脳サルコイドーシス

Neurosarcoidosis (Brain)

神経サルコイドーシスの中で，特に脳病変を中心にまとめた。脳神経障害，頭痛，意識障害，痙攣など複数の神経症候を呈することが多い。多岐にわたる疾患が鑑別すべき疾患に挙がり，結核，真菌感染症は適切に除外されなければならない。したがって，脳病変からの病理診断が極めて重要であり，積極的に考慮されるべきである。治療としてプレドニゾロンの投与が一般的であり，加えて腫瘍壊死因子αに対するインフリキシマブの有用性が示されている。

Abstract

This review focuses on neurosarcoidosis with pathological alterations in the brain. Patients with neurosarcoidosis develop several symptoms such as cranial nerve palsies, headache, consciousness disturbance, and seizures. It may be difficult to reach a definite diagnosis and carry out differential diagnosis. Sarcoidosis is characterized by noncaseating granuloma that may be seen at the dura, leptomeninges, brain (including the cranial nerves), spinal cord, and peripheral nerves. Epithelioid histocytes and multinucleated giant cells, including Langhans cells, are characteristic microscopic features. In some instances, an asteroid body and a Schaumann body may be seen. In particular, infectious diseases including tuberculosis and mycosis must be ruled out. Therefore, the pathologic diagnosis of brain lesions is the gold standard for diagnosing neurosarcoidosis. In addition to prednisolone therapy, infliximab, a monoclonal antibody against tumor necrosis factor-α, has received much attention for treating neurosarcoidosis.