Demyelinating polyneuropathy with preferentially-proximal involvement

A 47-year-old man showed progressive, symmetrical weakness in the limbs for 6 months. There was muscle atrophy, fasciculations, and acute denervation without motor conduction abnormalities below the elbows or knees, and motor neuron disease had once been suspected. However, compound muscle action potentials (CMAPs) after proximal stimulation showed an amplitude reduction between axilla and Erb’s point for the median and ulnar nerves on both sides. His weakness as well as the amplitude reduction improved after administration of prednisolone. Demyelinative conduction abnormalities can be limited to the proximal segments for at least several months in a conduction equivalent to chronic inflammatory demyelinating polyneuropathy (CIDP).