Table of Contents
Primary Category
Neuromuscular
P-Category
Secondary Category
S-Category
Authors:
Introduction
- Classified under the eponym Guillain-Barre syndrome (GBS)
- Other variants of GBS include Acute Motor Axonal Neuropathy (AMAN), Acute Motor and Sensory Axonal Neuropathy (ASMAN), and the Miller Fisher syndrome (MFS).
- AIDP is acute monophasic immune-mediated polyradiculoneuropathy provoked by a preceding infection
Epidemiology
- Mean age of onset of 40 years affecting slightly more males than females of all ages, races and nationalities
- Worldwide incidence of GBS ranges from 0.6 to 4.0/100,000 people
Etiology
- AIDP is most common form of GBS in North America, Europe and most of the developed world representing about 85% to 90% of cases
- ⅔ patients give history of antecedent respiratory tract or GI tract infection
- Campylobacter jejuni infection is most commonly observed in 25-50% of the adult patients, with higher frequency in Asian countries.
- Campylobacter associated GBS has worse prognosis, manifests slow recovery and with greater residual neurologic disability.
- Other precipitants include EBV, CMV, mycoplasma, pneumonia, and influenza-like illnesses
- Also has an association with HIV infection; predominantly in those who are not profoundly immunocompromised
Pathogenesis
- Molecular Mimicry: auto-antibodies cross-react with peripheral nerve components because of sharing of cross-reactive epitopes
- Immune response can be directed towards myelin or axon of peripheral nerve
- Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP): When directed against epitopes in myelin or Schwann cell membrane; cellular + humoral immune responses are involved
- Progression of disease for about two to four weeks
- In a study pop. of 494 adult patients, disease nadir was reached in 2 week in 80% and within 4 weeks in 97%
- If disease progression is more than 8 weeks, it is classified as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
- Subacute inflammatory demyelinating polyneuropathy (SIDP) is considered if nadir is reached between 4 and 8 weeks
Box 1: Clinical Features of AIDP
Box 1: Clinical Features of AIDP
Reaching nadir within 3-4 weeks
- Dysautonomia (~70%)
- Facial nerve Palsies (>50%)
- Oculomotor weakness (15%)
- Oropharyngeal weakness (50%)
- SIADH (5%; more in hospitalized patients)
- Paresthesias accompanying weakness (>80%)
- Weakness begins in arms and facial muscles (10%)
- Decreased or absent reflexes in affected arms or legs (90%)
- Severe respiratory muscle weakness requiring ventilatory support (10%-30%)
- Progressive, ascending & fairly symmetrical weakness starts in the legs (90%)
- Pain due to nerve root inflammation, typically in the back and extremities (66%)
Rare features
- Unusual clinical features of AIDP include: papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes.
- Posterior reversible encephalopathy syndrome, also called reversible posterior leukoencephalopathy syndrome has been associated with GBS in children and adults likely related to acute hypertension from dysautonomia
Investigations
- CSF Analysis
- Cytoalbuminologic dissociation: normal cell count and increased protein level
- In few cases (about 15%), mild increase in CSF cell count was seen
- Electrodiagnostic findings
- Absent H reflexes
- Decreased motor nerve conduction velocity
- Sural sparing pattern (early stages)
- Prolonged distal motor latency
- Increased F wave latency
- Conduction blocks
- Temporal dispersion
- Serial NCS over weeks are sometimes needed to reliably distinguish between AMAN and ADIP
- MRI
- Thickening and enhancement of the intrathecal spinal nerve roots and cauda equina
- Involvement of anterior spinal roots may be present, or both anterior and posterior roots
Diagnostic Criteria
In 1990, Asbury and Cornblath expanded the diagnostic criteria
Features required:
- Progressive motor weakness of more than one limb.
- Areflexia
Features strongly supportive of the diagnosis:
- Progression of symptoms and signs
- Relative symmetry
- Mild sensory symptoms or signs
- Cranial nerve involvement
- Recovery
- Autonomic dysfunction
- Pain
- No fever at the onset
- Elevated protein in CSF with cell count ≤50cells/mm3
- Electrodiagnostic abnormalities consistent with GBS
Features that make diagnosis of GBS doubtful:
- Sensory level
- Marked, persistent asymmetry of weakness
- Bowel and bladder dysfunction at onset
- Severe and persistent bowel and bladder dysfunction
- Severe pulmonary dysfunction with little or no limb weakness at onset
- Fever at onset
- CSF pleocytosis
Table 2: Differential Diagnosis of AIDP
Table 2: Differential Diagnosis of AIDP
Disease
Pattern of Paralysis
Investigation
Autonomic Dysfunction
Cranial Nerve Involvement
Sensory Involvement
Management
- Triage and Initial Management algorithm:
- Urgent intubation if clinical signs of respiratory distress, oxygen desaturation, or hypercapnia are present
- Admit to ICU and consider elective intubation if
- Moderate to severe or rapidly progressive appendicular weakness with bulbar dysfunction
- Symptoms of respiratory insufficiency
- Abnormal spirometry
- FVC < 20mL/Kg
- MIP > (-neg) 30 mmH2O
- MEP < 40 mmH2O
- or > 30% decrease from baseline value
- Evidence of aspiration
- Pronounced dysautonomia
- Monitor on the ward if none of the above four mentioned points
- Dysautonomia:
- Manifestations:
- Tachycardia
- Urinary retention
- Hypertension alternating with hypotension
- Orthostatic hypotension
- Bradycardia
- Arrhythmias
- Ileus
- Loss of sweating
- So the management must include:
- Management:
- Monitoring:
- Cardiac rhythm
- Close blood pressure monitoring
- Fluid status
- Intra-arterial monitoring if significant blood pressure fluctuations are present
- Daily abdominal auscultation to monitor for bowel silence and for adynamic ileus
- Treatment:
- Low-dose phenylepinephrine if fluids are not effective for hypotension (Only low doses of carefully titrated short-acting vasoactive agents should be used to avoid possible effects of denervation hypersensitivity)
- Labetalol, esmolol, or nitroprusside for MAP>125 mmHg
- Intervention with administration of atropine and cardiac pacing might be required in life-threatening arrhythmias like AV block and asystole
- Erythromycin or neostigmine may be effective for ileus
- Gabapentin or carbamazepine for ICU pain control in acute phase
- TCAs, gabapentin, carbamazepine, or pregabalin can be used for long-term pain management
- Main modalities of treating GBS:
- Plasmapheresis and IVIg are the mainstay of treatment for AIDP or for other variants of GBS
- Oral steroids and methylprednisolone have not shown any benefits in this disorder
- Combination of IVIg and plasma exchange is not significantly better than either alone
- Plasma exchange:
- Four to six rounds of plasmapheresis over 8-10 days.
- Evidence based conclusion:
- Median time to recover with plasma exchange was shorter than control group
- Time of onset of motor recovery in mild GBS was significantly shorter than control group
- More effective when started within 7 days of onset
- 4 rounds of treatment were superior to 2 in moderate severe GBS
- Adverse effects:
- Hypotension
- Sepsis
- Problems related to central venous catheter:
- Local hematoma
- Pneumothorax
- Line-related infection
- Mild coagulopathy
- Hypocalcemia
- Transfusion reaction (including transfusion related acute lung injury)
- Intravenous immune globulin (IVIg):
- 0.4 gram/kg per day for 5 days
- Evidence based results:
- Studies compared IVIg with plasmapheresis
- IVIg is as effective as plasma exchange for GBS
- Patients assigned to IVIg are significantly less likely to discontinue treatment than those on plasmapheresis
- Adverse effects:
- Aseptic meningitis
- Chest pain
- Infusion reaction:
- Headache
- Shivering
- Myalgia
- Anaphylaxis (if IgA deficient)
- Acute renal failure
- Hyperviscosity leading to stroke (rare)
- No evidence shows a second course of IVIg to be effective in patients of GBS that continue to deteriorate
Table 3: Complications of AIDP
Table 3: Complications of AIDP
Pulmonary
Cardiac
Gastrointestinal
Genitourinary
Prognosis
- Respiratory Failure:
- 15-30% of AIDP patients need ventilatory support
- Predictors of respiratory failure:
- Evidence based conclusion: mechanical ventilation was required in >85% patients with four out of the following six predictors
- Time of onset to admission <7 days
- Inability to cough
- Inability to stand
- Inability to lift the elbows
- Inability to lift the head
- Increase in LFTs
- Disability:
- 20% of patients are unable to walk after 6 months
- Most patients have residual pain and fatigue due to persistent axonal loss
- Mortality rate
- 3-10%
- Death can occur in acute progressive state most probably due to ventilatory insufficiency or pulmonary complications, or from dysautonomia like arrhythmia
- Death can also occur in later stage
- Treatment Related Fluctuations (TRF)
- 10% patients will deteriorate within the first 8 weeks after the start of IVIg which is called TRF
- Repeated treatment with IVIg has been shown to be beneficial in these patients
- CIDP with acute onset (A-CIDP)
- 5% of patients initially diagnosed with AIDP were eventually found to have acute onset CIDP
- Diagnosis should be considered in patients :
- Initially diagnosed with AIDP who have 3 or more periods with clinical deterioration
- Or when there is new deterioration after 8 weeks from onset of weakness
- TRF vs A-CIDP:
- The difference is significant management wise.
- There might be improvement with retreatment in TRF whereas patients with A-CIDP need chronic maintenance treatment with IVIg or a switch to corticosteroid treatment
- GBS Disability Score:
- It uses level of disability be documented using a scale from 0 to 6.
- EGOS:
- The Erasmus GBS Outcome Score (EGOS) is a prognostic model based on age, diarrhea, and GBS disability at 2 weeks after hospital admission
- It accurately predicts the chance of being able to walk independently at 6 months
- mEGOS:
- Modified EGOS was developed to apply at hospital admission and at day 7 of hospital stay as compared to original EGOS model which applied on 14th day of stay
- It requires Medical Research Council (MRC) Scale for Muscle Strength score instead of disability
- According to this study, poor outcome is associated with:
- Older age
- Rapid disease progression
- Severe disease indicated by GBS disability score or MRC sumscore
- C. jejuni or CMV positive serology
- Preceding respiratory tract infection
- NCS might also have prognostic value; patients with features of demyelination are more prone to need mechanical ventilation
- Low compound action potentials (CMAPs) are the most consistent findings predictive of poor outcome
- Online prognosis model: https://gbstools.erasmusmc.nl/prognosis-tool
Further reading
Comprehensive review article: Willison, H. J., Jacobs, B.C.. van Doorn, P. A. (2016). Guillain-Barré syndrome. The Lancet. 388:10045(717-727). https://doi.org/10.1016/S0140-6736(16)00339-1
Bibliography
- Willison, H. J. et. al (2016). Guillain-Barré syndrome. The Lancet. 388:10045(717-727). https://doi.org/10.1016/S0140-6736(16)00339-1
- Walgaard, C. et. al (2011). Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology. 76(11): 968-975. doi: 10.1212/WNL.0b013e3182104407
- Fokke, C. et. al (2014). Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria. Brain. 10.1093/brain/awt285. Epub 2013 Oct 26.
- Rees, J. H. et. al (1995). Campylobacter jejuni infection and Guillain–Barré syndrome. New England Journal of Medicine, 333(21), 1374-1379.
- Visser, L. H. et. al (1995). Guillain-Barré syndrome without sensory loss (acute motor neuropathy) A subgroup with specific clinical, electrodiagnostic and laboratory features. Brain, 118(4), 841-847.
- Jacobs B.C. et. al (1998). The spectrum of antecedent infections in Guillain-Barre syndrome: A case-control study. Neurology. 51(4):1110-1115.
- Dimachkie, M. M., & Barohn, R. J. (2013). Guillain-Barré syndrome and variants. Neurologic clinics, 31(2), 491-510.
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Written by
Board certified in Vascular Neurology, Neuroimaging, and Clinical Neurophysiology, Member of the American Academy of Neurology.