Evaluation of the EFNS/PNS diagnostic criteria in a cohort of CIDP patients

Abstract

Objective To evaluate the European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) in a cohort of patients diagnosed and treated for CIDP in a tertiary university hospital. Methods In a monocentric retrospective study of 203 CIDP patients, diagnosed according to expert opinion, we evaluated the EFNS/PNS diagnostic criteria. Clinical course and nerve conduction studies (NCS) over 1 year from first referral were studied. Secondarily, we compared the clinical and paraclinical characteristics, including nerve ultrasound, of patients who failed with those who fulfilled the criteria in order to identify clinically relevant differences. Results At 1 year, 182 (89.7%) patients fulfilled the criteria (156/76.9% definite, 22/10.8% probable, and 4/2% possible). Twenty‐one (10.3%) patients did not because the electrodiagnostic criteria remained negative. These still showed signs of demyelination but did not reach the cut‐off values. They also presented typical, albeit less pronounced, multifocal nerve enlargement in ultrasonography. Mean disability at presentation and 1 year after was significantly lower. Most importantly, a relevant proportion of these patients also responded to therapy (6/21 = 28.6% vs. 82/182 = 45.3% of those fulfilling the criteria). Interpretation CIDP diagnosis could be established for 89.7% of patients over the course of 1 year using EFNS/PNS criteria. The remaining patients (10.3%) presented with milder disability, less accentuated demyelination, but otherwise similar characteristics and still considerable probability of treatment response. Failure to fulfill diagnostic criteria should not automatically preclude treatment. Nerve ultrasound should be considered as a complementary diagnostic tool to detect signs of inflammation in CIDP.

Correlations between Electrophysiological Parameters, Lymphocyte Distribution and Cytokine Levels in Patients with Chronic Demyelinating Inflammatory Polyneuropathy

“…The study group consisted of 60 patients (52 male and eight female) with a mean age of 64.8 ± 11.2 (female 59.9 ± 11.1 and male 65.5 ± 11.1), who fulfilled the diagnostic criteria for CIDP according to The European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) guidelines [ 15 , 16 ]. Typical phenotypic variants of CIDP were included in the study.…”

Serum neurofilament light chain does not detect self‐reported treatment‐related fluctuations in chronic inflammatory demyelinating polyneuropathy

“…Patients with the diagnosis of CIDP or a CIDP variant, already receiving treatment with IVIG, were enrolled in a single-center, prospective, observational study (St. Josef-Hospital, University Hospital Bochum, Bochum, Germany). Patients were diagnosed in accordance with the diagnostic criteria of the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) [7,9]. No patient had a change in therapy in the previous 12 months.…”

Objectivity, practicality, and significance of practice guidelines for the practicing neurologists: What we learnt from consensus criteria in CIDP, Myasthenia Gravis and Inflammatory Myopathies

“…4 (2) In a German study, 10.3% among 182 CIDP patients did not fulfill the new EAN/ PNS criteria because of inadequate electrodiagnostic data, even though their patients had signs of demyelination and responded to therapy; they suggested the need to also include ultrasound as a complementary diagnostic tool. 5 (3) Among Dutch neurologists (that also included members of the Task Force) there was considerable variation in the diagnosis of CIDP with 77% not adhering to the EFNS/PNS criteria while only 50% followed treatment guidelines, suggesting the use of nerve imaging. 6 (4) A Japanese study raised concerns about the EAN/PNS criteria advising the use of peripheral nerve imaging with ultrasound and/or MRI to increase specificity.…”

“…(2) In a German study, 10.3% among 182 CIDP patients did not fulfill the new EAN/PNS criteria because of inadequate electrodiagnostic data, even though their patients had signs of demyelination and responded to therapy; they suggested the need to also include ultrasound as a complementary diagnostic tool. 5 …”