What is the initial treatment for patients with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presenting with sensory deficits?

Sensory Deficits Pattern and Initial Treatment in CIDP

The initial treatment for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presenting with sensory deficits should be intravenous immunoglobulin (IVIG), plasma exchange, or corticosteroids, with IVIG often preferred due to its favorable efficacy and side effect profile. 1, 2

Sensory Deficit Patterns in CIDP

Predominant Sensory Manifestations

• CIDP commonly presents with a predominant sensory axonal neuropathy, though motor and autonomic involvement can occur 3
• Large sensory nerve fibers are most commonly affected, leading to symmetrical length-dependent "dying back axonopathy" 3
• Sensory symptoms typically include acral pain, paraesthesia (tingling like pins and needles), dysaesthesia, allodynia, and hyperalgesia 3
• Sensory loss appears in a "glove and stocking type" distribution with impaired perception of light touch, vibration sense, pin prick, and proprioception 3

Small Fiber Involvement

• Small fiber neuropathy may also be present, affecting nerve terminals involved in temperature and pain perception 3
• This can lead to painful burning sensations in feet and/or hands, and lancinating pain 3
• Clinical examination reveals decreased pain perception and temperature sensation in affected areas 3

Progression Pattern

• Some patients initially present with pure sensory symptoms (chronic sensory demyelinating neuropathy) but may later develop motor deficits 4
• This indicates that a pure sensory presentation may sometimes be a transitional clinical stage of CIDP 4

Diagnostic Approach

Clinical Assessment

• Early detection is crucial for adequate management and preventing irreversible neuropathy 3
• Baseline and ongoing clinical evaluation (before every treatment cycle) of physical function is critical 3
• Neurological consultation is recommended if there is uncertainty in the clinical presentation 3

Neurophysiological Examination

• Conventional neurophysiological methods (EMG with nerve conduction studies) provide complementary information to clinical assessment 3
• Axonal degeneration is evident as progressive reduction of sensory nerve action potential amplitude 3
• In small fiber neuropathies, standard neurophysiological techniques may be normal 3
• MRI of the brachial or lumbosacral plexus can help identify focal or diffuse peripheral nerve abnormalities in CIDP variants 5

Initial Treatment Options

First-Line Therapies

• Three main first-line treatments are available for CIDP: 1
  • Intravenous immunoglobulin (IVIG)
  • Plasma exchange
  • Corticosteroids

IVIG Therapy

• IVIG is effective as an induction treatment for CIDP 1
• Repeat intravenous or subcutaneous immunoglobulin infusion therapy can be used as maintenance treatment 1
• Subcutaneous administration provides a new option that may increase independence and improve tolerability 2

Plasma Exchange

• Plasma exchange is effective as an induction treatment for CIDP 1
• It may be particularly beneficial in cases with rapid progression or severe symptoms 6

Corticosteroid Therapy

• Corticosteroid administration, such as daily oral or pulse therapies, may be effective for both induction and maintenance 1
• Methylprednisolone (2-4 mg/kg/day) followed by slow steroid taper is a reasonable approach 3

Treatment Considerations

Early Intervention

• Early treatment is crucial to prevent permanent nerve damage 2
• Treatment should be guided by evidence-based, proven-effective therapies that sequentially escalate depending on the response 6

Treatment Monitoring

• Serial clinical assessments are key to understanding the value of continued immunotherapy 6
• There is no reliable disease activity biomarker that can be used to guide treatment 6
• Objective outcome measures should be adopted to monitor treatment response 7

Treatment-Refractory Cases

• For patients not responding to first-line therapies, immunosuppressive agents may be considered 6
• These require balancing risk, cost, and unknown likelihood of benefit tailored to each patient's circumstances 6

Supportive Interventions

• Regardless of immunotherapy status, addressing residual deficits through supportive interventions is important 6
• These include physical therapy, adaptive equipment, pain management, and emotional support 6

Special Considerations

• Some patients may initially present with pure sensory symptoms but later develop motor involvement 4
• IVIG has been found effective in both pure sensory and sensorimotor types of CIDP 4
• Treatment strategies may include combinations of the aforementioned approaches for both induction and maintenance therapies 1