From the Guidelines
Demyelinating diseases, such as Guillain-Barré syndrome, should be managed with a comprehensive approach that includes early recognition, diagnosis, and treatment, as well as monitoring and management of disease progression and complications, as outlined in the ten-step guideline for the diagnosis and management of GBS 1.
Key Considerations
- Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections.
- Diagnosis of GBS is based on the patient history and neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations, and electrophysiological studies can distinguish between the subtypes of GBS: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN) 1.
- Treatment typically involves plasma exchange or intravenous immunoglobulin, and supportive care, such as mechanical ventilation and cardiac monitoring, is crucial for patients with severe disease.
Management and Treatment
- The ten-step guideline for the diagnosis and management of GBS provides a comprehensive approach to managing the disease, including early recognition and diagnosis, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae 1.
- High-dose corticosteroids, such as methylprednisolone, are not typically used to treat GBS, as their effectiveness is not well established, and other treatments, such as plasma exchange or intravenous immunoglobulin, are preferred 1.
- Symptom management is also important, with medications like baclofen for spasticity, amantadine for fatigue, and gabapentin for neuropathic pain, and physical therapy, occupational therapy, and psychological support are crucial components of comprehensive care.
Prognosis and Outcome
- The prognosis for patients with GBS is generally good, with 60-80% of patients able to walk independently 6 months after disease onset, with or without treatment, although some patients may experience residual weakness or other complications 1.
- Mortality is estimated at 3-10% for patients with GBS, even with the best medical care available, and is often due to complications, such as respiratory failure or cardiac arrhythmias 1.
From the Research
Demyelinating Diseases
- Demyelinating diseases, such as multiple sclerosis (MS), are characterized by inflammatory demyelination with axonal transection in the central nervous system 2.
- MS typically presents in young adults with unilateral optic neuritis, partial myelitis, sensory disturbances, or brainstem syndromes, and can lead to physical disability, cognitive impairment, and decreased quality of life 2.
Diagnosis and Treatment
- Diagnosis of MS is made based on a combination of signs and symptoms, radiographic findings, and laboratory findings, which are components of the 2017 McDonald Criteria 2.
- Nine classes of disease-modifying therapies (DMTs) are available for relapsing-remitting MS and secondary progressive MS with activity, including interferons, glatiramer acetate, and monoclonal antibodies 2.
- These DMTs reduce clinical relapses and MRI lesions, with efficacy rates ranging from 29%-68% 2.
Immunomodulatory Drugs
- Immunomodulatory drugs, such as interferon beta and glatiramer acetate, have been shown to be effective in preventing conversion from clinically isolated syndromes (CIS) to clinically definite MS (CDMS) 3.
- Early treatment with interferon beta was associated with a significant reduction in the proportion of patients converting to CDMS after one and two years of follow-up 3.
- Glatiramer acetate and interferon beta-1a have been shown to modulate the B cell system in MS patients, with increases in IL-10 and BAFF protein concentrations 4.
Innate Immunity
- Innate immunity has been neglected in MS immunopathology, but recent studies suggest that interferon beta-1a may modulate the immune response in MS by affecting monocyte function 5.
- Changes in innate immunity cell populations have been observed in MS patients treated with glatiramer acetate and interferon beta-1a, with statistically significant differences in monocyte counts 5.