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.