symptoms and physical examination, certain tests can be done to confirm the diagnosis. These tests are:
- Nerve conduction studies – electrodes are taped to the skin above the nerves under study. It shows a slowing or possible blockage of conduction
- Spinal tap – a small amount of fluid is withdrawn from the spinal canal in the lower back. It shows increased protein levels but a normal white blood cell count.
- Electromyography – thin needle electrodes are inserted into the muscles which the doctor wants to study. The electrodes measure nerve activity in the muscles.
- Electrocardiogram, and
- Pulmonary function test.
What are the treatment options available?
Following treatment options can speed up recovery and reduce the severity of illness:
- Plasmapheresis – This treatment includes the exchanging of the plasma of the patient with the recovered patient or with that of normal people. This method improves short-term and long-term outcomes in the patients, as per various studies.
- High dose intravenous Immunoglobulin therapy.
- Mechanical ventilation may be required in severe cases until the respiratory muscle function returns to normal.
- Pain control with non-steroidal anti-inflammatory drugs, carbamazepine, or gabapentin.
- Supportive care – monitoring for respiratory and autonomic complications, prevention of venous thrombosis, skin breakdown, and deconditioning.
What can happen if I have GBS?2
People with Guillain-Barre Syndrome may develop complications like:
- The weakness of the respiratory muscles
- Cardiac arrhythmias
- Residual numbness or other sensations
- Pressure sores
- Bowel and bladder problems
- Relapse: occurs in about 2-5% of cases
What is the disease course when it is left untreated?
Approximately 80% of the patients have a complete recovery. 3% of patients with GBS unfortunately may die. Neurologic problems persist in about 20% of patients, and one-half of these patients are severely disabled.1
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- AD W, G D. Guillain-Barré syndrome [Internet]. PubMed. 2021 [cited 1 March 2021]. Available from: https://pubmed.ncbi.nlm.nih.gov/23418763/#:~:text=Guillain%2DBarr%C3%A9%20syndrome%20consists%20of,to%201.79%20per%20100%2C000%20persons.
- Guillain-Barre syndrome – Symptoms and causes [Internet]. Mayo Clinic. 2021 [cited 1 March 2021]. Available from: https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793#:~:text=Guillain%2DBarre%20(gee%2DYAH,eventually%20paralyzing%20your%20whole%20body.