Guillain Barré Syndrome
Guillain Barré syndrome (GBS) is an autoimmune disorder. In GBS, the immune system specifically attacks the nerves. GBS is usually triggered by a viral or bacterial infection. GBS isn't contagious and it's not passed down through families (it isn't an inherited disease).
Symptoms of GBS may include the following:
Numbness or tingling in the hands or feet.
Muscle weakness (usually starts in the feet and moves upward).
Heart rate or blood pressure problems.
Diagnosis of GBS is based on the patient history, neurological electrophysiological, and cerebrospinal fluid (CSF) examinations.
Cerebrospinal fluid examination
CSF examination is mainly used to rule out causes of weakness other than GBS and should be performed during the initial evaluation of the patient. The classic finding in GBS is the combination of an elevated CSF protein level and a normal CSF cell count (known as albumin-cytological dissociation). However, protein levels are normal in 30–50% of patients in the first week after disease onset and 10–30% of patients in the second week. Therefore, normal CSF protein levels do not rule out a diagnosis of GBS.
Electrophysiological studies provide evidence of PNS dysfunction and can distinguish between the subtypes of GBS: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN). Electrophysiological measurements might be normal when performed early in the disease course (within 1 week of symptom onset) or in patients with initially proximal weakness, mild disease, slow progression, or clinical variants. In these patients, a repeat electrodiagnostic study 2–3 weeks later can be helpful.
Although GBS affects the peripheral nervous system, CSF is a potential source for biomarkers, because of its close contact with the nerve roots. Alterations in protein expression, post-translational modification, or turnover within the nervous system may be reflected in corresponding changes in CSF protein levels. Hp and HSp70 are significantly increased, and Cystatin C is downregulated in the CSF of patients with GBS. However, these biomarkers cannot differentiate AIDP type of GBS from AMAN type. The other biomarkers in GBS include myelin sheath associated glial (glia are the supporting cells in the nervous system) and neuronal markers (neuron specific enolase, 14-3-3 proteins, S100B, Hypocretin-1), and immunologic markers (TNF-α, chemokines).
Although most patients with GBS recover spontaneously, all of them need to be hospitalized for disease-modifying immunotherapy and multispecialty supportive care because of the unpredictable, potentially life-threatening and severely disabling course of GBS.
Studies have established plasma exchange (PE) and intravenous immunoglobulin (IVIg) as the disease-modifying treatment of GBS.
Plasma exchange (PE)
Plasma exchange improved outcome in a patient with GBS. The complications of PE comprise hypotension septicemia hypocalcemia and abnormal clotting.
Intravenous immunoglobulin (IVIg)
It is used extensively in the treatment of immunologically mediated disorders. The absolute contraindications to IVIg treatment include selective IgA deficiency, and anaphylaxis (life-threatening, severe allergic response to a drug) from previous Ig infusion, while the relative contraindications comprise cardiac (heart) and renal (kidney) failure.
The common adverse effects to IVIg include malaise, fever, chills, headache, nausea, vomiting, and rashes.
Since GBS has heterogeneous clinical presentation and a wide range of prognoses, it is a difficult disorder to diagnose and manage.