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Previous Volume 352:1343-1356 March 31, 2005 Number 13 Next

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Chronic inflammatory demyelinating polyneuropathy is a common, albeit underdiagnosed, and potentially treatable disease with an estimated prevalence of about 0.5 per 100,000 children¹ and 1 to 2 per 100,000 adults.^2,3 Clinical similarities to the acute variant of inflammatory demyelinating polyneuropathy (the Guillain–Barré syndrome) and the beneficial effects of immunosuppressive therapies suggest an immune-mediated pathogenesis. Since the first descriptions of patients with corticosteroid-responsive chronic polyneuropathies by Austin,⁴ Thomas et al.,⁵ and Dyck et al.,⁶ the spectrum of clinical presentation and the diagnostic armamentarium have enlarged, and further therapeutic options have evolved. The recognition of this disorder as distinct from other common . . . [Full Text of this Article]

Clinical Presentation

Classic Chronic Inflammatory Demyelinating Polyneuropathy

Demyelinating Neuropathies Distinct from Classic Chronic Inflammatory Demyelinating Polyneuropathy

            Distal Acquired Demyelinating Symmetric Neuropathy

            Multifocal Motor Neuropathy

            Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (the Lewis–Sumner Syndrome)

Other Neuropathies Similar to Chronic Inflammatory Demyelinating Polyneuropathy

Concurrent Diseases

Central Nervous System Involvement

Diagnostic Approach

Electrophysiological Diagnostic Procedures

Laboratory Examinations

Nerve Biopsy

MRI

Pathogenesis

Cellular Immune Response

Humoral Immune Response

Axonal Loss

Current Treatment

Conclusions

Source Information

From the Department of Neurology, Heinrich-Heine University, Düsseldorf, Germany.

Address reprint requests to Dr. Hartung at the Department of Neurology, Heinrich-Heine University, Moorenstr. 5, D-40225 Düsseldorf, Germany, or at hans-peter.hartung@uni-duesseldorf.de.

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