1. Guillain-Barré syndrome is an acute immune-mediated polyradiculoneuropathy characterized by rapidly progressive ascending paralysis.
2. It is considered an autoimmune disorder resulting from molecular mimicry following a bacterial or viral infection.
3. Clinical features include progressive symmetric weakness of the limbs and lack of reflexes with or without sensory involvement. Cranial nerve palsies and autonomic dysfunction may occur.
4. Diagnosis is based on the above clinical features along with elevated cerebrospinal fluid protein and electrodiagnostic studies showing demyelination.
3. Incidence : 1 in 4 cases per 100000 annually.
Males > females
It is acute frequently severe and fulminant
polyradiculopathy that is autoimmune in nature.
4. 70% cases- post infectious
◦ 1-3 weeks after an acute infectious process, usually respiratory
or GIT.
◦ 20 to 30 % cases by campylobacter jejuni infection
◦ others HHV, EBV, CMV, Mycoplasma pneumoniae
Recent immunisation- swine influenza, meningocccal
vaccination.
Can be seen in patients with lymphoma, HIV, SLE
5. An autoimmune basis
Both celluLar and humoral immunity involved.
T cell activation- elevated IL2, IL2 receptor in serum
and, IL6, TNF alpha, IFN gamma in CSF.
All GBS results from immune responses to non self
antigens [infectious agents, vaccines] that misdirect to
host nerve tissue through a resemblance of Epitope
[Molecular Mimicry]
Neural targets are gangliosides.
Anti ganglioside ab-GM1 [20% cases of C.jejuni]
Anti GQ1b ab more than 90% MFS
6.
7.
8. GM1 on nerves, Nodes of Ranvier
Anti GM1 ab as a part of Molecular mimicry
Complement mediated injury at Paranodal axon – glial
junction
Disrupts the cluster of Na channels
Conduction block
Flaccid paralysis
9. Respiratory or GIT infection prior to onset of weakness
about 1-3 weeks
H/o recent immunization [Rabies]
Sudden or progressive weakness over 2-3 days.
No fever at the onset of weakness
Fever and constitutional symptoms are absent at the
onset and if present, cast doubt on diagnosis.
10. Motor System
Rapidly evolving areflexic motor paralysis with or without sensory
disturbance.
Ascending type of paralysis
Rubbery legs
Weakness evolves over hours or days, Legs followed by arms
Accompanied by dysesthesias of extremities
Deep Tendon Reflexes
Reflexes attenuate, disappear in few days of onset
11. Sensory System
◦ Largely myelinated fibres are severely affected
◦ Proprioception is more affected than pain and temperature
sensation
Bladder
◦ Only in severe cases, transiently.
◦ If bladder dysfunction is a prominent feature and comes
early in the course, think other than GBS - spinal cord
disease.
12. Facial diparesis is seen in 50%
Lower cranial nerves affected - bulbar weakness,
difficulty in handling secretion, maintaining airway
Ophthalmoplegia in Miller Fischer variant
Pupillary paralysis
Optic atrophy
13. Deep aching pain may be present in the previous day in
weakened muscles.
Initially at onset- neck, shoulder, back, diffusely over
spine- 50% cases.
Dysesthetic pain in extremities
Self limited usually
Responds to analgesics
14. Common, seen even in mild cases
Wide fluctuation in blood pressure
Postural hypotension
Cardiac arrythmias
Close monitoring and management
Can be fatal
All require hospitalization
30% require ventilator support
15. Pattern of rapidly progressing ascending paralysis with
areflexia, initially of legs.
Usually doesn’t progress beyond 4 weeks after
reaching a plateau – nadir at 2-4 wks of onset.
Resolution of symptoms after the peak.
Death – 5%, due to respiratory paralysis or lung
infections.
16. Adults > children
Rapid recovery
antiGM1 ab (50%)
Demyelinating
First attack on Schwann cell surface
Widespread myelin damage
Lymphocyte infiltration
Variable sec axonal damage
17. Children, young adults
antiGD1a ab.
Rapid recovery
Axonal
First attack on motor nodes of ranvier
Macrophage activation
Axonal damage is variable
18. Mostly adults
Slow recovery, often incomplete
Closely related to AMAN
Also affects sensory nerves and roots
Axonal damage usually severe
19. Adults, children
antiGQ1b antibodies >90% [Not seen in
other forms of GBS unless there is EOM
involvement]
More of GQ1b gangliosides in EOM
Cause conduction block
Pupillary paralysis
Only 5% GBS
20. Pure sensory forms
Ophthalmoplegia with anti-GQ1b antibodies as part
of severe motor sensory GBS
GBS with severe bulbar and facial paralysis
sometimes associated with antecedent CMV infection
and anti GM2 antibodies
Acute pandysautonomia
21. CSF
◦ raised CSF protein, 1-10g/L[100-1000mg/dl]
◦ Without accompanying pleocytosis
Albuminocytological dissociation usually
normal <48hrs
Increased proteins at the end of first week
WBC-10-100/microL as transient elevation
If sustained pleocytosis, think of HIV,CMV
22. May be normal
Lag behind clinical events
Demyelination- prolonged distal wave
latencies, slowing of conduction velocity,
conduction block
It can be reduced amplitude of compound
action potential without conduction slowing
23. 1. Required for diagnosis
1. Progressive weakness in both arms and legs
2. Generalized hypo or areflexia
2. Supportive of diagnosis
Clinical features
◦ Progression of symptoms over days to 4 wks
◦ Relative symmetry of paresis
◦ Mild to moderate sensory signs
◦ Cranial nerve involvement
◦ Recovery 2-4 wks following plateau
◦ Autonomic dysfunction
◦ Preceeding GI illness or URTI common
24. CSF features supporting diagnosis
◦ Elevated or serial elevation of CSF protein
◦ CSF cell counts are <10 mononuclear cell/mm3
Electrodiagnostic medicine findings supportive of Dx
◦ 80% pts -NCV slowing or conduction block some time during
disease process
◦ NCV < 60% of normal
◦ Distal motor latency increase, > 3x of normal value
◦ F – wave indicate proximal NCV slowing
◦ 15 – 20 % - normal NCV
◦ No abnormality on NCS may be seen for several weeks
25. ◦ Assymetric weakness
◦ Failure of bowel/bladder symptoms to resolve
◦ Severe bowel/bladder dysfunction at initiation of dse
◦ Greater than 50 mononuclear cells/mm3 in CSF
◦ Well- demarcated sensory level
Exclusionary criteria
◦ Diagnosis of other causes of acute neuromuscular weakness
MG, Botulism, Poliomyelitis, toxic neuropathy
◦ Abnormal CSF cytology suggesting carcinomatous
invasion of nerve roots
26. Acute myelopathy – back pain, sphincter
disturbance.
Botulism – early loss of pupillary reactivity
Diphtheria – early oropharyngeal involvement
Lyme disease – polyradiculitis
Porphyria – abdominal pain, seizure, psychosis
28. Initiate as soon as possible
2 weeks after the first motor symptoms,
immunotherapy is no longer effective
IVIg – first choice, easy to administer
◦ 5 daily infusions, 2 g/kg body wt
Plasmapheresis
◦ 40-50 ml/kg , 4 times a week
Combination is not effective
29. Treatment reduced need for ventilation by
half, increases full recovery at an year
Glucocorticoids are not effective
Conservative management in mild cases
30. Critical care setting
◦ Attention to vital capacity
◦ Heart rhythm
◦ Blood pressure
◦ Nutrition
◦ DVT
◦ CV status
◦ Tracheostomy
◦ Chest physiotherapy – physiotherapy also important
◦ 30% require ventilation- some for prolonged time.
31. Acute, rapidly evolving areflexic ascending motor
paralysis with/out sensory disturbances.
Fever is absent at the onset of weakness
Bladder involvement in sever cases – transient
Campylobactor jejuni in 20-30% cases
Autoimmune basis – molecular mimicry
antiGM1ab [MC], antiGD1a, antiGQ1b – MFS
Autonomic involvement is common
Facial nerve – MC, optic nerve
32. 30% require ventilatory support
Course usually less than 4 weeks
Typical CSF profile shows high protein, no pleocytosis.
Electrographically, Conduction block present
Treatment as soon as possible
IVIg, plasmapheresis – both equally good.
Glucocorticoids are not effective
Think of MFS if opthalmoplegia, ataxia, areflexia.
33. Chronic course, Gradual onset
If GBS continues to deteriorates more than 9 weeks
from onset or more than 3 episodes of GBS after
relapse
Both motor and sensory involved
Can be asymmetric
Tremor in 10% cases, Death is uncommon
Biopsy reveals onion bulb changes [imbricated layers
of attenuated schwann cell process surrounding an
axon]
Responds to glucocorticoids.
