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.

Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database / G. Liberatore, A. De Lorenzo, C. Giannotta, F. Manganelli, M. Filosto, G. Cosentino, D. Cocito, C. Briani, A. Cortese, R. Fazio, G. Lauria, A.M. Clerici, T. Rosso, G.A. Marfia, G. Antonini, G. Cavaletti, M. Carpo, P.E. Doneddu, E. Spina, S. Cotti Piccinelli, E. Peci, L. Querol, E. Nobile-Orazio. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-3478. - 43:6(2022 Jun), pp. 3939-3947. [10.1007/s10072-021-05811-0]

Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database

A. De Lorenzo
Secondo
;
G. Lauria;E. Nobile-Orazio
Writing – Review & Editing
2022

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.
Anti-nerve antibodies; CIDP; Chronic inflammatory demyelinating polyradiculoneuropathy; Peripheral neuropathy; anti-ganglioside antibodies; paranodopathy; Ataxia; Autoantibodies; Cell Adhesion Molecules; Contactin 1; Humans; Nerve Growth Factors; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
Settore MED/26 - Neurologia
giu-2022
20-gen-2022
Article (author)
File in questo prodotto:
File Dimensione Formato  
LAVORO+anticorpi+anti-nervo+edo+04_08_21.pdf

Open Access dal 01/07/2023

Tipologia: Post-print, accepted manuscript ecc. (versione accettata dall'editore)
Dimensione 268.19 kB
Formato Adobe PDF
268.19 kB Adobe PDF Visualizza/Apri
s10072-021-05811-0.pdf

accesso riservato

Tipologia: Publisher's version/PDF
Dimensione 896.71 kB
Formato Adobe PDF
896.71 kB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/929102
Citazioni
  • ???jsp.display-item.citation.pmc??? 5
  • Scopus 10
  • ???jsp.display-item.citation.isi??? 8
social impact