Central nervous system involvement in multifocal demyelinating neuropathy with persistent conduction block

We report the case of a 27-year-old man treated for bilateral optic neuritis 5 and 3 years before who within a few months developed sensorimotor disorders of the arms and legs characterized by asymmetric distribution and distal prominence. In addition to sensorimotor defects, which were particularly marked in the left arm and right leg, clinical examination showed nearly generalized areflexia. Electrophysiological studies revealed a multifocal neuropathy with persistent distal and proximal conduction blocks associated with a considerable slowing of motor nerve conduction, as well as central nervous system involvement indicated by motor-, somatosensory-, and visual-evoked potentials. CSF analysis showed a mildly elevated protein level; anti-GM1 activity was negative. Sural nerve biopsy revealed onion-bulb-like formations, and cerebral MRI showed a small, isolated, and aspecific high signal for white matter. First described by Lewis and Sumner in 1982, multifocal neuropathy with persistent conduction blocks may be associated with central demyelination. Our case is compared with 3 similar ones in the literature, and the favorable effects of steroid therapy are emphasized.