慢性發炎性脫髓鞘神經病變與抗體

10.6666/ClinMed.2017.80.5.119

林恭平

慢性發炎性脫髓鞘神經病變(chronic inflammatory demyelinating polyradiculoneuropathy, CIDP) ； anti-contactin 1抗體 ； anti-neurofascin 155抗體

臨床醫學月刊

80卷5期（2017 / 11 / 24）

654 - 657

繁體中文

慢性發炎性脫髓鞘神經病變是免疫仲介多種抗體的神經疾病。臨床表現為肢體的近及遠端無力，主要是運動肌力的症狀，感覺症狀較少。雖然有明確的診斷標準，但是臨床表現上仍是多樣不一致。疾病背後的抗原抗體的免疫機致，仍需進一步的了解。至今知道的抗體的神經病變有anti-GM1抗體-多發局部運動神經病變合併傳導阻隔(multifocal motor neuropathy with conduction block), anti-myelin associated glycoprotein抗體-anti-MAG神經病變，未知抗體的有Lewis-Sumner症候群等。近三年來，又知道兩個-anti-contactin 1抗體及anti-neurofascin 155抗體。anti-contactin 1抗體造成的神經病變，病程進展快，神經軸索退化早，病徵嚴重，對免疫球蛋白反應差。anti-neurofascin 155抗體，發病年紀輕，早出現共濟失調(ataxia)，遠端肢體神經傳導速度慢，中樞神經系統有時也受影響。上述兩種抗體的神經病變，抗體主要是IgG4，對Rituximab的治療療效佳。

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