The Diagnostic Accuracy of the Second Revision of the European Academy of Neurology/Peripheral Nerve Society Guideline for Diagnosing Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Iris N. van Doorn, Adája E. Baars, Luuk Wieske, Bart C. Jacobs, Krista Kuitwaard, W. Ludo van der Pol, Pieter A. van Doorn, Ivo N. van Schaik, Filip Eftimov, H. Stephan Goedee, Judith Drenthen, Camiel VerhammeABSTRACT
Background
Diagnosing chronic inflammatory demyelinating polyneuropathy (CIDP) relies on clinical and electrodiagnostic features. In 2021, the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) revised the CIDP guideline, superseding the 2010 European Federation of Neurological Societies (EFNS)/PNS guideline. This study compares the diagnostic accuracy of both guidelines and evaluates the impact of specific criterion changes and nerve conduction study (NCS) extensiveness.
Methods
Patients with CIDP were included if they fulfilled the 2010 EFNS/PNS electrodiagnostic criteria for at least possible CIDP or had a presumptive clinical CIDP diagnosis with objective treatment response. Matched controls were patients with suspected chronic immune‐mediated neuropathy who received a diagnosis other than CIDP. Diagnostic accuracy was evaluated using two thresholds: one including possible CIDP and one restricted to (probable/definite) CIDP. The impact of electrodiagnostic criteria changes and NCS extensiveness on diagnostic performance was assessed.
Results
We included 339 patients and 339 matched controls. Sensitivity of the 2010 EFNS/PNS guideline was 92% when including possible CIDP and 91% for probable/definite CIDP, with specificities of 67% and 78%, respectively. For the 2021 EAN/PNS guideline, sensitivity was 94% when including possible CIDP and 83% for CIDP, with specificities of 66% and 86%, respectively. Differences were due to sensory criteria and changes in conduction block criteria. Unilateral NCS would miss 77 possible CIDP diagnoses.
Conclusion
Sensitivity and specificity were comparable when including possible CIDP, but the 2021 EAN/PNS guideline showed lower sensitivity and higher specificity for CIDP alone. Modifying criteria, including removal of sensory abnormalities, and more extensive NCS enhanced accuracy.