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Guillain-Barré Syndrome
GBS
1
Contents
• Definition
• Ethology
• Pathophysiology
• Risk factor
• Sign and symptom
• Complication
• Diagnosis
• Management / Treatment
• Prognosis
• Conclusion
• Reference
2
GBS
• Guillain–Barré syndrome (GBS) is an acute onset, usually
monophasic immune-mediated disorder of the peripheral
nervous system
• GBS is a rare disorder in which your body's immune system
attacks your nerves. Weakness and tingling in your hands and
feet are usually the first symptoms.
• These sensations can quickly spread, eventually paralyzing
your whole body. In its most severe form Guillain-Barre
syndrome is a medical emergency. Most people with the
condition must be hospitalized to receive treatment.
3
Ethology
• Although the cause of Guillain-Barré syndrome is not fully
understood, it is thought to be autoimmune.
• In about two thirds of patients, Guillain-Barré syndrome
begins 5 days to 3 weeks after a banal infectious disorder,
surgery, or vaccination. Infection is the trigger in > 50% of
patients; common pathogens include :
 Bacterial
 Campylobacter jejuni which causes diarrhea (often from undercooked
infected chicken)
 Haemophilus influenzae which causes chest infection
4
Cont..
 Virus
o Herpesviruses (including cytomegalovirus and Epstein-Barr
virus)
• cytomegalovirus which causes chest infection or
glandular fever
• Epstein-Barr virus which causes glandular fever
o Hepatitis E which causes jaundice
o Zika virus which causes fever, rash and joint pain
• In some patients, Guillain-Barré syndrome has developed
after a respiratory or a gastrointestinal infection, Zika virus
infection or after COVID-19.
5
Pathophysiology
• The cause of GBS is inflammation of the peripheral nerves. These
nerves normally take messages to and from the skin and muscles to
the brain and spinal cord.
• There is strong evidence that the cause is autoimmune. The
immune system produces an immune response to an infection
which cross-reacts with the nerves.
• It usually reacts with and damages the outer coating sheath of the
nerve fibers, called myelin. In more severely affected people, this
damage also affects the central conducting core of the nerve, called
the axon.
• In some people the axon is itself the main target of the
autoimmune response.
6
Cont..
There are three different forms of GBS:
• Acute inflammatory demyelinating polyradiculoneuropathy -
which predominantly affects the myelin. The commonest form
in America and Europe.
• Acute motor axonal neuropathy - which affects the axons of
the nerves going to the muscles. The commonest form in
some Asian countries.
• Acute motor and sensory axonal neuropathy - which affects
the axons of the motor and sensory nerves.
 The different forms have similar symptoms, signs and disease courses
except for the absence of sensory symptoms and signs in acute motor
axonal neuropathy. They are distinguished by nerve conduction tests.
7
Risk factors
• Guillain-Barre syndrome can affect all age groups, but your
risk increases as you age. It's also slightly more common in
males than females. GBS may be triggered by
o Most commonly, infection with campylobacter, a type of
bacteria often found in undercooked poultry
o Influenza virus, Cytomegalovirus, Epstein-Barr virus, Zika virus
o Hepatitis A, B, C and E
o HIV, the virus that causes AIDS
o Mycoplasma pneumonia
o Surgery
o Trauma
o COVID-19 virus
o COVID-19 Johnson & Johnson and AstraZeneca vaccine
8
Signs & Symptoms
• Guillain-Barre syndrome often begins with tingling and
weakness starting in your feet and legs and spreading to your
upper body and arms.
• Some people notice the first symptoms in the arms or face. As
Guillain-Barre syndrome progresses, muscle weakness can
turn into paralysis. Symptoms can progress over hours, days,
or weeks.
9
Cont…
Signs and symptoms of GBS may include:
o A pins and needles sensation in your fingers, toes, ankles or wrists
o Weakness in your legs that spreads to your upper body
o Unsteady walking or inability to walk or climb stairs
o Difficulty with facial movements, including speaking, chewing or
swallowing
o Double vision or inability to move the eyes
o Severe pain that may feel achy, shooting or cramplike and may be
worse at night
o Difficulty with bladder control or bowel function
o Rapid heart rate
o Low or high blood pressure
o Difficulty breathing
10
Complications
Guillain-Barre syndrome affects your nerves. Because nerves
control your movements and body functions, people with
Guillain-Barre may experience:
 Breathing difficulties - The weakness or paralysis can spread
to the muscles that control your breathing, a potentially fatal
complication.
 Heart and blood pressure problems - Blood pressure
fluctuations and irregular heart rhythms (cardiac arrhythmias)
are common side effects of Guillain-Barre syndrome.
 Bowel and bladder function problems - Sluggish bowel
function and urine retention may result from Guillain-Barre
syndrome
11
Cont..
 Blood clots - People who are immobile due to Guillain-Barre
syndrome are at risk of developing blood clots. Until you're
able to walk independently, taking blood thinners and
wearing support stockings may be recommended.
 Pain - One-third of people with Guillain-Barre syndrome
experience severe nerve pain, which may be eased with
medication.
• Severe, early symptoms of Guillain-Barre syndrome
significantly increase the risk of serious long-term
complications. Rarely, death may occur from complications
such as respiratory distress syndrome and heart attacks.
12
Diagnosis
Guillain-Barre syndrome can be difficult to diagnose in its earliest stages. Its
signs and symptoms are similar to those of other neurological disorders and
may vary from person to person.
 Diagnosis of Guillain-Barré syndrome is primarily clinical
 Examination
• ask about symptoms, such as how long they've lasted and whether
they're getting worse, muscle weakness that's getting worse over
time is a common sign of Guillain-Barré syndrome
• ask if recently been ill – Guillain-Barré syndrome often follows an
infection such as food poisoning or flu
• check reflexes, such as whether leg twitches when knee is tapped in a
particular place - people with Guillain-Barré syndrome usually have
no or reduced reflexes
13
Cont…
 Nerve tests
Tow tests may be carried out to see how well your nerves are
working:
Electromyography (EMG)
• Thin-needle electrodes are inserted into the muscles your
doctor wants to study. The electrodes measure nerve activity
in the muscles.
Nerve conduction studies
• Electrodes are taped to the skin above your nerves. A small
shock is passed through the nerve to measure the speed of
nerve signals.
14
Cont…
 Spinal tap (lumbar puncture)
• A small amount of fluid is withdrawn from the spinal canal in
your lower back. The fluid is tested for a type of change that
commonly occurs in people who have Guillain-Barre
syndrome
• The sample of fluid will be checked for signs of problems that
can cause similar symptoms to Guillain-Barré syndrome, such
as an infection
15
Management
Goal of therapy
 Monitor disease progression and the occurrence of complications
 Decrease mortality rate or to improves survival
 Reduce the symptoms and speed up recovery
 To control pain and other conditions that may be present
16
Supportive care
• If breathing becomes difficult, mechanical ventilation with a
breathing machine in an intensive care unit becomes
necessary. For this, a special plastic tube, called an
endotracheal tube, connects the person to the breathing
machine via the mouth or nose.
• Deep vein thrombosis prophylaxis, All patients should be
given subcutaneous fractionated or unfractionated heparin
and support stockings until they are able to walk
independently to prevent deep vein thrombosis
17
Cont..
• Pain and sensory symptoms are reported in majority of
patients with GBS and should be treated with analgesic
regimens, Gabapentin or carbamazepine are useful for pain
management in the acute phase of GBS
• Nutrition - Nasogastric or gastric tube feeding should be
instituted early and slowly. High energy (40–45 nonprotein
kcal) and high protein diet (2–2.5 g/kg) have been
recommended so has to reduce muscle wasting and assist
respiratory weaning
18
Treatment
• There's no cure for Guillain-Barre syndrome. But two types of
treatments can speed recovery and reduce the severity of the
illness:
1. Plasma exchange (plasmapheresis)
 A plasma exchange, also called plasmapheresis, is sometimes
used instead of IVIG
 This involves being attached to a machine that removes blood
from a vein and filters out the harmful antibodies that are
attacking your nerves before returning the blood to your body
• Most people need treatment over the course of around 5 days
19
Cont..
• Plasma exchange helps when done early; it is used if IVIG is
ineffective.
• Plasma exchange shortens the disease course and hospital
stay, and reduces mortality risk and incidence of permanent
paralysis.
• However, it may cause hypotension due to large fluid shifts,
and IV access may be difficult or cause complications.
• Plasma exchange removes any previously administered IVIG,
negating its benefits, and so should never be done during or
soon after use of IVIG.
20
Cont..
2. Immunoglobulin Therapy
 Immunoglobulin containing healthy antibodies from blood
donors is given through a vein (intravenously). High doses of
immunoglobulin can block the damaging antibodies that may
contribute to Guillain-Barre syndrome.
 Given early, IVIG 2 g/kg over 1 to 2 days or, more slowly, as
400 mg/kg IV once a day for 5 consecutive days is the
treatment of choice; it has some benefit up to 1 month from
disease onset.
21
Cont…
• IVIG is more convenient and more widely available than PE
but both are equally helpful. Combining the two does not
help more.
• Early treatment within the first two weeks after the onset of
GBS symptoms is preferable.
• Besides IVIg and plasma exchange, no other procedures or
drugs have been proven effective in the treatment of GBS.
• Corticosteroids would be expected to be beneficial in reducing
inflammation, But corticosteroids for GBS showed no
significant benefit, and treatment with oral corticosteroids
was even shown to have a negative effect on outcome
22
Prognosis
• Approximately 85% of patients with GBS achieve a full
functional recovery with in several months to a year
• The mortality rate is <5% in optimal setting; death usually
results from secondary pulmonary complications
• The outlook is worst in patients with sever proximal motor
and sensory axonal damage. Other factors that worsen the
outlook for recovery are advanced age, delayed in the onset
of treatment
• Between 5 to 10% of patients with typical GBS have one or
more late relapses; such case are then classified as chronic
inflammatory demyelinating polyneuropathy (CIDP)
23
Conclusion
• GBS is a monophasic immune-mediated neuropathy
characterized by acute onset of predominantly motor
weakness and is a common cause of respiratory paralysis.
• There are many variants described with different prognosis
and manifestations. Electrodiagnosis aids in the diagnosis.
• Immunotherapy definitely makes a difference in the recovery
of GBS patients and both PE and IVIg are equally effective.
IVIg may be preferred because of its low side-effect profile
and ease of administration.
• Supportive treatment is equally important in reducing the
morbidity and mortality in GBS.
24
Reference
• Guillain-Barré Syndrome ANNE D. WALLING, MD, ChB, and
GRETCHEN DICKSON, MD, MBA University of Kansas School of
Medicine, Wichita, Kansas
• https://www.mayoclinic.org/diseases-conditions/guillain-
barre-syndrome/symptoms-causes/syc-20362793
• https://www.msdmanuals.com/professional/neurologic-
disorders/peripheral-nervous-system-and-motor-unit-
disorders/guillain-barr%C3%A9-syndrome-gbs
• Harrison 21 edition
25
THANK YOU
26

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Guillain-Barré Syndrome.pptx

  • 2. Contents • Definition • Ethology • Pathophysiology • Risk factor • Sign and symptom • Complication • Diagnosis • Management / Treatment • Prognosis • Conclusion • Reference 2
  • 3. GBS • Guillain–Barré syndrome (GBS) is an acute onset, usually monophasic immune-mediated disorder of the peripheral nervous system • GBS is a rare disorder in which your body's immune system attacks your nerves. Weakness and tingling in your hands and feet are usually the first symptoms. • These sensations can quickly spread, eventually paralyzing your whole body. In its most severe form Guillain-Barre syndrome is a medical emergency. Most people with the condition must be hospitalized to receive treatment. 3
  • 4. Ethology • Although the cause of Guillain-Barré syndrome is not fully understood, it is thought to be autoimmune. • In about two thirds of patients, Guillain-Barré syndrome begins 5 days to 3 weeks after a banal infectious disorder, surgery, or vaccination. Infection is the trigger in > 50% of patients; common pathogens include :  Bacterial  Campylobacter jejuni which causes diarrhea (often from undercooked infected chicken)  Haemophilus influenzae which causes chest infection 4
  • 5. Cont..  Virus o Herpesviruses (including cytomegalovirus and Epstein-Barr virus) • cytomegalovirus which causes chest infection or glandular fever • Epstein-Barr virus which causes glandular fever o Hepatitis E which causes jaundice o Zika virus which causes fever, rash and joint pain • In some patients, Guillain-Barré syndrome has developed after a respiratory or a gastrointestinal infection, Zika virus infection or after COVID-19. 5
  • 6. Pathophysiology • The cause of GBS is inflammation of the peripheral nerves. These nerves normally take messages to and from the skin and muscles to the brain and spinal cord. • There is strong evidence that the cause is autoimmune. The immune system produces an immune response to an infection which cross-reacts with the nerves. • It usually reacts with and damages the outer coating sheath of the nerve fibers, called myelin. In more severely affected people, this damage also affects the central conducting core of the nerve, called the axon. • In some people the axon is itself the main target of the autoimmune response. 6
  • 7. Cont.. There are three different forms of GBS: • Acute inflammatory demyelinating polyradiculoneuropathy - which predominantly affects the myelin. The commonest form in America and Europe. • Acute motor axonal neuropathy - which affects the axons of the nerves going to the muscles. The commonest form in some Asian countries. • Acute motor and sensory axonal neuropathy - which affects the axons of the motor and sensory nerves.  The different forms have similar symptoms, signs and disease courses except for the absence of sensory symptoms and signs in acute motor axonal neuropathy. They are distinguished by nerve conduction tests. 7
  • 8. Risk factors • Guillain-Barre syndrome can affect all age groups, but your risk increases as you age. It's also slightly more common in males than females. GBS may be triggered by o Most commonly, infection with campylobacter, a type of bacteria often found in undercooked poultry o Influenza virus, Cytomegalovirus, Epstein-Barr virus, Zika virus o Hepatitis A, B, C and E o HIV, the virus that causes AIDS o Mycoplasma pneumonia o Surgery o Trauma o COVID-19 virus o COVID-19 Johnson & Johnson and AstraZeneca vaccine 8
  • 9. Signs & Symptoms • Guillain-Barre syndrome often begins with tingling and weakness starting in your feet and legs and spreading to your upper body and arms. • Some people notice the first symptoms in the arms or face. As Guillain-Barre syndrome progresses, muscle weakness can turn into paralysis. Symptoms can progress over hours, days, or weeks. 9
  • 10. Cont… Signs and symptoms of GBS may include: o A pins and needles sensation in your fingers, toes, ankles or wrists o Weakness in your legs that spreads to your upper body o Unsteady walking or inability to walk or climb stairs o Difficulty with facial movements, including speaking, chewing or swallowing o Double vision or inability to move the eyes o Severe pain that may feel achy, shooting or cramplike and may be worse at night o Difficulty with bladder control or bowel function o Rapid heart rate o Low or high blood pressure o Difficulty breathing 10
  • 11. Complications Guillain-Barre syndrome affects your nerves. Because nerves control your movements and body functions, people with Guillain-Barre may experience:  Breathing difficulties - The weakness or paralysis can spread to the muscles that control your breathing, a potentially fatal complication.  Heart and blood pressure problems - Blood pressure fluctuations and irregular heart rhythms (cardiac arrhythmias) are common side effects of Guillain-Barre syndrome.  Bowel and bladder function problems - Sluggish bowel function and urine retention may result from Guillain-Barre syndrome 11
  • 12. Cont..  Blood clots - People who are immobile due to Guillain-Barre syndrome are at risk of developing blood clots. Until you're able to walk independently, taking blood thinners and wearing support stockings may be recommended.  Pain - One-third of people with Guillain-Barre syndrome experience severe nerve pain, which may be eased with medication. • Severe, early symptoms of Guillain-Barre syndrome significantly increase the risk of serious long-term complications. Rarely, death may occur from complications such as respiratory distress syndrome and heart attacks. 12
  • 13. Diagnosis Guillain-Barre syndrome can be difficult to diagnose in its earliest stages. Its signs and symptoms are similar to those of other neurological disorders and may vary from person to person.  Diagnosis of Guillain-Barré syndrome is primarily clinical  Examination • ask about symptoms, such as how long they've lasted and whether they're getting worse, muscle weakness that's getting worse over time is a common sign of Guillain-Barré syndrome • ask if recently been ill – Guillain-Barré syndrome often follows an infection such as food poisoning or flu • check reflexes, such as whether leg twitches when knee is tapped in a particular place - people with Guillain-Barré syndrome usually have no or reduced reflexes 13
  • 14. Cont…  Nerve tests Tow tests may be carried out to see how well your nerves are working: Electromyography (EMG) • Thin-needle electrodes are inserted into the muscles your doctor wants to study. The electrodes measure nerve activity in the muscles. Nerve conduction studies • Electrodes are taped to the skin above your nerves. A small shock is passed through the nerve to measure the speed of nerve signals. 14
  • 15. Cont…  Spinal tap (lumbar puncture) • A small amount of fluid is withdrawn from the spinal canal in your lower back. The fluid is tested for a type of change that commonly occurs in people who have Guillain-Barre syndrome • The sample of fluid will be checked for signs of problems that can cause similar symptoms to Guillain-Barré syndrome, such as an infection 15
  • 16. Management Goal of therapy  Monitor disease progression and the occurrence of complications  Decrease mortality rate or to improves survival  Reduce the symptoms and speed up recovery  To control pain and other conditions that may be present 16
  • 17. Supportive care • If breathing becomes difficult, mechanical ventilation with a breathing machine in an intensive care unit becomes necessary. For this, a special plastic tube, called an endotracheal tube, connects the person to the breathing machine via the mouth or nose. • Deep vein thrombosis prophylaxis, All patients should be given subcutaneous fractionated or unfractionated heparin and support stockings until they are able to walk independently to prevent deep vein thrombosis 17
  • 18. Cont.. • Pain and sensory symptoms are reported in majority of patients with GBS and should be treated with analgesic regimens, Gabapentin or carbamazepine are useful for pain management in the acute phase of GBS • Nutrition - Nasogastric or gastric tube feeding should be instituted early and slowly. High energy (40–45 nonprotein kcal) and high protein diet (2–2.5 g/kg) have been recommended so has to reduce muscle wasting and assist respiratory weaning 18
  • 19. Treatment • There's no cure for Guillain-Barre syndrome. But two types of treatments can speed recovery and reduce the severity of the illness: 1. Plasma exchange (plasmapheresis)  A plasma exchange, also called plasmapheresis, is sometimes used instead of IVIG  This involves being attached to a machine that removes blood from a vein and filters out the harmful antibodies that are attacking your nerves before returning the blood to your body • Most people need treatment over the course of around 5 days 19
  • 20. Cont.. • Plasma exchange helps when done early; it is used if IVIG is ineffective. • Plasma exchange shortens the disease course and hospital stay, and reduces mortality risk and incidence of permanent paralysis. • However, it may cause hypotension due to large fluid shifts, and IV access may be difficult or cause complications. • Plasma exchange removes any previously administered IVIG, negating its benefits, and so should never be done during or soon after use of IVIG. 20
  • 21. Cont.. 2. Immunoglobulin Therapy  Immunoglobulin containing healthy antibodies from blood donors is given through a vein (intravenously). High doses of immunoglobulin can block the damaging antibodies that may contribute to Guillain-Barre syndrome.  Given early, IVIG 2 g/kg over 1 to 2 days or, more slowly, as 400 mg/kg IV once a day for 5 consecutive days is the treatment of choice; it has some benefit up to 1 month from disease onset. 21
  • 22. Cont… • IVIG is more convenient and more widely available than PE but both are equally helpful. Combining the two does not help more. • Early treatment within the first two weeks after the onset of GBS symptoms is preferable. • Besides IVIg and plasma exchange, no other procedures or drugs have been proven effective in the treatment of GBS. • Corticosteroids would be expected to be beneficial in reducing inflammation, But corticosteroids for GBS showed no significant benefit, and treatment with oral corticosteroids was even shown to have a negative effect on outcome 22
  • 23. Prognosis • Approximately 85% of patients with GBS achieve a full functional recovery with in several months to a year • The mortality rate is <5% in optimal setting; death usually results from secondary pulmonary complications • The outlook is worst in patients with sever proximal motor and sensory axonal damage. Other factors that worsen the outlook for recovery are advanced age, delayed in the onset of treatment • Between 5 to 10% of patients with typical GBS have one or more late relapses; such case are then classified as chronic inflammatory demyelinating polyneuropathy (CIDP) 23
  • 24. Conclusion • GBS is a monophasic immune-mediated neuropathy characterized by acute onset of predominantly motor weakness and is a common cause of respiratory paralysis. • There are many variants described with different prognosis and manifestations. Electrodiagnosis aids in the diagnosis. • Immunotherapy definitely makes a difference in the recovery of GBS patients and both PE and IVIg are equally effective. IVIg may be preferred because of its low side-effect profile and ease of administration. • Supportive treatment is equally important in reducing the morbidity and mortality in GBS. 24
  • 25. Reference • Guillain-Barré Syndrome ANNE D. WALLING, MD, ChB, and GRETCHEN DICKSON, MD, MBA University of Kansas School of Medicine, Wichita, Kansas • https://www.mayoclinic.org/diseases-conditions/guillain- barre-syndrome/symptoms-causes/syc-20362793 • https://www.msdmanuals.com/professional/neurologic- disorders/peripheral-nervous-system-and-motor-unit- disorders/guillain-barr%C3%A9-syndrome-gbs • Harrison 21 edition 25