What is Chronic Inflammatory Demyelinating Polyneuropathy (CIPD)?

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP is an acquired immune-mediated polyradiculoneuropathy characterized by progressive or relapsing symmetric proximal and distal weakness, sensory involvement, and decreased or absent deep tendon reflexes that progresses over more than 2 months, distinguishing it from acute conditions like Guillain-Barré syndrome. 1, 2

Clinical Presentation

• CIDP typically presents with progressive or relapsing symmetric weakness affecting both proximal and distal muscles, sensory dysfunction in at least two limbs, and reduced or absent tendon reflexes 2, 3
• Unlike Guillain-Barré syndrome which progresses over days to weeks, CIDP progresses over more than 2 months 4
• Common symptoms include paresthesias, progressive weakness, sensory dysfunction, and neuropathic pain 2
• Additional symptoms may include cranial nerve involvement, autonomic symptoms, and ataxia 2
• CIDP generally affects older individuals and has a male predominance 2

Diagnostic Criteria

• Diagnosis requires assessment of clinical, electrophysiological, and supportive criteria 3
• Cerebrospinal fluid analysis typically shows cytoalbuminologic dissociation (elevated protein with normal cell count) 4
• Electrophysiological studies show evidence of demyelination and help distinguish CIDP from other neuropathies 4, 5
• MRI of the brachial or lumbosacral plexus can help identify focal or diffuse peripheral nerve abnormalities 4
• Nerve biopsy may be useful in evaluating atypical forms of CIDP 4, 5

CIDP Variants

• Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM or Lewis-Sumner Syndrome) is characterized by asymmetric involvement with preserved reflexes in areas not affected by weakness 4
• Some variants may be predominantly or exclusively motor or sensory 5
• Chronic neurovisceral variants may present with slower progression of neurological symptoms 6

Differential Diagnosis

• Guillain-Barré syndrome (acute onset with progression over days to weeks) 4
• Diabetic polyradiculoneuropathy 4
• Leptomeningeal metastases 4
• Infectious causes: HIV, cytomegalovirus, Epstein-Barr virus, Lyme disease 4, 2
• Systemic inflammatory disorders like lupus 4
• Other peripheral neuropathies including those associated with metabolic disorders, toxins, or nutritional deficiencies 6

Treatment

• First-line therapies according to EAN/PNS guidelines include:

  • Corticosteroids (methylprednisolone 1-2 mg/kg/day) 1, 7
  • Intravenous immunoglobulin (IVIG) 1, 7
  • Subcutaneous immunoglobulin (SCIG) 1, 3
  • Plasma exchange 1, 7

• For severe or progressing symptoms:

  • Consider pulse corticosteroids (methylprednisolone 1g IV daily for 3-5 days) plus IVIG 2g/kg over 5 days 6
  • Plasmapheresis may be used in severe cases not responding to other therapies 6, 7

• Second-line options for refractory cases:

  • Immunosuppressants (methotrexate, cyclophosphamide, mycophenolate mofetil) 7, 2
  • Rituximab may be considered in consultation for cases with limited improvement 6, 1

• Treatment should be initiated early to prevent irreversible neurological deficits 7

• Serial clinical assessments are essential to monitor response and determine need for continued therapy 7

Prognosis and Monitoring

• Axon loss associated with demyelination is the most important factor affecting disability and treatment resistance 5
• Early treatment is associated with better outcomes and reduced risk of permanent disability 7
• Regular monitoring of clinical response is necessary as there are no reliable biomarkers of disease activity 7
• Some patients may develop residual and irreversible neurological deficits even after treatment of the inflammatory component 7

Supportive Care

• Physical therapy, adaptive equipment, and pain management are important components of comprehensive care 7
• Address residual deficits through supportive interventions regardless of immunotherapy status 7