CIDP diagnostic pitfalls and perception of treatment benefit

Objective: We aimed to explore the diagnosis and misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and to identify pitfalls that erroneously lead to a misdiagnosis.

Methods: A retrospective study of 59 consecutive patients referred with a diagnosis of CIDP was performed. Patients were classified as having or not having CIDP according to European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. Diagnostic and treatment data were compared in the 2 groups.

Results: Forty-seven percent of patients referred with a diagnosis of CIDP failed to meet minimal CIDP diagnostic requirements. All misdiagnosed patients who satisfied EFNS/PNS clinical criteria would be considered atypical as defined by the EFNS/PNS. CSF cytoalbuminologic dissociation was present in 50% of those without CIDP, although protein elevations were generally mild. Nerve conduction studies in patients without CIDP were heterogeneous, but generally showed demyelinating features better explained by a process other than CIDP. Patients frequently reported improvements after being treated with immunotherapy, even if the CIDP diagnosis was incorrect.

Conclusions: CIDP misdiagnosis is common. Over-reliance on subjective patient-reported perception of treatment benefit, liberal electrophysiologic interpretation of demyelination, and placing an overstated importance on mild or moderate cytoalbuminologic dissociation are common diagnostic errors. Utilization of clear and objective indicators of treatment efficacy might improve our ability to make informed treatment decisions.