From the Guidelines
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is diagnosed based on clinical presentation and progression, with key criteria including repeated clinical relapses and a chronic disease course.
Diagnostic Criteria
- Repeated clinical relapses, suggesting a more chronic disease process
- Three or more episodes of transient neurological worsening or improvement, also known as treatment-related fluctuations (TRFs)
- Clinical deterioration ≥8 weeks after disease onset, indicating a chronic rather than acute condition 1 This distinction is crucial in differentiating CIDP from other conditions like Guillain-Barré Syndrome (GBS), as the management and prognosis may vary significantly.
From the Research
Diagnostic Criteria for CIDP
The diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is based on a combination of clinical symptoms, nerve conduction study findings that indicate demyelination, and other supportive criteria 2. 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 in 2021, which includes clinical and electrodiagnostic criteria for CIDP variants, updated supportive criteria, and sensory criteria as an integral part of the electrodiagnostic criteria 2, 3.
Key Diagnostic Criteria
- Clinical features combined with electrophysiological evidence of demyelination 3, 4
- Nerve conduction velocities below the lower limits of normal, prolongation of F-waves beyond the normal range, or presence of conduction block or temporal dispersion 5
- Supportive criteria, such as sensory criteria, as an integral part of the electrodiagnostic criteria 2
Challenges in Diagnosis
- Clinical heterogeneity of the disease, its multifocality and predilection for proximal nerve segments, and the limitations of electrophysiologic and pathologic techniques 5
- Some patients do not fulfill the diagnostic criteria, such as those with severe early axonal damage 4
- Lack of objective and reliable tools to monitor the disease course 4
Diagnostic Approaches
- European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria, which combine clinical features with electrophysiological evidence of demyelination 3, 4
- Novel diagnostic imaging techniques and molecular approaches can help to solve diagnostic problems, but do not find enough implementation 4