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

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.

Keywords: European Academy of Neurology; Lewis-Sumner syndrome; Peripheral Nerve Society; chronic inflammatory demyelinating polyradiculoneuropathy; chronic inflammatory demyelinating polyradiculoneuropathy variants; diagnostic criteria; intravenous immunoglobulin; steroids.

FIGURE 1

Case selection flowchart. CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CISP, chronic immune sensory polyradiculopathy; EAN/PNS, European Academy of Neurology/Peripheral Nerve Society.

FIGURE 2

Clinical diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) according to the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for CIDP in 369 patients fulfilling the 2021 EAN/PNS electrodiagnostic criteria for possible CIDP.