Guillain-Barré syndrome is an uncommon disease but has been thrust into the spotlight with the Zika virus outbreak. The disease affects about 3 in 100,000 Americans and is more common in adults, particularly in the elderly. However, with the Zika outbreak there is concern that Guillain-Barré syndrome may be more commonly seen as it is a complication of Zika virus infection in adults. Nevertheless, it is an uncommon complication as most people with Zika virus infection do not experience any complications or even symptoms.
What is Guillain-Barré syndrome?
Guillain-Barré syndrome is an uncommon autoimmune disorder where the immune system targets and attacks the nerves. The exact cause is unknown but for some reason the immune system malfunctions and misidentifies normal tissue and attacks it. Guillain-Barré syndrome is not an infection but may be triggered by an infection, such as the Zika virus infection, an upper respiratory tract infection or viral gastroenteritis.
Due to the damage and inflammation to the nerve, Guillain-Barré syndrome usually presents with neurological symptoms such as abnormal sensations (paresthesia), numbness, muscle weakness and even paralysis. Guillain-Barré syndrome is not a single disorder as once thought. There are different forms of it and the symptoms may also vary to some extent between these different types.
The nerves outside of spinal cord and brain (peripheral nerves) are usually affected. Typically the first symptoms of abnormal sensations and weakness start in the feet and legs, and then gradually spread upwards.
Read more on peripheral neuropathy.
Causes of Guillain-Barré Syndrome
The exact cause of Guillain-Barré syndrome is unknown. It tends to appear after an infection and as a result of an immune response against nerve tissue. It appears that the immune system forms antibodies to target the antigens of the invading pathogen. These antibodies cross-react with nerve tissue and then directs the immune system to target this tissue. A number of different infections can trigger this immune-mediated response but infections are not the cause of the Guillain-Barré syndrome.
The immune system attacks the outer covering of the nerves known as the myelin sheath. This sheath is responsible for insulating the nerves. When this sheath is damaged it cannot transmit impulses as normal. As a result there is weakening, numbness or paralysis depending on which sensory (sensation) or motor (muscle) nerves are affected. However, in most cases this is not permanent and there is usually complete recovery. In a minority of cases, the weakening, numbness and paralysis may persist.
Zika Virus and Guillain-Barré syndrome
Guillain-Barré syndrome may be triggered by a host of different infections. The condition has gained significant attention in recent months due to it being associated with Zika virus infection. However, even with Zika virus infection, the onest of Guillain-Barré syndrome is uncommon. The condition is more likely to occur in adults and is rarely seen in children.
Read more on Zika virus outbreak.
Signs and Symptoms
The signs and symptoms of Guillain-Barré syndrome develop within days to weeks after an infection in most cases. However, it may sometimes arise without a person being able to remember a preceding infection. Symptoms arise gradually and peaks within 2 to 4 weeks of the first symptoms appearing. These signs and symptoms include:
- Tingling or prickling (“pins and needles”) in the hands and/or feet.
- Weakness of the limbs, usually starting in the legs and spreading upwards but sometimes starting in the face or arms.
- Problems with moving the eyes and/or face which makes actions like talking, chewing and swallowing difficult.
- Difficulty standing or walking without assistance due to weakness and poor balance. This may be most pronounced when walking up stairs or carrying a heavy object.
- Aching or throbbing pain that can be severe and most commonly felts on the shoulders, back, thighs and buttocks.
- Loss of bladder or bowel control.
While these symptoms are most noticeable, sometimes there may be other serious symptoms that can initially pass by unnoticed. It includes:
- Rapid heart rate with or without palpitations.
- Low or high blood pressure.
- Shortness of breath to overt difficulty breathing.
Symptoms may vary depending on the form of Guillain-Barré syndrome. It can affect muscles anywhere in the body. When the muscles of the heart, blood vessels or breathing muscles are affected that the outcome is most serious. However, most cases of Guillain-Barré syndrome are not serious and death from this condition is rare. Symptoms usually resolve several weeks after the symptoms peak.
Treatment of Guillain-Barré Syndrome
Hospitalization is usually required for Guillain-Barré syndrome until it can be established that the symptoms have reached its peak or are now reversing. Even cases with mild symptoms need to be carefully monitored as serious heart and breathing symptoms can arise and progress relatively suddenly.
Before commencing with treatment, Guillain-Barré syndrome has to be conclusively diagnosed. This can be difficult at times and certain diagnostic investigations may be necessary, such as a lumbar puncture (spinal tap), electromyography and nerve conduction studies.
There is no cure for this condition and treatment focuses on managing the symptoms as well as expediting a complete recovery. Treatment of Guillain-Barré syndrome involves:
- Plasma exchange (plasmapheresis) where the blood plasma is filtered out to remove or reduce the antibodies that direct the immune system to cause nerve damage.
- Immunoglobulin therapy where high doses of antibodies are used to block the existing antibodies that trigger nerve damage in Guillain-Barré syndrome.
Pain medication (analgesics) may also be prescribed to manage pain. Sometimes corticosteroids may be used but this does not appear to have any significant benefit. In addition, physical therapy, occupational therapy and speech therapy may also be required to help a patient cope with the symptoms and facilitate recovery from Guillain-Barré syndrome. Always consult with a medical professional if Guillain-Barré syndrome symptoms appear.
Recovery and Prognosis
Most people with Guillain-Barré syndrome will begin recovering within several weeks to months. Complete recovery occurs within months and in some cases within years. Only about 10% of patients have incomplete recovery or very delayed recovery. The prognosis is significantly better with early diagnosis and appropriate treatment as mentioned above. Death from Guillain-Barré syndrome is rare.