G Liberatore1, F Manganelli2, P E Doneddu1, D Cocito3, R Fazio4, C Briani5, M Filosto6, L Benedetti7,8, A Mazzeo9, G Antonini10, G Cosentino11,12,13, S Jann14, A Cortese12,13,15, G A Marfia16, A M Clerici17, G Siciliano18, M Carpo19, M Luigetti20, G Lauria21,22, T Rosso23, G Cavaletti24, L Santoro2, E Peci3, S Tronci4, M Ruiz5, S Cotti Piccinelli6, A Schenone7, L Leonardi10, A Toscano9, G Mataluni16, E Spina2, L Gentile9, E Nobile-Orazio1,25. 1. Neuromuscular and Neuroimmunology Service, IRCCS Humanitas Clinical and Research Institute, Milan, Italy. 2. Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples 'Federico II', Naples, Italy. 3. Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Turin, Italy. 4. Department of Neurology, Division of Neuroscience, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy. 5. Neurology Unit, Department of Neuroscience, University of Padova, Padova, Italy. 6. Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology, ASST 'Spedali Civili', University of Brescia, Brescia, Italy. 7. Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy. 8. Neurology Unit, Sant'Andrea Hospital, La Spezia, Italy. 9. Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy. 10. Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, Sant'Andrea Hospital, 'Sapienza' University of Rome, Rome, Italy. 11. Department of Experimental BioMedicine and Clinical Neurosciences (BioNeC), University of Palermo, Palermo, Italy. 12. IRCCS Foundation C. Mondino National Neurological Institute, Pavia, Italy. 13. Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy. 14. Department of Neuroscience, Niguarda Ca' Granda Hospital, Milan, Italy. 15. Molecular Neurosciences, University College London, London, UK. 16. Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy. 17. Neurology Unit, Circolo and Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy. 18. Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. 19. Neurology Unit, ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy. 20. Fondazione Policlinico Universitario A. Gemelli IRCCS, UOC Neurologia, Università Cattolica del Sacro Cuore, Rome, Italy. 21. Unit of Neuroalgology, IRCCS Foundation 'Carlo Besta' Neurological Institute, Milan, Italy. 22. Department of Biomedical and Clinical Sciences 'Luigi Sacco', University of Milan, Milan, Italy. 23. ULSS2 Marca Trevigiana, UOC Neurologia-Castelfranco Veneto, Treviso, Italy. 24. School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy. 25. Department of Medical Biotechnology and Translational Medicine, Milan University, Milan, Italy.
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.
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.