Acute Demyelinating Process: Peripheral to Proximal
Guillain-Barré Syndrome (GBS) is the most likely diagnosis for an acute demyelinating process progressing from peripheral to proximal, requiring prompt treatment with intravenous immunoglobulin or plasma exchange.
Diagnosis
GBS typically presents as a rapidly progressive, symmetrical ascending weakness that reaches maximum disability within 2-4 weeks of onset, with the characteristic pattern of progression from distal (peripheral) to proximal 1.
Key Diagnostic Features:
- Rapidly progressive bilateral weakness with ascending pattern (distal to proximal)
- Diminished or absent deep tendon reflexes
- Sensory symptoms (paresthesias, numbness)
- History of recent infection (respiratory or gastrointestinal) in 2/3 of cases
- Albuminocytological dissociation in CSF (elevated protein with normal cell count)
Diagnostic Workup:
- CSF Analysis: Look for albuminocytological dissociation 1, 2
- Electrophysiological Studies: To confirm diagnosis and distinguish subtypes 2
- MRI: Consider in atypical cases, particularly to rule out spinal cord pathology 2
- Serology:
- Anti-ganglioside antibodies (particularly anti-GQ1b for Miller Fisher variant)
- Nodal-paranodal antibodies if autoimmune nodopathy is suspected 2
Differential Diagnosis:
- Acute-onset Chronic Inflammatory Demyelinating Polyneuropathy (A-CIDP) - consider if progression continues beyond 8 weeks 2
- Immune checkpoint inhibitor-related polyneuropathy 3
- MOG antibody-associated demyelination 3
- Systemic lupus erythematosus with peripheral nervous system involvement 3
Treatment
First-line Treatment:
For patients who cannot walk unaided (moderate to severe disease):
- Intravenous Immunoglobulin (IVIg):
OR
- Plasma Exchange (PE):
Important Treatment Considerations:
- Do not use corticosteroids alone - they are ineffective in GBS 1, 2
- Do not routinely administer a second IVIg course in patients with poor prognosis 2
- Do not use PE followed immediately by IVIg 2
For Pain Management:
- Consider gabapentinoids, tricyclic antidepressants, or carbamazepine 2
Monitoring and Supportive Care
Respiratory Function:
- Monitor closely as 20% of patients develop respiratory failure requiring mechanical ventilation 1
- Consider using the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk 2
Autonomic Function:
- Monitor for blood pressure/heart rate instability, pupillary dysfunction, and bowel/bladder dysfunction 1
Additional Supportive Measures:
- Venous thromboembolism prophylaxis 1
- Early rehabilitation
- Nutritional support
Prognosis
- Mortality rate: 3-10% despite best medical care 1
- Recovery: 60-80% of patients able to walk independently 6 months after onset 1
- Most improvement occurs in the first year but can continue for >5 years 1
- Recurrence is rare (2-5% of patients) 1
Prognostic Assessment:
- Consider using the modified Erasmus GBS outcome score (mEGOS) to assess outcome 2
Special Considerations
Treatment-Related Fluctuations:
- About 10% of GBS patients have secondary deterioration within 8 weeks after IVIg 4
- May require repeated IVIg treatment
Acute-onset CIDP:
- About 5% of patients initially diagnosed with GBS develop A-CIDP 4
- Consider this diagnosis if progression continues beyond 8 weeks 2
Immune Checkpoint Inhibitor-Related Cases:
- For immune checkpoint inhibitor-related acute demyelinating polyneuropathy, hold the immunotherapy and consider high-dose corticosteroids (methylprednisolone 1-2 mg/kg/day) 3
- For severe cases, consider pulse corticosteroids plus IVIg or plasmapheresis 3
Early diagnosis and prompt initiation of immunotherapy are crucial for improving outcomes in patients with acute demyelinating polyneuropathy.