Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy

Area of Science:

• Neurology
• Clinical Electrophysiology
• Peripheral Neuropathy Research

Background:

• The frequency and underlying causes of chronic inflammatory demyelinating polyneuropathy (CIDP) underdiagnosis remain unclear.
• Assessing underdiagnosis is crucial for timely and appropriate patient management in specialist clinics.

Purpose of the Study:

• To determine the frequency and electroclinical characteristics of CIDP underdiagnosis before referral.
• To analyze the duration of diagnostic and treatment delays for patients with CIDP.

Main Methods:

• Retrospective analysis of 60 patients diagnosed with definite/probable CIDP between 2015-2019.
• Review of clinical and electrophysiological data against European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines.
• Identification of initial misdiagnoses, their causes, and associated diagnostic delays.

Main Results:

• 68.3% of patients initially received an alternative diagnosis, most commonly Guillain-Barré syndrome (GBS) (23.3%).
• Non-GBS underdiagnoses (45%) included genetic neuropathy, diabetic neuropathy, and chronic idiopathic axonal polyneuropathy.
• Key factors contributing to underdiagnosis were unrecognized proximal weakness (70.4%) and electrophysiological misinterpretation (85%). Mean diagnostic delay was 21.3 months.

Conclusions:

• Underdiagnosis of CIDP is common and results in substantial delays in diagnosis and treatment.
• Insufficient attention to typical electroclinical features and diagnostic criteria during initial evaluations contributes significantly to misdiagnosis.