Guillian Barre Syndrome is an autoimmune disease affecting the nerves due to plasma exchange. GBS disease may be triggered by cytomegalovirus or campylobacter.

Guillain-Barre Syndrome

Dr. Syed Intisar Hussain Clinic

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Syed Intisar Hussain

Overview

Guillain-Barre syndrome (GBS) is defined as a rare disorder in which the body&rsquo;s immune system attacks the nerves.

It is a rare polyradiculoneuropathy in which the immune system of the body attacks the nerves, resulting in weakness and diminished reflexes. The first symptoms of the syndrome typically include weakness and tingling in the motor nerves of the lower extremities. In the most severe form of the disease, the sensations spread upwards, and may eventually cause paralysis in the whole body.&nbsp;

While the exact cause of Guillain-Barre syndrome is inconclusive, in two-thirds of the patients, the syndrome is secondary to infection including Zika virus, gastrointestinal infection, respiratory infection, or <a href="https://www.merisehat.pk/disease/covid-19" target="_blank">COVID</a><a href="https://www.merisehat.pk/disease/covid-19" target="_blank">-19</a>. Unfortunately, there is currently no cure for Guillain-Barre syndrome. However, there are several treatments that can reduce the duration of the illness and alleviate symptoms.

Similarly, although most people recover from the disease, in severe cases it can also be fatal. Recovery may take several years but most people are able to walk six months after the onset of symptoms. Whereas some people may experience lasting symptoms, for example, weakness, numbness, and fatigue.&nbsp;

Normally, the immune system fights viruses and bacteria but in Guillain-Barre syndrome, the immune system attacks your own body. Thus, it is referred to as an autoimmune disorder, &ldquo;auto&rdquo; meaning &ldquo;self&rdquo;. In GBS, the immune system specifically attacks the nerves. The nerves are like wires that control the muscles and if the nerves are damaged, the body loses its ability to control the muscles. Thus, causing muscle weakness, tingling and numbness. The disease is characterized by weakness of the limbs, reduced deep tendon reflexes, sensory symptoms, and paresthesia (burning or prickling sensation) of the upper and lower extremities. Cranial nerves involvement with the facial and bulbar muscles being affected is also a common manifestation of GBS.&nbsp;

The incidence of Guillain-Barre syndrome increases with age and people 50 years and older are at the highest risk of developing GBS. An average of 3,000 to 6,000 people develop GBS each year in the United States<a href="#accordion">[1]</a>. Whereas in Pakistan, the overall incidence of GBS is found to be 1.1/100,00/year to 1.8/100,000/year<a href="#accordion">[2]</a>. However, this incidence increases after the age of 50 years from 1.7/100,00/year to 3.3/100,000/year<a href="#accordion">[2]</a>. There has been a significant increase in the incidence of GBS during the COVID-19 pandemic. Similarly, there have been several cases that have shown an association between GBS and <a href="https://www.merisehat.pk/disease/covid-19" target="_blank">COVID-19</a><a href="#accordion">[3]</a>. 
&nbsp;

GBS is classified into different clinical types, listed below:&nbsp; 
&nbsp;

<ul>
	<li>Classical Sensorimotor Guillain-Barre Syndrome: Classic GBS presents with numbness associated with pain, burning or a tingling sensation and sensory loss. It is often associated with weakness in the legs that progresses to the arms and cranial muscles (muscles of the head and neck). In most patients, the reflexes are either decreased or absent. Similarly, a malfunction of the autonomic nervous system is also common and may include blood pressure and heart rate instability, in addition to bowel or bladder dysfunction and pupillary dysfunction. The pain reported is often either muscular, radicular (affects the root of the spinal cord) or neuropathic (disturbance of function or change in several nerves). The onset of the disease is acute and typically patients reach disability within a maximum of 2 weeks.&nbsp; 
	&nbsp;</li>
	<li>Paraparetic Guillain-Barre Syndrome: This is a rare type of GBS which presents with leg weakness and absence of deep tendon reflexes (areflexia). Along with an absence of neurological involvement of the respiratory muscles, cranial nerves, and arms. The onset of the disease is characterized by lower back, buttock, or leg pain with flaccid paralysis in the absence of sensory disturbance. 
	&nbsp;</li>
	<li>Acute Inflammatory Demyelinating Polyneuropathy (AIDP): This type of GBS presents with progressive areflexic weakness and mild sensory changes. 
	&nbsp;</li>
	<li>Acute Motor Axonal Neuropathy (AMAN): Acute motor axonal neuropathy is a non-inflammatory disease type of Guillain-Barre syndrome in which the axons of the motor nerve cells are targeted selectively and destroyed by the body&rsquo;s immune system. This type of GBS is characterized by acute progressive motor weakness, oculomotor dysfunction, ataxia, areflexia and absence of sensory symptoms. Acute motor axonal neuropathy is typically preceded by an infection, for example, Haemophilus influenzae. 
	&nbsp;</li>
	<li>Miller Fisher Syndrome: Miller Fisher syndrome is a rare, acquired nerve disease and is considered a variant of Guillain-Barre syndrome. In this type, paralysis begins in the eye<a href="#accordion">[1]</a>. It is also characterized by the absence of tendon reflexes and abnormal muscle coordination. The symptoms, like Guillain-Barre syndrome, are preceded by a viral infection. Other symptoms may include muscle weakness and respiratory failure. 
	&nbsp;</li>
	<li>Pharyngeal Cervical Brachial: This is a rare variant of Guillain-Barre syndrome. It initially presents with muscle weakness that involves the upper extremities, neck, and oropharynx. 
	&nbsp;</li>
	<li>Bickerstaff Brainstem Encephalitis: This is a rare autoimmune disease of the peripheral and central nervous system and is considered a variant of Guillain-Barre syndrome. It is characterized by an acute triad of ataxia, ophthalmoplegia and encephalopathy. 
	&nbsp;</li>
</ul>

&nbsp;

Signs And Symptoms

Guillain-Barre syndrome is characterized by tingling and weakness in the feet and legs that spreads to the upper body and extremities. The symptoms may start in the arms or face, but the weakness can turn into paralysis as the syndrome progresses. The most significant weakness is typically experienced two weeks after the symptoms begin.

&nbsp;

The signs and symptoms of Guillain-Barre syndrome may include:&nbsp;

<ul>
	<li>Pins and needle sensation in the finger, toes, ankles, or wrists</li>
	<li>Weakness in the lower extremities that spreads to the upper extremities</li>
	<li>Inability to walk and climb the stairs&nbsp;</li>
	<li>Difficulty with chewing, swallowing, speaking, and other facial movements&nbsp;</li>
	<li>Inability to move the eyes and double vision&nbsp;</li>
	<li>Severe, cramp-like pain that may be worse at night</li>
	<li>Inability to control bowel function and bladder</li>
	<li>Increased heart rate&nbsp;</li>
	<li>High or low blood pressure&nbsp;</li>
	<li>Difficulty breathing</li>
</ul>

&nbsp;

Causes

The exact cause of Guillain-Barre syndrome is not yet elucidated, and the disorder typically precedes a respiratory or digestive tract infection. For example, cases of Guillain-Barre syndrome have been reported following infections with the Zika virus. The syndrome may also occur following infection with <a href="https://www.merisehat.pk/disease/covid-19" target="_blank">COVID-19</a>. It is also a rare reaction in those receiving the AstraZeneca and Johnson &amp; Johnson vaccines.&nbsp;

In Guillain-Barre syndrome, the immune system begins attacking the nerves and the nerves covering or the myelin sheath. This damage to the myelin sheath prevents the nerves from transmitting signals to the brain, causing weakness, paralysis, and numbness. While the exact cause of GBS is not yet elucidated, there are several factors that can trigger GBS, these include:&nbsp; 
&nbsp;

<ul>
	<li>Diarrhea Or A&nbsp; Respiratory Illness: Guillain-Barre syndrome is preceded by diarrhea or a respiratory illness, in about 2 in every 3 people.&nbsp; 
	&nbsp;</li>
	<li>Viral Infections: the flu viruses, cytomegalovirus, Zika virus, and Epstein Barr virus can also trigger GBS. 
	&nbsp;</li>
	<li>Vaccination: In very rare cases, people have developed GBS a few days or weeks post-COVID vaccinations (Ad.26.COV2.S (Janssen) vaccine). However, the benefits of getting vaccinated far outweigh the risks, for example, studies have shown the chances of getting GBS after the flu are higher than after getting vaccinated against the flu.&nbsp; 
	&nbsp;</li>
	<li>Campylobacter Jejuni: Infection with campylobacter jejuni which presents with diarrhea is a common cause of GBS. In the United States, 1 in every 1000 people with the infection get GBS<a href="#accordion">[4]</a>. In addition, 1 in every 20 people with GBS has a recent history of Campylobacter infection<a href="#accordion">[4]</a>. 
	&nbsp;</li>
</ul>

<h4>"Your respiratory muscles can slowly get affected in GBS. Head on over to the <a href="https://www.merisehat.pk/page/sehat-scan/" target="_blank">sehat scan</a> and see how your respiratory rate is doing!" </h4>

Diagnosis

The diagnosis of GBS is based on clinical history, examination, and investigations in the absence of specific disease biomarkers. The investigations include CSF examination and electrodiagnostic studies. The diagnostics criteria for GBS were developed by the National Institute of Neurologic Disorders and Stroke (NINDS) in 1978 and revised in 1990. The diagnostics criteria were further updated by the Brighton Collaboration in 2011. Both the criteria were designed for investigating the epidemiological association between GBS and vaccinations but are now used for clinical studies and trials alike.&nbsp; 
&nbsp;

<h5>Laboratory Investigations&nbsp;</h5>

Laboratory testing is advised in all patients suspected of GBS. The tests include:&nbsp;

<ul>
	<li>Complete blood counts</li>
	<li>Blood tests for glucose&nbsp;</li>
	<li>Electrolytes&nbsp;</li>
	<li>Kidney function&nbsp;</li>
	<li>Liver enzymes&nbsp;</li>
</ul>

The results of the tests are used for ruling out other causes of acute flaccid paralysis, for example, metabolic or electrolyte dysfunctions. Further specific tests may be carried out to exclude other diseases that can mimic GBS.&nbsp;

A positive test result on serum levels of antiganglioside antibodies is helpful when the diagnosis is in doubt. However, a negative test result does not rule out the diagnosis of GBS. In 90% of the patients, anti-GQ1b antibodies are found in Miller Fisher syndrome, thus, having a greater diagnostic value in patients with this variant<a href="#accordion">[5]</a>.&nbsp;

&nbsp;

<h5>Cerebrospinal Fluid (CSF) Examination</h5>

CSF examination is performed during the initial evaluation of the patient and is used for ruling out other causes of weakness.&nbsp;Classic findings of GBS include elevated CSF protein level and a normal CSF count, which is also known as albumin-cytological dissociation. However, in 30-50% of the patients, the protein levels are normal during the first week of disease onset and in 10-30% of the patients in the second week<a href="#accordion">[6]</a>.&nbsp;

Normal CSF protein levels, therefore, do not rule out the possibility of GBS. An increased white cell count in CSF examination, though consistent with the diagnosis of GBS, suggests other pathologies, for example, leptomeningeal malignancy or other inflammatory or infectious diseases of the spinal cord and nerve roots.&nbsp; 
&nbsp;

<h5>Electrodiagnostic Studies&nbsp;</h5>

Electrodiagnostic studies, although not required for the diagnosis of GBS, are recommended to support the diagnosis in patients with an atypical presentation. In addition, it is used for differentiating between the three classical subtypes of GBS: AIDP, AMAN and acute motor and sensory axonal neuropathy.&nbsp;&nbsp;

&nbsp;

<h5>Imaging&nbsp;</h5>

Although MRI is not a routine diagnostic criterion of GBS, it can be helpful for excluding other differential diagnoses, for example, leptomeningeal malignancy, nerve root compression, spinal cord or anterior horn cell inflammation, stroke, and brainstem infection.

Similarly, the presence of nerve root enhancement on MRI can support the diagnosis of GBS, especially in young children when both clinical and electrophysiological assessment can be challenging.&nbsp;

MRI can further be used to distinguish between acute flaccid myelitis and GBS in young children. Ultrasound imaging of the peripheral nerves early in the disease course can highlight spinal root inflammation and help establish a diagnosis of GBS.&nbsp; 
&nbsp;

Treatment

The most common treatment approaches for Guillain-Barre syndrome include:&nbsp; 
&nbsp;

<ul>
	<li>Intravenous Immunoglobulin (IVIG): In this treatment, donated blood that contains healthy antibodies is transfused into a vein to help stop the harmful antibodies from damaging the nerves.&nbsp; 
	&nbsp;</li>
	<li>Plasma Exchange (Plasmapheresis): A plasma exchange is used instead of IVIG in which a machine is used to remove blood from a vein to filter out harmful antibodies that are attacking the nerves. 
	&nbsp;</li>
</ul>

Other treatments in the hospital may include:&nbsp;

<ul>
	<li>Ventilator if the patient has difficulty breathing</li>
	<li>A feeding tube if the patient has difficulty swallowing&nbsp;</li>
	<li>Painkillers for pain&nbsp;</li>
	<li>Catheter in the urethra to carry out urine from the body if the patient has difficulty urinating</li>
	<li>Laxatives for constipation</li>
	<li>Medication and leg stockings to prevent blood clots 
	&nbsp;</li>
</ul>

<ul>
	<li>Breathing difficulties&nbsp;</li>
	<li>Numbness&nbsp;</li>
	<li>Irregular heart rhythms&nbsp;</li>
	<li>Pain&nbsp;</li>
	<li>Bowel and bladder function problems&nbsp;</li>
	<li>Blood clots&nbsp;</li>
	<li>Pressure sores&nbsp;</li>
	<li>Relapse&nbsp;</li>
</ul>

&nbsp;

GBS can affect all age groups, however, the risk increases as you age. Other factors that may increase the risk of GBS include:&nbsp;

<ul>
	<li>Campylobacter infection&nbsp;</li>
	<li><a href="https://www.merisehat.pk/disease/influenza-116" target="_blank">Influenza </a>virus&nbsp;</li>
	<li>Cytomegalovirus&nbsp;</li>
	<li>Epstein-Barr virus&nbsp;</li>
	<li>Zika virus&nbsp;</li>
	<li><a href="https://www.merisehat.pk/disease/hepatits-abcde-59" target="_blank">Hepatitis A</a>, <a href="https://www.merisehat.pk/disease/hepatitis-b-113" target="_blank">B</a>, <a href="https://www.merisehat.pk/disease/hepatitis-c-114" target="_blank">C </a>and <a href="https://www.merisehat.pk/disease/hepatits-abcde-59" target="_blank">E&nbsp;</a></li>
	<li><a href="https://merisehat.pk/disease/hiv" target="_blank">HIV&nbsp;</a></li>
	<li><a href="https://www.merisehat.pk/disease/pneumonia" target="_blank">Pneumonia&nbsp;</a></li>
	<li><a href="https://www.merisehat.pk/disease/hodgkins-lymphoma" target="_blank">Hodgkin&rsquo;s lymphoma&nbsp;</a></li>
	<li><a href="https://www.merisehat.pk/disease/covid-19" target="_blank">COVID-19</a> virus&nbsp;</li>
	<li>Johnson &amp; Johnson and AstraZeneca vaccine (rare)&nbsp;</li>
	<li>Influenzas vaccination (rare)</li>
	<li>Surgery&nbsp;</li>
	<li>Trauma</li>
</ul>

&nbsp;

Key Points

<ul>
	<li>Guillain-Barre syndrome is a rare polyradiculoneuropathy in which the immune system of the body attacks the nerves with resultant weakness and diminished reflexes.</li>
	<li>The incidence of Guillain-Barre syndrome increases with age and people 50 years and older are at the highest risk of developing GBS.</li>
	<li>In Pakistan, the overall incidence of GBS is found to be 1.1/100,00/year to 1.8/100,000/year. There has been a significant increase in the incidence of GBS during the COVID-19 pandemic.</li>
	<li>Guillain-Barre syndrome is characterized by tingling and weakness in the feet and legs that spreads to the upper body and extremities. The symptoms may start in the arms or face, but the weakness can turn into paralysis as the syndrome progresses.</li>
	<li>The most common treatment approaches for Guillain-Barre syndrome include intravenous immunoglobulin and plasmapheresis. Researchers have not yet determined how to prevent Guillain-Barre syndrome.</li>
</ul>

&nbsp;

[1] Centers for Disease Control and Prevention, 2022. Guillain-Barre Syndrome and Vaccines.

[2] Samar, S.S., Ahmed, S.I. and Bareeqa, S.B., 2018. Guillain–Barré syndrome in Pakistan: A short review of literature. J Neurol Neurorehabil Res. 2018; 3 (1): 34-35. J Neurol Neurorehabil Res 2018 Volume 3 Issue, 1.

[3] Randhawa, J., Randhawa, H.S. and Toor, P., 2021. Pharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome in a Patient of COVID-19 Infection. Cureus, 13(9).

[4] Centers for Disease Control and Prevention, 2022. Guillain-Barre Syndrome.

[5] Yoshikawa, K., Kuwahara, M., Morikawa, M., Fukumoto, Y., Yamana, M., Yamagishi, Y. and Kusunoki, S., 2018. Varied antibody reactivities and clinical relevance in anti-GQ1b antibody–related diseases. Neurology-Neuroimmunology Neuroinflammation, 5(6).

[6] Fokke, C., van den Berg, B., Drenthen, J., Walgaard, C., van Doorn, P.A. and Jacobs, B.C., 2014. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain, 137(1), pp.33-43.

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