Guillain-Barré Syndrome Overview Prevalence Cause

The pathologic hallmark of the disease is loss of myelin in peripheral nerves due to an acute and progressive inflammation of unknown cause. It is suggested that it is an autoimmune disease, in which the sufferer's immune system is triggered into damaging the nerve covering. There is some support for this in that half of all cases occur soon after a microbial infection or respiratory or gastrointestinal viral infection. Many cases developed in people who received the 1976 swine flu vaccine.

Peripheral nerves originate in the spinal cord and proceed to their target tissues (mainly muscle, skin and all internal organs). Their most proximal parts emerging from the spinal cord are called nerve roots and the inflammation in most (but not all) typical Guillain-Barré syndrome cases starts in these roots. Therefore, this condition is also referred to as acute polyradiculoneuritis.

Recent studies on the disease have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss. The cases indicating the demyelinating form (AIDP) are called "acute motor and sensory axonal neuropathy" (AMSAN); the cases showing only motor symptoms (diffuse weakness) are called "acute motor axonal neuropathy" (AMAN). In a different and infrequent variant called Miller Fisher syndrome , patients develop ataxia, loss of tendon reflexes, and difficulty moving eye muscles but not weakness or sensory loss. All variants of Guillain-Barré syndrome are now supposed to be an autoimmune disease caused by antibodies against a variety of ganglioside s found in abundant amounts in the peripheral nerve tissue.

2 Prevalence

GBS is a rare disease affecting about 1 to 2 people in every 100,000 annually. It does not discriminate with regard to the age or sex of sufferers.

3 Cause

About one half of patients have a history of preceding viral infection. Guillain-Barré syndrome may also be associated with immunizations, recent surgery or trauma, pregnancy, Hodgkin's disease and connective tissue diseases. The most frequently associated viral agents are cytomegalovirus (CMV), HIVThe Human immunodeficiency virus HIV is a frequently mutating retrovirus that attacks the human immune system and which has been shown to cause acquired immune deficiency syndrome (AIDS). History HIV was discovered and identified as the agent for AIDS by, measlesMeasles also known as rubeola is a common disease caused by a virus of the genus Morbillivirus''. Reports of measles go back to at least 700, however, the first scientific description of the disease and its distinction from smallpox is attributed to the M and herpes simplex virusThe Herpes simplex virus infection (common names: herpes cold sores is a common, contagious, incurable, and in some cases sexually transmitted disease caused by a double-stranded DNA virus. The infection can also affect the brain, in which case the conseq. A bacteria called Campylobacter jejuniCampylobacter jejuni is a species of curved, rod-shaped bacteria commonly found in animal faeces. It is one of the most common causes of human diarrhoea in developed countries. Food poisoning caused by Campylobacter species can be severely debilitating bu has recently been shown to be closely related with certain subtypes of the disease.

4 Signs and symptoms

Extensive damage of myelin causes disturbances in peripheral nerve functions, which can be classified as motor (affecting the muscle), sensory (affecting the skin) or autonomic (affecting the internal organs). Therefore, patients usually show two or more of the following symptoms: weakness (often symmetrical, in ascending fashion, leading to respiratory failure in one-third of cases), decreased sensation (numbness, loss of position sense), severe fluctuations in blood pressure, irregularities of heart rate, constipation and incontinence. Additional symptoms may be blurred vision, difficulty moving facial muscles, difficulty swallowing, and drooling.

The symptoms are ascending weakness with abnormal sensations and then paralysis of the legs, arms, face and possibly breathing muscles. It is rarely fatal but there is no direct cure and recovery may need care in an intensive care unit and can take years (although people can recover in a few weeks as well).

5 Diagnosis

The diagnosis is established by electromyographyElectromyography (EMG) is a medical technique for measuring muscle response to nervous stimulation. EMG is performed using an instrument called an electromyograph, to produce a record called an electromyogram. An electromyograph detects the electrical pot examination, nerve conduction studies (NCS), and cerebrospinal fluidCerebrospinal fluid CSF in short, is the clear fluid that occupies the subarachnoid space (the space between the skull and cortex of the brain). It acts as a "cushion" or buffer for the cortex. Also, CSF occupies the ventricular system of the brain and th (CSF) examination. Electromyography and NCS show slowing of conduction velocities, indicating myelin loss; CSF examination reveals high proteinmyoglobin, showing coloured alpha helices. This protein was the first to have its structure solved by X-ray crystallography by Max Perutz and Sir John Cowdery Kendrew in 1958, which led to them receiving a Nobel Prize in Chemistry. A protein is a complex, content with usually normal or slightly elevated cell count, indicating severe nerve damage. These findings are usually prominent after the first week of the disease, so the clinical symptoms and findings are more valuable in the early stages.

6 Treatment

Supportive care with monitoring of all vital functions is the cornerstone of successful management. Because the immune mechanisms play a role in pathogenesis, plasma exchange or intravenous immunoglobulins may improve the outcome. Although the corticosteroidIn physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydras may be used in treatment, they are not usually considered the drug of first choice because they may occasionally worsen the symptoms.

7 Prognosis

Approximately 80% of patients have a complete recovery and about 5-10% recover with severe disability. However this is a grave disease and despite all improvements in treatment and supportive care, the death rate among patients with this disease is still about 2-3% even in the best intensive care units.

8 History

The disease was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré and Andre Strohl discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

9 External links

Guillain-Barré syndrome forum and information

Eponymous diseases Autoimmune diseases Neurology

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1 Overview