Opinion
Video
Overview of CIDP
Author(s):
Panelists discuss how recognizing the diverse clinical presentations and underlying immune mechanisms of chronic inflammatory demyelinating polyneuropathy (CIDP) is essential for accurate diagnosis, differentiating subtypes, and guiding effective, individualized treatment strategies.
Summary for Physicians:
This discussion provides a clinical overview of CIDP, focusing on its presentation, subtypes, and underlying pathophysiology to support accurate diagnosis and effective treatment planning.
Key Points:
- Typical and Atypical Forms of CIDP:
CIDP is a rare, immune-mediated neuropathy characterized by progressive, symmetrical weakness and sensory dysfunction in both proximal and distal muscles. The typical form presents with both motor and sensory deficits over a period of at least 8 weeks.
Atypical variants may include:
- Distal acquired demyelinating symmetric neuropathy—primarily affects distal limbs.
- Multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome)—presents asymmetrically and may mimic mononeuritis multiplex.
- Pure sensory or pure motor CIDP—less common and can be more challenging to diagnose.
Symptoms generally include limb weakness, areflexia, sensory loss, and fatigue. CIDP can vary in severity and may progress gradually or have relapsing-remitting features.
- Pathophysiology of CIDP:
CIDP is believed to result from autoimmune mechanisms targeting peripheral nerve myelin. Both cellular (T cell–mediated) and humoral (autoantibody-mediated) immune responses contribute to demyelination. The inflammatory process disrupts nerve conduction, leading to the characteristic motor and sensory symptoms.
In some patients, nodal and paranodal pathology (targeting proteins like neurofascin and contactin) has been identified, which may help explain some atypical presentations and differential treatment responses. Ongoing research into these mechanisms is shaping future diagnostic and therapeutic approaches.
In summary, CIDP encompasses a spectrum of clinical phenotypes, and understanding its immunopathogenesis is key to improving patient outcomes. Early recognition and differentiation from other neuropathies are essential for timely and effective treatment.
