chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Introduction

A group of chronic progressive or relapsing inflammatory demyelinating peripheral neuropathies.

Classification

Two major forms of CIDP:

• monoclonal IgM that reacts with myelin-associated glycoprotein (MAG)
  • occurs in 1/2 of patients with IgM gammopathy & neuropathy
  • more sensory than motor
  • slowly progressive
• osteosclerotic myeloma
  • monoclonal IgA or IgG that do not react with MAG
  • predominantly motor
  • often indolent in nature
  • may eventually produce severe wasting
  • sensory & autonomic phenomena are uncommon

Etiology

• autoimume inflammatory disorder
• rarely associated with solid tumors
  • breast cancer
  • lung cancer
  • colon cancer
• rarely associated with Waldenstrom's macroglobulinemia, heavy chain disease, or lymphoma
• may occur in association with diabetes mellitus, paraproteinemia or HIV1 infection

Pathology

• segmental loss of myelin with preservation of axons

Clinical manifestations

• insidious, progressive symmetric neuropathy evolving over months
• symptoms stabilize (but may persist) after 3 months
• mixed sensory & motor neuropathy
  • symmetric distal & proximal muscle weakness, chronic progressive or relapsing
  • symmetric sensory loss, distal sensory paresthesias
• diffuse areflexia

Laboratory

• lumbar puncture with CSF analysis:
  • elevation of cerebrospinal fluid protein
  • no CSF pleocytosis
• serum protein electrophoresis & CSF protein electrophoresis (high resolution)
  • rule out multiple sclerosis
  • identify monoclonal gammopathy
• bone marrow biopsy

Diagnostic procedures

• nerve conduction study
  • slowed nerve conduction velocities
    • including motor nerves
• electromyography:
  • dispersion of compound muscle action potential
• sural nerve biopsy may be normal if demyelination is proximal

Radiology

• nerve ultrasound is complementary to nerve conduction studies in improving sensitivity for diagnosis of CIDP

Differential diagnosis

• Guillain Barre syndrome
  • distinguished from Guillain Barre syndrome by
    • insidious rather than acute time course
      • Guillain Barre syndrome reaches a nadir of symptoms within 4 weeks
    • more prominent involvement of sensory nerves
    • lack of involvement of autonomic nerves
    • responsiveness to immunotherapy
• POEMS syndrome
  • lambda monoclonal gammopathy, hepatosplenomegaly & skin changes suggest POEMS syndrome
• multiple sclerosis
• diabetic amyotrophy

Management

• long-term therapy
• glucocorticoids
• plasmapheresis
• IV immunoglobulin
• immunosuppressive therapy
  • methotrexate
  • cytoxan
  • azathiaprine
  • cyclosporin
• efgartigimod alfa (Vyvgart) in combination with hyaluronidase-qvfc (Vyvgart Hytrulo) reduces risk of CIDP relapse
• treat associated malignancy

More general terms

• inflammatory demyelinating polyneuropathy
• chronic neurologic disease

More specific terms

• distal acquired demyelinating symmetric neuropathy (DADS)

Additional terms

• Guillain-Barre syndrome (GBS)
• multiple sclerosis (MS); includes clinically isolated syndrome
• POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome

Patient information

chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patient information