Recognized since the early 1800s, Guillain-Barré syndrome is a rare, paralyzing and potentially catastrophic disorder of the peripheral nerves. It involves a sudden attack on the nerves that control movement - both voluntary, like walking, and involuntary, like breathing. People may spend months in hospital, not knowing if or when they will recover. Some face long-term disability. The disease is of variable severity; in mild cases the victim can still walk, while in the most severe sufferers are reduced to a state of near-paralysis. Four out of five victims eventually recover fully but it takes several years for the injured nerves to completely repair themselves. Younger people recover more quickly. There is around a five percent mortality rate, usually from respiratory complications or heart problems.
The cause of the disease is unknown, but about two-thirds of cases are preceded by a respiratory or gastrointestinal infection. A significant minority of cases follow infection by two herpes viruses, cytomegalovirus or Epstein-Barr virus, the cause of infectious mononucleosis. Some cases have been triggered by immunizations against infections such as influenza.
The name derives from the names of two French army doctors who wrote the first description of the disease in 1916.
Myelin, the fatty protective sheath around the nerves, is inflamed and patchily destroyed, disrupting messages from the nerves to the muscles, which become weak and non-functional. The first symptom is usually a tingling sensation and numbness in the feet, legs and arms. Over the next few days there is progressive loss of sensation and muscle power affecting the whole body. Symptoms may continue to worsen for two to three weeks. In the worst cases, the muscles that control speech, eye movements and breathing may be paralysed (one-third of patients develop breathing problems and one-fifth require artificial ventilation). Most experts believe the syndrome represents an autoimmune attack in which antibodies in the blood mistakenly attach themselves to normal tissue and destroy it. However, the usual treatments for autoimmune disorders - steroids and anti-inflammatory medications -- do not help and may make things worse. Plasma exchange, involving removal and replacement of the liquid part of the blood that contains the antibodies with a slat and protein solution, or pooled gamma globulin, which contains antibodies from blood donors, give the best results.
Guillain-Barre syndrome most often strikes people over 60, but it can be contracted by younger and vigorous people, such as Olympic swimmers. The author Joseph Heller, wrote of his experiences in his novel "No Laughing Matter". It is the most common cause of paralysis in the young and affects several hundred people in the UK each year.