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- Title
Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database.
- Authors
Liberatore, Giuseppe; De Lorenzo, Alberto; Giannotta, Claudia; Manganelli, Fiore; Filosto, Massimiliano; Cosentino, Giuseppe; Cocito, Dario; Briani, Chiara; Cortese, Andrea; Fazio, Raffaella; Lauria, Giuseppe; Clerici, Angelo Maurizio; Rosso, Tiziana; Marfia, Girolama Alessandra; Antonini, Giovanni; Cavaletti, Guido; Carpo, Marinella; Doneddu, Pietro Emiliano; Spina, Emanuele; Cotti Piccinelli, Stefano
- Abstract
Objective: To investigate the frequency and clinical correlates of anti-nerve autoantibodies in an unselected series of Italian patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) Methods: Sera from 276 CIDP patients fulfilling the EFNS/PNS criteria and included in the Italian CIDP database were examined for the presence of anti-nerve autoantibodies. Results were correlated with the clinical data collected in the database. Results: Anti-neurofascin155 (NF155) antibodies were found in 9/258 (3.5%) patients, anti-contactin1 (CNTN1) antibodies in 4/258 (1.6%) patients, and anti-contactin-associated protein1 (Caspr1) in 1/197 (0.5%) patients, while none had reactivity to gliomedin or neurofascin 186. Predominance of IgG4 isotype was present in 7of the 9 examined patients. Anti-NF155 patients more frequently had ataxia, tremor, and higher CSF protein levels than antibody-negative patients. Anti-CNTN1 patients more frequently had a GBS-like onset, pain, and ataxia and had more severe motor impairment at enrollment than antibody-negative patients. They more frequently received plasmapheresis, possibly reflecting a less satisfactory response to IVIg or steroids. IgM antibodies against one or more gangliosides were found in 6.5% of the patients (17/260) and were more frequently directed against GM1 (3.9%). They were frequently associated with a progressive course, with a multifocal sensorimotor phenotype and less frequent cranial nerve involvement and ataxia. Conclusions: Anti-paranodal and anti-ganglioside antibodies are infrequent in patients with CIDP but are associated with some typical clinical association supporting the hypothesis that CIDP might be a pathogenically heterogeneous syndrome possibly explaining the different clinical presentations.
- Subjects
CHRONIC inflammatory demyelinating polyradiculoneuropathy; IMMUNOGLOBULINS; JOHN Cunningham virus
- Publication
Neurological Sciences, 2022, Vol 43, Issue 6, p3939
- ISSN
1590-1874
- Publication type
Article
- DOI
10.1007/s10072-021-05811-0