A case of neurosarcoidosis monitored by computerized tomography

A 21-year-old man complaining of impaired visual acuity was admitted to the hospital. Physical examinations showed asymptomatic bilateral hilar lymphadenopathy and cardiomyopathy. Neurological 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 disorder developed during a month though he was given steroids. The more deteriorated the clinical course, the larger the suprasellar mass with expanding hydrocephalus in repeated computerized tomographies. After the ventriculo-peritoneal shunt operation, consciousness improved. Diabetes insipidus also improved after Diabenese administration. 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 the admission, whereas blindness never recovered. He died of developed status epilepticus eleven months after the admission. The authors reviewed neuroradiological findings of neurocarcoidosis based on pathological findings in the literature, and emphasized that computerized tomography was the most useful for diagnosis and treatment of neurosarcoidosis. (author)