December 2022
What is in the Literature focuses on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a neuropathy with challenges in diagnosis and treatment. A recent revision of diagnostic criteria (EFN/PNS criteria) has helped define clinical features of typical and atypical variants and what is not considered CIDP. Initiating pathologic factors is not known for typical CIDP or variants. New treatment approaches are based on immunologic mechanisms.
Rare patients with a CIDP-like clinical pattern are found to have antibodies to proteins at and around the node of Ranvier and are not considered to be CIDP but a nodal-paranodopathy. Although occurring mainly in adults, CIDP also occurs in children.
CIDP may have clinical and electrodiagnostic features that overlap with hereditary neuropathies, and the latter might show some response to treatment. Articles published in the past year that address these issues are discussed in this review.
Chronic inflammatory demyelinating polyneuropathy (CIDP) can be thought of as the chronic form of AIDP. It is primarily distinguished both by its time course—by definition, symptoms must persist for at least 8weeks—and its responsiveness to corticosteroid treatment (AIDP, you may recall, is not steroid responsive). It tends to be monophasic, with a relatively gradual onset and even more gradual recovery, but some patients can present with a relapsing and remitting course. Otherwise, CIDP closely resembles AIDP, with predominantly distal, symmetric, ascending motor weakness and decreased or absent reflexes. Albuminocytologic dissociation in the CSF is a hallmark finding, as with AIDP. IVIG, PLEX, or pulse high- dose corticosteroids are first- line treatment.
There are several CIDP variants, including sensory- predominant and pure motor forms, that can be harder to recognize and diagnose. CSF analysis and electrophysiologic testing are helpful.