Guillain-Barré Syndrome PDF

Summary

This document provides an overview of Guillain-Barré syndrome, including its causes, symptoms, diagnosis, and treatment. It details the autoimmune inflammatory disease affecting the peripheral nervous system.

Full Transcript

Guillain–Barré syndrome It is an autoimmune inflammatory disease of the PNS and is the most common cause of acute flaccid paralysis. GBS occurs more frequently in males than in females. All age groups can be affected. Patients with GBS typically present with weakness and sensory signs in the l...

Guillain–Barré syndrome It is an autoimmune inflammatory disease of the PNS and is the most common cause of acute flaccid paralysis. GBS occurs more frequently in males than in females. All age groups can be affected. Patients with GBS typically present with weakness and sensory signs in the legs that progress to the arms and cranial muscle. Miller Fisher syndrome is a rare, nerve disease that is considered a variant of Guillain-Barré syndrome. It is characterized by abnormal muscle coordination, paralysis of the eye muscles. Like Guillain-Barré syndrome, symptoms may be preceded by a viral illness. Although the cause is unknown, the underlying mechanism involves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation. Sometimes this immune dysfunction is triggered by an infection or, less commonly, by surgery, and rarely, by vaccination. The diagnosis is usually based on the signs and symptoms through the exclusion of alternative causes and supported by tests such as nerve conduction studies and examination of the cerebrospinal fluid. The first symptoms of Guillain–Barré syndrome are numbness, tingling, and pain, alone or in combination. This is followed by weakness of the legs and arms that affects both sides equally and worsens over time. The weakness can take half a day to over two weeks to reach maximum severity, and then becomes steady. In one in five people, the weakness continues to progress for as long as four weeks. Involvement of the muscles that control the bladder and anus is unusual. Pain-related symptoms affect more than half, and include back pain, painful tingling, muscle pain, and pain in the head and neck. Many people with Guillain–Barré syndrome have experienced the signs and symptoms of an infection in the 2–4 weeks before the onset of the neurological symptoms. This may consist of upper respiratory tract infection (rhinitis, sore throat), or diarrhea. On neurological examination, characteristic features are weakness of muscles, hypotonia and reduced or absent tendon reflexes (hypo- or areflexia). In the Miller Fisher variant of Guillain–Barré syndrome, weakness of the eye muscles and abnormalities in coordination can be found. The level of consciousness is normally unaffected in Guillain–Barré syndrome. Respiratory failure A quarter of all people with Guillain–Barré syndrome develop weakness of the breathing muscles leading to respiratory failure, the inability to breathe adequately to maintain healthy levels of oxygen, and/or carbon dioxide in the blood. Autonomic dysfunction The autonomic, which is involved in the control of body functions such as heart rate and blood pressure, is affected in two-thirds of people with Guillain–Barré syndrome. Twenty percent may experience severe blood-pressure fluctuations and irregularities in the heartbeats, sometimes to the point that the heart beats stop and requires pacemaker-based treatment. Infection onset Two-thirds of people with Guillain–Barré syndrome have experienced an infection before the onset of the condition. Most commonly, these are episodes of gastroenteritis or a respiratory tract infection. Vaccine onset GBS cases occurred in 362 patients during the 6 weeks after influenza vaccination The swine flu vaccination-induced GBS; small increases in incidence Diagnosis The diagnosis of Guillain–Barré syndrome depends on findings such as rapid development of ascending muscle paralysis, absent reflexes, absence of fever, and absence of a likely cause. Cerebrospinal fluid analysis and nerve conduction studies are supportive investigations commonly performed in the diagnosis of GBS. An abnormally low level of sodium in the blood is often encountered in Guillain–Barré syndrome. This has been attributed to the inappropriate secretion of antidiuretic hormone, leading to relative retention of water. In many cases, magnetic resonance imaging of the spinal cord is performed to distinguish between Guillain–Barré syndrome and other conditions causing limb weakness, such as spinal cord compression. Spinal fluid Cerebrospinal fluid examination Characteristic findings in Guillain–Barré syndrome are an elevated protein level, usually greater than 0.55 g/L, and fewer than 10 white blood cells per cubic millimeter of fluid (albuminocytological dissociation). Neurophysiology Nerve conduction of electrical impulses can exclude other causes of acute muscle weakness. Electromyography (EMG) Neurophysiology studies are not required for the diagnosis. Treatment Immunotherapy Plasmapheresis and intravenous immunoglobulins (IVIG) are the two main treatments for GBS. Plasmapheresis attempts to reduce the body's attack on the nervous system by filtering antibodies out of the bloodstream. Similarly, administration of IVIG neutralizes harmful antibodies and inflammation. These two treatments are equally effective, but a combination of the two is not significantly better than either alone. Plasmapheresis speeds recovery when used within four weeks of the onset of symptoms. IVIG works as well as plasmapheresis when started within two weeks of the onset of symptoms and has fewer complications. IVIG is usually used first because of its ease of administration and safety. Glucocorticoids alone have not been found to be effective in speeding recovery and could potentially delay recovery. Respiratory failure Respiratory failure may require intubation of the trachea and breathing support through mechanical ventilation, generally on an intensive care unit. Pain While pain is common in people with Guillain–Barré syndrome, studies comparing different types of pain medication are insufficient to make a recommendation as to which should be used. Rehabilitation Following the acute phase, around 40% of people require intensive rehabilitation with the help of a multidisciplinary team to focus on improving activities of daily living (ADLs). Rehabilitation improves long- term symptoms. Teams may include physical therapists, occupational therapists, speech language pathologists, social workers, psychologists, other allied health professionalsand nurses.

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