Giuseppe Liberatore1, Alberto De Lorenzo2, Claudia Giannotta2, Fiore Manganelli3, Massimiliano Filosto4, Giuseppe Cosentino5,6, Dario Cocito7, Chiara Briani8, Andrea Cortese6,9, Raffaella Fazio10, Giuseppe Lauria11,12, Angelo Maurizio Clerici13, Tiziana Rosso14, Girolama Alessandra Marfia15, Giovanni Antonini16, Guido Cavaletti17, Marinella Carpo18, Pietro Emiliano Doneddu2, Emanuele Spina3, Stefano Cotti Piccinelli4, Erdita Peci7, Luis Querol19, Eduardo Nobile-Orazio2,20. 1. Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Manzoni 56, 20089, Rozzano, Italy. giuseppe.liberatore@humanitas.it. 2. Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Manzoni 56, 20089, Rozzano, Italy. 3. Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples "Federico II", Naples, Italy. 4. Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology, ASST "Spedali Civili", University of Brescia, Brescia, Italy. 5. Department of Experimental BioMedicine and Clinical Neurosciences (BioNeC), University of Palermo, Palermo, Italy. 6. IRCCS Foundation C. Mondino National Neurological Institute, Pavia, Italy. 7. Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Turin, Italy. 8. Neurology Unit, Department of Neuroscience, University of Padua, Padua, Italy. 9. Molecular Neurosciences, University College London, London, UK. 10. Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy. 11. Unit of Neuroalgology, IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy. 12. Department of Biomedical and Clinical Sciences "Luigi Sacco", University of Milan, Milan, Italy. 13. Neurology Unit, Circolo & Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy. 14. ULSS2 Marca Trevigiana, UOC Neurologia-Castelfranco Veneto, Treviso, Italy. 15. Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy. 16. Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, "Sapienza" University of Rome, Sant'Andrea Hospital, Rome, Italy. 17. School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy. 18. ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy. 19. Neuromuscular Diseases Unit, Autoimmune Neurology, Neuromuscular Laboratory, Neurology Department, Hospital de la Santa Creu i Sant Pau, Institut de Recerca Biomèdica Sant Pau, Barcelona, Spain. 20. Department of Medical Biotechnology and Translational Medicine, Milan University, Milan, Italy.
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.
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.
Authors: Luis Querol; Gisela Nogales-Gadea; Ricardo Rojas-Garcia; Jordi Diaz-Manera; Julio Pardo; Angel Ortega-Moreno; Maria Jose Sedano; Eduard Gallardo; Jose Berciano; Rafael Blesa; Josep Dalmau; Isabel Illa Journal: Neurology Date: 2014-02-12 Impact factor: 9.910
Authors: Judy King Man Ng; Joachim Malotka; Naoto Kawakami; Tobias Derfuss; Mohsen Khademi; Tomas Olsson; Christopher Linington; Masaaki Odaka; Björn Tackenberg; Harald Prüss; Jan M Schwab; Lutz Harms; Hendrik Harms; Claudia Sommer; Matthew N Rasband; Yael Eshed-Eisenbach; Elior Peles; Reinhard Hohlfeld; Nobuhiro Yuki; Klaus Dornmair; Edgar Meinl Journal: Neurology Date: 2012-10-24 Impact factor: 9.910
Authors: Emily K Mathey; Susanna B Park; Richard A C Hughes; John D Pollard; Patricia J Armati; Michael H Barnett; Bruce V Taylor; P James B Dyck; Matthew C Kiernan; Cindy S-Y Lin Journal: J Neurol Neurosurg Psychiatry Date: 2015-02-12 Impact factor: 10.154