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1. Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants

Author

De&nbsp, Lorenzo, A, Liberatore, G, Doneddu, P, Manganelli, F, Cocito, D, Briani, C, Fazio, R, Mazzeo, A, Schenone, A, Di&nbsp, Stefano, V, Cosentino, G, Marfia, G, Benedetti, L, Carpo, M, Filosto, M, Antonini, G, Clerici, A, Luigetti, M, Matà, S, Rosso, T, Lucchetta, M, Siciliano, G, Lauria&nbsp, Pinter, G, Cavaletti, G, Inghilleri, M, Cantisani, T, Notturno, F, Ricciardi, D, Habetswallner, F, Spina, E, Peci, E, Salvalaggio, A, Falzone, Y, Strano, C, Gentile, L, Vegezzi, E, Mataluni, G, Cotti&nbsp, Piccinelli, S, Leonardi, L, Romano, A, Nobile-Orazio, E, Lorenzo A., Liberatore G., Doneddu P. E., Manganelli F., Cocito D., Briani C., Fazio R., Mazzeo A., Schenone A., Stefano V., Cosentino G., Marfia G. A., Benedetti L., Carpo M., Filosto M., Antonini G., Clerici A. M., Luigetti M., Matà S., Rosso T., Lucchetta M., Siciliano G., Pinter G., Cavaletti G., Inghilleri M., Cantisani T., Notturno F., Ricciardi D., Habetswallner F., Spina E., Peci E., Salvalaggio A., Falzone Y., Strano C., Gentile L., Vegezzi E., Mataluni G., Piccinelli S., Leonardi L., Romano A., Nobile-Orazio E., De&nbsp, Lorenzo, A, Liberatore, G, Doneddu, P, Manganelli, F, Cocito, D, Briani, C, Fazio, R, Mazzeo, A, Schenone, A, Di&nbsp, Stefano, V, Cosentino, G, Marfia, G, Benedetti, L, Carpo, M, Filosto, M, Antonini, G, Clerici, A, Luigetti, M, Matà, S, Rosso, T, Lucchetta, M, Siciliano, G, Lauria&nbsp, Pinter, G, Cavaletti, G, Inghilleri, M, Cantisani, T, Notturno, F, Ricciardi, D, Habetswallner, F, Spina, E, Peci, E, Salvalaggio, A, Falzone, Y, Strano, C, Gentile, L, Vegezzi, E, Mataluni, G, Cotti&nbsp, Piccinelli, S, Leonardi, L, Romano, A, Nobile-Orazio, E, Lorenzo A., Liberatore G., Doneddu P. E., Manganelli F., Cocito D., Briani C., Fazio R., Mazzeo A., Schenone A., Stefano V., Cosentino G., Marfia G. A., Benedetti L., Carpo M., Filosto M., Antonini G., Clerici A. M., Luigetti M., Matà S., Rosso T., Lucchetta M., Siciliano G., Pinter G., Cavaletti G., Inghilleri M., Cantisani T., Notturno F., Ricciardi D., Habetswallner F., Spina E., Peci E., Salvalaggio A., Falzone Y., Strano C., Gentile L., Vegezzi E., Mataluni G., Piccinelli S., Leonardi L., Romano A., and Nobile-Orazio E.

Abstract

Background and purpose: There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. Methods: We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. Results: According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. Conclusions: The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.

Published
2024

5. Unclassified clinical presentations of chronic inflammatory demyelinating polyradiculoneuropathy

Author

Doneddu, P, Akyil, H, Manganelli, F, Briani, C, Cocito, D, Benedetti, L, Mazzeo, A, Fazio, R, Filosto, M, Cosentino, G, Di Stefano, V, Antonini, G, Marfia, G, Inghilleri, M, Siciliano, G, Clerici, A, Carpo, M, Schenone, A, Luigetti, M, Lauria, G, Mata, S, Rosso, T, Minicuci, G, Lucchetta, M, Cavaletti, G, Liberatore, G, Spina, E, Campagnolo, M, Peci, E, Germano, F, Gentile, L, Strano, C, Cotti Piccinelli, S, Vegezzi, E, Leonardi, L, Mataluni, G, Ceccanti, M, Schirinzi, E, Romozzi, M, Nobile-Orazio, E, Doneddu P. E., Akyil H., Manganelli F., Briani C., Cocito D., Benedetti L., Mazzeo A., Fazio R., Filosto M., Cosentino G., Di Stefano V., Antonini G., Marfia G. A., Inghilleri M., Siciliano G., Clerici A. M., Carpo M., Schenone A., Luigetti M., Lauria G., Mata S., Rosso T., Minicuci G. M., Lucchetta M., Cavaletti G., Liberatore G., Spina E., Campagnolo M., Peci E., Germano F., Gentile L., Strano C., Cotti Piccinelli S., Vegezzi E., Leonardi L., Mataluni G., Ceccanti M., Schirinzi E., Romozzi M., Nobile-Orazio E., Doneddu, P, Akyil, H, Manganelli, F, Briani, C, Cocito, D, Benedetti, L, Mazzeo, A, Fazio, R, Filosto, M, Cosentino, G, Di Stefano, V, Antonini, G, Marfia, G, Inghilleri, M, Siciliano, G, Clerici, A, Carpo, M, Schenone, A, Luigetti, M, Lauria, G, Mata, S, Rosso, T, Minicuci, G, Lucchetta, M, Cavaletti, G, Liberatore, G, Spina, E, Campagnolo, M, Peci, E, Germano, F, Gentile, L, Strano, C, Cotti Piccinelli, S, Vegezzi, E, Leonardi, L, Mataluni, G, Ceccanti, M, Schirinzi, E, Romozzi, M, Nobile-Orazio, E, Doneddu P. E., Akyil H., Manganelli F., Briani C., Cocito D., Benedetti L., Mazzeo A., Fazio R., Filosto M., Cosentino G., Di Stefano V., Antonini G., Marfia G. A., Inghilleri M., Siciliano G., Clerici A. M., Carpo M., Schenone A., Luigetti M., Lauria G., Mata S., Rosso T., Minicuci G. M., Lucchetta M., Cavaletti G., Liberatore G., Spina E., Campagnolo M., Peci E., Germano F., Gentile L., Strano C., Cotti Piccinelli S., Vegezzi E., Leonardi L., Mataluni G., Ceccanti M., Schirinzi E., Romozzi M., and Nobile-Orazio E.

Abstract

Background To assess the ability of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include within their classification the whole spectrum of clinical heterogeneity of the disease and to define the clinical characteristics of the unclassifiable clinical forms. Methods The 2021 EAN/PNS clinical criteria for CIDP were applied to 329 patients fulfilling the electrodiagnostic (and in some cases also the supportive) criteria for the diagnosis of CIDP. Clinical characteristics were reviewed for each patient not strictly fulfilling the clinical criteria ('unclassifiable'). Results At study inclusion, 124 (37.5%) patients had an unclassifiable clinical presentation, including 110 (89%) with a typical CIDP-like clinical phenotype in whom some segments of the four limbs were unaffected by weakness ('incomplete typical CIDP'), 10 (8%) with a mild distal, symmetric, sensory or sensorimotor polyneuropathy confined to the lower limbs with cranial nerve involvement ('cranial nerve predominant CIDP') and 4 (1%) with a symmetric sensorimotor polyneuropathy limited to the proximal and distal areas of the lower limbs ('paraparetic CIDP'). Eighty-one (65%) patients maintained an unclassifiable presentation during the entire disease follow-up while 13 patients progressed to typical CIDP. Patients with the unclassifiable clinical forms compared with patients with typical CIDP had a milder form of CIDP, while there was no difference in the distribution patterns of demyelination. Conclusions A proportion of patients with CIDP do not strictly fulfil the 2021 EAN/PNS clinical criteria for diagnosis. These unclassifiable clinical phenotypes may pose diagnostic challenges and thus deserve more attention in clinical practice and research.

Published
2023

6. Risk of disease relapse, safety and tolerability of SARS-CoV-2 vaccination in patients with chronic inflammatory neuropathies

Author

Doneddu, P. E., Briani, C., Cocito, D., Manganelli, F., Fabrizi, G. M., Matà, S., Mazzeo, A., Fazio, R., Benedetti, L., Luigetti, M., Inghilleri, M., Ruiu, E., Siciliano, G., Cosentino, G., Marfia, G. A., Carpo, M., Filosto, M., Antonini, G., Notturno, F., Sotgiu, S., Cucurachi, L., Dell'Aquila, C., Bianchi, E., Rosso, T., Giordano, A., Fernandes, M., Campagnolo, M., Peci, E., Spina, E., Tagliapietra, M., Sperti, M., Gentile, L., Strano, C., Germano, F., Romozzi, M., Moret, F., Zarbo, I. R., Viola, D. V., Vegezzi, E., Mataluni, G., Cotti-Piccinelli, S., Leonardi, L., Carta, A., and Nobile-Orazio, E.

Subjects
COVID-19, SARS-CoV-2, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, vaccination, Settore MED/26
Published
2023

7. Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP

Author

Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., Manganelli F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., and Manganelli F.

Abstract

Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.

Published
2022

8. The neurophysiological lesson from the Italian CIDP database

Author

Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., Manganelli F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, Manganelli, F, Spina E., Doneddu P. E., Liberatore G., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Peci E., Tronci S., Ruiz M., Piccinelli S. C., Schenone A., Leonardi L., Gentile L., Piccolo L., Mataluni G., Santoro L., Nobile-Orazio E., and Manganelli F.

Abstract

Introduction: Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database. Methods: We calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP. Results: The ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively. Conclusion: Our findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP.

Published
2022

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

Author

Liberatore, G, De Lorenzo, A, Giannotta, C, Manganelli, F, Filosto, M, Cosentino, G, Cocito, D, Briani, C, Cortese, A, Fazio, R, Lauria, G, Clerici, A, Rosso, T, Marfia, G, Antonini, G, Cavaletti, G, Carpo, M, Doneddu, P, Spina, E, Cotti Piccinelli, S, Peci, E, Querol, L, Nobile-Orazio, E, Liberatore G., De Lorenzo A., Giannotta C., Manganelli F., Filosto M., Cosentino G., Cocito D., Briani C., Cortese A., Fazio R., Lauria G., Clerici A. M., Rosso T., Marfia G. A., Antonini G., Cavaletti G., Carpo M., Doneddu P. E., Spina E., Cotti Piccinelli S., Peci E., Querol L., Nobile-Orazio E., Liberatore, G, De Lorenzo, A, Giannotta, C, Manganelli, F, Filosto, M, Cosentino, G, Cocito, D, Briani, C, Cortese, A, Fazio, R, Lauria, G, Clerici, A, Rosso, T, Marfia, G, Antonini, G, Cavaletti, G, Carpo, M, Doneddu, P, Spina, E, Cotti Piccinelli, S, Peci, E, Querol, L, Nobile-Orazio, E, Liberatore G., De Lorenzo A., Giannotta C., Manganelli F., Filosto M., Cosentino G., Cocito D., Briani C., Cortese A., Fazio R., Lauria G., Clerici A. M., Rosso T., Marfia G. A., Antonini G., Cavaletti G., Carpo M., Doneddu P. E., Spina E., Cotti Piccinelli S., Peci E., Querol L., and Nobile-Orazio E.

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.

Published
2022

10. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore, G., Manganelli, F., Doneddu, P. E., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Mazzeo, A., Antonini, G., Cosentino, G., Jann, S., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Santoro, L., Peci, E., Tronci, S., Ruiz, M., Cotti Piccinelli, S., Schenone, A., Leonardi, L., Toscano, A., Mataluni, G., Spina, E., Gentile, L., Nobile-Orazio, E., Gallia, F., Beronio, A., Piccolo, L., Fierro, B., Verrengia, E. P., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Sabatelli, M., Dacci, P., Schirinzi, E., Balducci, C., Liberatore, G., Manganelli, F., Doneddu, P. E., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Mazzeo, A., Antonini, G., Cosentino, G., Jann, S., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Santoro, L., Peci, E., Tronci, S., Ruiz, M., Cotti-Piccinelli, S., Schenone, A., Leonardi, L., Toscano, A., Mataluni, G., Spina, E., Gentile, L., Nobile-Orazio, E., Gallia, F., Beronio, A., Piccolo, L., Fierro, B., Verrengia, E. P., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Sabatelli, M., Dacci, P., Toni, G., Schirinzi, E., Balducci, C., Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, and Balducci, C

Subjects
Pediatrics, medicine.medical_specialty, Response to therapy, Databases, Factual, Neural Conduction, Settore MED/26, 03 medical and health sciences, 0302 clinical medicine, Peripheral nerve, Retrospective Studie, Medicine, Humans, Medical history, In patient, 030212 general & internal medicine, Peripheral Nerves, Retrospective Studies, chronic inflammatory demyelinating polyradiculoneuropathy, business.industry, Polyradiculoneuropathy, Polyradiculopathy, medicine.disease, electrophysiology, Settore MED/26 - NEUROLOGIA, Neurology, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Clinical diagnosis, Peripheral Nerve, diagnostic criteria, National database, Neurology (clinical), business, 030217 neurology & neurosurgery, Human
Abstract

Background and purpose The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). Conclusions Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Published
2021

11. Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

Author

Liberatore, G., Manganelli, F., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Sabatelli, M., Lauria, G., Rosso, T., Nobile Orazio, E., Doneddu, P. E., Gallia, F., Nobile-Orazio, E., Peci, E., Tronci, S., Santoro, L., Spina, E., Ruiz, M., Piccinelli, S. C., Beronio, A., Toscano, A., Gentile, L., Mataluni, G., Piccolo, L., Callegari, I., Fierro, B., Pinuccia, V. E., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Schenone, A., Luigetti, M., Dacci, P., Leonardi, L., Schirinzi, E., Balducci, C., Cavaletti, G., Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore, G., Manganelli, F., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Sabatelli, M., Lauria, G., Rosso, T., Nobile Orazio, E., Doneddu, P. E., Gallia, F., Nobile-Orazio, E., Peci, E., Tronci, S., Santoro, L., Spina, E., Ruiz, M., Piccinelli, S. C., Beronio, A., Toscano, A., Gentile, L., Mataluni, G., Piccolo, L., Callegari, I., Fierro, B., Pinuccia, V. E., Bianchi, E., Beghi, E., Scrascia, F., Garnero, M., Schenone, A., Luigetti, M., Dacci, P., Leonardi, L., Schirinzi, E., Balducci, C., and Cavaletti, G.

Subjects
medicine.medical_specialty, peripheral neuropathy, Response to therapy, Motor nerve, CIDP, Settore MED/26, chronic inflammatory demyelinating neuropathy, diagnostic criteria, EMG, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, In patient, Nerve biopsy, medicine.diagnostic_test, business.industry, General Neuroscience, Polyradiculoneuropathy, medicine.disease, Chronic inflammatory demyelinating neuropathy, Diagnostic criteria, Peripheral neuropathy, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Clinical diagnosis, Neurology (clinical), business, 030217 neurology & neurosurgery, Sensory nerve
Abstract

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

Published
2020

12. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, Balducci, C, Liberatore G., Manganelli F., Doneddu P. E., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Mazzeo A., Antonini G., Cosentino G., Jann S., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Santoro L., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Schenone A., Leonardi L., Toscano A., Mataluni G., Spina E., Gentile L., Nobile-Orazio E., Gallia F., Beronio A., Piccolo L., Fierro B., Verrengia E. P., Bianchi E., Beghi E., Scrascia F., Garnero M., Sabatelli M., Dacci P., Schirinzi E., Balducci C., Liberatore, G, Manganelli, F, Doneddu, P, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Gallia, F, Beronio, A, Piccolo, L, Fierro, B, Verrengia, E, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Sabatelli, M, Dacci, P, Schirinzi, E, Balducci, C, Liberatore G., Manganelli F., Doneddu P. E., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Mazzeo A., Antonini G., Cosentino G., Jann S., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Luigetti M., Lauria G., Rosso T., Cavaletti G., Santoro L., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Schenone A., Leonardi L., Toscano A., Mataluni G., Spina E., Gentile L., Nobile-Orazio E., Gallia F., Beronio A., Piccolo L., Fierro B., Verrengia E. P., Bianchi E., Beghi E., Scrascia F., Garnero M., Sabatelli M., Dacci P., Schirinzi E., and Balducci C.

Abstract

Background and purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Published
2021

13. Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

Author

Doneddu, P, Cocito, D, Manganelli, F, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Bianchi, E, Jann, S, Mazzeo, A, Antonini, G, Cosentino, G, Marfia, G, Cortese, A, Clerici, A, Carpo, M, Schenone, A, Siciliano, G, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Beghi, E, Liberatore, G, Santoro, L, Spina, E, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Verrengia, E, Gentile, L, Leonardi, L, Mataluni, G, Piccolo, L, Nobile-Orazio, E, Doneddu P. E., Cocito D., Manganelli F., Fazio R., Briani C., Filosto M., Benedetti L., Bianchi E., Jann S., Mazzeo A., Antonini G., Cosentino G., Marfia G. A., Cortese A., Clerici A. M., Carpo M., Schenone A., Siciliano G., Luigetti M., Lauria G., Rosso T., Cavaletti G., Beghi E., Liberatore G., Santoro L., Spina E., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Verrengia E. P., Gentile L., Leonardi L., Mataluni G., Piccolo L., Nobile-Orazio E., Doneddu, P, Cocito, D, Manganelli, F, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Bianchi, E, Jann, S, Mazzeo, A, Antonini, G, Cosentino, G, Marfia, G, Cortese, A, Clerici, A, Carpo, M, Schenone, A, Siciliano, G, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Beghi, E, Liberatore, G, Santoro, L, Spina, E, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Verrengia, E, Gentile, L, Leonardi, L, Mataluni, G, Piccolo, L, Nobile-Orazio, E, Doneddu P. E., Cocito D., Manganelli F., Fazio R., Briani C., Filosto M., Benedetti L., Bianchi E., Jann S., Mazzeo A., Antonini G., Cosentino G., Marfia G. A., Cortese A., Clerici A. M., Carpo M., Schenone A., Siciliano G., Luigetti M., Lauria G., Rosso T., Cavaletti G., Beghi E., Liberatore G., Santoro L., Spina E., Peci E., Tronci S., Ruiz M., Cotti Piccinelli S., Verrengia E. P., Gentile L., Leonardi L., Mataluni G., Piccolo L., and Nobile-Orazio E.

Abstract

Objectives To determine the prevalence of different comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and their impact on outcome, treatment choice and response. Methods Using a structured questionnaire, we collected information on comorbidities from 393 patients with CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria included in the Italian CIDP database. Results One or more comorbidities were reported by 294 patients (75%) and potentially influenced treatment choice in 192 (49%) leading to a less frequent use of corticosteroids. Response to treatment did not differ, however, from that in patients without comorbidities. Diabetes (14%), monoclonal gammopathy of undetermined significance (MGUS) (12%) and other immune disorders (16%) were significantly more frequent in patients with CIDP than expected in the general European population. Patients with diabetes had higher disability scores, worse quality of life and a less frequent treatment response compared with patients without diabetes. Patients with IgG-IgA or IgM MGUS had an older age at CIDP onset while patients with other immune disorders had a younger age at onset and were more frequently females. IgM MGUS was more frequent in patients with motor CIDP than in patients with typical CIDP. Conclusions Comorbidities are frequent in patients with CIDP and in almost 50% of them have an impact on treatment choice. Diabetes, MGUS and other immune diseases are more frequent in patients with CIDP than in the general population. Only diabetes seems, however, to have an impact on disease severity and treatment response possibly reflecting in some patients a coexisting diabetic neuropathy.

Published
2020

14. Impact of environmental factors and physical activity on disability and quality of life in CIDP

Author

Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Beghi, E, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Schenone, A, Liberatore, G, Peci, E, Spina, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Gentile, L, Piccolo, L, Leonardi, L, Mataluni, G, Ruiz, M, Sabatelli, M, Santoro, L, Nobile-Orazio, E, Gallia, F, Velardo, D, Topa, A, Cotti-Piccinelli, S, Todeschini, A, Cabona, C, Zuppa, A, Callegari, I, Verrengia, E, Scrascia, F, Garnero, M, Luigetti, M, Dacci, P, Schirinzi, E, Balducci, C, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Beghi E., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Schenone A., Liberatore G., Peci E., Spina E., Tronci S., Cotti Piccinelli S., Toscano A., Gentile L., Piccolo L., Leonardi L., Mataluni G., Ruiz M., Sabatelli M., Santoro L., Nobile-Orazio E., Gallia F., Velardo D., Topa A., Cotti-Piccinelli S., Todeschini A., Cabona C., Zuppa A., Callegari I., Verrengia E. P., Scrascia F., Garnero M., Luigetti M., Dacci P., Schirinzi E., Balducci C., Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Beghi, E, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Schenone, A, Liberatore, G, Peci, E, Spina, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Gentile, L, Piccolo, L, Leonardi, L, Mataluni, G, Ruiz, M, Sabatelli, M, Santoro, L, Nobile-Orazio, E, Gallia, F, Velardo, D, Topa, A, Cotti-Piccinelli, S, Todeschini, A, Cabona, C, Zuppa, A, Callegari, I, Verrengia, E, Scrascia, F, Garnero, M, Luigetti, M, Dacci, P, Schirinzi, E, Balducci, C, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Beghi E., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Schenone A., Liberatore G., Peci E., Spina E., Tronci S., Cotti Piccinelli S., Toscano A., Gentile L., Piccolo L., Leonardi L., Mataluni G., Ruiz M., Sabatelli M., Santoro L., Nobile-Orazio E., Gallia F., Velardo D., Topa A., Cotti-Piccinelli S., Todeschini A., Cabona C., Zuppa A., Callegari I., Verrengia E. P., Scrascia F., Garnero M., Luigetti M., Dacci P., Schirinzi E., and Balducci C.

Abstract

A few observational studies and randomized trials suggest that exercise and rehabilitation may improve activity limitation and quality of life (QoL) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but the impact of other modifiable factors on the severity of the disease is not well understood. Using a structured questionnaire, we collected data on lifestyle and dietary habits of the patients included in the Italian CIDP database to investigate the possible influence of modifiable lifestyle factors on disability and QoL. Questionnaire data were available for 323 patients. The effect of lifestyle and dietary exposures on impairment, disability and QoL was evaluated using logistic regression models, adjusting for age, sex, disease duration, physical activity and smoking. Physical activity was associated with lower sensory impairment by the ISS scale, less disability by the INCAT and RODS scale and a better QoL in all the domains of EURO-QoL scale with the exception of anxiety/depression. None of the other parameters had an impact on these scales. This study adds evidence to the possible role of physical activity in improving symptom severity, disability and QoL in patients with CIDP. None of the other environmental factors investigated appeared to have an impact on the severity and health perception of CIDP.

Published
2020

15. Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

Author

Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore G., Manganelli F., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Sabatelli M., Lauria G., Rosso T., Nobile Orazio E., Doneddu P. E., Gallia F., Nobile-Orazio E., Peci E., Tronci S., Santoro L., Spina E., Ruiz M., Piccinelli S. C., Beronio A., Toscano A., Gentile L., Mataluni G., Piccolo L., Callegari I., Fierro B., Pinuccia V. E., Bianchi E., Beghi E., Scrascia F., Garnero M., Schenone A., Luigetti M., Dacci P., Leonardi L., Schirinzi E., Balducci C., Cavaletti G., Liberatore, G, Manganelli, F, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Sabatelli, M, Lauria, G, Rosso, T, Nobile Orazio, E, Doneddu, P, Gallia, F, Nobile-Orazio, E, Peci, E, Tronci, S, Santoro, L, Spina, E, Ruiz, M, Piccinelli, S, Beronio, A, Toscano, A, Gentile, L, Mataluni, G, Piccolo, L, Callegari, I, Fierro, B, Pinuccia, V, Bianchi, E, Beghi, E, Scrascia, F, Garnero, M, Schenone, A, Luigetti, M, Dacci, P, Leonardi, L, Schirinzi, E, Balducci, C, Cavaletti, G, Liberatore G., Manganelli F., Cocito D., Fazio R., Briani C., Filosto M., Benedetti L., Antonini G., Cosentino G., Jann S., Mazzeo A., Cortese A., Marfia G. A., Clerici A. M., Siciliano G., Carpo M., Sabatelli M., Lauria G., Rosso T., Nobile Orazio E., Doneddu P. E., Gallia F., Nobile-Orazio E., Peci E., Tronci S., Santoro L., Spina E., Ruiz M., Piccinelli S. C., Beronio A., Toscano A., Gentile L., Mataluni G., Piccolo L., Callegari I., Fierro B., Pinuccia V. E., Bianchi E., Beghi E., Scrascia F., Garnero M., Schenone A., Luigetti M., Dacci P., Leonardi L., Schirinzi E., Balducci C., and Cavaletti G.

Abstract

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

Published
2020

16. Sensitivity and specificity of a commercial ELISA test for anti-MAG antibodies in patients with neuropathy

Author

Liberatore, G, Giannotta, C, Sajeev, B, Morenghi, E, Terenghi, F, Gallia, F, Doneddu, P, Manganelli, F, Cocito, D, Filosto, M, Antonini, G, Cosentino, G, Marfia, G, Clerici, A, Lauria, G, Rosso, T, Cavaletti, G, Nobile-Orazio, E, Liberatore G., Giannotta C., Sajeev B. P., Morenghi E., Terenghi F., Gallia F., Doneddu P. E., Manganelli F., Cocito D., Filosto M., Antonini G., Cosentino G., Marfia G. A., Clerici A. M., Lauria G., Rosso T., Cavaletti G., Nobile-Orazio E., Liberatore, G, Giannotta, C, Sajeev, B, Morenghi, E, Terenghi, F, Gallia, F, Doneddu, P, Manganelli, F, Cocito, D, Filosto, M, Antonini, G, Cosentino, G, Marfia, G, Clerici, A, Lauria, G, Rosso, T, Cavaletti, G, Nobile-Orazio, E, Liberatore G., Giannotta C., Sajeev B. P., Morenghi E., Terenghi F., Gallia F., Doneddu P. E., Manganelli F., Cocito D., Filosto M., Antonini G., Cosentino G., Marfia G. A., Clerici A. M., Lauria G., Rosso T., Cavaletti G., and Nobile-Orazio E.

Abstract

For the diagnosis of anti-MAG polyneuropathy the commercial ELISA manufacturer currently recommends a cut-off of 1000 Bühlmann Titer Units (BTU). We analyzed sera from 80 anti-MAG neuropathy patients and 383 controls (with other neuropathies or healthy controls) to assess the ELISA sensitivity and specificity at different thresholds. A better combination of sensitivity/specificity was found at a threshold >1500 BTU than at >1000 BTU. The best value of specificity was obtained at threshold >7000 BTU. There was a diagnostic grey area between 1500 and 7000 BTU in which the clinical phenotypes as well as electrophysiological studies need to be carefully assessed particularly to differentiate CIDP and anti-MAG neuropathy.

Published
2020

17. Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database

Author

Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Luigetti, M, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Beghi, E, Liberatore, G, Santoro, L, Peci, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Piccolo, L, Verrengia, E, Leonardi, L, Schirinzi, E, Mataluni, G, Ruiz, M, Dacci, P, Nobile-Orazio, E, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Luigetti M., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Beghi E., Liberatore G., Santoro L., Peci E., Tronci S., Cotti Piccinelli S., Toscano A., Piccolo L., Verrengia E. P., Leonardi L., Schirinzi E., Mataluni G., Ruiz M., Dacci P., Nobile-Orazio E., Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Luigetti, M, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Beghi, E, Liberatore, G, Santoro, L, Peci, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Piccolo, L, Verrengia, E, Leonardi, L, Schirinzi, E, Mataluni, G, Ruiz, M, Dacci, P, Nobile-Orazio, E, Doneddu P. E., Bianchi E., Cocito D., Manganelli F., Fazio R., Filosto M., Mazzeo A., Cosentino G., Cortese A., Jann S., Clerici A. M., Antonini G., Siciliano G., Luigetti M., Marfia G. A., Briani C., Lauria G., Rosso T., Cavaletti G., Carpo M., Benedetti L., Beghi E., Liberatore G., Santoro L., Peci E., Tronci S., Cotti Piccinelli S., Toscano A., Piccolo L., Verrengia E. P., Leonardi L., Schirinzi E., Mataluni G., Ruiz M., Dacci P., and Nobile-Orazio E.

Abstract

Background and purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender-matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls. Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex-matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1–42 days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events. Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease.

Published
2020

18. Clinical Reasoning: A case of COVID-19-associated pharyngeal-cervical-brachial variant of Guillain-Barré syndrome

Author

Liberatore, G., De Santis, T., Doneddu, P. E., Gentile, F., Albanese, Alberto, Nobile-Orazio, E., Albanese A. (ORCID:0000-0002-5864-0006), Liberatore, G., De Santis, T., Doneddu, P. E., Gentile, F., Albanese, Alberto, Nobile-Orazio, E., and Albanese A. (ORCID:0000-0002-5864-0006)

Abstract

In March 2020, a 49-year-old man was admitted through our emergency department due to a 1-week history of fever (39–40°C) and cough. His medical history included arterial hypertension and a testicular seminoma in 2011 treated with surgery and platinum-based chemotherapy. Laboratory tests revealed increased C-reactive protein, mild lymphopenia, and thrombocytopenia. ChestCT showedmultifocal ground-glass opacities and nasopharyngeal swab was positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), leading to a diagnosis of coronavirus disease 2019 (COVID-19). The patient was hospitalized and treatment with hydroxychloroquine, lopinavir/ritonavir, and ceftriaxone was started.

Published
2020

19. Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

Author

Doneddu, P. E., Cocito, D., Manganelli, F., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Bianchi, E., Jann, S., Mazzeo, A., Antonini, Gabriele, Cosentino, G., Marfia, G. A., Cortese, A., Clerici, Anna Marina, Carpo, M., Schenone, A., Siciliano, Giovanni, Luigetti, Marco, Lauria, G., Rosso, T., Cavaletti, G., Beghi, E., Liberatore, G., Santoro, Luca, Spina, E., Peci, E., Tronci, S., Ruiz, M., Cotti Piccinelli, S., Verrengia, E. P., Gentile, L., Leonardi, L., Mataluni, G., Piccolo, L., Nobile-Orazio, E., Antonini G., Clerici A. M., Siciliano G., Luigetti M. (ORCID:0000-0001-7539-505X), Santoro L., Doneddu, P. E., Cocito, D., Manganelli, F., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Bianchi, E., Jann, S., Mazzeo, A., Antonini, Gabriele, Cosentino, G., Marfia, G. A., Cortese, A., Clerici, Anna Marina, Carpo, M., Schenone, A., Siciliano, Giovanni, Luigetti, Marco, Lauria, G., Rosso, T., Cavaletti, G., Beghi, E., Liberatore, G., Santoro, Luca, Spina, E., Peci, E., Tronci, S., Ruiz, M., Cotti Piccinelli, S., Verrengia, E. P., Gentile, L., Leonardi, L., Mataluni, G., Piccolo, L., Nobile-Orazio, E., Antonini G., Clerici A. M., Siciliano G., Luigetti M. (ORCID:0000-0001-7539-505X), and Santoro L.

Abstract

Objectives To determine the prevalence of different comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and their impact on outcome, treatment choice and response. Methods Using a structured questionnaire, we collected information on comorbidities from 393 patients with CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria included in the Italian CIDP database. Results One or more comorbidities were reported by 294 patients (75%) and potentially influenced treatment choice in 192 (49%) leading to a less frequent use of corticosteroids. Response to treatment did not differ, however, from that in patients without comorbidities. Diabetes (14%), monoclonal gammopathy of undetermined significance (MGUS) (12%) and other immune disorders (16%) were significantly more frequent in patients with CIDP than expected in the general European population. Patients with diabetes had higher disability scores, worse quality of life and a less frequent treatment response compared with patients without diabetes. Patients with IgG-IgA or IgM MGUS had an older age at CIDP onset while patients with other immune disorders had a younger age at onset and were more frequently females. IgM MGUS was more frequent in patients with motor CIDP than in patients with typical CIDP. Conclusions Comorbidities are frequent in patients with CIDP and in almost 50% of them have an impact on treatment choice. Diabetes, MGUS and other immune diseases are more frequent in patients with CIDP than in the general population. Only diabetes seems, however, to have an impact on disease severity and treatment response possibly reflecting in some patients a coexisting diabetic neuropathy.

Published
2020

20. Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database

Author

Doneddu, P. E., Bianchi, E., Cocito, D., Manganelli, F., Fazio, R., Filosto, M., Mazzeo, A., Cosentino, G., Cortese, A., Jann, S., Clerici, A. M., Antonini, G., Siciliano, G., Luigetti, M., Marfia, G. A., Briani, C., Lauria, G., Rosso, T., Cavaletti, G., Carpo, M., Benedetti, L., Beghi, E., Liberatore, G., Santoro, L., Peci, E., Tronci, S., Cotti Piccinelli, S., Toscano, A., Piccolo, L., Verrengia, E. P., Leonardi, L., Schirinzi, E., Mataluni, G., Ruiz, M., Dacci, P., Nobile-Orazio, E., Luigetti M. (ORCID:0000-0001-7539-505X), Doneddu, P. E., Bianchi, E., Cocito, D., Manganelli, F., Fazio, R., Filosto, M., Mazzeo, A., Cosentino, G., Cortese, A., Jann, S., Clerici, A. M., Antonini, G., Siciliano, G., Luigetti, M., Marfia, G. A., Briani, C., Lauria, G., Rosso, T., Cavaletti, G., Carpo, M., Benedetti, L., Beghi, E., Liberatore, G., Santoro, L., Peci, E., Tronci, S., Cotti Piccinelli, S., Toscano, A., Piccolo, L., Verrengia, E. P., Leonardi, L., Schirinzi, E., Mataluni, G., Ruiz, M., Dacci, P., Nobile-Orazio, E., and Luigetti M. (ORCID:0000-0001-7539-505X)

Abstract

Background and purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender-matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls. Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex-matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1–42 days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events. Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease.

Published
2020

21. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore, G, Manganelli, F, Doneddu, P E, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G A, Clerici, A M, Siciliano, G, Carpo, M, Luigetti, Marco, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, Luigetti, M (ORCID:0000-0001-7539-505X), Liberatore, G, Manganelli, F, Doneddu, P E, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Mazzeo, A, Antonini, G, Cosentino, G, Jann, S, Cortese, A, Marfia, G A, Clerici, A M, Siciliano, G, Carpo, M, Luigetti, Marco, Lauria, G, Rosso, T, Cavaletti, G, Santoro, L, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Schenone, A, Leonardi, L, Toscano, A, Mataluni, G, Spina, E, Gentile, L, Nobile-Orazio, E, and Luigetti, M (ORCID:0000-0001-7539-505X)

Abstract

Background and purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Published
2020

25. LIFESTYLE AND DIETARY HABITS AS PREDISPOSING FACTORS FOR THE ONSET AND PROGRESSION OF CIDP: A CASE-CONTROL STUDY FROM THE ITALIAN CIDP DATABASE

Author

Doneddu, P. E., Cocito, D., Santoro, L., Fazio, R., Filosto, M., Mazzeo, A., Jann, S., Cortese, A., Beghi, E., Carpo, M., Clerici, M., Luigetti, M., Lauria, G., Fierro, B., Antonini, G., Briani, C., Cavaletti, G., Rosso, T., Benedetti, L., Marfia, G., Liberatore, G., Peci, E., Manganelli, F., Velardo, D., Todeschini, A., Toscano, A., Verrengia, E. P., Piccolo, L., and Eduardo Nobile-Orazio

Subjects
Settore MED/26 - Neurologia
Published
2017

26. FREQUENCY AND DIAGNOSTIC CRITERIA FOR ATYPICAL CIDP: DATA FROM THE ITALIAN DATABASE ON CIDP

Author

Doneddu, P. E., Cocito, D., Fazio, R., Filosto, M., Jann, S., Santoro, L., Cortese, A., Carpo, M., Clerici, M., Luigetti, M., Mazzeo, A., Lauria, G., Fierro, B., Antonini, G., Cavaletti, G., Rosso, T., Benedetti, L., Marfia, G., Briani, C., Peci, E., Liberatore, G., Velardo, D., Todeschini, A., Toscano, A., Verrengia, E. P., Manganelli, F., Piccolo, L., Gallia, F., and Eduardo Nobile-Orazio

Subjects
Settore MED/26 - Neurologia
Published
2017

27. FREQUENCY, PROGRESSION AND THERAPY OF ATYPICAL CIDP: DATA FROM THE ITALIAN DATABASE ON CIDP

Author

Doneddu, P. E., Cocito, D., Santoro, L., Fazio, R., Filosto, M., Mazzeo, A., Jann, S., Cortese, A., Beghi, E., Carpo, M., Clerici, M., Luigetti, M., Lauria, G., Fierro, B., Antonini, G., Briani, C., Cavaletti, G., Rosso, T., Benedetti, L., Marfia, G., Liberatore, G., Peci, E., Manganelli, F., Velardo, D., Todeschini, A., Toscano, A., Verrengia, E. P., Piccolo, L., and Eduardo Nobile-Orazio

Subjects
Settore MED/26 - Neurologia
Published
2017

28. Sleep palsy involving different nerves: Role of ultrasound

Author

Iodice, Francesco, Doneddu, P. E., Costantini, Emanuele Maria, Bizzarro, Alessandra, Coraci, D, and Padua, Luca

Subjects
Settore MED/26 - NEUROLOGIA, Nerve entrapment, Ultrasound, Settore MED/34 - MEDICINA FISICA E RIABILITATIVA
Published
2015

29. Paradoxical Effect of Levetiracetam in Newly Diagnosed Type II Focal Cortical Dysplasia

Author

Fois, Chiara, Rassu, Anna Laura, Mandia, Daniele, Sechi, Elia, Doneddu, Pietro E., and Sechi, GianPietro

Abstract

A paradoxical effectof antiepileptic drugs was defined as an increased seizure frequency or severity occurring shortly after introducing a drug considered effective for that kind of epilepsy. In addition, this effect should occur at nontoxic drug serum levels. So far, pathophysiological mechanisms underlying this phenomenon have not been clarified. Recent evidence suggests that the variability of drug effects may depend on precise intrinsic properties of dynamic networks involving the drug and its binding site. Although several reports of paradoxical seizure exacerbation have been reported for levetiracetam (LEV), a possible association with focal cortical dysplasia has never been described nor investigated. In this report, we document a paradoxical effect induced by LEV monotherapy in a patient with type II focal cortical dysplasia at LEV serum levels within the therapeutic range. A hint of pathophysiological hypothesis underlying this potential relationship will be also suggested.

Published
2016
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30. Response to: SARS‐CoV‐2 associated Guillain‐Barré syndrome in 62 patients.

Author

Doneddu, P. E., De Sanctis, P., Viganò, L., Selmi, C., Gentile, F., and Nobile‐Orazio, E.

Subjects
*GUILLAIN-Barre syndrome, *SARS-CoV-2, *COVID-19, *ZIKA virus infections, *NEUROLOGICAL disorders, *INCUBATION period (Communicable diseases)
Abstract

Response to: SARS-CoV-2 associated Guillain-Barré syndrome in 62 patients Keywords: Covid; cranial nerves; cranial neuropathy; Guillain-Barre syndrome; neurological disorders; peripheral neuropathies EN Covid cranial nerves cranial neuropathy Guillain-Barre syndrome neurological disorders peripheral neuropathies e9 e9 1 12/28/20 20210101 NES 210101 Reply We thank Finsterer and colleagues for their useful comment and for their very useful update on the reported new cases of Guillain-Barré syndrome (GBS) in the context of SARS-CoV-2 infection. However, since the incubation period of COVID-19 can last up to 24 days [2] while GBS mostly occurs 10 and 14 days [3] after a preceding infection, it is possible that GBS in some patients may develop before the clinical appearance of COVID-19. [Extracted from the article]

Published
2021
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31. The neurophysiological lesson from the Italian CIDP database

Author

Marco Luigetti, Andrea Cortese, Erdita Peci, Angelo Maurizio Clerici, Raffaella Fazio, Anna Mazzeo, Dario Cocito, Gabriele Siciliano, S. Jann, Laura Piccolo, Girolama Alessandra Marfia, Giovanni Antonini, Pietro Emiliano Doneddu, Luca Leonardi, Chiara Briani, Lucio Santoro, Fiore Manganelli, Giuseppe Lauria, Giuseppe Cosentino, Marinella Carpo, Stefano Cotti Piccinelli, Massimiliano Filosto, Giorgia Mataluni, Emanuele Spina, Angelo Schenone, Giuseppe Liberatore, Luca Gentile, Eduardo Nobile-Orazio, Tiziana Rosso, Marta Ruiz, Stefano Tronci, Luana Benedetti, Guido Cavaletti, Spina, E., Doneddu, P. E., Liberatore, G., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Peci, E., Tronci, S., Ruiz, M., Piccinelli, S. C., Schenone, A., Leonardi, L., Gentile, L., Piccolo, L., Mataluni, G., Santoro, L., Nobile-Orazio, E., Manganelli, F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, and Manganelli, F

Subjects
medicine.medical_specialty, Diagnostic criteria, Nerve conduction, Neurology, Polyradiculoneuropathy, Neural Conduction, Neurophysiology, Motor nerve, CIDP, Dermatology, Settore MED/26, computer.software_genre, Humans, Medicine, Peripheral Nerves, Chronic Inflammatory Demyelinating, Ulnar nerve, Ulnar Nerve, Neuroradiology, Database, business.industry, Curve analysis, Peroneal Nerve, General Medicine, medicine.disease, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Psychiatry and Mental health, Peripheral Nerve, Original Article, Neurology (clinical), Neurosurgery, business, computer, Human
Abstract

Introduction Electrophysiological diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may be challenging. Thus, with the aim ofproviding some practical advice in electrophysiological approach to a patient with suspected CIDP, we analyzed electrophysiological data from 499 patients enrolled inthe Italian CIDP Database. Methods We calculated the rate of each demyelinating feature, the rate of demyelinating features per nerve, the diagnostic rate for upper andlower limb nerves, and, using a ROC curve analysis, the diagnostic accuracy of each couple of nerves and each demyelinating feature, for every CIDP subtype.Moreover, we compared the electrophysiological data of definite and probable CIDP patients with those of possible and not-fulfilling CIDP patients, and by a logisticregression analysis, we estimated the odds ratio (OR) to make an electrophysiological diagnosis of definite or probable CIDP. Results The ulnar nerve had the highestrate of demyelinating features and, when tested bilaterally, had the highest diagnostic accuracy except for DADS in which peroneal nerves were the most informative.In possible and not-fulfilling CIDP patients, a lower number of nerves and proximal temporal dispersion (TD) measurements had been performed compared to definiteand probable CIDP patients. Importantly, OR for each tested motor nerve and each TD measurement was 1.59 and 1.33, respectively. Conclusion Our findingsdemonstrated that the diagnosis of CIDP may be missed due to inadequate or incomplete electrophysiological examination or interpretation. At the same time, thesedata taken together could be useful to draw a thoughtful electrophysiological approach to patients suspected of CIDP.

Published
2021
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32. Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database

Author

Giuseppe Liberatore, Alberto De Lorenzo, Claudia Giannotta, Fiore Manganelli, Massimiliano Filosto, Giuseppe Cosentino, Dario Cocito, Chiara Briani, Andrea Cortese, Raffaella Fazio, Giuseppe Lauria, Angelo Maurizio Clerici, Tiziana Rosso, Girolama Alessandra Marfia, Giovanni Antonini, Guido Cavaletti, Marinella Carpo, Pietro Emiliano Doneddu, Emanuele Spina, Stefano Cotti Piccinelli, Erdita Peci, Luis Querol, Eduardo Nobile-Orazio, Liberatore, G., De Lorenzo, A., Giannotta, C., Manganelli, F., Filosto, M., Cosentino, G., Cocito, D., Briani, C., Cortese, A., Fazio, R., Lauria, G., Clerici, A. M., Rosso, T., Marfia, G. A., Antonini, G., Cavaletti, G., Carpo, M., Doneddu, P. E., Spina, E., Cotti Piccinelli, S., Peci, E., Querol, L., Nobile-Orazio, E., Liberatore, G, De Lorenzo, A, Giannotta, C, Manganelli, F, Filosto, M, Cosentino, G, Cocito, D, Briani, C, Cortese, A, Fazio, R, Lauria, G, Clerici, A, Rosso, T, Marfia, G, Antonini, G, Cavaletti, G, Carpo, M, Doneddu, P, Spina, E, Cotti Piccinelli, S, Peci, E, Querol, L, and Nobile-Orazio, E

Subjects
anti-ganglioside antibodie, Chronic inflammatory demyelinating polyradiculoneuropathy, Anti-nerve antibodies, Peripheral neuropathy, Dermatology, General Medicine, CIDP, anti-ganglioside antibodies, Settore MED/26, paranodopathy, Anti-nerve antibodie, Psychiatry and Mental health, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Contactin 1, Humans, Ataxia, Neurology (clinical), Nerve Growth Factors, Cell Adhesion Molecules, Autoantibodies
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.

Published
2022
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33. Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP

Author

Spina, Emanuele, Doneddu, Pietro Emiliano, Liberatore, Giuseppe, Cocito, Dario, Fazio, Raffaella, Briani, Chiara, Filosto, Massimiliano, Benedetti, Luana, Antonini, Giovanni, Cosentino, Giuseppe, Jann, Stefano, Mazzeo, Anna, Cortese, Andrea, Marfia, Girolama Alessandra, Clerici, Angelo Maurizio, Siciliano, Gabriele, Carpo, Marinella, Luigetti, Marco, Lauria, Giuseppe, Rosso, Tiziana, Cavaletti, Guido, Peci, Erdita, Tronci, Stefano, Ruiz, Marta, Piccinelli, Stefano Cotti, Schenone, Angelo, Leonardi, Luca, Gentile, Luca, Piccolo, Laura, Mataluni, Giorgia, Santoro, Lucio, Nobile-Orazio, Eduardo, Manganelli, Fiore, Gallia, Francesca, Rozzano, Null, Raffaele, San, Pisano, Enrica, Beronio, Alessandro, Toscano, Antonio, Vegezzi, Elisa, Fierro, Brigida, Pinuccia, Verrengia Elena, Bianchi, Elisa, Beghi, Ettore, Scrascia, Federica, Garnero, Martina, Sabatelli, Mario, Dacci, Patrizia, Schirinziand, Rika, Balducci, Claudia, Spina, E., Doneddu, P. E., Liberatore, G., Cocito, D., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Antonini, G., Cosentino, G., Jann, S., Mazzeo, A., Cortese, A., Marfia, G. A., Clerici, A. M., Siciliano, G., Carpo, M., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Peci, E., Tronci, S., Ruiz, M., Piccinelli, S. C., Schenone, A., Leonardi, L., Gentile, L., Piccolo, L., Mataluni, G., Santoro, L., Nobile-Orazio, E., Manganelli, F., Spina, E, Doneddu, P, Liberatore, G, Cocito, D, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Antonini, G, Cosentino, G, Jann, S, Mazzeo, A, Cortese, A, Marfia, G, Clerici, A, Siciliano, G, Carpo, M, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Peci, E, Tronci, S, Ruiz, M, Piccinelli, S, Schenone, A, Leonardi, L, Gentile, L, Piccolo, L, Mataluni, G, Santoro, L, Nobile-Orazio, E, and Manganelli, F

Subjects
0301 basic medicine, medicine.medical_specialty, Neurology, Databases, Factual, Population, Neural Conduction, Polyradiculoneuropathy, Neurophysiology, Chronic inflammatory demyelinating polyneuropathy, CIDP, Wrist, Settore MED/26, Diagnostic accuracy, 03 medical and health sciences, Databases, 0302 clinical medicine, medicine, Humans, Carpal tunnel, Carpal tunnel syndrome, education, Chronic Inflammatory Demyelinating, demyelination, diagnostic accuracy, neurophysiology, Factual, Demyelination, Median Nerve, Carpal Tunnel Syndrome, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, education.field_of_study, Original Communication, business.industry, Median Neuropathy, medicine.disease, Median nerve, 030104 developmental biology, medicine.anatomical_structure, Neurology (clinical), Radiology, business, 030217 neurology & neurosurgery, Human
Abstract

Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.

Published
2022

34. Acute and chronic inflammatory neuropathies and COVID-19 vaccines: practical recommendations from the task force of the Italian Peripheral Nervous System Association (ASNP)

Author

Chiara Briani, Gian Maria Fabrizi, Emanuele Spina, Pietro Emiliano Doneddu, Fiore Manganelli, Eduardo Nobile-Orazio, Doneddu, P. E., Spina, E., Briani, C., Fabrizi, G. M., Manganelli, F., and Nobile-Orazio, E.

Subjects
medicine.medical_specialty, 2019-20 coronavirus outbreak, COVID-19 Vaccines, Coronavirus disease 2019 (COVID-19), Guillain-Barre Syndrome, inflammatory neuropathies, 03 medical and health sciences, 0302 clinical medicine, vaccine, medicine, Humans, In patient, Intensive care medicine, Societies, Medical, COVID-19, coronavirus disease, vaccination, Task force, business.industry, General Neuroscience, Vaccination, Increased risk, Italy, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, 030220 oncology & carcinogenesis, Expert opinion, Practice Guidelines as Topic, Neurology (clinical), Best evidence, business, 030217 neurology & neurosurgery, inflammatory neuropathie
Abstract

Background and aims To develop recommendations for vaccination for coronavirus-19 (COVID-19) in patients with inflammatory neuropathies. Methods Key questions were formulated in order to perform a literature review on the safety and efficacy of vaccines in patients with inflammatory neuropathies. Based on the best evidence and expert opinion, a list of recommendations was formulated to inform decision on vaccination for COVID-19 in patients with inflammatory neuropathies and increase adherence to vaccination programmes. Results Recommendations addressing safety and efficacy of vaccination in patients with inflammatory neuropathies were formulated. No data are currently available on the safety and efficacy of COVID-19 vaccines in patients with inflammatory neuropathies or other immune-mediated conditions. There is only sparse data on the safety of previous available vaccines in patients with inflammatory neuropathies, but studies on other autoimmune disorders indicate that these are safe and mostly efficacious. Patients with inflammatory neuropathies might be at increased risk for severe illness from COVID-19. Interpretation Patients with inflammatory neuropathies should be encouraged to adhere to the vaccination campaign for COVID-19. These recommendations provide guidance on the management of vaccinations for COVID-19 in patients with inflammatory neuropathies. More research is needed regarding the safety and efficacy of vaccination in patients with inflammatory neuropathies and other immune conditions.

Published
2021

35. Guillain-Barré syndrome and COVID-19: an observational multicentre study from two Italian hotspot regions

Author

Giovanni De Maria, Francesca Castellani, Gabriella Zara, Barbara Frigeni, Stefano Gazzina, Giuseppe Natalini, Maurizio Osio, Francesco Palmerini, Alessandro Padovani, Enrico Marchioni, Pietro Emiliano Doneddu, Eugenio Magni, Sabrina Ravaglia, Frank Rasulo, C. Foresti, Andrea Bellomo, Maria Cotelli, Marinella Carpo, Laura Broglio, Eduardo Nobile-Orazio, Anna Maria Perotti, Chiara Briani, Ubaldo Del Carro, Massimo Filippi, Giuseppe Cosentino, Stefano Cotti Piccinelli, Antonino Uncini, Andrea Rasera, Raffaella Fazio, Gian Maria Fabrizi, Giovanna Squintani, Elena Grappa, Loris Poli, Ugo Leggio, Valeria Bertasi, Sergio Ferrari, Nicola Latronico, Francesca Caprioli, Maria Sessa, Laura Bertolasi, Massimiliano Filosto, Francesca Bianchi, Federico Ranieri, Giuseppe Scopelliti, Maria Cristina Servalli, Filosto, M., Cotti Piccinelli, S., Gazzina, S., Foresti, C., Frigeni, B., Servalli, M. C., Sessa, M., Cosentino, G., Marchioni, E., Ravaglia, S., Briani, C., Castellani, F., Zara, G., Bianchi, F., Del Carro, U., Fazio, R., Filippi, M., Magni, E., Natalini, G., Palmerini, F., Perotti, A. M., Bellomo, A., Osio, M., Scopelliti, G., Carpo, M., Rasera, A., Squintani, G., Doneddu, P. E., Bertasi, V., Cotelli, M. S., Bertolasi, L., Fabrizi, G. M., Ferrari, S., Ranieri, F., Caprioli, F., Grappa, E., Broglio, L., De Maria, G., Leggio, U., Poli, L., Rasulo, F., Latronico, N., Nobile-Orazio, E., Padovani, A., and Uncini, A.

Subjects
Adult, Male, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Clinical Neurology, GBS, Guillain-Barre Syndrome, Settore MED/17 - MALATTIE INFETTIVE, law.invention, 03 medical and health sciences, 0302 clinical medicine, law, Internal medicine, medicine, Humans, Guillain-Barré syndrome (GBS), 030212 general & internal medicine, Referral and Consultation, Aged, Retrospective Studies, Guillain-Barre syndrome, SARS-CoV-2, business.industry, Incidence, GBS increased incidence, GBS, Guillain Barrè Syndrome, COVID-19, SARS-CoV-2, COVID-19, Outbreak, Retrospective cohort study, Middle Aged, medicine.disease, Guillain Barrè Syndrome, Intensive care unit, SARS-CoV-2 outbreak, Hospitalization, Psychiatry and Mental health, Blood pressure, Italy, Neuromuscular, Cohort, Female, Observational study, Surgery, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

ObjectiveSingle cases and small series of Guillain-Barré syndrome (GBS) have been reported during the SARS-CoV-2 outbreak worldwide. We evaluated incidence and clinical features of GBS in a cohort of patients from two regions of northern Italy with the highest number of patients with COVID-19.MethodsGBS cases diagnosed in 12 referral hospitals from Lombardy and Veneto in March and April 2020 were retrospectively collected. As a control population, GBS diagnosed in March and April 2019 in the same hospitals were considered.ResultsIncidence of GBS in March and April 2020 was 0.202/100 000/month (estimated rate 2.43/100 000/year) vs 0.077/100 000/month (estimated rate 0.93/100 000/year) in the same months of 2019 with a 2.6-fold increase. Estimated incidence of GBS in COVID-19-positive patients was 47.9/100 000 and in the COVID-19-positive hospitalised patients was 236/100 000. COVID-19-positive patients with GBS, when compared with COVID-19-negative subjects, showed lower MRC sum score (26.3±18.3 vs 41.4±14.8, p=0.006), higher frequency of demyelinating subtype (76.6% vs 35.3%, p=0.011), more frequent low blood pressure (50% vs 11.8%, p=0.017) and higher rate of admission to intensive care unit (66.6% vs 17.6%, p=0.002).ConclusionsThis study shows an increased incidence of GBS during the COVID-19 outbreak in northern Italy, supporting a pathogenic link. COVID-19-associated GBS is predominantly demyelinating and seems to be more severe than non-COVID-19 GBS, although it is likely that in some patients the systemic impairment due to COVID-19 might have contributed to the severity of the whole clinical picture.

Published
2020

36. Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

Author

Pietro Emiliano, Doneddu, Dario, Cocito, Fiore, Manganelli, Raffaella, Fazio, Chiara, Briani, Massimiliano, Filosto, Luana, Benedetti, Elisa, Bianchi, Stefano, Jann, Anna, Mazzeo, Giovanni, Antonini, Giuseppe, Cosentino, Girolama Alessandra, Marfia, Andrea, Cortese, Angelo Maurizio, Clerici, Marinella, Carpo, Angelo, Schenone, Gabriele, Siciliano, Marco, Luigetti, Giuseppe, Lauria, Tiziana, Rosso, Guido, Cavaletti, Ettore, Beghi, Giuseppe, Liberatore, Lucio, Santoro, Emanuele, Spina, Erdita, Peci, Stefano, Tronci, Marta, Ruiz, Stefano, Cotti Piccinelli, Elena Pinuccia, Verrengia, Luca, Gentile, Luca, Leonardi, Giorgia, Mataluni, Laura, Piccolo, Eduardo, Nobile-Orazio, Mario, Sabatelli, Doneddu, P. E., Cocito, D., Manganelli, F., Fazio, R., Briani, C., Filosto, M., Benedetti, L., Bianchi, E., Jann, S., Mazzeo, A., Antonini, G., Cosentino, G., Marfia, G. A., Cortese, A., Clerici, A. M., Carpo, M., Schenone, A., Siciliano, G., Luigetti, M., Lauria, G., Rosso, T., Cavaletti, G., Beghi, E., Liberatore, G., Santoro, L., Spina, E., Peci, E., Tronci, S., Ruiz, M., Cotti Piccinelli, S., Verrengia, E. P., Gentile, L., Leonardi, L., Mataluni, G., Piccolo, L., Nobile-Orazio, E., Doneddu, P, Cocito, D, Manganelli, F, Fazio, R, Briani, C, Filosto, M, Benedetti, L, Bianchi, E, Jann, S, Mazzeo, A, Antonini, G, Cosentino, G, Marfia, G, Cortese, A, Clerici, A, Carpo, M, Schenone, A, Siciliano, G, Luigetti, M, Lauria, G, Rosso, T, Cavaletti, G, Beghi, E, Liberatore, G, Santoro, L, Spina, E, Peci, E, Tronci, S, Ruiz, M, Cotti Piccinelli, S, Verrengia, E, Gentile, L, Leonardi, L, Mataluni, G, Piccolo, L, and Nobile-Orazio, E

Subjects
Male, Diabetic neuropathy, Comorbidity, 030204 cardiovascular system & hematology, Adrenal Cortex Hormone, Monoclonal Gammopathy of Undetermined Significance, 0302 clinical medicine, Immunologic Factor, Diabetic Neuropathies, Adrenal Cortex Hormones, 80 and over, Age of Onset, Chronic Inflammatory Demyelinating, Child, Aged, 80 and over, education.field_of_study, diabetes, Plasma Exchange, Immunoglobulins, Intravenous, Diabetes Mellitu, Middle Aged, Settore MED/26 - NEUROLOGIA, Psychiatry and Mental health, Treatment Outcome, Italy, MGUS, Female, Intravenous, Human, Adult, medicine.medical_specialty, Adolescent, Population, Polyradiculoneuropathy, Immunoglobulins, CIDP, Settore MED/26, Autoimmune Disease, Autoimmune Diseases, 03 medical and health sciences, Young Adult, Internal medicine, Diabetes mellitus, Diabetes Mellitus, medicine, Humans, Immunologic Factors, Sex Distribution, education, Aged, business.industry, Retrospective cohort study, medicine.disease, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, CIDP, neuropathy, diabates, Immunoglobulins, Intravenou, Quality of Life, Surgery, Diabetic Neuropathie, Neurology (clinical), Age of onset, business, 030217 neurology & neurosurgery, Monoclonal gammopathy of undetermined significance
Abstract

ObjectivesTo determine the prevalence of different comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and their impact on outcome, treatment choice and response.MethodsUsing a structured questionnaire, we collected information on comorbidities from 393 patients with CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria included in the Italian CIDP database.ResultsOne or more comorbidities were reported by 294 patients (75%) and potentially influenced treatment choice in 192 (49%) leading to a less frequent use of corticosteroids. Response to treatment did not differ, however, from that in patients without comorbidities. Diabetes (14%), monoclonal gammopathy of undetermined significance (MGUS) (12%) and other immune disorders (16%) were significantly more frequent in patients with CIDP than expected in the general European population. Patients with diabetes had higher disability scores, worse quality of life and a less frequent treatment response compared with patients without diabetes. Patients with IgG-IgA or IgM MGUS had an older age at CIDP onset while patients with other immune disorders had a younger age at onset and were more frequently females. IgM MGUS was more frequent in patients with motor CIDP than in patients with typical CIDP.ConclusionsComorbidities are frequent in patients with CIDP and in almost 50% of them have an impact on treatment choice. Diabetes, MGUS and other immune diseases are more frequent in patients with CIDP than in the general population. Only diabetes seems, however, to have an impact on disease severity and treatment response possibly reflecting in some patients a coexisting diabetic neuropathy.

Published
2020

37. Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database

Author

Chiara Briani, Giuseppe Lauria, Pietro Emiliano Doneddu, Giovanni Antonini, Andrea Cortese, Marco Luigetti, Eduardo Nobile-Orazio, Guido Cavaletti, Luca Leonardi, Dario Cocito, G Marfia, Ettore Beghi, S. Jann, Fiore Manganelli, Laura Piccolo, S. Cotti Piccinelli, Giorgia Mataluni, Tiziana Rosso, Patrizia Dacci, Marinella Carpo, Giuseppe Cosentino, Gabriele Siciliano, Angelo Maurizio Clerici, Elisa Bianchi, Elena Pinuccia Verrengia, Erika Schirinzi, Lucio Santoro, Antonio Toscano, Massimiliano Filosto, Marta Ruiz, Stefano Tronci, Luana Benedetti, Giuseppe Liberatore, Raffaella Fazio, Anna Mazzeo, Erdita Peci, Doneddu, P, Bianchi, E, Cocito, D, Manganelli, F, Fazio, R, Filosto, M, Mazzeo, A, Cosentino, G, Cortese, A, Jann, S, Clerici, A, Antonini, G, Siciliano, G, Luigetti, M, Marfia, G, Briani, C, Lauria, G, Rosso, T, Cavaletti, G, Carpo, M, Benedetti, L, Beghi, E, Liberatore, G, Santoro, L, Peci, E, Tronci, S, Cotti Piccinelli, S, Toscano, A, Piccolo, L, Verrengia, E, Leonardi, L, Schirinzi, E, Mataluni, G, Ruiz, M, Dacci, P, Nobile-Orazio, E, Doneddu, P. E., Bianchi, E., Cocito, D., Manganelli, F., Fazio, R., Filosto, M., Mazzeo, A., Cosentino, G., Cortese, A., Jann, S., Clerici, A. M., Antonini, G., Siciliano, G., Luigetti, M., Marfia, G. A., Briani, C., Lauria, G., Rosso, T., Cavaletti, G., Carpo, M., Benedetti, L., Beghi, E., Liberatore, G., Santoro, L., Peci, E., Tronci, S., Piccinelli, S. C., Toscano, A., Piccolo, L., Verrengia, E. P., Leonardi, L., Schirinzi, E., Mataluni, G., Ruiz, M., Dacci, P., and Nobile-Orazio, E.

Subjects
Adult, Male, medicine.medical_specialty, lifestyle, Databases, Factual, Disease, computer.software_genre, Settore MED/26, chronic inflammatory demyelinating neuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, diet, epidemiology, infections, vaccination, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, Epidemiology, medicine, Humans, 030212 general & internal medicine, Risk factor, Child, Life Style, Database, business.industry, Antecedent variable, Polyradiculoneuropathy, Feeding Behavior, Middle Aged, medicine.disease, infection, Settore MED/26 - NEUROLOGIA, Antecedent (behavioral psychology), Italy, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Neurology, Female, Neurology (clinical), Complication, business, Lifestyle habits, computer, 030217 neurology & neurosurgery
Abstract

Background and purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender-matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls. Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex-matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1–42days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events. Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease.

Published
2020

38. Sensitivity and specificity of a commercial ELISA test for anti-MAG antibodies in patients with neuropathy

Author

Girolama Alessandra Marfia, Giovanni Antonini, Giuseppe Cosentino, Tiziana Rosso, Giuseppe Lauria, Fiore Manganelli, Francesca Gallia, Angelo Maurizio Clerici, Dario Cocito, Pietro Emiliano Doneddu, Guido Cavaletti, Massimiliano Filosto, F. Terenghi, Eduardo Nobile-Orazio, Claudia Giannotta, Emanuela Morenghi, Blesson Punnen Sajeev, Giuseppe Liberatore, Liberatore, G., Giannotta, C., Sajeev, B. P., Morenghi, E., Terenghi, F., Gallia, F., Doneddu, P. E., Manganelli, F., Cocito, D., Filosto, M., Antonini, G., Cosentino, G., Marfia, G. A., Clerici, A. M., Lauria, G., Rosso, T., Cavaletti, G., Nobile-Orazio, E., Liberatore, G, Giannotta, C, Sajeev, B, Morenghi, E, Terenghi, F, Gallia, F, Doneddu, P, Manganelli, F, Cocito, D, Filosto, M, Antonini, G, Cosentino, G, Marfia, G, Clerici, A, Lauria, G, Rosso, T, Cavaletti, G, and Nobile-Orazio, E

Subjects
0301 basic medicine, medicine.medical_specialty, anti-MAG polyneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, ELISA, sensitivity, specificity, autoantibodies, case-control studies, enzyme-linked immunosorbent assay, humans, myelin-associated glycoprotein, polyneuropathies, retrospective studies, Immunology, Anti-MAG polyneuropathy, Enzyme-Linked Immunosorbent Assay, Settore MED/26, Gastroenterology, Polyneuropathies, 03 medical and health sciences, Sensitivity, 0302 clinical medicine, Internal medicine, medicine, Humans, Immunology and Allergy, In patient, Autoantibodies, Retrospective Studies, Anti-MAG polyneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, biology, business.industry, Anti mag, Specificity, medicine.disease, Autoantibodie, Myelin-Associated Glycoprotein, Titer, 030104 developmental biology, Polyneuropathie, nervous system, Neurology, Case-Control Studies, Elisa test, biology.protein, Neurology (clinical), Antibody, Case-Control Studie, business, Sensitivity (electronics), Polyneuropathy, 030217 neurology & neurosurgery, Human
Abstract

For the diagnosis of anti-MAG polyneuropathy the commercial ELISA manufacturer currently recommends a cut-off of 1000 Bühlmann Titer Units (BTU). We analyzed sera from 80 anti-MAG neuropathy patients and 383 controls (with other neuropathies or healthy controls) to assess the ELISA sensitivity and specificity at different thresholds. A better combination of sensitivity/specificity was found at a threshold >1500 BTU than at >1000 BTU. The best value of specificity was obtained at threshold >7000 BTU. There was a diagnostic grey area between 1500 and 7000 BTU in which the clinical phenotypes as well as electrophysiological studies need to be carefully assessed particularly to differentiate CIDP and anti-MAG neuropathy.

Published
2020
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39. Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

Author

Liberatore G, Manganelli F, Doneddu PE, Cocito D, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Antonini G, Cosentino G, Jann S, Cortese A, Marfia GA, Clerici AM, Siciliano G, Carpo M, Luigetti M, Lauria G, Rosso T, Cavaletti G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Schenone A, Leonardi L, Toscano A, Mataluni G, Spina E, Gentile L, and Nobile-Orazio E

Subjects
Databases, Factual, Humans, Neural Conduction, Peripheral Nerves, Retrospective Studies, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating diagnosis
Abstract

Background and Purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria., Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed., Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%)., Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies., (© 2020 European Academy of Neurology.)

Published
2021
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40. Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database.

Author

Doneddu PE, Bianchi E, Cocito D, Manganelli F, Fazio R, Filosto M, Mazzeo A, Cosentino G, Cortese A, Jann S, Clerici AM, Antonini G, Siciliano G, Luigetti M, Marfia GA, Briani C, Lauria G, Rosso T, Cavaletti G, Carpo M, Benedetti L, Beghi E, Liberatore G, Santoro L, Peci E, Tronci S, Cotti Piccinelli S, Toscano A, Piccolo L, Verrengia EP, Leonardi L, Schirinzi E, Mataluni G, Ruiz M, Dacci P, and Nobile-Orazio E

Subjects
Adult, Child, Databases, Factual, Female, Humans, Infections complications, Italy epidemiology, Male, Middle Aged, Risk Factors, Feeding Behavior, Life Style, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating epidemiology
Abstract

Background and Purpose: The role of lifestyle and dietary habits and antecedent events has not been clearly identified in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)., Methods: Information was collected about modifiable environmental factors and antecedent infections and vaccinations in patients with CIDP included in an Italian CIDP Database. Only patients who reported not having changed their diet or the lifestyle habits investigated in the study after the appearance of CIDP were included. The partners of patients with CIDP were chosen as controls. Gender-matched analysis was performed with randomly selected controls with a 1:1 ratio of patients and controls., Results: Dietary and lifestyle data of 323 patients and 266 controls were available. A total of 195 cases and 195 sex-matched controls were used in the analysis. Patients eating rice at least three times per week or eating fish at least once per week appeared to be at decreased risk of acquiring CIDP. Data on antecedent events were collected in 411 patients. Antecedent events within 1-42 days before CIDP onset were reported by 15.5% of the patients, including infections in 12% and vaccinations in 1.5%. Patients with CIDP and antecedent infections more often had an acute onset of CIDP and cranial nerve involvement than those without these antecedent events., Conclusions: The results of this preliminary study seem to indicate that some dietary habits may influence the risk of CIDP and that antecedent infections may have an impact on the onset and clinical presentation of the disease., (© European Academy of Neurology 2019.)

Published
2020
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