Guillain-Barre Syndrome

: Guillain-Barre Syndrome

p Specifications : 6 Pages , 6 Sources , APA Style

br Describe Guilllain-Barre Syndrome (GBS , including the causes pathology , assessment diagnosis and treatments . Also briefly discuss the different variances , such as Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP , and Miller Fisher Variant

Guillain-Barre Syndrome (GBS ) or 'acute idiopathic polyneuritis ' is an rapidly progressing autoimmune dis (condition the individual 's immune system attacks the tissues ) of the peripheral nerve tissues usually following an infection (bacterial or viral (about 60 to 70 of all cases , and resulting in tiredness , muscle weakness , abnormal sensations

in the lower limbs (and upper limbs , paralysis , respiratory difficulties , etc (NINDS , 2007 . In rare cases , portions of the brain and the spinal nerves may be involved with the disease (Griffin , 2000 pp . 2192 and Mayo Clinic , 2006 . Very rarely does GBS occur after surgery or vaccination (Cull , 1996 , pp . 1106

The dis can occur in both sexes , and individuals of all age and ethnic groups . The condition is very rare and can occur once in every 100 , 000 individuals in the US every year (Mayo Clinic , 2006 . The infection usually follows a infection of the digestive tract or the respiratory system . The condition usually progresses gradually over the next few days or weeks (NINDS , 2007 . Acute versions of the disease are more common in all regions of the World , other than the US and Europe (Griffin , 2000 , pp . 2192

The exact cause of GBS is not understood , but studies suggest that is basically due to defect in the immune system that may be initiated by a viral or bacterial infection . The body 's defense mechanism may begin to attack the myelin sheath that covers the nerve resulting in its degeneration . These myelin sheaths helps transmit nerve signals . The point at which myelin is absent at the nerve is known as 'node of Ranvier , and the nerve signals jump from one node of Ranvier to another , enabling speedy transmission of the nerve signals . When the myelin sheath is degenerated , the nerve signals cannot be transmitted resulting in inability of the muscles to respond efficiently to the signals generated from the brain . The nerves that carry sensory information from the sense organs to the brain also get demyelinated resulting in improper decoding of sensory information . Several sensory abnormalities such a loss of sensations , abnormal sensations , stinging etc , are experienced by the individual (NINDS , 2007

GBS may occur following a viral infection or a bacterial infection Some of the common infections in which GBS can occur include C . Jejuni infection , infectious mononucleosis , Cytomegalovirus infection , Epstein Bar Virus infection , Herpes infection and Myoplasma infection . The C jejuni infection usually tends to be the most severe type (Griffin 2000 , pp . 2193 . A study conducted demonstrated that about 1 .17 individuals out of every 1000 affected with the bacteria would develop GBS , every year , and the incidence was about 77 more than the normal incidence rates . About 2 in every 10 , 000 individuals who have suffered from the bacterial infection would develop GBS within the next two months (Tam , 2006 . Once the cells are affected with the virus , a defective immune mechanism begins to act . Several defense cells of the body such as the lymphocytes and the macrophages tend to attack cells present in the nervous system resulting in the degeneration of the myelin sheath . Antibodies are produced by the B-lymphocytes against the myelin on stimulation by the T-lymphocytes (NINDS , 2007 . The spinal nerve roots and the peripheral nerves are pathologically invaded with lymphocytes resulting in inflammation . The macrophages cause degeneration of the myelin sheath and secondary degeneration of the axon (Griffin , 2000 , pp . 2193

The individual initially experiences paraesthesia and weakness in the muscles of the lower limbs . The muscle weakness progresses from the distal end to the proximal end , whereas the paraesthesia progress from the proximal end to the distal end (Cull , 1996 , pp . 1106 . Frequently the upper limbs are also involved . The individual may lose reflexes in various parts of the body . Gradually the symptoms worsen , until the individual is unable to move the limbs resulting in paralysis . About half the number of individuals has facial weakness on either side of the body (Cull , 1996 , pp . 1106 . Eye movements , mastication , speaking swallowing , etc , may get involved with the disease process . Bladder and bowel functions are also affected (Mayo Clinic , 2006 . In certain cases , the breathing , heart beat and the blood pressure may become abnormal (with the disease process ) resulting in life-threatening conditions and requiring emergency care (NINDS , 2007 . Some individuals may experience the symptoms for a certain amount of time , and slowly regain the lost muscle strength and the sensations . The process of recovery takes longer (several years ) and requires active rehabilitation programs (Mayo Clinic , 2006

The diagnosis of GBS may be difficult as the presentation of the symptoms and signs are varied and depend on several factors . The diagnosis is usually made based on the history , symptoms , signs , nerve conduction velocity tests , lumbar puncture , electromyography , blood tests , etc . The symptoms such as muscle weakness , absence of knee reflexes , paraesthesia , etc , should be observed on either side of the body . Blood tests are performed to determine a rise in the lymphocyte count . Electromyography (EMG ) is performed to study the electrical activity of the muscles on nerve stimulation , and the rate at which it conducts the nerve signal (Mayo Clinic , 2006 . Nerve conduction tests are performed to determine the rate at which nerve signals are transmitted (usually reduced in GBS . Lumbar puncture is utilized to determine the protein level in the CSF (usually raised in GBS (NINDS 2007 . The proteins levels in the CSF are usually normal in the first week of the dis (Cull , 1996 , pp . 1106

Currently , no specific treatment is available for GBS . Symptomatic relief is provided to ensure that the individuals are comfortable However , techniques have been developed to reduce the severity of complications . GBS patients should be continuously monitored for lung functions (especially vital capacity , swallowing activity , autonomic functions , etc . Some of the measures that may help include plasmapheresis , corticosteroids , immunoglobulin , and rehabilitation therapy (NINDS , 2007 . Corticosteroids are not effective when administered in GBS , independently (Griffin , 2000 , pp . 2193

Usually plasmapheresis (plasma exchange ) is performed along with immunoglobulin therapy . The exact mechanism by which plasmapheresis works in GBS is still not understood , properly . But it is suggested that certain immune-mediated substances may be present in the plasma which brings about the symptoms of GBS . The blood is passed through the machine which helps to separate out the blood cells . The plasma is filtered out . The blood cells are reconstituted with an appropriate substitute which is injected back into the individual . Immunoglobulins help to attack microorganisms that stimulate GBS . Studies have also shown that they destroy antibodies that enable the GBS pathological process (Mayo Clinic , 2006 . Corticosteroid therapy may be useful in treating the symptoms of GBS , but studies have demonstrated that it is usually not very effective (NINDS , 2007

If the individual develops episodes of unproductive cough , an acute respiratory problem could exist , requiring immediate respiratory assistance (as respiratory failure can develop within a very short period . The condition may be fatal if immediate ventilation is not provided (Griffin , 2000 , pp . 2193 . Individuals may have to be admitted to the hospital to treat emergency conditions . The condition of the heart and the lungs has to be closely monitored

After an attack occurs , the individual may require physical therapy and hydrotherapy to aid reducing the effect of muscle weakness (NINDS , 2007 and Mayo Clinic , 2006 . Individuals admitted to intensive care units require constant monitoring of blood pressure , breathing , formation of the clots , etc . Usually , the individual is able to recover from this condition , but some amount of weakness in the muscles may continue to exist (NINDS , 2007

The outcome of GBS differs depending on several factors such age of the individual , severity and the extent of demyelination . The individual may have involvement of the respiratory system with the disease (resulting in respiratory paralysis (Griffin , 2000 , pp . 2193 . It takes about 3 months to recover from such an episodes (if plasmapheresis is performed ) and 6 months in case plasmapheresis is not conducted About 80 of individuals treated are able to recover (Cull , 1996 , pp 1106 . Some individual are able to recover faster than the others About 10 arrive at fatal outcomes , and another 10 develop severe neurological disabilities (Cull , 1996 , pp . 1106 . Recurrences are usually treated with the help of plasmapheresis and immunoglobulin therapy . Education and motivation plays a very important role in improving the outcome of GBS . It helps to prevent rapid degeneration of the motor and the sensory functions . The individual is taught the manner in which communication is possible during a respiratory emergency situation (Griffin , 2000 , pp . 2193 . There are no known ways of preventing the dis (Mayo Clinic , 2006

Some of the different types of GBS include Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP , multifocal motor neuropathy and Miller Fisher Syndrome

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP ) is an acute version of GBS . The pathology , clinical features , etc are similar to that of GBS . The course of the disease and certain episodes that occur in CIDP may be dissimilar compared to GBS . The immunological mechanism that results in CIPD is different . CIPD can occur in individuals of any age group . There is a slow muscle weakens in the legs , areflexia (absence of reflex actions , etc . Several parts of the body may be involved . The large nerve fibers are usually involved first . CIDP usually progresses at a much slower rate compared to GBS Nerve conduction studies help to demonstrate the slow nerve conduction rate (suggesting demyelination ) and lumbar puncture suggest increased proteins in the CSF . CIPD is usually treated with corticosteroids as they are effective . Sometimes , plasmapheresis and immunoglobulin therapy seems to be useful in the condition . Corticosteroids are administered in lower doses first . The plasmapheresis should be repeated every few weeks (Griffin , 2000 , pp . 2193

Another variant similar to CIPD is multifocal motor neuropathy . It is a pure neuropathy as no sensory nerves are involved with the condition The individual experiences a drop in the wrist and the foot due to the involvement of the radial nerve and the peroneal nerve , respectively The tendon reflexes are also lost . The nerves tend to be involved in a stepwise fashion . The pathological features of multifocal motor neuropathy are similar to that of CIPD . However , this condition tends to remain highly localized , and does not involve the sensory nerve . The diagnosis is usually made by the nerve conduction tests that demonstrate decreased nerve condition (due to focal demyelination . The CSF is normal , and usually blood tests demonstrate an increase in the IgM and the anti-ganglioside antibodies in the blood . The condition is usually treated by administering immunoglobulin and chemotherapeutic agents Corticosteroids and plasmapheresis are usually not very effective in treating multifocal motor neuropathy (Griffin , 2000 , pp . 2193

Miller Fisher Syndrome (MFS ) is a rare variant of GBS in which the individual develops poor coordination of the muscles (ataxia , paralysis of the muscles of the eye (ophthalmoplegia , and loss of the tendon reflexes (areflexia . It is a type of acute polyneuropathy . Besides respiratory problem and muscle weakness also exist . The condition is usually associated with a viral infection . Blood tests performed usually demonstrate the presence of a specific antibody . Immunoglobulin therapy and plasmapheresis help in treating MFS . Supportive care may be required to aid in respiration . The outcome of MFS is usually better than GBS , as the individual recover within a few weeks after the disease has actually begun . The chances of recurrences are usually very minimal (NINDS , 2007

References

Cull , R . E , and Will , R . G (1996 . Diseases of the Nervous System , In Edwards , C . R . A , Bouchier , I . A . D Haslett , C (Ed , Davidson 's Principles and Practice of Medicine (17th Ed , Edinburgh : Churchill Livingstone

Griffin , J . W (2000 . Immune-mediated Neuropathies , Goldman , L . and Bennett . J . C (Ed ) Cecil Textbook of Medicine (21st ed , Vol . 2 Philadelphia : W . B . Saunders

Mayo Clinic staff (2006 . Guillain-Barre Syndrome . Retrieved on April 11 , 2007 , from Mayo Clinic Web site : HYPERLINK "http /www .mayoclinic .com /health /guillain-barre-syndrome /DS00413 /DSECTI ON 1 http /www .mayoclinic .com /health /guillain-barre-syndrome /DS00413 /DSECTIO N 1

National Institute of Neurological Diss and Stroke (2007 Guillain-Barre Syndrome Fact Sheet . Retrieved on April 11 , 2007 , from NINDS Web site : HYPERLINK "http /www .ninds .nih .gov /diss /gbs /detail_gbs .htm http /www .ninds .nih .gov /diss /gbs /detail_gbs .htm

National Institute of Neurological Diss and Stroke (2007 . NINDS Miller Fisher Syndrome Information Page . Retrieved on April 11 , 2007 from NINDS Web site : HYPERLINK "http /www .ninds .nih .gov /diss /miller_fisher /miller_fisher .htm http /www .ninds .nih .gov /diss /miller_fisher /miller_fisher .htm

Tam , C . C , Rodrigues , L . C , Petersen , I . Et al (2006 'Incidence of Guillain-Barre syndrome among patients with Campylobacter infection : a general practice research database study ' J Infect Dis , 194 (1 , 95-97 HYPERLINK "http /www .ncbi .nlm .nih .gov /entrez /query .fcgi ?cmd Retrieve db PubMed do pt Abstract list_uids http /www .ncbi .nlm .nih .gov /entrez /query .fcgi ?cmd Retrieve db PubMed dop t Abstract list_uids...