[A case of Crow-Fukase syndrome which developed seven years following myelopathy of unknown origin].

A rare case of Crow-Fukase syndrome was reported, which developed 7 years following myelopathy of unknown origin. In September 1983, a 31-year-old man came to our department for progressive gait disturbance and numbness in both lower extremities. Examination on admission showed hyperreflexia with clonus and moderated muscle weakness of legs, and paresthesia below Th9 level, but myelography and CT indicated no abnormality. Thereafter, he was doing as well as walkable with a cane, but in 1989 he developed hypertrichosis, skin pigmentation, leg edema and gynecomastia with aggravation of numbness of lower extremity and was rehospitalized in October. Detailed examination on the present admission indicated compression of the conus medullaris due to an osteosclerotic lesion of vertebral body L1, which did not correspond to the possible site of pyramidal sign. Thus, the origin of the myelopathy remained unknown. After hospitalization, polyneuropathy newly developed, making him confined to his bed. Thus, the diagnosis was established as Crow-Fukase syndrome. Subtotal extirpation of L1 vertebral body and steroid therapy (PSL 10 mg/day) caused improvements of polyneuropathy and numbness of lower extremity, making him walkable again and return to his social work 3 months later. Pathohistological examination of the osteosclerotic lesion proved partial plasmacytoma. In MRI findings of the osteosclerotic lesion, the osteosclerotic and plasmacytomatous regions were outlined as low and high signals, respectively for both T1 and T2-weighted. MRI examination was very useful for grasping the tumorous extent within the osteosclerotic lesion as observed with Crow-Fukase syndrome and for determining the extent of indication for surgery and radiotherapy.