Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?
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.
Disclosure of conflicts of interest
E.N.O. reported personal fees for advisory or scientific boards from Kedrion, Italy, Baxter, Italy, Novartis, Switzerland, CSL Behring, Italy, LFB, France, Astellas, the Netherlands, outside the submitted work and travel grants to attend scientific meetings from Baxter, Grifols, Kedrion and Novartis, Italy. P.E.D. reported travel grants to attend scientific meetings from CSL Behring and Kedrion. G.L. reported travel grants to attend scientific meetings from CSL Behring and Kedrion. D.C. reported honoraria for lecturing from Shire, CSL Behring and Kedrion and travel grants to attend scientific meetings from Shire, Kedrion and CSL Behring. E.P. reported travel grants to attend scientific meetings from CSL Behring. R.F. has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion and CSL Behring to attend scientific meetings. M.C. reported travel grants to attend scientific meetings from Kedrion. A.M. reported travel grants from Kedrion and CSL Behring to attend scientific meetings. C.B. has served on scientific advisory boards for Pfizer and has received travel grants from Kedrion and CSL Behring to attend scientific meetings. G.C. reported travel grants to attend scientific meetings from CSL Behring and Kedrion. B.F. reported travel grants to attend scientific meetings from CSL Behring and a liberal contribution from CSL Behring for the neuromuscular diseases center, outside the submitted work. A.C. reported travel grants to attend scientific meetings from Kedrion. M.L. reported honoraria for a scientific board from Pfeizer and Alnylam and travel grants from Pfeizer, Grifols and Kedrion to attend scientific meetings. L.S. reported personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. F.M. reported personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. G.C. reported honoraria for lecturing and travel grants to attend scientific meetings from Kedrion. M.F. has served on scientific advisory boards for CSL Behring and Sarepta Therapeutics and has received travel grants from Sanofi Genzyme, Kedrion, Baxter and CSL Behring to attend scientific meetings. S.J. reported research grants from Grifols, outside this work, and travel grants from Grifols and Kedrion. G.A.M. reported consultancy fees and travel funding from CSL Behring, Kedrion, Shire and Grifols. G.A. reported honoraria for lecturing from Kedrion and Sanofi Genzyme, travel grants from Kedrion, Sanofi Genzyme and LJ Pharma. G.M. reported consultancy fees and travel funding from CSL Behring, Kedrion, Shire and Grifols. The other authors declare no conflict of interest.
Open Research
Data availability statement
The data that support the findings of this study are available from the corresponding author upon reasonable request.
