OVERPRODUCTION OF VASCULAR ENDOTHELIAL GROWTH-FACTOR - VASCULAR-PERMEABILITY FACTOR IS CAUSATIVE IN CROW-FUKASE-(POEMS)-SYNDROME

Crow-Fukase or POEMS syndrome of polyneuropathy, organomegaly, endocri nopathy, M-protein, and skin changes is a rare multisystem disorder of obscure pathogenesis that is associated with microangiopathy, neovasc ularization, and accelerated vasopermeability. We examined the levels of the vascular endothelial growth factor/vascular permeability factor (VEGF) in the serum and cerebrospinal fluid (CSF) from 10 patients wi th this syndrome. Serum VEGF levels were about 15-30 times those in co ntrol subjects or patients with Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and other neurologi cal disorders. The CSF VEGF levels, however, were similar to those fou nd in GBS and CIDP, Elevated VEGF levels in the serum decreased in 7 p atients with Crow-Fukase syndrome after conventional therapy. The prin cipal isoform of VEGF in Crow-Fukase syndrome was VEGF165, Elevated VE GF was independent of M-protein, Our results suggest that the overprod uction of VEGF is important in the pathogenesis of this disorder. (C) 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1390-1397, 1998.