What are the typical symptoms, age of onset, progression, and treatment options for a patient with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Clinical Features and Management

Typical Symptoms

CIDP presents with progressive, symmetric proximal and distal muscle weakness, sensory loss (numbness and tingling), and reduced or absent deep tendon reflexes developing over at least 2 months. 1

Key clinical features include:

• Motor symptoms: Gradual weakness affecting both proximal and distal muscles, typically symmetric 1, 2
• Sensory symptoms: Numbness, tingling, and sensory loss in extremities 2
• Reflex changes: Reduced or absent deep tendon reflexes 1, 2
• Dysphagia: May occur in some cases 1

Atypical Presentations

Approximately 50% of pediatric cases present atypically, and similar variants occur in adults 3:

• Distal variant: Predominantly distal weakness and sensory loss 3
• Pure motor variant: Weakness without sensory involvement 3
• Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM/Lewis-Sumner): Asymmetric involvement with preserved reflexes in unaffected areas 1
• Pure sensory variant: Rare presentation with only sensory symptoms 3

Age of Onset

CIDP can occur at any age, but typical onset is in middle to older adulthood, with pediatric cases ranging from early childhood through adolescence. 3, 2

• Adult cases commonly present in the 5th-7th decades 2
• Pediatric cases documented from age 3.5 years through adolescence (up to 17-18 years) 3
• The condition affects both sexes equally 3

Disease Progression

CIDP is distinguished from Guillain-Barré syndrome by its slower progression over more than 2 months, compared to the acute progression over days to weeks seen in GBS. 1

Disease course characteristics:

• Progression timeline: Symptoms develop gradually over at least 8 weeks (>2 months), which is the key diagnostic criterion separating it from acute inflammatory demyelinating polyneuropathy 1
• Variable course: Some patients experience chronic progressive worsening, while others have a relapsing-remitting pattern 4
• Long-term outcomes: In pediatric series, 51.4% achieved complete remission, 10.8% had remission with minimal residual deficits, and 37.8% required ongoing treatment with gradual improvement 3

Treatment Approach

First-Line Therapies

For severe or progressing CIDP, initiate pulse corticosteroids (methylprednisolone 1g IV daily for 3-5 days) plus IVIG 2g/kg over 5 days. 1

The three established first-line options are 5, 4:

• Intravenous immunoglobulin (IVIG): 2g/kg divided over 2-5 days; can also be given subcutaneously 1, 4
• Corticosteroids: Pulse methylprednisolone (1g IV daily for 3-5 days) or oral prednisone 1, 5
• Plasma exchange (PLEX): Typically 5-10 treatments over 2-4 weeks 1, 5

Treatment Selection Strategy

Start with IVIG or corticosteroids based on:

• IVIG preferred when: Rapid response needed, avoiding steroid side effects is priority, or patient has contraindications to steroids 5
• Corticosteroids preferred when: Cost is a major factor or long-term maintenance therapy is anticipated 5
• Combination therapy: Use pulse steroids plus IVIG for severe, rapidly progressive cases 1

Second-Line and Escalation Therapies

For inadequate response to first-line therapy or as steroid-sparing agents, consider immunosuppressants or rituximab. 1, 4

Options include:

• Azathioprine: Most commonly used immunosuppressant in pediatric and adult cases 3, 4
• Methotrexate: Alternative immunosuppressant 3, 4
• Rituximab: Reserved for refractory cases with limited improvement from conventional therapies 1, 3
• Plasma exchange: For severe cases not responding to IVIG or steroids 1, 6

Monitoring and Duration

Treatment response should be monitored through:

• Clinical assessment: Strength testing, functional mobility, sensory examination 5
• Electrodiagnostic studies: Repeat nerve conduction studies to assess demyelination 5
• Functional scales: Document baseline and serial measurements 5

Many patients require long-term maintenance therapy, with treatment duration ranging from months to years. 3, 4

Critical Pitfalls to Avoid

• Misdiagnosis is common: Ensure proper electrodiagnostic confirmation and CSF analysis showing cytoalbuminologic dissociation before committing to long-term immunotherapy 5, 2
• Plasma exchange complications: Monitor for thrombotic events, particularly in patients with prothrombotic risk factors, as multifocal cerebral infarcts have been reported 2
• Premature treatment discontinuation: Approximately 38% of pediatric patients require ongoing treatment; assess for relapse risk before stopping therapy 3