From the Research
Diagnostic Criteria for CIDP
The European Federation of Neurological Societies (EFNS) diagnostic criteria for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) combine clinical features with electrophysiological evidence of demyelination 1. The criteria include:
- Clinical symptoms such as symmetric involvement of the proximal and distal portions of the limbs
- Nerve conduction study findings that indicate demyelination
- Other supportive criteria such as cytoalbuminologic dissociation in cerebrospinal fluid (CSF)
EFNS/PNS Diagnostic Criteria
The EFNS/Peripheral Nerve Society (PNS) criteria define "typical CIDP" and "atypical CIDP" variants, including multifocal acquired demyelinating sensory and motor (MADSAM), distal acquired demyelinating symmetric (DADS), pure sensory, pure motor, and focal subtypes 2. The criteria also include clinical and electrodiagnostic criteria for CIDP variants, updated supportive criteria, and sensory criteria as an integral part of the electrodiagnostic criteria 3.
Evaluation of EFNS/PNS Diagnostic Criteria
A study evaluating the EFNS/PNS diagnostic criteria in a cohort of CIDP patients found that 89.7% of patients fulfilled the criteria, while 10.3% did not due to negative electrodiagnostic criteria 4. However, these patients still showed signs of demyelination and responded to therapy, suggesting that failure to fulfill diagnostic criteria should not automatically preclude treatment.
Challenges in Diagnosis
The diagnosis of CIDP poses numerous challenges, including heterogeneous presentations, complexity of interpreting electrodiagnostic criteria, and misdiagnoses 5, 3. Early recognition and treatment are important to reduce the risk of irreversible axonal damage, which may lead to permanent disability. 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 3.