Electrophysiological Responsiveness to Long-Term Therapy in Chronic Inflammatory Demyelinating Polyneuropathy: Case...

Electrophysiological studies are essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP), but the utility of nerve conduction studies in monitoring outcomes in individual CIDP patients is controversial. Electrophysiological improvements after short-term treatment have been described in large cohorts of CIDP patients, but the magnitude of the changes is small and might be obscured in individual patients due to the variation inherent in nerve conduction testing. We present the case of a CIDP patient treated successfully with immunosuppression and followed for 31 years with serial standardized clinical and electrophysiological evaluations. Improvement in electrophysiological parameters lagged clinical changes for up to 2 years, but all motor parameters improved (distal motor and F wave latencies, conduction velocities, and compound muscle action potential amplitudes) even with evidence of initial axonal damage. Worsening of electrophysiological parameters, specifically increasing F wave latencies, heralded clinical relapse by as much as a year. Electrophysiological parameters do improve with treatment in CIDP patients, although the changes can take up to 2 years, and also worsening electrophysiological parameters can herald clinical relapse and might help guide therapeutic decisions.