Chronic inflammatory demyelinating polyneuropathy

Kronična upalna demijelinizacijska polineuropatija (eng. chronic inflammatory demyelinating polineuropathy, CIDP) je stečena autoimuna bolest perifernih živaca i živčanih korijena. U patogenezi bolesti ključnu ulogu ima interakcija celularne i humoralne imunosti. Kliničke značajke ove bolesti su simetrična proksimalna i distalna slabost, senzorna disfunkcija te arefleksija. Težina kliničke slike može varirati od blagih do teških neuroloških deficita koji mogu rezultirati invalidnošću bolesnika. Osim tipične prezentacije simptoma, CIDP se može prezentirati atipičnim simptomima koji su dio atipičnih oblika bolesti, a oni uključuju čisti motorni oblik, čisti senzorni oblik, fokalni oblik, predominanto distalni oblik, Lewis Sumner sindrom i multifokalnu motornu neuropatiju (MMN). Dijagnoza CIDP-a se postavlja uz pomoć ekstenzivne elektromioneurografske (EMNG) obrade, a dodatne pretrage uključuju analizu cerebrospinalnog likvora, magnetsku rezonancu (MR), razne pretrage za detektiranje komorbiditeta i nasljednih polineuropatija te biopsiju perifernog živca. U svrhu ispravne dijagnoze CIDP-a razvijene su različite grupe dijagnostičkih kriterija među kojima su najviše korišteni EFNS/PNS (eng. European Federation of Neurological Societies/ Peripheral Nerve Society) kriteriji. CIDP se uglavnom uspješno liječi, a u terapiji se koriste kortikosteroidi, intravenska primjena imunoglobulina (IVIG) te plazmafereza.Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disorder of peripheral nerves and nerve roots. Interaction between cellular and humoral immunity play the main role in CIDP pathogenesis. Clinical features of the disease are symmetrical proximal and distal weakness, sensory dysfunction and areflexia. Severity of the symptoms may vary from mild to severe neurologic dysfunction that may lead to patient's disability. Besides typical presentation, CIDP can present with atypical symptoms that are part of atypical forms of CIDP. These include pure motor CIDP, pure sensory CIDP, focal CIDP, DADS (distal acquired demyelinating symmetric neuropathy), Lewis Sumner syndrome and multifocal motor neuropathy (MMN). CIDP diagnosis is based upon an extensive electromyoneurography (EMNG) procedure, with additional diagnostic tools including cerebrospinal fluid (CSF) analysis, magnetic resonance imaging (MRI), various tests for detection of comorbidities and hereditary polyneuropathies and peripheral nerve biopsy. Different groups of diagnostic criteria for CIDP have been developed, among which EFNS/ PNS (European Federation of Neurological Societies/ Peripheral Nerve Society) criteria are most commonly used. CIDP is treated successfully in most cases and the major treatment options are corticosteroids, intravenous immunoglobulins (IVIG) and plasma exchange