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Ⅰ.はじめに
Sarcoidosisは,原因不明のmultisystem granuloma—tous disorderであり9)主として肺,眼,皮膚に好発し20),中枢神経を侵すことは稀である4,14)。われわれはsarcoidosisの経過中,視力障害、尿崩症,意識障害を伴ない,痙攣重積で死亡した症例にCT scanにて経時的に観察し,脳生検で組織学的にsarcoid病変を確認した。
NeurosarcoidosisのCT像についての報告は少なく,わずかにKendall et al10)とBahr et al2)の論文がみられるのみである。文献的に中枢神経sarcojd病変の病理学的特徴と神経放射線学的所見を総括し,とくにCT像による診断および治療方法について考察した。
A 21-year-old man complaining of impaired visual acuity was admitted to the hospital. Physical ex-aminations showed asymptomatic bilateral hilar lymphadenopathy and cardiomyopathy. Neuro-logical findings disclosed left blindness and right temporal hemianopsia. Computerized tomography, pneumoencephalography and carotid angiography revealed a suprasellar mass.
After the admission, the following symptoms deteriorated rapidly: diabetes insipidus, anterior pituitary dysfunction, visual loss of the right eye and hepatomegaly, subsequently consciousness dis-order developed during a month though he was given steroids. The more deteriorated the clinical course, the larger the suprasellar mass with ex-panding hydrocephalus in repeated computerized tomographies. After the ventriculo-peritoneal shunt operation, consciousness improved. Diabetes insipidus also improved after Diabenese adminis-tration.
On the operation, adhesive arachnoiditis over all the frontotemporal cortex and swollen purplishly red optic chiasm were exposed. Microscopically the specimen from the optic chiasm evidenced a sarcoid granuloma which composed of epitheroid cells, lymphocytes and multi-nucleated giant cells with numerous hemosiderin droplets. The specimen from the surface of the left frontal lobe showed thick fibrosis in the subarachnoid space.
By steroids therapy, diabetes insipidus and hepatomegaly disappeared five months after theadmission, whereas blindness never recovered. He died of developed status epilepticus eleven months after the admission. The authers reviewed neuro-radiological findings of neurocarcoidosis based on pathological findings in the literature, and empha-sized that computerized tomography was the most useful for diagnosis and treatment of neuro-sarcoidosis.
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