Challenges in the Early Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Adults: Current Perspectives

Authors: van Doorn, Iris N.; Eftimov, Filip; Wieske, Luuk; van Schaik, Ivo N.; Verhamme, Camiel.

Affiliations: Department of Neurology and Clinical Neurophysiology, Amsterdam Neuroscience and University of Amsterdam, Amsterdam UMC, location AMC, the Netherlands; Department of Clinical Neurophysiology, Sint Antonius Hospital, Nieuwegein, the Netherlands; 3 Sanquin Blood Supply Foundation, Amsterdam, the Netherlands

Publication: Therapeutics and Clinical Risk Management. 2024. 111–126.

ABSTRACT: Diagnosing Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) poses numerous challenges. The heterogeneous presentations of CIDP variants, its mimics, and the complexity of interpreting electrodiagnostic criteria are just a few of the many reasons for misdiagnoses. Early recognition and treatment are important to reduce the risk of irreversible axonal damage, which may lead to permanent disability. The diagnosis of CIDP is based on a combination of clinical symptoms, nerve conduction study findings that indicate demyelination, and other supportive criteria. In 2021, the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) published a revision on the most widely adopted guideline on the diagnosis and treatment of CIDP. This updated guideline now includes clinical and electrodiagnostic criteria for CIDP variants (previously termed atypical CIDP), updated supportive criteria, and sensory criteria as an integral part of the electrodiagnostic criteria. Due to its many rules and exceptions, this guideline is complex, and misinterpretation of nerve conduction study findings remain common. CIDP is treatable with intravenous immunoglobulins, corticosteroids, and plasma exchange. The choice of therapy should be tailored to the individual patient’s situation, taking into account the severity of symptoms, potential side effects, patient autonomy, and past treatments. Treatment responses should be evaluated as objectively as possible using disability and impairment scales. Applying these outcome measures consistently in clinical practice aids in recognizing the effectiveness (or lack thereof) of a treatment and facilitates timely consideration of alternative diagnoses or treatments. This review provides an overview of the current perspectives on the diagnostic process and first-line treatments for managing the disease.