中枢神経サルコイドーシス

Sarcoidosis in Central Nervous System

サルコイドーシスの中でも中枢神経サルコイドーシスは非常に稀である。しかし，脳腫瘍や髄膜（脳）炎，脊髄症などで鑑別診断の1つに挙げられる機会は多く，日常診療での重要度は高い。難治例が多く，永続的な後遺症をきたしやすいが，本症に対するインフリキシマブの高い治療効果が近年多く報告され，予後改善が期待される。

Abstract

Sarcoidosis with lesions in the central nervous system is a heterogeneous condition with diverse clinical presentations and various degrees of neurological disability. Although extremely rare among sarcoidosis cases, it is of relevance to routine clinical practice because it may be a differential diagnosis in brain tumors, meningitis, and myelopathy of unknown origin. Encephalic and myelopathic lesions of sarcoidosis are often resistant or only partially responsive to immunotherapies, including high-dose corticosteroids and immunosuppressants. This may lead to residual neurological disability in a certain number of cases. Several retrospective findings have recently shown superior therapeutic effects with infliximab. However, high-level evidence is warranted to confirm its efficacy to treat neurosarcoidosis.