[Electrophysiologic aspects of Crow-Fukase (POEMS) syndrome--significance in early diagnosis and insights into the pathophysiology].
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement. The presence of polyneuropathy is mandatory for the diagnosis of POEMS syndrome, and progressive neuropathy results in the deterioration of patients' quality of life. Although the pathophysiology of nerve damage has not yet been elucidated, nerve conduction abnormalities exhibit characteristic, patterns that can be summarized by a number of features including (1) slow nerve conduction diffusely distributed in the intermediate nerve segment, (2) relatively preserved nerve conduction near the distal nerve terminals, (3) prominent axonal loss in distal lower extremity nerves, and (4) no conduction blocks. These features are useful in differential diagnosis of POEMS syndrome from chronic inflammatory demyelinating polyneuropathy (CIDP) and neuropathy associated with anti-myelin-associated glycoprotein antibody. The pathogenesis of POEMS syndrome is not well understood; however overproduction of vascular endothelial growth factor (VEGF), probably secreted by plasmacytoma, may be responsible for most of the characteristic symptoms, including neuropathy. The patterns of nerve conduction abnormalities suggest that demyelination and axonal degeneration are caused by some serum neurotoxic substances in serum that cannot access the nerve parenchyma under physiological conditions. Elevated serum VEGF level would result in increased permeability and breakdown of the blood-nerve barrier. In addition, endoneurial or perineurial vascular proliferation with altered hematocoaglabilty could partly play a role in the pathogenesis of neuropathy in POEMS syndrome.
Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.
Sonoko
S
Misawa
D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D000068258:Bevacizumab; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D004594:Electrophysiology; D006801:Humans; D009389:Neovascularization, Pathologic; D009431:Neural Conduction; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D036102:Peripheral Blood Stem Cell Transplantation; D010525:Peripheral Nerves / Q000098:blood supply / Q000503:physiopathology; D010954:Plasmacytoma / Q000378:metabolism; D013792:Thalidomide / Q000627:therapeutic use; D042461:Vascular Endothelial Growth Factor A / Q000097:blood / Q000378:metabolism
D020533:Angiogenesis Inhibitors; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab; D013792:Thalidomide