What is Guillain-Barré Syndrome?
Guillain-Barré Syndrome is another name of acute polyneuropathy. This is a disorder which affects the peripheral nervous system. The most common symptom of this syndrome is ascending paralysis and weakness beginning in the upper and lower extremities and radiating towards the trunk. This syndrome can cause some serious complications, particularly if the muscles of the respiratory system get involved as well as the autonomic nervous system. This syndrome usually develops with an infection.
Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy is diagnosed by EMG/nerve conduction studies as well as CSF analysis. With prompt IVIG treatment or plasmapheresis, the majority of people get cured completely. This syndrome is very rare but is the most common cause of nontraumatic paralysis.
Symptoms of Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy is illustrated by proportional weakness affecting the lower extremities in the beginning and then quickly moves in an upward direction. People typically experience weakness in their lower extremities to the point where the legs tend to give way under them. As the weakness moves in an upward fashion over a period of some days, the upper extremities and the face also get affected. Normally, the lower cranial nerves also get involved resulting in dysphagia, drooling, etc. Most people need hospital admission and around one fourth of them require ventilatory support for respiratory distress. Also very common is the weakness of the facial muscles.
There may also be loss of position sense and absence of reflexes. There is also loss of sensation of temperature but it is very mild. People with GBS usually have an aching pain in the muscles. There have been some cases of bowel and bladder dysfunction with GBS as well.
With severe form of GBS, there is autonomic function deficiency which presents as blood pressure variations, postural hypotension, and irregular heartbeat.
Causes of Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
- GBS develops due to immune response of the body to infectious agents. The most common infection with which GBS develops is from the bacteria Campylobacter jejuni.
- Additionally, cytomegalovirus has been known to be associated with GBS, although it is impossible to identify a particular cause. There are some cases where GBS was triggered by influenza virus.
- GBS, unlike multiple sclerosis or amyotrophic lateral sclerosis, is a peripheral nerve ailment and does not cause injury to the brain or the spinal cord.
Diagnosis of Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
- Diagnosing GBS is dependent on findings of development of paralyzed muscles etc. Some testing to diagnose GBS is CSF analysis or EMG/nerve conduction studies
- During CSF analysis, if a person has GBS, there may be albuminocytological dissociation.
- For a person with GBS, the EMG/nerve conduction studies will show delayed distal latencies, conduction block, and temporal spreading of compound motor unit action potentials. F waves and H-reflexes may also be delayed or even absent.
- Some of the signs that strongly suggest a diagnosis of GBS is weakness of the extremities, areflexia, sensory loss, facial muscle weakness, autonomic dysfunction, elevated CSF protein, and the electrodiagnostic testing results as described above.
Types of Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
There are six forms or subsets of Guillain-Barré Syndrome (GBS), which are the following:
- AIDP: This is the most widespread variant of GBS. This process presents itself with areflexia, weakness, and sensory changes.
- Miller Fisher Syndrome: This is a rare form of GBS. Less than 10% of GBS cases are identified as this form of the disease. Unlike normal GBS, in this syndrome the paralysis is in a descending direction. It affects the muscles of the eyes first and presents itself with ophthalmoplegia, ataxia, and absence of reflexes.
- Acute Motor Axonal Neuropathy: This form of GBS is probably because of autoimmune response against the peripheral nerves.
- Acute Motor Sensory Axonal Neuropathy: This is similar to motor axonal neuropathy but in this form also affected are the sensory nerves.
- Acute Panautonomic Neuropathy: This is rarest form of GBS. It sometimes occurs with associated encephalopathy. Since there is cardiovascular involvement with this form of GBS, it has a high mortality rate. Some symptoms of this form of GBS are dysphagia, constipation alternating with diarrhea, dry itchy skin, etc.
- Bickerstaff's Brainstem Encephalitis: In this form of GBS, symptoms involve acute onset of ophthalmoplegia, ataxia, altered consciousness, and increased reflexes. It can also be relapsing-remitting.
Treatment for Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
Supportive care forms the basis of successful treatment. One of the concerns in GBS is the paralysis of the diaphragm which may lead to acute respiratory failure. Ensuing treatment involves attempts at curtailing the damage to the nervous system by plasmapheresis or by administration of IVIG to take care of the harmful antibodies present. This treatment is generally started the moment a diagnosis of GBS is confirmed.
Following the above mentioned treatment, rehabilitation is done with aim at improving activities of daily living. Occupational therapy is used to provide equipment like wheelchair to assist in ADLs.
Prognosis of Guillain-Barré Syndrome (GBS) or Acute Polyneuropathy
Within a month of treatment, a person with GBS starts to recover. About 90% of patients recover completely in some months, although there may be some minor abnormalities remaining. There are about 10% of people who recover but with severe disabilities. There are again about 10% of people who may have a relapse which has been classified as Chronic Inflammatory Demyelinating Polyneuropathy or CIDP. The prognosis generally starts becoming poor for people over the age of 40.