Primary meningeal lymphoma presenting solely with blindness: a report of an autopsy case

A 64-year-old woman was admitted because of progressive paraplegia. She had a history of unexplained blindness which had developed gradually 1 year earlier. The contrast-enhanced CT showed a small mass at the sella turcica. The funduscopy showed merely atrophic discs. The lumbar puncture revealed marked pleocytosis (cell count: 10.4 x 10(9)/l) and a diagnosis of meningeal lymphoma was made by the cytology of the cerebrospinal fluid. No lymphomatous lesions were detected outside of the central nervous system (CNS) and the patient received whole brain irradiation together with intrathecal chemotherapy. However, there was no improvement in vision or other neurological activities. Eventually the patient died of interstitial pneumonia 2 months after admission. The autopsy revealed a residual B-cell lymphoma existing only in the leptomeninges of medulla oblongata. The optic nerves were atrophic macroscopically and massive gliosis was seen microscopically. In addition, cytomegalovirus infection was observed in the lungs and adrenal glands but not in the CNS. This patient must have had a primary meningeal lymphoma. The mechanism of the visual loss of this patient is discussed.