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GBS Guillain Barre Syndrome

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GBS Guillain Barre Syndrome

What is GBS?

(GBS) Guillain Barre Syndrome is a rare rapid onset neurological condition in which the body’s immune system attacks peripheral nerve cells, which could lead to paralysis.

It affects the arms and legs and the early symptoms include pain and weakness in addition to numbness. It first begins as a tingling sensation and then progresses to paralysis of the limbs in the form of quadriplegia (affecting all four limbs).

Patients may also experience weakness of the respiratory muscles. If that happens, it turns into a medical emergency requiring mechanical or assisted ventilation. Damage to the nerve cells supplying the organs (autonomic nervous system) may affect heart function. This can lead to complications such as low blood pressure and irregular heart rate and rhythm.

 

How Common is GBS Guillain Barre Syndrome?

The prevalence of GBS Guillain Barre Syndrome in the US is 1-2 cases per 1000,000 individuals. The risk of hospitalization increases proportional to the patient’s age, with 9 cases per 100,000 people in 70 years and above. The incidence rate for pregnant women in the US is 2.4 cases per million. It includes patients that develop GBS during and 40 days post-delivery.

 

Causes

The exact reason for GBS is unknown. Possible causes include a history of viral or bacterial infection. Researchers believe that infection triggers a heightened immune response in already autoimmune patients that destroys myelin- the protective sheath of nerves. Once the protective sheath is damaged, the nerves go haywire and result in GBS symptoms.  

Possible causes of GBS include:

  • Bacterial Infection Caused By Campylobacter Jujeni, A Bacteria Causing Diarrheal Illness. One Of The Risk Factors For Getting This Infection Include Eating Undercooked Poultry.
  • Viral Illness Caused By Hepatitis, Hiv, Influenza, Cytomegalovirus, Covid-19, And Zika Virus
  • Cancers Such As Hodgkin’s Lymphoma
  • Trauma Or Surgery

 

Diagnostic Tests for GBS

Common tests include cerebrospinal fluid testing for albuminocytologic dissociation and nerve conduction studies.

Cerebrospinal Fluid (CSF)

Cerebrospinal Fluid (CSF) is obtained through a procedure called Lumbar Puncture (LP) in which a needle is inserted into the spine to gain CSF. 

CSF is then analyzed to see the number of cells (mainly immune or white cells) and proteins. 

GBS Guillain Barre Syndrome causes albuminocytologic dissociation, which means an increase in the concentration of the cerebrospinal proteins without an associated increase in the white blood cells. An increase in protein concentration is directly proportional to an increase in inflammation of the nerve cells. This condition is particularly prominent in the acute phase of the disease.

Nerve Conduction Studies

Nerve conduction studies test how the nerves respond when exposed to electrical current. In GBS, this test shows dampened response of nerve fibers when exposed to electrical current.

Other typical signs seen during clinical examination include muscle weakness, hyporeflexia (diminished muscle reflexes), or areflexia (absent reflexes). Other immediately apparent symptoms of GBS include reduced coordination and trouble walking.

 

Subtypes of GBS Guillain Barre Syndrome

The symptoms of GBS tend to vary depending on the disease subtypes. There are two main subtypes:

  • Acute Inflammatory Demyelinating Polyneuropathy (AIDP): The demyelinating subtype that attacks the myelin sheath.
  • Acute Motor Axonal Neuropathy (AMAN):  This type affects the core structure of the nerve cells called axons. The outer covering (myelin) might remain unaffected.

The symptoms of these variants often overlap, so it is difficult to characterize the precise subtype. However, the treatment for these subtypes is the same with varying dosages of IVIG depending on the patient’s condition.

 

GBS and Pregnancy

GBS Guillain Barre Syndrome in pregnancy can lead to respiratory and neurological disruption. Patients often require respiratory support and monitoring, especially during labor.

Around 34.5% of pregnant women with GBS require ventilator support for an average of 126 days. Pregnancy termination is not common but cases have been reported. Around 1 out of 3 women with pregnancy termination get bedridden for 20 weeks. Overall, women require 8 to 20 weeks for a full recovery. The overall survival rate is 95.7%.

A rare case of a GBS-positive child born to an infected mother was also reported in which the infant required respiratory support by day 12 and presented with paralysis of limbs and loss of tendon reflexes. This was possibly due to the damage to fetal nerves by the rogue maternal antibodies.

 

IVIG Infusion for GBS

Intravenous immunoglobulin (IVIG) infusion is a potent technique to help modulate the body’s immune system. In this technique, antibodies are injected through the veins.

Scientists do not completely understand how IVIG helps with GBS Guillain Barre Syndrome. However, the following are the possible mechanisms through which IVIG might help improve GBS symptoms:

  • IVIG Suppress Immune Cells: B and T lymphocytes are the key cell lines involved in autoimmune process causing GBS. IVIG suppresses the growth, differentiation, and activity of these immune cells.
  •  IVIG Neutralizes Antibodies: Immune cells are triggered by an infection or other factors lead to an increased production of antibodies, which can target the human nerve tissues. IVIG helps to dilute and neutralize the effects of these antibodies.
  • IVIG Dampens Inflammatory Response: One of the key pathologies during GBS is heightened inflammation resulting from B and T cell activation. The inflammatory chemicals, called cytokines, damage the sensitive myelin sheath of nerve fibers. IVIG blocks the chemical processes involved in the production of these cytokines.

Several other mechanisms are also involved but the nutshell is that IVIG helps to soothe the damage and inflammation caused by immune response targeted towards nerves.

Many of the effects of IVIG are due to its ability to increase the levels of immunoglobulin G (IgG). The body’s response to IVIG and recovery from GBS is recorded by the blood IgG levels.

 

Dosage of IVIG for GBS Guillain Barre Syndrome

Intravenous infusion of immunoglobulins is the treatment of choice for patients diagnosed with GBS. It is injected at the rate of 2 g per kg of body weight with 0.4 g per kg of body weight per day regularly for a total of 5 days.

It is important to have the right IgG levels in the blood, a lower increase in serum IgG levels can result in slower recovery. The IgG antibodies possibly work by neutralizing the action of the autoantibodies, which play a role in the autoimmune progression of the disease. 

The IgG obstructs the activation of the T and B cells, which can then produce autoantibodies, thereby significantly reducing the inflammation.

 

IVIG Side-effects

This IVIG treatment for GBS can cause side effects, which may be immediate or delayed.

The immediate side effects may manifest as fever, headache, skin reactions, hypertension, and arrhythmia.

On the other hand, delayed side effects include thrombotic and renal impairments but are reported in only 1% of the patients who receive the treatment.

For GBS, IVIG is effective but for pregnant women diagnosed with the syndrome, plasma exchange might be more effective. For patients with hemodynamic instability or ambulatory dependence, intravenous infusion is preferred.

 

Second Dose Administration

Cases have been reported where patients do not show any improvement. In such cases, the patients might require a second dose. Often the second dose is administered 7 to 9 days after primary treatment. Research suggests that patients unresponsive to the first treatment often have lower IgG levels. If the second dose is given, it can lead to higher IgG levels and better clinical response.

However, there is inconsistent evidence to support the use of a second dose of IVIG for GBS. Some research suggests that second IVIG only causes a small increase in IgG levels and may worsen the prognosis.

The second IVIG dose causes a 25% increase in the risk of thrombocytopenia. Thrombocytopenia is a condition in which the body has a significantly low platelet count. The loss of platelets results in slow clothing of the blood, which results in bleeding in severe cases. The bleeding incurred differs from patient to patient.

Therefore, the use of a second dose for IVIG is not commonly recommended. The second dose of IVIG should only be administered under expert guidance. The use of a second dose might increase the risk of side effects that may outweigh the benefits of treatment. 

 

Combination Treatment- Ivig With Other Drugs

Other therapeutic approaches combine immunoglobulin infusion with steroids in the early stage of the disease. This combination is effective against the demyelinating subtype of the disease. Another approach is combination treatment with methylprednisolone, which has shown short-term benefits but has no evidence for long-term advantages.

What to Expect as the Result of IVIG Treatment?

GBS Guillain Barre Syndrome is a rare autoimmune condition that damages the myelin sheath of nerves. It can cause a multitude of symptoms ranging from simple nerve damage to paralysis.

IVIG treatment can be given as ambulatory or non-ambulatory infusions. Ameri Pharma offers both types of solutions. Each infusion lasts three to four hours and each treatment course lasts for up to five days. The recovery from GBS can be variable. It is often slow and can take between six and twelve months to improve. In some cases, the improvement of GBS symptoms as the result of IVIG treatment can take up to three years.

In addition, most of the symptoms result in a degree of remnant symptoms. The degree of persistent symptoms can range from simple nerve damage to permanent limb weakness. However, some people might make full recovery.

Regardless of the severity of symptoms, if you or someone you know has GBS, contact the Ameri Pharma infusion specialist services to book a consultation with experts.

 

FAQs

Q: How do you catch Guillain-Barre Syndrome?
A: The exact etiology is unknown but researchers believe that GBS might be the result of infections caused by Campylobacter Jujeni or viruses such as HIV, COVID-19, influenza, and hepatitis.

Q: Can GBS Guillain Barre Syndrome be cured?
A: GBS symptoms improve over months to years. Treatments such as IVIG, steroids, and plasma exchange can help speed up the recovery.

Q: How does Guillain-Barre syndrome affect the body?
A:
GBS causes damage to peripheral nerves. The symptoms can include tingling, numbness, muscle weakness, and paralysis. It can damage nerve to organs such as heart causing an irregular heartbeat.

Q: Can IVIG cure GBS?
A: IVIG can help improve GBS symptoms. However, the speed of recovery can vary greatly between individuals.

Q: How many doses of IVIG are needed for GBS?
A: One dose over 5 years is recommended. However, a second dose might be needed in certain cases. If IVIG does not work, plasma exchange might be needed as an alternative.