Clinical and Electrophysiological Patterns of Chronic Dysimmune Polyneuropathy

Overview

Chronic dysimmune neuropathies (CDN) are a heterogenous group of acquired inflammatory demyelinating neuropathies including chronic inflammatory demyelinating polyneuropathies (CIDP), Lewis-Sumner Syndrome (LSS), multifocal motor neuropathy (MMN) and other rare entities. Despite their relatively low prevalence, CDN lead to substantial costs for patients and society. CDN are usually misdiagnosed due to progressive nature of the disease with little known data regarding disease activity and treatment response

CIDP is the most common treatable CDN worldwide. Its prevalence is ranging between approximately 1 to 8.9 cases per 100.000 . In addition, there is often the tendency to diagnose CIDP in order to attempt a treatment option. This ultimately leads to overdiagnosis and overtreatment in some patients Although the different CDN have varying underlying pathophysiology and clinical characteristics, they are all potentially treatable with immunomodulatory drugs. To date, diagnostic criteria, measurement of disease activity and treatment response of CDN are mainly based on clinical, electrophysiological, and patient related outcome parameters. The commonly used EFNS/PNS diagnostic criteria for CIDP seems to have the best sensitivity among different sets of diagnostic criteria

Conditions

Eligibility

Outcomes