What Is Guillain-Barre Syndrome?
Approximately 5000-6000 cases of Guillain-Barre Syndrome occur in USA annually and it can affect any age group. It is one of the commonest forms of acute polyneuropathy.
Almost two out of every three cases occur 1-3 weeks after a respiratory infection or gastrointestinal infection (like diarrhea). It is an autoimmune disease, i.e. the immune system that is supposed to attack foreign substances like bacteria; starts attacking cells of own body, in this case the nerves. The immune system produces special molecules, called the antibodies that are mainly responsible for damage to nerve cells in Guillain-Barre Syndrome.
Guillain-Barre syndrome is mainly a motor neuropathy, i.e. it mainly affects the nerves supplying the muscles. The paralysis often starts in legs and slowly progressing upwards, it may ultimately result in paralysis of arms, face and even eye muscles. In limbs it is often symmetric, i.e. both sides are more or less equally affected. The polyneuropathy may involve nerves controlling blood pressure and heart rate resulting in rapid fluctuations in them and sometimes cardiac dysrhythmias also. Bladder involvement (thus problem in voiding urine) is rarely seen, and if it occurs, it is temporary. Sensory system is mildly affected; persons affected often only have a tingling sensation in the extremities. Fever is almost always absent when symptoms first appear. Shoulder pain, Neck pain, or diffuse back pain also common in beginning and occurs in almost half of all cases. There may also be mild aching pain in affected muscles. In some variants of Guillain-Barre Syndrome (Miller Fisher Syndrome), there is predominant eye muscles weakness and lack of coordination in muscle movements (ataxia) with no limb weakness. The muscle weakness in Guillain-Barre syndrome keeps increasing slowly for about 1-4 weeks then becomes constant for 2-4 weeks before recovery starts.
The treatment in Guillain-Barre Syndrome should be started immediately after the diagnosis is made. Mainstay of treatment is either intravenous immune globulin (IVIg) or plasmapharesis. In plasmapharesis, the blood is taken out of the body and then the plasma is processed to remove the antibodies and then plasma is returned to the body. In a variant of this process, the separated plasma is thrown away and new fresh plasma from a donor is infused (plasma exchange). Both IVIg and plasmapharesis are not necessary, and either of them works equally well alone and there is no additional advantage of giving both. Generally IVIg is a better choice because it's easier to administer and safer than plasmapharesis. One can expect significant improvement in symptoms after 1 week but sometimes it may be delayed. Unlike other autoimmune disease, corticosteroids have almost no effect on the course of disease.
Other than the above treatment, in severe cases of Guillain-Barre Syndrome, supportive therapy under critical care is of paramount importance. If the diaphragm is paralyzed, patient is placed on mechanical ventilation. About one out of every three patients may need mechanical ventilation. Blood pressure, pulse and nutrition are monitored closely. Frequent turning and skin care to prevent bed sores is also important, so is daily range-of-motion exercises of joints to prevent joint contractures.
Recovery is generally good, with serious problems occurring only in less than ten percent of cases. About five percent of the cases are fatal despite proper treatment. Full functional recovery may take many months and sometimes a year.
Almost two out of every three cases occur 1-3 weeks after a respiratory infection or gastrointestinal infection (like diarrhea). It is an autoimmune disease, i.e. the immune system that is supposed to attack foreign substances like bacteria; starts attacking cells of own body, in this case the nerves. The immune system produces special molecules, called the antibodies that are mainly responsible for damage to nerve cells in Guillain-Barre Syndrome.
Guillain-Barre syndrome is mainly a motor neuropathy, i.e. it mainly affects the nerves supplying the muscles. The paralysis often starts in legs and slowly progressing upwards, it may ultimately result in paralysis of arms, face and even eye muscles. In limbs it is often symmetric, i.e. both sides are more or less equally affected. The polyneuropathy may involve nerves controlling blood pressure and heart rate resulting in rapid fluctuations in them and sometimes cardiac dysrhythmias also. Bladder involvement (thus problem in voiding urine) is rarely seen, and if it occurs, it is temporary. Sensory system is mildly affected; persons affected often only have a tingling sensation in the extremities. Fever is almost always absent when symptoms first appear. Shoulder pain, Neck pain, or diffuse back pain also common in beginning and occurs in almost half of all cases. There may also be mild aching pain in affected muscles. In some variants of Guillain-Barre Syndrome (Miller Fisher Syndrome), there is predominant eye muscles weakness and lack of coordination in muscle movements (ataxia) with no limb weakness. The muscle weakness in Guillain-Barre syndrome keeps increasing slowly for about 1-4 weeks then becomes constant for 2-4 weeks before recovery starts.
The treatment in Guillain-Barre Syndrome should be started immediately after the diagnosis is made. Mainstay of treatment is either intravenous immune globulin (IVIg) or plasmapharesis. In plasmapharesis, the blood is taken out of the body and then the plasma is processed to remove the antibodies and then plasma is returned to the body. In a variant of this process, the separated plasma is thrown away and new fresh plasma from a donor is infused (plasma exchange). Both IVIg and plasmapharesis are not necessary, and either of them works equally well alone and there is no additional advantage of giving both. Generally IVIg is a better choice because it's easier to administer and safer than plasmapharesis. One can expect significant improvement in symptoms after 1 week but sometimes it may be delayed. Unlike other autoimmune disease, corticosteroids have almost no effect on the course of disease.
Other than the above treatment, in severe cases of Guillain-Barre Syndrome, supportive therapy under critical care is of paramount importance. If the diaphragm is paralyzed, patient is placed on mechanical ventilation. About one out of every three patients may need mechanical ventilation. Blood pressure, pulse and nutrition are monitored closely. Frequent turning and skin care to prevent bed sores is also important, so is daily range-of-motion exercises of joints to prevent joint contractures.
Recovery is generally good, with serious problems occurring only in less than ten percent of cases. About five percent of the cases are fatal despite proper treatment. Full functional recovery may take many months and sometimes a year.
