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特集 傍腫瘍性神経筋疾患update
文献概要
はじめに
傍腫瘍性神経症候群（paraneoplastic neurological syndromes：PNS）は，腫瘍の転移や直接浸潤，あるいは化学療法や放射線療法による副作用・代謝障害・日和見感染によらず，腫瘍の遠隔効果を原因として出現するさまざまな神経症候群の総称である。
1980年代から現在に至るまで，腫瘍に発現するさまざまな神経由来抗原を標的とした抗神経抗体（onconeural antibodies）が多数同定され，腫瘍と神経系を同時に標的とする抗神経抗体をはじめとした“なんらかの免疫因子”がPNSの発症に関与していると推測されている。PNSにおける抗神経抗体は，悪性腫瘍の存在を示唆する重要な情報である一方，PNS特有の神経症状を有するが，既存の抗神経抗体を持たない症例も存在することが知られている。また，PNSでは腫瘍免疫が存在するために，神経症状は著しく重度であるにもかかわらず，腫瘍自体が小さいことが多く，先行する神経症状が腫瘍発見の第1の足がかりになる場合もある。
そこで本稿ではPNSの系統的診断アプローチと治療マネージメントについて，ヨーロッパ神経学会（European Federation of Neurological Societies：EFNS）とParaneoplastic Neurological Syndrome Euronetwork（PNS Euronetwork）の合同task forceにより新たに提案された診断ガイドライン1，2）（以下，EFNS-PNS Euronetwork診断ガイドライン）を中心に，最新の知見を加え，概説する。
傍腫瘍性神経症候群（paraneoplastic neurological syndromes：PNS）は，腫瘍の転移や直接浸潤，あるいは化学療法や放射線療法による副作用・代謝障害・日和見感染によらず，腫瘍の遠隔効果を原因として出現するさまざまな神経症候群の総称である。
1980年代から現在に至るまで，腫瘍に発現するさまざまな神経由来抗原を標的とした抗神経抗体（onconeural antibodies）が多数同定され，腫瘍と神経系を同時に標的とする抗神経抗体をはじめとした“なんらかの免疫因子”がPNSの発症に関与していると推測されている。PNSにおける抗神経抗体は，悪性腫瘍の存在を示唆する重要な情報である一方，PNS特有の神経症状を有するが，既存の抗神経抗体を持たない症例も存在することが知られている。また，PNSでは腫瘍免疫が存在するために，神経症状は著しく重度であるにもかかわらず，腫瘍自体が小さいことが多く，先行する神経症状が腫瘍発見の第1の足がかりになる場合もある。
そこで本稿ではPNSの系統的診断アプローチと治療マネージメントについて，ヨーロッパ神経学会（European Federation of Neurological Societies：EFNS）とParaneoplastic Neurological Syndrome Euronetwork（PNS Euronetwork）の合同task forceにより新たに提案された診断ガイドライン1，2）（以下，EFNS-PNS Euronetwork診断ガイドライン）を中心に，最新の知見を加え，概説する。
参考文献
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