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      [Crow-Fukase syndrome and VEGF].

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          Abstract

          Crow-Fukase syndrome is diagnosed based on the presence of chronic sensori-motor polyneuropathy along with other characteristic generalized symptoms denoted by the acronym of POEMS which stands for polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. In this syndrome, the serum levels of the vascular endothelial growth factor (VEGF) are abnormally elevated, and this is a predictive factor for its diagnosis. Although the causes of CFS/POEMS remain unknown, VEGF is evidently correlated with its pathogenesis. Human glioblastoma cells are known to express VEGF. In models of CFS/POEMS, mice that are peritoneally transplanted with human glioblastomas exhibit high serum levels of VEGF, prominent edema with increased circulation volume, and pathological findings in the liver, spleen, and kidney. VEGF that is highly concentrated in platelets may be released in massive amounts due to coagulation in the peripheral tissue and may thus exert its maximal physiological effects and produce the abovementioned diffuse pathological findings. The correlation between polyneuropathy and elevated VEGF remains unclear. However, VEGF may affect the blood-nerve barrier by increased microvascular hyperpermeability, upregulated cytokines such as matrix metalloproteases may induce blood-nerve barrier breakdown and demyelination of the peripheral nerve. Furthermore, microangiopathy due to proliferative endothelial cells and hypercoagulated occlusion also affect axonal damage. Novel strategies that have recently been proposed for the management of this disease include high-dose chemotherapy combined with autologous peripheral blood stem cell transplantation (PBSCT) and molecular-targeted therapy against plasma cells and VEGF. Notably, PBSCT exerts a dramatic effect on polyneuropathy; such an effect has rarely been achieved by the previously described modalities of low-dose melphallan and steroid therapy. PBSCT is observed to induce a rapid and persistent decrease in the serum VEGF levels. In conclusion, VEGF is not only the primary molecule involved in the pathogenesis of CSF, but also an important marker for both the diagnosis and treatment of this disease.

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          Author and article information

          Journal
          Brain Nerve
          Brain and nerve = Shinkei kenkyu no shinpo
          1881-6096
          1881-6096
          Jun 2008
          : 60
          : 6
          Affiliations
          [1 ] Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medicine, 8-35-1 Sakuragaoka, Kagoshima 890-8544, Japan.
          Article
          18567356
          eaba5120-1795-49ee-9e2f-98b933b466c9
          History

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