This document provides information about Guillain-Barré syndrome (GBS), including: 1. GBS is an acute immune-mediated polyneuropathy that affects the peripheral nervous system and can be life-threatening. It has several subtypes with different clinical features. 2. The cause is unknown but is thought to involve molecular mimicry between microbial antigens and peripheral nerve antigens, triggering an autoimmune response. 3. Treatment involves supportive care to address symptoms like weakness, respiratory issues, and pain. Immunotherapy with IVIG or plasmapheresis within 2 weeks of onset can improve outcomes.

1. Dr:
Samir Mohammed Mounir
Lecturer of Pediatrics
Minia University

2. Other names
 LANDRY’S ASCENDING PARALYSIS
 ACUTE INFLAMMATORY DEMYELINATING
 POLYNEUROPATHY (AIDP)
 ACUTE IDIOPATHIC POLYRADICULONEURITIS
 ACUTE IDIOPATHIC POLYNEURITIS
 FRENCH POLIO
 LANDRY GUILLAIN BARRE SYNDROME

4. Georges Guillain, together with Barré and Strohl, described two cases of self-
limiting acute paralysis with peculiar changes in the cerebrospinal fluid. 1916

6. Nerve fibers include a solitary axon and numerous
dendrites.
Larger axons are enclosed by sheaths of myelin
provided by Schwann cells and are myelinated
fibers.
Neurons can be grouped in two ways: on the basis
of structural differences (bipolar, unipolar, and
multipolar neurons), and by functional differences
(sensory neurons, interneurons, and motor
neurons).
A nerve is a bundle of nerve fibers held together
by layers of connective tissue.
Nerves can be sensory, motor, or mixed, carrying
both sensory and motor fibers.

10.  Guillain–Barré syndrome (GBS) is acute progressive
potentially life-threatening idiopathic autoimmune (
immune mediated) polyneuropathy
 Affects about 100 000 people every year worldwide.
 Both sexes and all parts of the world have similar rates
of disease.
 Under the umbrella term of GBS are several
recognizable variants with different clinical and
pathological features.
 Diagnostic biomarkers are not available for most
variants of the syndrome.

12.  Many people have overlapping symptoms that can make
the classification difficult in individual cases
 All types have partial forms. Other forms of Miller Fisher
syndrome (anti-GQ1b antibody syndrome) and "acute
ataxic hypersomnolence" where coordination problems
and drowsiness are present but no muscle weakness can be
detected
 Isolated eye-movement or coordination problems
 Congenital?
 Neonatal?
 Paraplegic?8%
 Bickerstaff brainstem encephalitis (drowsiness, sleepiness,
or coma)

13. Cause Pathology
 No clear cause.
 Neither contagious nor hereditary
 Peripheral nerve myelin is target of an immune attack
 Aberrant autoimmune response targeting peripheral nerves and their spinal
roots
 Eventually get widespread patchy demyelination = increased paralysis
 Destruction of the myelin sheath surrounding largest, most myelinated
sensory and motor fibers, resulting in disrupted proprioception and weakness
 The immune dysfunction is triggered (not by direct infection) of peripheral
nerves or nerve roots by an infection, surgery or vaccination
 Molecular mimicry between microbial and nerve antigens
 ■■Molecular mimicry, antiganglioside antibodies and, likely, complement
activation Lymphocytic infiltration of spinal roots/peripheral nerves & then
macrophage-mediated, multifocal stripping
 A potential role for genetic susceptibility requires further investigation
 Campylobacter jejuni (different lipopolysaccharides) , CMV, EBV, VZV, HEV
and the bacterium Mycoplasma pneumoniae. influenza virus and potentially
influenza vaccine
 27% have no preceding illness

14. Epidemiology
 Nondiscriminatory: can affect persons of any gender
(Males?), age, or ethnic background
 • Emerged as the major cause of AFP.
 • GBS affects 2 per 100,000 annually (1,500 people/year)
 The incidence <18 years is 0.34 to 1.34 cases per
100,000/year
 The average age is 4 to 8 years. rare below the age of 2 years
 • Males more susceptible
 • More common in developing countries.

15. Course
 Initial phase
 – Gradually increasing involvement lasts 10-30 days
 (less than 4 weeks)
 Plateau phase
 – Short phase (within 2 weeks)
 – Long plateau phase → poor prognosis
 Recovery phase
 – Within months
 – Usually complete
 – Motor sequelae (5-25%)
 – Relapse & late recurrences (3%)

16. Clinical Features
Weakness
 Onset is gradual and progresses over weeks
 fairly symmetric muscle weakness(asymmetry 9% )
 Lower extremities (unable/refusal to walk) trunks upper limbs bulbar
muscles flaccid tetraplegia = Landry Ascending Paralysis
 Progression: from distal to proximal while recovery is the reverse
10% will 1st develop weakness in face or arms
-severe resp muscle weakness in 10-30% pts
-oropharyngeal weakness in ~ 50%
Muscle tenderness – At the onset : Often prominent severe pain in lower back
Absent or depressed DTR. Areflexia (83%)
 Exaggerated DTR, in ACUTE PHASE: hyperreflexia (Seneviratne, 2000)
 AMAN: with retained or brisk reflexes (Winer, 2008)
 Preservation of cutaneous relfexes (Bounduelie, 1998)
Sensory System:
 Largely myelinated fibres are severely affected.
 Paraesthesias – in some cases in hands and feet *(Gloves and stocking )
 Proprioception is more affected than pain temperature sensation.
 sensory ataxia can also occur

17.  Bulbar involvement (50%)
 o Dysphagia and facial weakness – signs of impending respiratory failure
 o Interfere with eating. Increase risk of aspiration
 Cranial nerve involvement (50%)
 Cranial Nerve deficit (Franz, 2000) in (III- VII, IX- XII) with facial palsy
 Ptosis, opthalmoparesis (diplopia), facial weakness, dysarthria, dysphagia
with pooling of secretions.
 Pupillary paralysis?
 Optic atrophy?
 Autonomic involvement (Dysautonomia )
 Common
 Seen even in mild cases
 Wide fluctuation in blood pressure
 Postural hypotension
 Urinary retention or incontinence (20% of cases, usually transient)
 Ileus
 Cardiac dysrryhthmias, Occasional asystole
 Close monitoring and management
 Can be fatal.
 All require hospitalisation
 30% require ventilatory support

18.  Symptoms of viral meningitis / meningoencephalitis
In young children
 CNS involvement
ataxia
papilledema
 Miller-Fisher syndrome
External ophtalmoplegia
ataxia
areflexia
 What is the patient experiencing?!
-----Fear and anxiety
-----Depression and guilt
-----Pain: may before and can impede correct diagnosis

21.  REQUIRED :
 1.progressive weakness of 2 or more limbs due to neuropathy.
 2.areflexia
 3.disease course < 4 weeks
 4 exclusion of other causes (vasculitis,PAN,SLE,churg strauss
 syndrome,toxins,lead,botulism,diptheria,porphyria.,cauda
 equinal syndrome)
 SUPPORTIVE:
 1.relatively symmetric weakness
 2.mild sensory involvement
 3.facial nerve or other cranial nerve involvement
 4.absence of fever
 5.typical CSF profile(aceelualr,increase in protein level)
 6.electrophysiologic evidence of demyelination

22. Investigations
Lumbar puncture – cerebrospinal fluid (CSF)
 Usually normal in <48 hrs
 Albuminocytologic dissociation: elevated CSF protein w/ normal WBC (10
cells/mm)
 o Glucose level normal
 o Bacterial and viral culture is negative
 Neurophysiology:
 May be normal
 Lags behind clinical events
 MRI
 (exclude others
 . enhancement of the nerve roots)
 Exclude decrease serum K
 Low serum Na (inappropriate secretion of antidiuretic hormone)

23. Serum Creatine Kinase
 o Elevated or normal
Muscle biopsy
 o appear normal in early stages
 o show evidence of denervation atrophy in chronic stages
Sural nerve biopsy
segmental demyelination, focal inflammation.
Serology
Antiganglioside antibodies: Antiganglioside
antibodies,mainly against GM1 and GD1, are sometimes
elevated

24. Subdivision of GBS
 Subdivision Clinical manifestation
 Sporadic GBS (AIDP): The traditional form (85-90% )
 Most patients have a demyelinating neuropathy, but primarily axonal degeneration is
documented in some cases.
 Motor, bilat, facial and pharyngeal, occasional sensory and autonomic disturbances
 Acute motor-sensory axonal neuropathy (AMSAN)
 Relatively infrequent
 Severe degeneration of motor and sensory axons
 Little demyelination
 Fulminant, extensive and severe weakness with delayed and incomplete recovery
 Acute motor-axonal neuropathy (AMAN)
 Severe pure motor axonal neuropathy
 Clinical course and recovery is similar to AIDP
 Miller-Fisher syndrome
 Triad: ophthalmoplegia, ataxia, & areflexia
 Chronic IDP (CIDP)
 Neurologic symptoms are slower (>4 weeks)

26. DDx of Polyneuropathy:
 Acute myelopathy – back pain,sphincter disturbances
 Botulism –early loss of pupillary activity,descending paralysis
 Diphtheria –early oropharyngeal involvement
 Lyme disease polyradiculitis
 Porphyria –abdominal pain,seizure,psychosis
 Vasculitic neuropathy
 Poliomyelitis with fever,meningeal signs
 Brain stem ischemia
 Critical illness neuropathy
 Myasthenia gravis
 OP poisoningArsenic poisoning
 N-Hexane (glue sniffing)
 Vasculitis
 Lyme Disease
 Tick paralysis
 Sarcoidosis
 Leptomeningeal Dz
 Paraneoplastic Dz
 Critical Illness

30. Treatment
 No known cure.
 As soon as possible
 Conservative if mild.
 Supportive Care :
 Each day counts
 Monitor: Respiration. Cardiac status
 Phsyiotherapy

32. Supportive Care
 Monitor Resp status closely (follow NIFs), up to 30%
may req ventilatory support
 In severe cases, intrarterial monitoring may be
necessary given the gisngifcant blood pressure
fluctuations
 Neuropathic pain plagues most, often managed w/
Gabapentin or Carbamazepine

34. Disease Modifying Treatment
 2 weeks after the first motor symptoms – immunotherapy is no
longer effective.
 IVIg-first choice,easy to administer. 0.4gm/kg/day for 5
consecutive day (may need to extend course depending on
response)
 Plasmapheresis and/or immunosuppressive drugs are
alternatives if IVIG is ineffective.
 Plasmapheresis: usually 4-6 treatments over 8-10 days
 Plasmapheresis 40-50ml/kg four times a week
 The choice b/w plasma exchange and IVIG is dep on
availability, pt contraindications, etc. Because of ease of
administration, IVIG is frequently preferred. The cost and
efficacy of the 2 treatments are comparable.
Neither treatment found to be more effective
 Combination is not effective
 Glucocorticoids are not effective in GBS.

35. Plasmapheresis
 Better outcomes : Reduced risk for respiratory failure
- Decreased time to recover walking
- -- Reduced necessity and duration of ventilator use!
- Full muscle strength recover after 1 year
 Adverse effects : hypotension, septicemia, pneumonia,
abnormal clotting and hypocalcemia.
 Contraindications: hemostatic disorder, unstable
cardiovascular state, active infection, and pregnancy.

36. Intravenous Immunoglobulins
 Side effects:
 CHF
 Stroke
 Myocardial ischemia
 Renal failure
 Thrombocytopenia
 Hemolysis
 Anaphylactic shock.

37. Prognosis
 Overall, 80% usually recover completely
 Recovery : 2 months to 2 years
 ~25% of patients require artificial ventilation
 20% are unable to walk after 6 months
 5-10% have prolonged course w/ incomplete recovery, ~3%
wheelchair bound
 Approx 5% die despite ICU care
 2% will develop chronic relapsing Chronic Inflammatory
Demyelinating Polyradiculoneuropathy (CIDP)
 Rehabiliation can take up to 1 year, though residual effects
(other residual features (Pain. sensory disturbances and
fatigue) can be present 3-6 years. !
 Patient disability is seen in 20%-30%.

39. Prognosis
 Prompt treatment will lead to good recovery in the
majority. Recovery may take weeks to years.
 About a third have some permanent weakness. Globally
death occurs in about 7.5% of those affected.
 If the disease continues to progress beyond four weeks, or
there are multiple fluctuations in the severity (more than
two in eight weeks), the diagnosis may be chronic
inflammatory demyelinating polyneuropathy
 poor prognostic outcome in GBs are high age (aged 40
years and over), preceding diarrhoea (or C jejuni infection
in the past 4 weeks), and high disability at nadir

41. Treatment related fluctuation(TRFs) and
acute onset chronic inflammatory
demyelinating neuropathy
 About 10% of patients treated with IV Ig or plasma
exchange will deteriorate after initial improvement or
stabilization—ie, they will have a TRF. These TRFs usually
occur within the first 8 weeks after start of treatment.
Repeated treatment (2 g IV Ig/kg in 2–days) has been
observed to be beneficial
 5% of patients initially diagnosed with Guillain-Barre
 syndrome were eventually found to have acute onset
especially if progression exceeding 4 weeks
 CIDP should be suspected when a patient with GBS
deteriorates after eight weeks from onset, or when
deterioration occurs three times or more after that time

42.  A-CIDP was even more likely if a patient could still walk
unaided, had no cranial nerve dysfunction, and had
electrophysiological features compatible with CIDP
 Some clinical and electrophysiological features also can be
helpful for distinguishing A-CIDP from GBS, but cannot be
used to define the disorder.
 “Although time course is the most important factor
distinguishing the two diseases at present, biomarkers such
as auto-antibodies are likely to be identified in future
studies. (Kurt Samson: Relapse Patterns May Help Discern Guillain-Barré from
Acute CIDP: NEUROLOGY TODAY | MAY 20, 2010

44. Future
 Immunosuppressive drug mycophenolate mofetil,
brain-derived neurotrophic factor and interferon beta
(IFN-β) have not demonstrated benefit to support
their widespread use.
 Agents have shown promise in animal models :
glatiramer acetate, quinpramine, fasudil and the heart
drug flecainide. An antibody targeted against the anti-
GD3 antiganglioside antibody has shown benefit in
laboratory research.
 Complement inhibitors (such as eculizumab) may be
effective

45. Summary
 1.acute rapidly evolving areflexic ascending motor
 paralysis with or without sensory disturbances.
 2.fever is absent at the onset of weakness
 3.bladder involvement in severe cases –transient
 4.campylobacter jejuni 20-30 % cases
 5.autoimmune basis ,molecular mimicry
 6.anti GM1 ab (MC),anti GD1a,anti GQ1b (MFS)
 7.autonomic involvement is common
 8.facial nerve is the one most commonly affected,optic
 nerve.
 9.30% require ventilatory support.
 10.course is usually < 4 weeks
 11 .typical CSF profile shows high protein,no pleocytosis
 12.electrographically conduction block present
 13.treatment as soon as possible
 14.IVIg,plasmapheresis –both are equally good
 15.glucocorticoids are not effective in GBS
 16.think of miller fischer variant in presence of
 ophthalmoplegia,ataxia,areflexia.

47.  Your brain keeps developing until your late 40s
 New Brain Connections Are Created Every Time You Form a
Memory
 The human brain is composed of about 100 billion neurons plus
a trillion glial cells
 The brain can't actually feel pain despite its billions of neurons
 Lack of sleep may shrink your brain
 When awake, the human brain produces enough electricity to
power a small light bulb
 When you learn something new, the structure of your brain
changes.
 Neurons send info to your brain at more than 150 miles (241
kilometers) per hour