Liberatore, Giuseppe;
De Lorenzo, Alberto;
Giannotta, Claudia;
Manganelli, Fiore;
Filosto, Massimiliano;
Cosentino, Giuseppe;
Cocito, Dario;
... Nobile-Orazio, Eduardo; + view all
(2022)
Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database.
Neurological Sciences
, 43
(6)
10.1007/s10072-021-05811-0.
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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.
| Type: | Article |
|---|---|
| Title: | Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database |
| Location: | Italy |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1007/s10072-021-05811-0 |
| Publisher version: | https://doi.org/10.1007/s10072-021-05811-0 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences, Neurosciences & Neurology, Chronic inflammatory demyelinating polyradiculoneuropathy, CIDP, Peripheral neuropathy, Anti-nerve antibodies, paranodopathy, anti-ganglioside antibodies, INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY, NEUROFASCIN, IGG4, AUTOANTIBODIES |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10218528 |
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