Symposium on neurosciences
Guillain-Barré Syndrome

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Abstract

Guillain-Barré syndrome is an acute inflammatory immune-mediated polyradiculoneuropathy presenting typically with tingling, progressive weakness, and pain. Variants and formes frustes may complicate recognition. The best known variant is the sensory ataxic form of Miller Fisher syndrome, which also affects the oculomotor nerves and the brain stem. Divergent pathologic mechanisms lead to demyelinating, axonal, or mixed demyelinating-axonal damage. In the demyelinating form, yet to be identified antigens are inferred by complement activation, myelin destruction, and macrophage-activated cleanup. In the axonal and Miller Fisher variants, gangliosides (GM1, GD1a, GQ1b) are targeted by immunoglobulins and share antigenic epitopes with some bacterial and viral antigens. Campylobacter jejuni infection is associated with an axonal-onset variant; affected patients commonly experience more rapid deterioration. Many other antecedent infectious agents have been recognized including the most recently identified, Zika virus. Supportive care remains the mainstay of therapy. Plasma exchange or intravenous immunoglobin hastens recovery. Combination immunotherapy is not more effective, and the efficacy of prolonged immunotherapy is unproven. One in 3 patients will have deterioration severe enough to require prolonged intensive care monitoring or mechanical ventilation. Full recovery is often seen; most patients regain ambulation, even in severe cases, but disability remains in up to 10% and perhaps more. Numerous challenges remain including early identification and control of infectious triggers, improved access of modern neurointensive care worldwide, and translating our understanding of pathogenesis into meaningful preventive or assistive therapies. This review provides a historical perspective at the centenary of the first description of the syndrome, insights into its pathogenesis, triage, initial immunotherapy, and management in the intensive care unit.

Section snippets

The Eponym and 100 Years of Clinical Advances

George Guillain (ghee-lain6) was a student of Pierre Marie, the recipient of the Charcot chair at the Salpêtrière (Figure 1, A). Guillain was one of his most prolific pupils and would become his successor in 1925. In 1916, George Guillain,1 Jean-Alexandre Barré, and André Strohl described acute and progressive limb weakness causing major difficulty in 2 soldiers they examined in an army hospital during the battle of the Somme (northern France, World War I). The key features were (1) the

Pathogenesis

Multiple antecedent and potentially triggering events have been reported.22 The association with infections is established in not only C jejuni but also cytomegalovirus, Epstein-Barr virus, influenza A, Mycoplasma pneumoniae, Haemophilus influenzae, hepatitis (A, B, and E). and Zika virus.23, 24, 25, 26 The risk of GBS from influenza vaccine varies from 3 cases per million to as low as zero.27, 28, 29 Surgery may predispose patients to GBS (more likely in patients with prior malignant or

Clinical Presentation

The diagnosis of GBS is fairly typical, and the differential diagnosis is narrow. Symptoms and signs usually progress within 1 to 2 weeks. Severe back pain and distal limb paresthesias with a “tight band” feeling are common presenting signs, and the paresthesias gradually scatter over the limbs and move proximally. Although tingling is often the presenting symptom, sensory modalities remain normal to mildly impaired. Weakness begins in the more proximal muscles, causing difficulty with climbing

Mimicking Disorders

Very few disorders mimic GBS because their symptoms do not ascend, are not rapid, and are not postinfectious. The more likely candidates are presented in Table 1.59 Other disorders to consider are acute heavy metal intoxication, severe rhabdomyolysis (eg, rhabdomyolysis due to statin use), severe hypokalemia or hypophosphatemia, and intermittent porphyria. Guillain-Barré syndrome can be a first manifestation of chronic inflammatory demyelinating polyneuropathy, which typically worsens over 2

Laboratory Tests

Cerebrospinal fluid analysis typically reveals high protein levels with a normal white blood cell count (the classic albuminocytologic dissociation). Although unusual, more than 10 white blood cells per high-power field may be seen but is more common in associated disorders such as Lyme disease, sarcoidosis, and AIDS. Because CSF can include a normal cell count and normal protein level in weak patients early in presentation, its usefulness is questionable. Lumbar puncture may be more

General Management

Many patients with GBS become bedbound, and this immobilization requires expert nursing care. Skin, eye, and mouth care is crucial to the comfort of quadriplegic patients.

ICU Management

The management of patients with GBS is difficult because of its unpredictable course, potential for rapid deterioration, and high chances for respiratory failure. Any patient with worsening weakness on initial evaluation or presentation requires admission and observation in an ICU (Figure 4). However, only 1 in 3 patients will experience deterioration severe enough to mandate further or prolonged close monitoring and possibly endotracheal intubation.22, 72

Respiratory failure and GBS can be

Outcome

Severe manifestations of GBS admitted to the neurosciences intensive care unit involves long-term respiratory care, management of dysautonomia (wide blood pressure swings and cardiac arrhythmias) and management of major systemic complications. Although supposedly benign when described initially, far more serious and critical appearences became recognized.81 Several patients accounts of the experience have been published varying from titles such “My worst nightmare” (Byron Comp) to “Happily ever

Precautions

Questions about vaccinations after GBS are very common but difficult to definitively answer because there might be some risk with any type of vaccination. No cases of GBS or recurrent GBS after any type of vaccination were found in a Kaiser Permanente study.84 The benefit of influenza vaccination is great and in particular in elderly patients with pulmonary disease. The decision to proceed with vaccination is at the discretion of the physician in discussion of pro's and con's with the patient.

Future Directions and Immediate Challenges

Although GBS was initially described 100 years ago, its recent occurrence in association with the Zika virus emphasizes the importance of ongoing study of this disorder.87 In those affected, the most common presentation was that of acute motor axonal neuropathy, with 19% having novel autoantibodies to the glycolipid GA1. The virus is spread by mosquito-borne infection and sexual transmission and will most likely spread to the Americas. Epidemiological research to identify and control outbreaks

Conclusion

Guillain-Barré syndrome is a well-recognized, acute, disabling neurologic illness. Management is supportive with immunomodulating therapy but may involve prolonged intensive care and long-term neurorehabilitation. Without a full understanding of its pathophysiology, it will be difficult to find a treatment that dramatically hastens the slow recovery trajectory. Guillain-Barré syndrome can profoundly affect a patient's life and family because it may take years to resolve completely. Patients

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