Guillain Barre Syndrome  - Know It All!

Editorial

All you need to know about Guillain-Barre Syndrome.

Know your ailment well, so you can manage it better!!

Here we come with Guillain-Barre Syndrome today!

Guillain Barre Syndrome 

What is Guillain-Barre Syndrome?

Guillain-Barré syndrome (GBS) is a rare neurological condition in which part of the peripheral nervous system, the network of nerves beyond the brain and spinal cord, is mistakenly attacked by the body’s immune system. GBS can range from a very mild case of brief weakness to almost devastating paralysis, leaving the individual unable to independently breathe. Luckily, most individuals fully recover from even the most extreme cases of GBS. Some individuals may continue to have some degree of impairment after recovery.

In this, the nerves are attacked by the body’s immune system. The first signs generally include fatigue and tingling in the extremities.

Anyone may be affected by Guillain-Barré syndrome It may occur at any age (although in adults and older people it is more frequent) and both sexes are equally vulnerable to the disorder. It is estimated that GBS affects around one in 100,000 individuals per year.

These sensations will spread rapidly, paralysing the entire body eventually. Guillain-Barre syndrome is a medical emergency in its most severe form. To receive medication, most persons with the disorder must be hospitalised.

There is no known exact cause of Guillain-Barre syndrome. But in the intervening six weeks, two-thirds of patients show signs of infection which includes respiratory or gastrointestinal or Zika virus infections.

Guillain-Barre syndrome has no proven cure, although some therapies can relieve symptoms and minimise the duration of the disease. The mortality rate is 4 percent to 7 percent, but most individuals recover from Guillain-Barre syndrome. 60–80% of people are able to walk at six months of age. Patients, such as weakness, numbness or exhaustion, can suffer residual effects from it.

Guillain Barre Syndrome 

What are the causes of Guillain-Barre Syndrome?

A post-postinfectious, immune-mediated disease affecting peripheral nerves is known to be GBS. Prior to the onset of neurologic symptoms, up to two-thirds of patients record a previous bacterial or viral disease. Most commonly, respiratory infections are recorded, followed by gastrointestinal infections. GBS has also been linked with the administration of many vaccines and other systemic diseases. There are case reports about various drugs and procedures; however it is unclear if there is any causal connection.

C Jejuni

In several studies, the most frequently isolated pathogen in GBS was C jejuni. In a Dutch GBS trial, serology studies reported 32 percent of patients as having a recent C jejuni infection, while studies recorded infection rates as high as 60 percent in northern China.

Infections of C jejuni may be observed with gastrointestinal and upper respiratory tract symptoms. Infections with C jejuni can also occur subclinically, resulting in patients with no recorded infectious symptoms prior to GBS development. Patients with a history of C jejuni infection who develop GBS often have a more severe course, with rapid progression and prolonged, incomplete recovery. A good clinical association between infections with C jejuni and pure motor and axonal forms of GBS has been noted.

It is assumed that the virulence of C jejuni stems from the presence of unique antigens that are shared with nerves in its capsule. Antibodies that cross-react with myelin to cause demyelination are produced by immune responses directed against capsular lipopolysaccharides.

Infections with C jejuni also produce anti-ganglioside antibodies, including GM1, GD1a, GalNac-GD1a, and GD1b gangliosides, which are usually found in patients with GBS axonal subtypes, AMAN and AMSAN. (However, patients with C. jejuni enteritis who do not have GBS complications do not develop specific anti-ganglioside antibodies.)

However, even in the subgroup of GM1 antibody patients, the clinical manifestations vary. In the development of GBS after infectious disease, host vulnerability is possibly one determinant.

While GM1 antibodies can also be identified in patients with demyelinating GBS, in these cases, they are far less frequent. C. Infection with jejuni may also develop antibodies to the myelin portion of the oculomotor nerve, ganglioside GQ1b, which is associated with MFS.

CMV

Infections with cytomegalovirus (CMV) are the second most frequently identified infections that precede GBS, with the most common viral cause of GBS being CMV. In 13 percent of patients, the aforementioned Dutch GBS study found CMV to be present.

Infections of CMV are present as infections of the upper respiratory tract, pneumonia, and nonspecific flu like diseases. GBS patients with prior CMV infections also have prominent sensory and cranial nerve involvement. CMV infections are significantly associated with ganglioside GM2 antibodies.

COVID-19

There is evidence that the occurrence of neurological complications, including GBS, is related to coronavirus disease 2019 (COVID-19). By April 20, 2020, one case of GBS had been identified from China in a patient with COVID-19 and five such cases were reported from Italy. A study on Italian cases stated that 5–10 days after diagnosis of COVID-19, GBS emerged, with three of the patients having a demyelinating form of GBS and the other two having an axonal variant emerging.

The first US case study of a patient with COVID-19-associated GBS was released in June 2020. Soon after the person became infected with serious acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus that causes COVID-19, the syndrome progressed, with test results showing that he had the demyelinating form of GBS.

Additional infections:

Epstein-Barr virus (EBV), Mycoplasma pneumonia and varicella-zoster virus are other significant, but less frequently detected, infectious agents in GBS patients. It is also well known that there is a correlation between GBS and acute human immunodeficiency virus (HIV) infection.

Patients with GBS have been shown to have infections with Haemophilus influenza, Borrelia burgdorferi, para-influenza virus type 1, influenza A virus, influenza B virus, adenovirus, and herpes simplex virus, but not more often than in controls.

There has been speculation that GBS may be caused by the Zika virus. During the Zika virus outbreak that was detected there in 2015, confirmed cases of the syndrome started to rise in Brazil, with hundreds of cases of GBS reported that year. In July 2015, for example, of 76 patients diagnosed with neurological syndromes in the state of Bahia, 42 were confirmed to have GBS, with a history of symptoms consistent with Zika virus infection in 26 of these confirmed cases.

Increases in GBS cases were also documented by other Latin American countries to which the Zika outbreak spread, including Colombia, Venezuela, and El Salvador. In El Salvador, where an average of 14 GBS cases are reported per month, between December 1, 2015 and January 6, 2016, 46 cases were reported. [44] Barbi et al’s literature review reported that 1.23 percent of cases of Zika virus could grow into GBS.

To settle speculation about the Zika-GBS connection, however, additional research will be needed. The reliability of the diagnosis of Zika virus outside the United States is not understood. The only infectious disease laboratories capable of making this diagnosis in the United States are at the US Centers for Disease Control and Prevention (CDC) and a few departments of state or local health. There is no commercially available Zika virus test at present.

The Vaccines

Vaccinations were related by the temporal association to GBS. For example, adjusted relative risk of 1.7 cases per 1 million influenza vaccinations was found in a study reviewing GBS cases during the 1992–1993 and 1993–1994 influenza seasons.

However in most cases, with the exception of the rabies vaccine prepared from infected brain tissue and the 1976 swine flu vaccine, no definite causal association has been identified between vaccines and GBS. (However after the swine flu vaccination, the increased risk of GBS was only one extra case per 100,000 vaccinations.

In addition, a study of all GBS post-vaccination cases from 1990–2005 did not show a rise in mortality for GBS post-vaccination cases relative to other causes.

Medicines

In a case-controlled study, GBS patients indicated more frequent use of penicillin and antimotility drugs and less frequent use of oral contraceptives. No clear relationships of cause and effect have been identified, however.

In the background of tumour necrosis factor antagonist agents used in rheumatoid arthritis, case reports exist. There are also case reports on, among others, streptokinase, isotretinoin, danazol, captopril, gold, and heroin.

A study by Ali showed that fluoroquinolone antibiotic therapy is also associated with the development of GBS. Using cases reported to the US Food and Drug Administration (FDA) Adverse Reporting System between 1997 and 2012, he determined that 9 percent of 539 reports of peripheral neuropathy associated with fluoroquinolone treatment were for patients with GBS.

Other Factors

As potential causes of GBS, various events, such as surgery, trauma, and pregnancy, have been identified, but these associations remain largely anecdotal.

Associations between bariatric and other gastric procedures, renal transplantation, and epidural anaesthesia are cited in case reports.

Systemic lupus erythematosus, sarcoidosis, lymphoma, and snakebite have anecdotal connections.

With increased expression, tumour necrosis factor-alpha polymorphisms are associated with many autoimmune and inflammatory diseases and may increase susceptibility to subtypes of axonal GBS. The position of these polymorphisms in GBS, however, remains unclear and justifies further study.

Guillain Barre Syndrome 

What are the types of Guillain-Barre Syndrome?

  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the most common form in North America and Europe. The most common sign of AIDP is muscle weakness that starts in the lower part of your body and spreads upward.
  • Miller Fisher syndrome (MFS), in which paralysis starts in the eyes. MFS is also associated with an unsteady gait. MFS is less common in the U.S. but more common in Asia.
  • Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are less common in the U.S. But AMAN and AMSAN are more frequent in China, Japan and Mexico.

What are the symptoms of Guillain-Barre Syndrome?

Tingling and weakness starting in your feet and legs and spreading to your upper body and arms often begin with Guillain-Barre syndrome. Symptoms begin in the arms or face in about 10 percent of individuals with the condition. Muscle weakness can develop into paralysis as Guillain-Barre syndrome progresses.

There may be signs and symptoms of Guillain-Barre syndrome:

  • Feelings of prickling, pins and needles in your fingers, toes, ankles or wrists
  • Weakness that extends to the upper body in the legs
  • Unstable walking or being unable to walk or climb stairs
  • Facial movements trouble, like speaking, chewing or swallowing
  • Double vision or eye control failure
  • Extreme pain that may feel achy, shot, or cramped, which may be worse at night
  • Bladder management or bowel function problems
  • Fast Heart Rate
  • Blood pressure that is low or high
  • Breathing Difficulty
  • After symptoms begin, people with Guillain-Barre syndrome usually experience their most significant weakness within two weeks.

What are the risk factors of Guillain-Barre Syndrome?

Acute flaccid myelitis mainly affects young children.

What are the complications of Guillain-Barre Syndrome?

Guillain-Barre syndrome can affect all age groups. But your risk increases as you age. It’s also more common in males than females.

Guillain-Barre syndrome may be triggered by:

  • Most commonly, infection with campylobacter, a type of bacteria often found in undercooked poultry
  • Influenza virus
  • Cytomegalovirus
  • Epstein-Barr virus
  • Zika virus
  • Hepatitis A, B, C and E
  • HIV, the virus that causes AIDS
  • Mycoplasma pneumonia
  • Surgery
  • Trauma
  • Hodgkin’s lymphoma
  • Rarely, influenza vaccinations or childhood vaccinations
  • COVID-19 infection
Guillain Barre Syndrome 

How is Guillain-Barre Syndrome diagnosed?

The initial signs and symptoms of GBS are various, and with common symptoms, there are many disorders. Doctors can also find it hard to diagnose GBS in its earliest stages.

Doctors will notice whether the symptoms occur on both sides of the body (a common finding in Guillain-Barré syndrome) and how rapidly the symptoms appear (muscle weakening will progress over months rather than days or weeks in other disorders). Normally, deep tendon reflexes in the legs, such as knee jerks, are lost in GBS. In the arms, reflexes can also be absent. A nerve conduction velocity test (NCV, which tests the capacity of the nerve to transmit a signal) may provide clues to support the diagnosis, since the signals flowing through the nerve are sluggish. In people with GBS, there is a change in the cerebrospinal liquid that bathes the spinal cord and brain. Researchers also discovered that the fluid contains more protein than normal (measured by white blood cells) but very few immune cells. A physician may therefore decide to conduct a spinal tap or lumbar puncture to obtain a spinal fluid sample to be analysed. A needle is inserted into the lower back of the person in this operation and a small volume of cerebrospinal fluid is removed from the spinal cord. Normally, this procedure is healthy, with rare complications.

Primary results for diagnosis include:

  • Recent onset of symmetrical weakness within days to at most four weeks, usually beginning in the legs
  • Abnormal sensations that follow or even occur before fatigue, such as pain, numbness and tingling in the feet
  • In weak muscles, missing or reduced deep tendon reflexes
  • It can take up to 10 days from the start of symptoms to produce an elevated cerebrospinal fluid protein without an elevated cell count.
  • Abnormal findings of nerve conduction level, such as slow conduction of the signal
  • A new viral infection or diarrhoea, occasionally.
Guillain Barre Syndrome 

What is the treatment for Guillain-Barre Syndrome?

Guillain-Barre syndrome has no treatment. But two types of therapies can speed recovery and decrease the severity of the disease:

Exchange of plasma (plasmapheresis).

Your blood cells extract and isolate the liquid part of your blood (plasma). Then the blood cells are inserted back into the body, which creates more plasma to compensate for what has been lost. Plasmapheresis can operate by ridding plasma of some antibodies that lead to the attack on the peripheral nerves by the immune system.

Therapy of immuno-globulin.

Blood donor immunoglobulin containing safe antibodies is delivered through a vein (intravenously). High doses of immunoglobulin may be able to block toxic antibodies that may lead to Guillain-Barre syndrome.

Equally successful are these therapies. It is no more productive to combine them or to administer one after the other than to use either form alone.

Medication is also likely to be issued to:

Pain relief, which can be serious,

Prevent the production of blood clots when you are immobile.

Before and during recovery, people with Guillain-Barre syndrome require physical assistance and rehabilitation.

Your treatment can involve the following:

  • Before recovery, caregivers shift your arms and legs to help keep your muscles flexible and powerful.
  • Physical therapy to help you deal with exhaustion during rehabilitation and regain strength and proper movement
  • Training with adaptive equipment to give you mobility and self-care skills, such as a wheelchair or braces

How to Cope:

A Guillain-Barre syndrome diagnosis can be emotionally complicated. Although most individuals do completely recover, the disorder is normally painful and requires hospitalisation and months of rehabilitation. You must adapt to restricted mobility and tiredness.

Consider some recommendations in order to handle the burden of recovery from Guillain-Barre syndrome:

  • Maintain a powerful friend and family support system
  • Contact a support group for yourself or for family members
  • Discuss with a psychologist about your concerns

References:

All Images used are for representation purposes and are obtained from google search and we do not intend to violate copyrights, all credits are due with respective content owners. If you wish to take credit or intend to remove the image, Kindly let us know in the comments.

https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/diagnosis-treatment/drc-20363006

https://emedicine.medscape.com/article/315632-differential

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet

https://www.webmd.com/brain/what-is-guillain-barre#1