Neurológia pre prax 1/2016

Chronic inflammatory demyelinating neuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic dysimmune polyneuropathy. It belongs to rare diseases, as the prevalence is reported between 0.8–8.9/100 000. Progression of signs and symptoms beyond 2 months represents the key diagnostic criterion. Motor signs and symptoms usually prevails to sensory ones. Beside typical form of CIDP representing 2/3 of cases the wide clinical spectrum of CIDP comprises also atypical forms, the most frequent being s.c Lewis-Sumner syndrome (15% of cases) and a sensory form (10% of cases). Diagnostics of CIDP is based, in addition to clinical criteria and course, on electrodiagnostic criteria of multifocal demyelinating neuropathy and some other laboratory findings, especially hyperproteinorrhachia, magnetic resonance finding of thickening and gadolinium enhancement of nerve roots and brachial and lumbar plexuses, and improvement after immunomodulating treatment. Corticosteroids and intravenous humane immunoglobulin are recommended treatment options of first order, followed by therapeutic plasma exchange in case of inefficacy or intolerability of first-order treatment. Immunomodulating drugs (especially cyclosporin, azathioprine, mycophenolate mofetil, cyclophosphamide and methotrexate) are used as additive treatment.

Keywords: chronic inflammatory demyelinating neuropathy, diagnostics, therapy, diagnostic criteria