A 59-year-old man with IgA myeloma diagnosed 12 months previously was seen because of weakness, confusion, and intermittent fevers. He had dysarthria, dysphasia, normal fundi, and hepatosplenomegaly. Investigation showed circulating plasma cells, IgA paraproteinemia (6.9 g/dL), and hyperviscous plasma. His CSF contained plasma cells and a monoclonal protein band identical with that in the serum. Treatment with irradiation to the cranium and intrathecally administered thiotepa produced some improvement, but Pseudomonas septicemia developed from which he recovered only to die with Aspergillus pneumonia and Candida septicemia. Autopsy showed extensive infiltration of the meninges with abnormal plasma cells, which were shown by immunohistologic staining to be monoclonal IgA-producing myeloma cells. This case is compared with the five previously reported cases of meningeal involvement in patients with systemic myeloma; to our knowledge, this is the only case in which meningeal disease has been proved by immunologic techniques.
(Arch Intern Med 140:256-259, 1980)