Parkinsonism as a late presentation of lymphomatosis cerebri following high-dose chemotherapy with autologous stem cell transplantation for primary central nervous system lymphoma.

Abstract

Primary central nervous system lymphoma is an aggressive form of non-Hodgkin lymphoma arising in the eyes, meninges, spinal cord, or brain. Treatment of primary CNS lymphoma with a combination of high-dose chemotherapy and autologous stem cell transplantation has been shown to have high rates of remission which is frequently sustained for multiple years. Recurrence of primary CNS lymphoma generally presents with one or multiple contrast enhancing lesions on MRI. In rare cases, lymphoma cells may proliferate diffusely within the brain parenchyma without mass formation, a pattern termed lymphomatosis cerebri. Lymphomatosis cerebri presents a significant diagnostic challenge, and has not been reported to present with parkinsonism. Here, we present a case of initially mass forming, contrast-enhancing primary CNS lymphoma which remitted following chemotherapy and autologous stem cell transplantation, and recurred 7years post-transplant with symptoms of parkinsonism and a lack of typical lesions on imaging, with lymphomatosis cerebri confirmed at autopsy.