Gbs

1. FROM PEDIATRICS WARD
15/07/2015 E.C
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2. Guillain-Barré syndrome (GBS)
GROUP MEMBERS
1.NITSUKAL
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3. Presentation outline
• Definition
• Ethology/cause
• Pathophysiology
• Subtypes of GBS
• Risk factor
• Clinical presentation
• Sign and symptoms
• Diagnosis
• Treatment
• Complication
• Prognosis
• Clinical summary
• Reference
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4. Guillain-Barré syndrome (GBS)
• A collection of clinical syndromes that manifests as an acute inflammatory
polyradiculoneuropathy with resultant weakness and diminished reflexes.
• 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 GBS is a medical emergency. Most people
with the condition must be hospitalized to receive treatment.
• Sensory, autonomic, and brainstem abnormalities may also be seen.
• With the eradication of poliomyelitis, GBS is the most common cause of
acute motor paralysis in children.
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5. Ethology/cause
• The exact cause of Guillain-Barre syndrome is unknown.
• Increasing data indicate that it is an autoimmune disease, often triggered:
• Vaccination against the:
 Flu
 Rabies
 Meningitis
• are documented precipitating factors
• By a preceding viral or bacterial
infection with organisms such as:
 Campylobacter jejuni
 Cytomegalovirus
 Epstein-Barr virus
 Mycoplasma pneumoniae.
 COVID-19, Zika virus
• Two-thirds of patients report symptoms of an infection in the six weeks
preceding. These include a COVID-19, respiratory or a gastrointestinal
infection or Zika virus.
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6. Pathophysiology
• Two pathophysiological forms have been described:
 Demyelinating form of GBS
 Axonal forms of GBS
Demyelinating form of GBS :
• Segmental demyelination of peripheral nerves is due to immune mediated
involving both humoral and cell-mediated immune mechanisms
Axonal forms of GBS
• axonal degeneration may occur without demyelination or inflammation.
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7. Pathophysiology (CONT’D)
• The mechanism of disease possibly involves an abnormal T-cell response
precipitated by an infection which activate CD4+ helper-inducer T cells.
• 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.
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8. Subtypes of GBS
• The clinical spectrum of GBS, which includes individual variation and
variable severity of presentation, comprises the following:
1. Acute inflammatory demyelinating polyradiculoneuropathy
(AIDP)
2. Acute motor axonal neuropathy (AMAN)
3. Acute motor and sensory axonal neuropathy (AMSAN)
4. Miller-Fisher syndrome (MFS)
5. Polyneuritis cranialis
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9. 1.Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP)
• Accounts for 80-90% of GBS cases ( Europe and North America)
• Characterized by an immune-mediated attack on myelin with infiltration of
lymphocytes and macrophages with segmental stripping of myelin.
• Motor and sensory fibres are usually affected simultaneously, producing
corresponding deficits.
• Electrophysiology shows:
1. Slow nerve conduction velocity
2. Prolonged F waves.
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10. 2.Acute motor axonal neuropathy (AMAN)
• Most commonly seen in China and Japan (50-60% of cases), as
apposed toWestern countries (10-20% of cases).
• In this form, axonal degeneration occurs by immune attack within 1-2
weeks after infection.
• Specific antibodies to axonal membranes of motor fibres attack the nodes of
Ranvier.
• This, in turn, activates complement and intrusion of macrophages into
periaxonal space, resulting in destruction of axons.
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11. 2.Acute motor axonal neuropathy (AMAN) {CONT’D}
• C jejuni is the most common preceding infection, and antiganglioside
antibodies are usually found in this type.
• Electrophysiology shows:
1. Reduction in muscle action potentials with relatively preserved
motor nerve conduction velocity
2. Normal sensory nerve action potentials and F waves
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12. 3.Acute motor and sensory axonal neuropathy
(AMSAN)
• This type is rare and resembles AMAN except sensory nerves
are also affected.
• This type is associated with a severecourse and poor prognosis.
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13. 4.Miller-Fisher syndrome (MFS)
• The involvement of CNs is very distinct in this form of GBS.
• Ocular motor nerves (oculomotor, trochlear, and abducens) are affected
and produce a triad of ophthalmoplegia, ataxia, and areflexia.
• Electrophysiology is normal.
• The characteristic autoantibodies are against gangliosides GQ1b and
GT1a.
• GQ1b plays a key role in the pathogenesis of MFS.
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14. Polyneuritis cranialis
• This is an acute onset of Multiple Cranial nerves. (usually bilateral CN
VII with sparing of CNs I and II)
o Elevated cerebrospinal fluid protein
o Slowed nerve conduction velocity
o Uncomplicated recovery.
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15. Risk factors
 Most commonly, infection with campylobacter, a type of bacteria often
found in undercooked poultry
 Influenza virus, Cytomegalovirus, Epstein-Barr virus, Zika virus
 Males than females
 Risk increases as you age increases
 Hepatitis A, B, C and E
 HIV, the virus that causes AIDS
 Mycoplasma pneumonia
 Surgery
 Trauma
 COVID-19 virus
 COVID-19 Johnson & Johnson and AstraZeneca vaccine
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16. Clinical Presentation
• 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.
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17. Signs and symptoms of GBS may include:
 A pins and needles sensation in your fingers, toes, ankles or wrists
 Weakness in your legs that spreads to your upper body
 Unsteady walking or inability to walk or climb stairs
 Difficulty with facial movements, including speaking, chewing or swallowing
 Double vision or inability to move the eyes
 Severe pain that may feel achy, shooting or cramplike and may be worse at night
 Difficulty with bladder control or bowel function
 Rapid heart rate
 Low or high blood pressure
 Difficulty breathing
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18. 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.
• Physical Examination and medical history is our major diagnosis criteria.
 Physical Examination
• Examine hands, feet or limb to check for numbness
• Recent history of GBS secondary to food poisoning or flu
• Check for reflexes
• Ask for duration and severity of symptoms
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19. Diagnosis(CONT’D)
• The diagnosis of GBS is typically based on the presence of :
o Progressive ascending weakness
o Areflexia
• Findings on :
 Lumbar puncture
 Electrodiagnostic studies
 MRI (occasionally)
• Can give support for the diagnosis.
• Abnormalities on these studies do not develop until days to weeks after onset
of symptoms.
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20. Lumbar Puncture
• Typically, the LP findings are suggestive of demyelination (i.e., increased
protein >45 mg/dL within 3 weeks of onset) without evidence of active
infection (lack of CSF pleocytosis),
• The CSF findings may be normal within the first 48 hours of
symptoms
• Occasionally the protein may not rise for a week.
• Usually by 10 days of symptoms, elevated CSF protein findings will be
most prominent.
• Most patients have fewer than 10 leukocytes per milliliter, but
occasionally a mild elevation (i.e., 10-50 cells/mL) is seen.
• Greater than 50 mononuclear cells/mL of CSF makes the diagnosis of
GBS doubtful.
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21. Electrodiagnostic Studies
• Within the first week of the onset of symptoms, electrodiagnostic studies
in at least two limbs reveal the following:
• A dispersed, impersistent, prolonged, or absent OF response (88%)
• Increased distal latencies (75%)
• Conduction block (58%) or temporal dispersion of compound muscle
action potential (CMAP)
• Reduced conduction velocity (50%) of motor and sensory nerves
• Criteria for axonal forms include:
• Lack of neurophysiologic evidence of demyelination
• Loss of amplitude of CMAP or sensory nerve action potentials to at least
less than 80% of lower limit of normal values for age
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22. MRI
 Nearly 2 weeks after presentation of symptoms, lumbosacral MRI can
show enhancement of the nerve roots with gadolinium.
 This imaging study has been described to be 83% sensitive for acute
GBS, with nerve root enhancement present in 95% of typical cases
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23. differential diagnosis:
• Spinal cord lesions may be considered in the differential diagnosis:
• Transverse myelitis
• Vascular malformations
• Epidural abscess
• Cord infarctions
• Tumors
• Cord compression
• Enteroviral infections of the anterior horn cells
• Lumbosacral disk syndromes
• Poliomyelitis
• Trauma
• Hopkins syndrome
Spinal cord lesions may be considered in the differential diagnosis:
• Transverse myelitis • Vascular malformations
• Epidural abscess • Cord infarctions
• Tumors • Cord compression
• Enteroviral infections of the
anterior horn cells
• Lumbosacral disk
syndromes
• Poliomyelitis • Trauma
• Hopkins syndrome
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24. Peripheral neuropathies from the following may produce a GBS-like
picture:
• Vincristine • Glue sniffing
• Heavy metals poisoning • Organophosphate pesticides
• HIV infection • Diphtheria
• Lyme disease • Inborn errors of metabolism
• Leigh disease • Tangier disease
• Porphyria
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25. Treatment
In pediatrics, the most effective form of therapy is generally considered tobe:
• intravenous immunoglobulin (IVIG)
• Plasmapheresis or
• Immunosuppressive drugs
• Patients in early stages of this acute disease should be admitted to the
hospital for observation because the ascending paralysis can rapidly
involve respiratory muscles and cause respiratory failure and autonomic
instability
• Steroids are not effective for weakness but may help with pain.
• Supportive care, such as respiratory support, prevention of pressure sores,
nutritional support, pain management, prevention of deep vein thrombosis,
and treatment of secondary bacterial infections is important.
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26. Intravenous immunoglobulin (IVIG)
• Severe or rapidly progressive muscle weakness is treated with intravenous
immunoglobulin (IVIG); common protocols include:
• IVIG 0.4 g/kg/day for 5 consecutive days or 1g/kg/day for 2 days.
• IVIG is a treatment made from donated blood that contains healthy
antibodies. These are given to help stop the harmful antibodies damaging
your nerves.
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27. Plasmapheresis
• A plasma exchange, also called plasmapheresis, is sometimes used As an
alternative 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
• 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.
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28. Other treatments
• While in hospital, you'll be closely monitored to check for any problems with
your lungs, heart or other body functions.
• You'll also be given treatment to relieve your symptoms and reduce the risk of
further problems. This may include:
• a breathing machine (ventilator) if you're having difficulty breathing
• a feeding tube if you have swallowing problem.
• painkillers if you're in pain
• being gently moved around on a regular basis to avoid bed sores and keep your
joints healthy
• a thin tube called a catheter in your urethra if you have difficulty peeing
• laxatives if you have constipation.
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29. Complications of GBS
• The most common serious complications are:
1. Weakness of the respiratory muscles
2. Autonomic instability
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Other important potential complications include:
• Pneumonia • Ileus
• Adult respiratory distress
syndrome
• constipation
• Septicemia • gastritis
• Pressure sores • dysesthesias
• Pulmonary embolus • Nephropathy

30. Prognosis
• In general, the outcome of GBS is more favourable in children than in adults
• the recovery period is long, oftenweeks to months
• Rarely, it can be fatal in 5-10% of patients with respiratory failure and cardiac
arrhythmia
• Recurrence of GBS occurs in approximately 5% of cases
• Overall mortality rate in childhood GBS is estimated to be less
than 5%
• Deaths are usually caused by respiratory failure, often
in association with :
 Cardiac arrhythmias
 Dysautonomia
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31. Clinical Summary
• Features required for diagnosis are:
(1) Progressive weakness of more than one extremity
(2) Hyporeflexia or areflexia
(3) Elevated cerebrospinal fluid protein (>45 mg/dL) after 1 week
following onset of symptoms
(4) Slow conduction velocity or prolonged F wave on
electrophysiology testing.
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32. • Features that rule out the diagnosis include:
(1) A current history of hexacarbon abuse
(2) Abnormal porphyria metabolism
(3) Recent diphtheria infection
(4) Evidence of polio, botulism, toxic neuropathy, tic paralysis,
or organophosphate poisoning.
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Clinical Summary (Cont.)

33. REFERENCE
• Nelson textbook of pediatrics.
• MSD MANUAL.
• 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
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