A 66-year-old man developed
paresthesia of the distal parts of the bilateral lower limbs a week after his upper respiratory
infection, followed by the weakness with the legs and
paresthesia with the lip area, tongue and finger
tips. Those symptoms gradually became worse to the point that he was unable to walk 10 days later. Although skin pigmentation,
edema, and lymph node swelling were not found, we made a diagnosis of
Crow-Fukase syndrome (CFS) because of clinical features of
polyneuropathy,
IgG-lambda type M proteinemia, endocrinological abnormality, elevated plasma level of
vascular endothelial growth factor (
VEGF) and extramedullary
plasmacytoma in his abdomen. Following
intravenous immunoglobulin therapy (
IVIg), he showed marked improvement. However, his
neurologic symptoms deteriorated acutely just after open biopsy together with the elevation of
VEGF level, and a few days later he was in the state of flaccid
quadriparesis. We tried
IVIg therapy again and his
neurologic symptoms were markedly improved. We speculated that an elevated
VEGF, released from plasma cells induced by the bioprocedure, might have caused an increase in microvascular permeability and affected the blood-nerve-barrier, thereby his
neurologic symptoms deteriorated. It is thought that this case may support the hypothesis that a significant role is played by
VEGF in the pathomechanism of the development of CFS. Additionally we experienced that
IVIg was very effective to the
neurologic symptoms, and we think that
IVIg will be able to be one of the future
therapy of the CFS. To our knowledge, there has been no report of CFS which manifested acute deterioration of his
neurologic symptoms just after open biopsy with acute onset with
Guillain-Barré syndrome like symptoms.