Compared and contrast the treatments of Guillaine barre syndrome

GUILLAINE-BARRE SYNDROME TREATMENTS

Comparison of treatments of Guillaine-Barre syndrome

Introduction

Guillaine-Barre syndrome (GBS ) is an acute immune mediated inflammatory or post infective demyelinating polyneuropathy . It causes a rapidly progressive , symmetrically ascending flaccid paralysis . It is the most common cause of flaccid paralysis . Around 5 of the patients die because of the disease and 10 receive severe motor disability (Raphael , C 1999 . It is a relatively rare disease which affects only 3 people out of 100 ,000 .more than two out of three times , it is preceded by n infection . These

can be bacterial or viral

Common organisms include Campylobacter jejuni or cytomegalovirus . Its starting symptoms include weakness of the distal part of the appendages i .e . fingers or toes . From here , the parasthesia progresses upwards worsening in condition . By around the 3rd week , areflexia may be present (McLean , S ,2008 . Some sensory involvement can occur . And there is a risk of autonomic involvement as well . Autonomic involvement leads to hypotension , urinary retention , impaired papillary responses and cardiac arrhythmias . In the most severe cases , involvement of the bulbar muscles causes difficulty with chewing and swallowing with an increased risk of aspiration (McLean , S ,2008 . We can categorize GBS according to the types of nerves it affects . Acute motor axonal neuropathy (AMAN is one type . It affects only the motor system and is responsible for more than 75 of the cases (Winer , J .B 2002 . Another type of GBS is the Acute Motor and Axonal Neuropathy (AMSAN . This type is rarer and occurs with the involvement of both motor and sensory neurons . These include supportive care and active treatment modalities (Winer , J .B 2002

Supportive care

The integration of supportive care management with active therapy aimed at limiting and /or reversing neurological damage , the mortality rate of GBS has greatly reduced from 30 to a mere percent (Winer , J .B 2002 Prophylaxis for deep venous thrombosis (DVT ) through use of heparin and pressure stockings , physiotherapy , G .I care , respiratory support through positive pressure ventilation has influenced the prognosis of GBS patients a great deal . Pain control through analgesics is also an important component of the support care

Plasma exchange

This was the first immunomodulatory treatment that was known to be beneficial in the case of GBS . Multiple randomized studies have been conducted to look into GBS treatment . A study was conducted in North America to compare randomized patients response to plasma exchange when compared with support care alone . Only the patients who were unable to walk due to disease severity were included in the study . This relates to the clinical grade 3 of the disease . Upon use of the treatment , it was found that plasma exchange increases the likelihood of reducing the clinical grade in 28 days time . It shortened the time required for the recovery from 44 days to 30 days

2 studies have conducted to see the effects of increasing the number of plasma exchanges on the recovery of patients suffering from GBS . 304 patients who could stand on their own were included in the study and were randomized into groups which either received 2 exchanges or 4 exchanges

It was noted that the patient who received 4 exchanges recovery relatively quicker when compared with patients receiving 2 plasma exchanges . 4 plasma exchanges were found to be the optimal number of exchanges to be used in moderate to severe cases . It was found even effective against moderate to severe forms of the disease . The study further showed that further increase in the number of plasma exchanges were of no added benefit to the patient . It was noted that patients who underwent 4 plasma exchanges had a relatively reduced time on ventilator support and their hospital stay time was reduced , compared to patients who underwent 2 exchanges (Winer , J .B 2002

Intravenous immunoglobulin (IVIg

Response to IVIg in Guillaine-Barre syndrome was first reported in 1988 .not many trials are available on comparison of IVIg with supportive management . Three significant studies have been conducted for comparison of IVIg with plasma exchange (PE . One study provided randomized selection of GBS patients with either PE or IVIg within 14 days of the appearance of the symptoms . An over all quicker recovery in patients undergoing IVIg treatment was seen . It was concluded that IVIg may be as effective as PE and visible efficacy trend towards the IVIg treatment may be due to variations of baseline variables

Another study conducted a similar study and found IVIg to be as effective in GBS treatment as PE . No speedier recovery was seen in patients who underwent IVIg treatment . A Meta analysis conducted on studies comparing IVIg with PE gave 398 patients . The IVIg was assessed on the basis of changes in the disability scale , as was used in older studies . The Meta Analysis concluded that there was no significant difference between IVIg and PE in change of disability grade or in time to walk unaided , mortality , or proportion of patients unable to walk at one year (Winer , J .B 2002 . It has also been noted that the relapse rate is higher in patients who undergo IVIg when compared with Plasma exchange

Corticosteroid

Corticosteroid drugs have a strong anti inflammatory effect . This knowledge leads may lead one to believe that corticosteroids have help in reducing and limiting the nerve damage in case of GBS . Many trials have been conducted to evaluate the effectiveness of corticosteroids in the treatment of GBS . From the six trials which had 587 participants the overall results showed no difference in disability grade , in patients treated with corticosteroids and non corticosteroids

Four trials were conducted with 120 participants using oral corticosteroids . The results showed significantly less improvement in patients who had undergone oral corticosteroids treatment when compared with those who underwent non steroidal treatment for GBS . In two large trials which compared intravenous corticosteroids compared with placebo It was shown that they hasten recovery when given with IVIg . The study review concluded that oral corticosteroids may delay recovery from Guillaine-Barry syndrome whereas intravenous corticosteroids may hasten recovery when given with intravenous immunoglobulin but do not affect the long-term outcome (Hughes RAC , 1999

Interferon

Certain new treatment modalities have come into light which may prove to be of superior efficacy when compared with plasma exchange and IVIg These according to researchers include use of Interferon beta Cerebrospinal fluid infiltration and administration of nerve growth factor to hasten nerve repair and regeneration . The most promising future treatment according to researchers may be the use of interferon beta . It has the capacity to reduce the adhesion process as well as transmigration of lymphocytes , hence potentially providing us with a beneficial therapeutic effect (Gersh , H , 2007 . In view of the potential therapeutic effects of interferon usage , a study was conducted on 19 non ambulant GBS patients . This double blind study compared randomized patients receiving either interferon beta 1a or placebos in addition to IVIg . The treatment continued for up to 24 weeks . It was noted that combining IVIg with interferon had no additional significant effect on the rate of recovery in the patients who received IVIg when compared with placebo administered patients (Pritchard , J , 2003 . Other immunomodulatory and cytotoxic drugs have also been used in trials with no added benefit in recovery process . A cross over trial of interferon on GBS patients revealed that interferon was of no extra benefit (Hughes , R .A .C , 2002

Combination treatments for use in GBS

Even though studies have shown that no benefits of using corticosteroids alone , there is evidence through a Dutch study which compared intravenous methyl prednisolone with IVIg maybe beneficial when compared with IVIg alone (Hughes , R .A .C , 2002

Plasma exchange was the first treatment known to be proven beneficial in the case of GBS patients . However , studies have shown no added benefit from combining plasma exchange treatment with intravenous immunoglobin Studies have also noted that patients who undergo plasma exchanges are more frequently seen with complications such as cardiac arrhythmias (Hughes , R .A .C , 2002

The drawback to the plasma exchange is that it requires maintenance of a central line . This can lead to future risks of development of hematoma or pneumothorax . It also increases the risk of a septicemia . Multiple plasma exchange can lead to a potential coagultopathic state and this can lead to the patients needing clotting factor and fibrinogen transfusions

IVIg according to a Dutch trail is a slightly more superior treatment option when compared with PE . This study compared 4 trials on 495 patients . It also found no significant differences when comparing IVIg alone with IVIg and PE , in reducing the disability grade of GBS patients (Hughes , R .A .C , 2002 . IVIg is easier to administer in patents when compared with PE . Therefore most often , it is the choice of treatment in many hospitals . There is no evidence to support the theory that IVIg may also help hasten the recovery in patients who come for treatment 2 weeks after the symptoms have started (Hughes , R .A .C , 2002

IVIg is linked to its own set of adverse effects . The less severe effects include joint pains , muscle pains and headaches . A more serious effect can occur in the form of transient hypercoagulobilty state During IVIg treatment , patients must be monitored for this condition as this can lead to an increased risk of thrombosis . Increase in volume can lead to pulmonary edema . IgA deficient patients are at risk for potentially more serious complications including kidney failure , aseptic meningitis and hepatitis

Conclusion

Even through PE and IVIg are commonly used as a mainstay for treatment sufficient dats is not present on the other treatment options including use of interferons , cytotoxic drugs , CSF filtration techniques etc inadequate data is available to weigh the side effects of the use of these treatment options . Further research to explore the adverse effects the available treatments need to be undertaken . Not much data is available on patients who come for treatment more than 2 weeks after symptoms appear . Trials with large sample sizes need to be conducted in this area in the future to reduce long term disability and so that the patients proper patient treatment and care

REFFERENCES

Gersh , H (2007 , New Horizons in Guillaine-Barry Treatment . Retrieved on March 4 , 2008 , from HYPERLINK "http /www .consultantlive .com http /www .consultantlive .com

Hughes , R .A .C (2002 . Systemic reviews of the treatments for inflammatory demyelinating neuropathy . Journal of Anatomy , 200 , 331-339 Retrieved March 4 , 2008 , from HYPERLINK "http /www .pubmedcentral .nih .gov /articlerender .fcgi ?artid 1570692 http /www .pubmedcentral .nih .gov /articlerender .fcgi ?artid 1570692

Hughes RAC , Swan AV , van Koningsveld R , van Doorn PA (1999 Corticosteroids for Guillaine-Barry syndrome . Cochrane Reviews Retrieved March 4 , 2008 , from HYPERLINK "http /www3 .interscience .wiley .com http /www3 .interscience .wiley .com

J .B . Winer (2002 , Treatment of Guillaine-Barry syndrome . QJ M : An international journal of medicine . 95 , 717-721 . Retrieved March 4 , 2008 from HYPERLINK "http /qjmed .oxfordjournals .org /cgi /content /full /95 /11 /717 http /qjmed .oxfordjournals .org /cgi /content /full /95 /11 /717

McLean , S (2008 . Childhood Guillaine-Barre Syndrome : Comparing Intravenous Immunoglobulin treatment with supportive retrieved on March 4 , 2008 from HYPERLINK "http /www .tcd .ie /tsmj /2005 /6067CGBS .pdf http /www .tcd .ie /tsmj /2005 /6067CGBS .pdf

Pritchard , J , Gray , I , A , Idrissova , Z , R , Lecky , B , R , F Sutton , I .J , Swan , A .V , Willison , H .J , et all (2003 . A randomized controlled trial of recombinant interferon-beta 1a in Guillaine-Barry syndrome , Neurology , 61 , 1282-1284

Raphael , C , Chevret , S , Auriant , L , Sharshar , T , Bouget , J Bolgert , F (1999 . Treatment of the adult Guillaine-Barry syndrome indications for plasma exchange . Transfusion science . 20 , 53-61

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GUILLAINE-BARRE SYNDROME TREATMENTS ...