How are sensory deficits in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) managed?

Management of Sensory Deficits in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

First-line treatment options for sensory deficits in CIDP include corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange, with treatment selection based on symptom severity and individual patient factors. 1, 2

Sensory Deficit Patterns in CIDP

• CIDP typically presents with symmetrical sensory axonal neuropathy affecting large sensory nerve fibers in a length-dependent "dying back axonopathy" pattern 1
• Common sensory symptoms include acral pain, paresthesia, dysesthesia, allodynia, and hyperalgesia 1
• Sensory loss appears in a "glove and stocking" distribution with impaired perception of light touch, vibration sense, pin prick, and proprioception 1
• Small fiber involvement may also occur, affecting temperature and pain perception, leading to painful burning sensations and lancinating pain 1, 3

Diagnostic Approach for Sensory Deficits

• Early detection is crucial to prevent irreversible neuropathy, requiring baseline and ongoing clinical evaluation of sensory function 1
• Neurological consultation is recommended when clinical presentation is uncertain 1
• Electromyography (EMG) with nerve conduction studies provides complementary information, with axonal degeneration evident as progressive reduction of sensory nerve action potential amplitude 1
• MRI of brachial or lumbosacral plexus can help identify peripheral nerve abnormalities in CIDP variants 1
• Small fiber involvement may require specialized assessment techniques as conventional nerve conduction studies may be normal 3

Treatment Options

First-Line Treatments

• Corticosteroids:

  • Daily oral prednisone (1 mg/kg/day) or pulse therapy may be effective for both induction and maintenance 3, 4
  • Methylprednisolone (2-4 mg/kg/day) followed by slow steroid taper is a reasonable approach 1
  • High-dose monthly oral dexamethasone is an alternative to daily oral prednisolone 5

• Intravenous Immunoglobulin (IVIg):

  • Produces more short-term improvement than placebo (high-quality evidence) 5
  • Can be administered as repeat intravenous or subcutaneous infusions for maintenance therapy 4
  • Subcutaneous administration provides a new option that may increase independence and improve tolerability 6

• Plasma Exchange:

  • Twice-weekly plasma exchange produces short-term improvement in disability (moderate-quality evidence) 5
  • Complications occur in approximately 3.9% of procedures 5

Treatment Considerations

• Treatment should be initiated early to prevent irreversible nerve damage 6
• Regular monitoring of sensory function is essential, including assessment of light touch, vibration sense, pin prick sensation, and proprioception 3
• Optimization of first-line treatments is needed to determine ideal dosing for individual patients 7
• Serial clinical assessments are key to understanding the value of continued immunotherapy 7

Second-Line Options

• For patients not responding to first-line treatments, immunosuppressants may be considered, though evidence is limited 2
• According to low-quality evidence, methotrexate did not allow more participants to reduce corticosteroid or IVIg doses 5
• It is uncertain whether azathioprine added to prednisone improves outcomes (very low-quality evidence) 5

Management of Residual Sensory Deficits

• Equally important to immunotherapy is addressing residual deficits through supportive interventions 7
• Management should include physical therapy, adaptive equipment, and pain management strategies 7
• Long-term treatment decisions should be based on individual response and the need for continued therapy 7

Monitoring and Follow-up

• Regular assessment of sensory function is essential for tracking treatment response 3
• No reliable disease activity biomarker exists to guide treatment, making serial clinical assessments crucial 7
• Treatment may need to be continued long-term in some patients, while others may not require ongoing therapy 7