The advent of plasma exchange and intravenous immunoglobulins has dramatically improved the prognosis of patients with GBS. Despite this fact, mortality and morbidity rates remain unacceptably high. Until better therapies are developed, the appropriate utilization of immune-modulating therapy and careful attention to supportive care issues will help to minimize these unfavorable outcomes

1. GB SYNDROME
ICU MANAGEMENT
Presenter: Dr. Ravi Kumar
Moderator: Dr. Aruna Bharti

2. Introduction
• Guillain-Barre syndrome (GBS) is a demyelinating
disorder of the peripheral nervous system, which is
monophasic (single peak) with spontaneous
remission.
• Acute
• Fulminant
• Demyelinating
• Inflammatory
• Polyradiculoneuropathy

3. • Guillain-Barre and
Strohl
• 1916
• acute areflexic paralysis
followed by recovery

4. Epidemiology
• Population based studies suggest a crude average
annual incidence of rates from 0.4 to 1.7/100,000
population.
• Incidence is higher in males than females as well as
in older (age > 60 years) compared with younger
people (age < 18 years).
• The occurrence rate is higher for whites than for
blacks.

5. Etiology
• Post infectious
1. One to three weeks After an acute GI infection
2. Compylobactor jejuni
3. Other Agents HHV(EBV, CMV)
4. Mycoplasma Pneumoniae
• Recent immunization - Swine influenza vaccine,
Older rabies vaccine
• Lymphoma, HIV +ve, SLE

6. Pathogenesis
• An autoimmune basis.
• 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 gangliosides ab- GM1(20-50% cases of C.
jejuni)
• Anti GQ1ab - >90% MFS

8. • GBS has been subdivided into the clinical
variants –
1. Acute Inflammatory Demyelinating
Polyradiculoneuropathy (AIDP)
2. Acute Motor Axonal Neuropathy (AMAN)
3. Acute Motor Sensory Axonal Neuropathy
(AMSAN).

9. AIDP (Acute Inflammatory Demyelinating
Polyradiculoneuropathy )
• Adults>children
• Rapid recovery
• Anti GM1 ab ( 50%)
• Demyelinating
• First attack on schwan cell surface
• Wide spread myelin damage
• Variable sec axonal damage

10. AMAN (Acute Motor Axonal
Neuropathy)
• Children, young adults
• Axonal
• First attack on node of ranvier
• Macrophage infiltration
• Axonal damage is variable

11. Miller fischer variant
• Adult, children
• Anti GQ1 b ab > 90%
• Pupillary paralysis
• Only 5 % GBS
OPHTHALMOPLEGIA
AREFLEXIA
ATAXIA

12. Clinical features
MOTOR SYSTEM
 Rapidly evolving areflexic motor paralysis with or
without sensory disturbance
 Ascending type of paralysis
 Typically starts in proximal legs
 10% will 1st develop weakness in face or arms
 severe resp muscle weakness in 10-30% pts
 oropharyngeal weakness in ~ 50%

13. DEEP TENDON REFLEXES
 Attenuate or disappear in a few days after onset
CRANIAL NERVES
 Facial diparesis – 50% affected individuals
 Ophthalmoplegia – Miller Fischer variant
 Pupillary paralysis
 Optic atrophy

14. SENSORY SYSTEM
 Myelinated fibres severely affected
 Proprioception is more affected than pain &
temperature
BLADDER
 Only in severe cases, transiently
 If bladder dysfunction is a prominent features and
comes early in the course, think other than GBS –
Spinal Cord Disease.

15. PAIN
 Deep aching pain may be present
 Dysesthetic pain in extremities
 Self limiting usually
 Respond to analgesics

16. AUTONOMIC INVOLVEMENT
 Common
 Seen even in mild cases
 Wide fluctuation in blood pressure
 Postural hypotension
 Cardiac dysrrythmias
 Can be fatal
 All require monitoring
 30% require ventilatory support

17. Diagnosis
• The diagnosis is based on -
• Clinical presentation (progressive symmetric limb
weakness following an acute infectious illness)
• Results of nerve conduction studies (slowing of
nerve conduction due to demyelination)
• Cerebrospinal fluid analysis (elevated protein
content in 80%)

18. CSF ANALYSIS
• Raised CSF protein (100-1000mg/dl)
• Without accompanied by pleocytosis
• Albuminocytological dissociation present
• Slow nerve conduction velocity
• prolonged or absent F waves
• Prolonged or absent F waves are pathognomonic
and reflect demyelination at level of nerve roots
ELECTROMYOGRAPHY

19. Treatment
• The treatment of Guillain-Barré syndrome mostly
involves supportive care.
• Initiate as soon as possible
• 2 weeks after the first motor symptoms-
immunotherapy is no longer effective
• plasmapheresis or intravenous immunoglobulin G
are equally effective in producing short-term
improvement.

20. Immunoglobulin G infusion (0.4 g/kg/day for 5 days)
• Neutralize autoantibodies or cytokines, saturate macrophage
Fc receptors, or inhibit complement activation.
Plasmapheresis 40-50 ml/kg four times a week up to 2 weeks
• Nonselectively removes immunoglobulins, complement, and
cytokines, all of which may play a role in the pathogenesis of
GBS.
Immunoglobulin G is often preferred because it is easiest to
administer

21. Glucocorticoids are not effective in GBS
• Meta-analysis of relevant studies shown no
advantage of IV methylprednisolone & suggested
less improvement in patients treated with oral
corticosteroids.
[Hughes RAC, Swan AV, Raphae¨l JC, Annane D, van Koningsveld R, van Doorn PA.
Immunotherapy for GuillainBarre´ syndrome: a systematic review. Brain. 2007;130(9):
2245-2257.]

22. Severe cases
INTENSIVE CARE UNIT
-labile dysautonomia
-forced vital capacity of less than 20 mL/kg
-severe bulbar palsy
-Any patients exhibiting clinical signs of respiratory
compromise
[Walgaard C, Lingsma HF, Ruts L, Drenthen J, van Koningsveld R, Garssen MJ, et al.
Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol. 2010 Jun.
67(6):781-7.]
[Hughes RA, Rees JH. Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect
Dis. 1997 Dec. 176 Suppl 2:S92-8.]

23. • Monitor Resp status closely (follow VC ), up to 30%
may req ventilatory support
• The most sensitive measure of respiratory muscle
strength is the maximum inspiratory pressure
(Pimax)
• A Pimax <30 cm H2O is evidence of severe
respiratory muscle weakness
• If VC has fallen below 15 mL/Kg or Pimax – 25cm
H2O – intubation
[Wijdicks EFM, Borel CO. Respiratory management in acute neurologic illness. Neurology.
1998;50(1):11-20.]

24. Respiratory Consequences of
Neuromuscular Weakness

25. • Drug-induced neuromuscular blockade is
sometimes needed to manage ventilator-
dependent patients who are agitated and difficult
to ventilate.
• Hypostatic pneumonia : Anti-microbial therapy,
nebulization, ventilation
• Venous thromboembolism : LMWH &
thromboguards used to prevent DVT and
consequent pulmonary thromboembolism

26. Dysautonomia
• Autonomic dysfunction occurs to some degree in
65% of patients with GBS.
• Manifestations includes brady- or tachy-
arrhythmias, episodic hypertension, orthostatic
hypotension, abnormal hemodynamic responses to
vasoactive medications, gastrointestinal
dysfunction, and sweating abnormalities.

27. • Patients with autonomic complications are managed in
ICU with continuous cardiac and blood pressor
monitoring.
• If fluctuations are severe enough to cause end-organ
damage, quickly titratable, short-acting medications
are recommended to avoid hypotension.
• Cessation of enteral feeding, gastric decompression,
promotility agents, reduced opiate medications, and
parental nutrition may be needed to overcome
gastrointestinal autonomic dysfunctions.

28. • To minimize infection risks, intermittent catheterization
is preferred over indwelling urinary catheters
Malnutrition
• Patients with GBS are at high risk for inadequate
nutrition throughout the course of their illness.
• Progressive bulbar dysfunction or adynamic ileus can
limit or eliminate oral intake.
• nutritional support should begin as quickly as possible
by appropriate means (eg, modified diet, nasogastric
tube, or parenteral nutrition).

29. IMMUNOMODULATION
• Immunomodulatory treatment in GBS has been
used to hasten recovery.
• Intravenous immunoglobulin (IVIG) and plasma
exchange have proved equally effective

30. Other Immobility Complications
• Careful body positioning, appropriate bracing,
pressure point padding, and frequent position
changes are all warranted.
• Patients with incomplete eye closure from facial
weakness are also at risk for exposure keratitis.
• Good corneal hygiene with artificial tears,
lubricants & careful lid-taping is essential

31. Psychiatric Complications
• This dramatic loss of independence & prolonged
hospitalization may produces several psychiatric
complications.
• Anxiety occurs in 82% of patients, with moderate or
severe depression occurring in two thirds of
patients.
• Selective serotonin reuptake inhibitors (SSRIs) and
anxiolytics are often helpful
[Weiss H, Rastan V, Mu¨llges W, Wagner RF, Toyka KV. Psychotic symptoms and
emotional distress in patients with Guillain-Barre´ syndrome. Eur Neurol. 2002;47(2):74-
78.]