Chronic inflammatory demyelinating polyradiculoneuropathy: update on clinical features, phenotypes and treatment...

In this review, we focus on less recognised signs and symptoms in typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and on the clinical presentation, course of disease and response to treatment of the atypical CIDP phenotypes.

Frequently occurring and often disabling symptoms in CIDP such as fatigue, pain and tremor have recently been emphasised, whereas autonomic dysfunction, if present, is usually mild. Sensory CIDP is probably the most frequent atypical CIDP phenotype and diagnosis can be difficult in the absence of clear demyelinating features on nerve conduction studies. The most important study comparing intravenous immunoglobulin treatment with intravenous methylprednisolone showed a lower rate of discontinuation due to inefficacy, adverse events or intolerance with intravenous immunoglobulin treatment. However, improvement after corticosteroids seems to be more long-lasting than after intravenous immunoglobulin suggesting superior long-term immunosuppressive and immunomodulating effect of corticosteroids in CIDP.

Symptoms other than the classical motor and sensory symptoms can lead to significant disability in CIDP patients. Based on limited evidence from largely small retrospective case series, we conclude that atypical CIDP phenotypes often have a different course of disease and sometimes response to treatment when compared with typical CIDP. Prospective multicentre cohort studies using standardised clinical description, electrophysiological parameters and outcome measures are needed to study the natural disease course of these phenotypes including response to different treatments.