臨床神経学

<シンポジウム17―1>難治性末梢神経疾患の治療戦略

慢性炎症性脱髄性多発根ニューロパチーの治療反応性

飯島 正博

名古屋大学神経内科〔〒466―8550 名古屋市昭和区鶴舞町65〕

CIDP is autoimmune-associated peripheral neuropathy characterized by motor and sensory disturbances in each limb. While various phenotypes have been reported in CIDP, the essential pathogenesis is not elucidated yet. Clinicopathological study indicated axonal dysfunction (muscle atrophy and decreased compound muscular action potentials) is one of the most important factors in IVIg Non-responders. Furthermore, single nucleotide polymorphism (SNP) haplotype/diplotype analysis within a linkage disequilibrium block indicates transient axonal glycoprotein 1 (TAG-1), which controls proper distribution of potassium channels in juxtaparanode, is an important factor for IVIg responsiveness. Gene expression analysis of biopsied nerves supported the hypothesis that CIDP pathogenesis is involved in humoral and cellular immune system. With respect to IVIg responsiveness, expression profiles indicate whole CIDP patients need conventional immune-modulating therapies in somewhat, while we should re-consider how to use them. From aspects of gene expression results, Non-responders need not only conventional immune-modulating therapies but also other original modalities which could intervene the pathogenesis except Schwann/inflammatory cells while Responders with IVIg dependence should need stronger and longer immune-suppression.
Full Text of this Article in Japanese PDF (188K)

(臨床神経, 51:1012−1014, 2011)
key words:CIDP,経静脈免疫グロブリン(IVIg),一塩基多型(SNP),遺伝子発現,治療反応性

(受付日:2011年5月19日)