What are the symptoms and treatment options for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Symptoms and Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP is characterized primarily by symmetric motor-predominant peripheral neuropathy with both distal and proximal weakness, with large-fiber abnormalities (weakness and ataxia) predominating over small-fiber abnormalities (autonomic dysfunction and pain). 1

Clinical Presentation

Primary Symptoms

• Motor symptoms (most common):
  • Progressive or relapsing symmetric weakness affecting both proximal and distal muscles
  • Muscle weakness in arms and legs
  • Decreased or absent deep tendon reflexes (areflexia)

Sensory Symptoms

• Numbness and tingling (paresthesias)
• Sensory ataxia (impaired coordination due to sensory loss)
• Vibration and proprioception deficits (large fiber involvement)
• Pain (less common than in small fiber neuropathies)

Autonomic Symptoms (less common)

• May include orthostatic hypotension
• Resting tachycardia
• Gastrointestinal disturbances

Clinical Variants

• Typical CIDP: Symmetric polyneuropathy with proximal and distal weakness
• Distal CIDP: Predominantly distal involvement
• Multifocal CIDP: Asymmetric, localized involvement 2

Diagnostic Approach

Key Diagnostic Criteria

1. Clinical features: Progressive or relapsing course over at least 8 weeks
2. Electrophysiologic evidence of peripheral nerve demyelination:
   • Slowed conduction velocities
   • Temporal dispersion
   • Conduction block
3. Cerebrospinal fluid analysis: Typically shows elevated protein levels without pleocytosis
4. Exclusion of other causes of neuropathy 2

Important Differential Diagnoses

• Guillain-Barré syndrome (more acute onset)
• Diabetic neuropathy
• Multifocal motor neuropathy
• Anti-myelin-associated glycoprotein (MAG) neuropathy
• Paraproteinemic neuropathies
• Hereditary neuropathies

Treatment Approach

First-Line Treatments

1. Intravenous Immunoglobulin (IVIG):

   • Most experts use IVIG as first-line therapy 1
   • Can be administered intravenously or subcutaneously
   • Typically given as loading dose followed by maintenance therapy

2. Corticosteroids:

   • Effective but with significant side effect profile
   • Various regimens (daily, alternate day, or pulse therapy)
   • Requires monitoring for metabolic complications

3. Plasma Exchange (PLEX):

   • Considered when IVIG and corticosteroids are ineffective or contraindicated
   • Usually requires specialized centers for administration 3

Second-Line/Refractory Cases

For patients who don't respond adequately to first-line treatments (approximately 20-30% of cases):

1. Immunosuppressants:

   • Azathioprine
   • Mycophenolate mofetil
   • Methotrexate
   • Cyclosporine

2. Rituximab:

   • Anti-CD20 monoclonal antibody
   • Particularly useful in certain subtypes 3

3. Emerging Therapies:

   • Complement inhibitors (e.g., Eculizumab) for refractory cases 4
   • Fc receptor-targeting therapies currently in clinical trials 3

Treatment Monitoring and Adjustment

Response Assessment

• Treatment response should be judged by objective measures:
  • Improvement in neurological examination
  • Improvement in electrophysiological parameters 1
  • Functional improvement in activities of daily living

Treatment Duration

• Treatment often needs to be individualized based on:
  • Disease severity
  • Response to initial therapy
  • Relapse pattern
  • Side effects

Prognosis and Long-term Management

Most patients with CIDP respond to immunomodulatory therapy, but the disease often follows a chronic course requiring long-term treatment. Some patients may achieve remission, while others require ongoing maintenance therapy to prevent relapses.

Common Pitfalls to Avoid

1. Misdiagnosis: Failing to distinguish CIDP from other chronic neuropathies
2. Inadequate treatment trials: Not giving sufficient time to assess response
3. Overlooking comorbidities: Not screening for associated conditions like diabetes, paraproteinemias, or HIV
4. Inappropriate treatment discontinuation: Stopping treatment too early in responders
5. Missing CIDP variants: Focusing only on classic presentation and missing atypical forms