Extensive demyelinating changes in the peripheral nerves of Crow-Fukase syndrome: a pathological study of one autopsied case

Summary Pathological changes of the peripheral nervous system in one autopsied case of Crow-Fukase syndrome (POEMS syndrome) was systemically examined. Distally accentuated myelinated axon loss was observed in the peripheral nerve trunks, ventral and dorsal spinal roots, but was not observed in the fasciculus gracilis. Segmental demyelination and remyelination associated with focal excessive myelin outfolds were the most characteristic features, the distribution of which was more prominent in the proximal nerve trunks and the spinal nerve roots. Endoneurial edema was present, and focal perivascular T lymphocyte accumulation was occasionally observed in the spinal nerve roots and proximal nerve trunks. Neurons in the sympathetic ganglia, dorsal root ganglia and ventral horns were well preserved.Supported by grants from the Ministry of Welfare and Health of Japan