With respect to the Guillain Barre Syndrome (GBS):

  • Outline how you would distinguish between GBS and Critical Illness Polymyoneuropathy (CIP).
  • What are the current treatment options in GBS? Briefly outline the supporting evidence
  • What is the prognosis of GBS and what factors are associated with a worse outcome?

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College Answer

GBS

CIP

History and 
Examination

Recent GI or resp illness.
Progressive bilateral symmetric 
paralysis. Subtypes can be more 
localized e.g. MF opthalmoplegia 
and ataxia. 
Sensory involvement is common. 
Areflexic. 
Autonomic involvement may be 
present

Always occurs in association with a critical illness in 
particular severe sepsis. May have an association 
encephalopathy in early stages. It is a symmetrical 
weakness. May have muscle tenderness, hyporeflexic, 
diminished distal sensation Not associated with 
autonomic involvement

Investigations 
Blood

Albuminocytologic dissociation in CSF. 
Identification of infection with 
campylobacter, mycoplasma, 
EBV,Varicella, CMV. 
Elevated csf IGG levels and 
possible serum antiganglioside 
antibodies

Elevated CK which may be 
transient.

Nerve 
conduction 
studies and 
EMG

When demyelinating form is 
present, you get a reduction in 
conduction velocity as well as 
reduction in CMAP. 
In axonal forms however it is 
only the distribution of the 
findings that helps determine the 
diagnosis.

A axonal neuropathy resulting in a decreased 
CMAP without a reduction in conduction velocity

b)

  • Plasma exchange and IVIG are both better than no therapy in hastening recovery from an episode and are equally effective. No value in both being given routinely.

c)

  • - 5% mortality from medical complications in hospital.
      • Up to 20% of patients are still significantly disabled at 6 months and 15% still have significant functional disability at 1 year.
    • Recurrence rate is 7% with the mean interval between recurrences being 7 years.
  • Poor prognosis associated with:
    • Older age
    • Rapid onset (<7days) prior to presentation
    • Severe muscle weakness at presentation
    • Need for mechanical ventilation
    • EMG showing average distal motor response amplitude <20% normal
    • Preceding diarrhoeal illness (proven Campylobacter)

Discussion

The distinctions between GBS and CIPN:

  • CIPN is associated with a severe illness, whereas GBS is associated with a mild one.
  • CIPN does not have autonomic dysfunction, whereas GBS does.
  • CIPN will not have any specific CSF findings, whereas GBS will have raised CSF protein and perhaps even a monocytosis
  • CIPN nerve conduction studies will not show any decrease in conduction velocity, whereas in GBS the cardinal feature is decreased conduction velocity.

As for management, Oh's Manual recommends either plasma exchanges or IV immunoglobulin, but there is no benefit in giving both together.

A discussion of the approach to the ICU patient with generalised weakness is available elsewhere.

A generic exam-oriented approach to Guillain Barre syndrome is presented in the "Required Reading" section.

An even better table than the CICM college answer is available in this 2006 article fom Respiratory Care; it is reproduced below.

Clinical Features and Laboratory Findings 
Critical-Illness Polyneuropathy, Guillain-Barre´ Syndrome, and Critical Illness Myopathy
 

Critical illness polyneuropathy

Guillain-Barre

Critical Illness Myopathy

Clinical setting 
 

Sepsis 
Multiorgan failure

Septic encephalopathy

Antecedent viral illness

Surgery

Campylobacter jejuni

HIV

Neuromuscular blocking drugs

Corticosteroids

Asthma

Organ transplant

Motor weakness

Generalised and distal

Generalised and ascending

Generalised and proximal

Reflexes

Diminished

Absent

Preserved but weak

Cranial nerve palsy

Rare

Common

Absent

Dysautonomia

Normal autonomic function

Frequent dysautonomia

Normal autonomic function

Sensory deficit

Distal

Normal sensation, or slightly altered

Normal sensation

CK level

Normal

Normal

Elevated

Nerve conduction

Reduced CMAP and SNAP amplitude

(compound muscle action potentials and sensory nerve action potentials)

Marked slowing, conduction block

Reduced CMAP amplitude
Normal SNAP amplitude

Needle EMG

Abnormal spontaneous activity

Reduced recruitment

Large polyphasic motor unit potentials (MUPs)

Abnormal spontaneous activity

Reduced recruitment

Normal MUPs (early in disease)

Minimal spontaneous activity

Early recruitment

Small polyphasic MUPs;

Direct muscle stimulation

Normal

Normal

Absent or reduced

Muscle biopsy

Neuropathic changes

Neuropathic changes

Myopathic changes

Thick myosin filament loss

Muscle fiber necrosis

References

References

Oh's Intensive Care manual

Chapter   51   (pp. 568)  Acute  cerebrovascular  complications by Bernard  Riley  and  Thearina  de  Beer

Chapter   57   (pp. 617)  Neuromuscular  diseases  in  intensive  care by George  Skowronski  and  Manoj  K  Saxena

Yuki, Nobuhiro, and Hans-Peter Hartung. "Guillain–Barré syndrome." New England Journal of Medicine 366.24 (2012): 2294-2304.

Jani, Charu. "Critical Illness Neuropathy." Medicine (2011): 237.

Dhand, Upinder K. "Clinical approach to the weak patient in the intensive care unit." Respiratory care 51.9 (2006): 1024-1041.