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RESEARCH PRODUCT
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database
Doneddu Pietro EmilianoCocito DarioManganelli FioreFazio RaffaellaBriani ChiaraMassimiliano FilostoBenedetti LuanaMazzeo AnnaMarfia Girolama AlessandraCortese AndreaFierro BrigidaJann StefanoEttore BeghiClerici Angelo MaurizioCarpo MarinellaSchenone AngeloMarco LuigettiGiuseppe LauriaGiovanni AntoniniRosso TizianaSiciliano GabrieleGuido CavalettiLiberatore GiuseppeSantoro LucioPeci ErditaTronci StefanoRuiz MartaCotti Piccinelli StefanoToscano AntonioMataluni GiorgiaPiccolo LauraCosentino GiuseppeSabatelli MarioNobile-orazio EduardoBalducci Claudiasubject
AdultMaleTreatment responselewis-sumner syndromeAdolescentDatabases FactualDisease durationchronic inflammatory demyelinating polyradiculoneuropathy; CIDP; diagnostic criteria; distal acquired demyelinating symmetric neuropathy; lewis-sumner syndrome; Surgery; Neurology (clinical); Psychiatry and Mental HealthKaplan-Meier EstimateCIDPcomputer.software_genreDisease courseYoung Adultlewis–sumner syndrome03 medical and health sciences0302 clinical medicineHumansMedicineIn patientChildAgedRetrospective StudiesAged 80 and overchronic inflammatory demyelinating polyradiculoneuropathyRetrospective reviewdistal acquired demyelinating symmetric neuropathyDatabasebusiness.industryPolyradiculoneuropathyMiddle Agedchronic inflammatory demyelinating polyradiculoneuropathy; CIDP; diagnostic criteria; distal acquired demyelinating symmetric neuropathy; lewis-sumner syndromemedicine.diseasePsychiatry and Mental healthchronic inflammatory demyelinating polyradiculoneuropathy; CIDP; diagnostic criteria; distal acquired demyelinating symmetric neuropathy; lewis-sumner syndrome; surgery; neurology ; psychiatry and mental healthItalyPolyradiculoneuropathy Chronic Inflammatory Demyelinatingdiagnostic criteriaDisease ProgressionFemaleSettore MED/26 - NeurologiaSurgeryProgression rateNeurology (clinical)CIDP; chronic inflammatory demyelinating polyradiculoneuropathy; diagnostic criteria; distal acquired demyelinating symmetric neuropathy; lewis–sumner syndromebusinesscomputer030217 neurology & neurosurgerydescription
ObjectivesA few variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been described, but their frequency and evolution to typical CIDP remain unclear. To determine the frequency and characteristics of the CIDP variants, their possible evolution to typical CIDP, and treatment response.MethodsWe applied a set of diagnostic criteria to 460 patients included in a database of Italian patients with CIDP. Clinical characteristics and treatment response were reviewed for each patient. The Kaplan-Meier curve was used to estimate the progression rate from atypical to typical CIDP.ResultsAt the time of inclusion, 376 (82%) patients had a diagnosis of typical CIDP while 84 (18%) had atypical CIDP, including 34 (7%) with distal acquired demyelinating symmetric neuropathy (DADS), 17 (4%) with purely motor, 17 (4%) with Lewis-Sumner syndrome (LSS) and 16 (3.5%) with purely sensory CIDP. Based on retrospective review of the symptoms and signs present at onset and for at least 1 year, 180 (39%) patients had an initial diagnosis compatible with atypical CIDP that in 96 (53%) patients evolved to typical CIDP. Mean disease duration was longer in patients evolving to typical CIDP than in those not evolving (p=0.0016). Patients with DADS and LSS had a less frequent response to immunoglobulin than those with typical CIDP, while patients with purely motor and sensory CIDP had a similar treatment response.ConclusionsThe proportion of patients with atypical CIDP varies during the disease course. DADS and LSS have a less frequent response to intravenous immunoglobulin compared with typical CIDP, raising the possibility of a different underlying pathogenetic mechanism.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2019-01-01 | Journal of Neurology, Neurosurgery & Psychiatry |