Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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Contents 1 Introduction 2 Classification 3 Etiology 4 Pathology 5 Clinical-manifestations 6 Laboratory 7 Differential-diagnosis 8 Management 9 More General Terms 10 Additional Terms 11 References

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

Pathology

• segmental loss of myelin with preservation of axons

Clinical-manifestations

• insidious, progressive symmetric neuropathy evolving over months
• symmetric proximal muscle weakness, chronic progressive or relapsing
• sensory loss, distal sensory paresthesias

Laboratory

• lumbar puncture with CSF analysis:

• elevation of cerebrospinal fluid (CSF) protein b ) no CSF pleocytosis

• serum protein electrophoresis & CSF protein electrophoresis (high resolution)

• rule out multiple sclerosis
• identify monoclonal gammopathy

• bone marrow biopsy Specia laboratory:
• nerve conduction study

• slowed nerve conduction velocities

• electromyography:

• dispersion of compound muscle action potential

• sural nerve biopsy may be normal if demyelination is proximal

Differential-diagnosis

• Guillain Barre syndrome

• distinguished from Guillain Barre syndrome by

• insidious rather than acure time course
• more prominent involvement of sensory nerves
• lack of involvement of autonomic nerves
• responsiveness to immunotherapy

• POEMS syndrome
• multiple sclerosis

Management

• long-term therapy
• glucocorticoids
• plasmapheresis
• IV immunoglobulin
• immunosuppressive therapy

• methotrexate
• cytoxan
• azathiaprine
• cyclosporin

• treat associated malignancy

More General Terms

• peripheral nerve disease; peripheral neuropathy
• inflammatory demyelinating polyneuropathy
• chronic neurologic disease

Additional Terms

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

References

1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 625-26, 2464
2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, American College of Physicians, Philadelphia 1998, 2006, 16
3. NINDS Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Information Page [1]
4. National Guideline Clearinghouse European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. European Federation of Neurological Societies ngc-guideline: [2]

chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)