(aka Acute Inflammatory Demyelinating Polyneuropathy, AIDP)
Epidemiology
- Incidence
- Approximately 3500 cases per year in USA and Canada
- GBS is the most common etiology of acute paralysis with neuromuscular ventilatory failure presenting to acute care hospitals
- Age: typically occurs in younger patient group than chronic inflammatory demyelinating polyneuropathy (CIDP) (see Chronic Inflammatory Demyelinating Polyneuropathy, [[Chronic Inflammatory Demyelinating Polyneuropathy]])
- Association with Infection
- GBS follows a viral infection by 1-3 weeks in 66% of cases
- Preceding upper respiratory illness or gastrointestinal illness about 1 month before presentation is common
Predisposing Factors
Infection/Vaccination
- Campylobacter Jejuni Enteritis (see Campylobacter Jejuni, [[Campylobacter Jejuni]]): 26% of cases have positive stool cultures or positive serology (IgA, IgM, and IgG) for C. Jejuni
- Cytomegalovirus (CMV) (see Cytomegalovirus, [[Cytomegalovirus]])
- Epstein-Barr Virus (EBV) (see Epstein-Barr Virus, [[Epstein-Barr Virus]])
- Human Immunodeficiency Virus (HIV) (see Human Immunodeficiency Virus, [[Human Immunodeficiency Virus]])
- Mycoplasma (se Mycoplasma, [[Mycoplasma]])
- Post-Swine Flu Vaccination: 500 cases reported in 1976
- Incubation Period: 1-6 weeks
- Vaccinia Virus (see Vaccinia Virus, [[Vaccinia Virus]])
Hematologic Disease
- Bone Marrow Transplant (see Bone Marrow Transplant, [[Bone Marrow Transplant]] [MEDLINE]
- Hodgkin’s Disease (see Hodgkin’s Disease, [[Hodgkins Disease]])
- Non-Hodgkin’s Lymphoma (NHL) (see Non-Hodgkin’s Lymphoma, [[Lymphoma]])
Other
- Post-Surgery (5-10% of cases): occurs within 1-4 weeks
- Systemic Lupus Eryhthematosus (SLE) (see Systemic Lupus Eryhthematosus, [[Systemic Lupus Eryhthematosus]])
Physiology
- Acquired demyelinating polyneuropathy (probably immune)
- Hypoventilation leads to hypoxia/ acidosis -> pulmonary vasoconstriction
Diagnosis
- ABG
- Early: hypoxemia (with elevated A-a gradient), hypocapnia
- Later: hypoxemia/hypercapnia (occurs when VC falls to <33% of normal)
- PFT’s: restrictive pattern
- Decreased VC (typically 40-70% predicted/occurs due to muscle weakness, decreased lung compliance, and decreased chest wall compliance)
- VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
- Decreased TLC
- Increased RV: due to inability to overcome recoil of chest wall outward at volumes below FRC
- Decreased MVV
- Decreased MIP/MEP
- VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
- Decreased Lung Compliance: due to chronically low lung volumes, microatelectasis, reduced surface tension, and altered interstitial elastic fibers
- Transdiaphragmatic Pressure: using NG balloon (Pga-Pes)/ normal change >25 cm H2O (referenced to TLC), usually 2-20 cm H2O in bilateral paralysis
- Decreased VC (typically 40-70% predicted/occurs due to muscle weakness, decreased lung compliance, and decreased chest wall compliance)
- CXR: may demonstrate aspiration pneumonia or low lung volumes
- Lumbar Puncture (LP): normal cell count with elevated total protein
- Elevated protein occurs by end of first week of symptoms
- EMG/NCV: predominantly demyelinating polyneuropathy
- Decreased NCV’s in motor and sensory nerves
- Evidence of demyelination is more common in patients who will require mechanical ventilation (85% of cases) vs those who did not (51% of cases) [MEDLINE]
- Diaphragmatic EMG/NCV: NCV abnormal at time of maximal weakness, but may be normal early (may help to rule in neuropathy and rule out myopathy and anterior horn cell disease)
- IgG Anti-GQ1b Antibodies: predicts the need for mechanical ventilation [MEDLINE]
Clinical Manifestations
Neurologic Manifestations
- Acute Progressive, Ascending Motor Paralysis
- Limb/truncal muscle weakness
- Difficult to differentiate from CIDP on clinical symptoms and signs alone (however, onset of polyneuropathy symptoms usually precedes the onset of respiratory failure)
- Mild Sensory Deficits
- Dysarthria
- Areflexia
Pulmonary Manifestations
- Dyspnea/Tachypnea (see Dyspnea, [[Dyspnea]])
- Cough (see Cough, [[Cough]])
- Acute/Chronic Hypoventilation (see Acute Hypoventilation, [[Acute Hypoventilation]] and Chronic Hypoventilation, [[Chronic Hypoventilation]]): 33% of cases develop acute ventilatory failure
- Aspiration Pneumonia (see Aspiration Pneumonia, [[Aspiration Pneumonia]])
- Increased Risk of Deep venous Thrombosis (DVT) and Acute Pulmonary Embolism (PE) (see Deep Venous Thrombosis, [[Deep Venous Thrombosis]] and Acute Pulmonary Embolism, [[Acute Pulmonary Embolism]])
Other Manifestations
- Dysphagia (see Dysphagia, [[Dysphagia]])
- Decubitus Ulcers
- Impaired Nutrition
Treatment
- Plasmapheresis
- Decreases hospital stay and duration of mechanical ventilation
- Most effective when used in the first 2 weeks of illness
- Not shown to be of benefit in children
- Cannot be used in patients with hemodynamic instability (such as recent MI/angina/cardiovascular dysautonomia associated with GBS)
- Intravenous Immunoglobulin (IVIG): may be effective (however, more trials are required)
- Mechanical Ventilation: 33% of cases require mechanical ventilation
- Predictors of Requirement for Mechanical Ventilation
- VC and MIP/MEP (these are the best tests to follow serially): VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
- EMG/NCV Evidence of Demyelination: more common in patients who will require mechanical ventilation (present in 85% of cases) vs those who did not require mechanical ventilation (present in 51% of cases) [MEDLINE]
- Presence of Cranial Nerve involvement + IgG Anti-GQ1b Antibodies: predicts the need for mechanical ventilation [MEDLINE]
- Average Duration of Mechanical Ventilation: 58 days (range: 10-104 days)
- Early Tracheostomy: should consider due to usual prolonged course of ventilation required
- Predictors of Requirement for Mechanical Ventilation
- Corticosteroids: probably not effective
- Anti-T-cell Antibody: not effective in decreasing disease severity
Prognosis
- Mortality Rate: 3-8%
- Near Complete Recovery Rate: 85% of cases make near complete recovery
- However, 5-10% of survivors remain serious disabled
- More severe axonal disease may be predicted by presence of a preceding upper respiratory or gastrointestinal illness
References
- Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001 Jun;58(6):893-8 [MEDLINE]
- Anti-GQ1b antibody as a factor predictive of mechanical ventilation in Guillain-Barré syndrome. Neurology. 2004 Mar 9;62(5):821-4 [MEDLINE]
- Guillain–Barre syndrome associated with rapid immune reconstitution following allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplantation (2006) 37, 617–619 [MEDLINE]
- Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome. Lancet Neurol. 2006 Dec;5(12):1021-8 [MEDLINE]