Mayo Clinic Alix School of Medicine, 13400 E. Shea Blvd, Scottsdale, AZ, 85259, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 1st St. SW, Rochester, MN, 55905, USA.
J Neurol. 2020 Aug;267(8):2239-2244. doi: 10.1007/s00415-020-09819-y. Epub 2020 Apr 15.
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 7 years post-transplant with symptoms of parkinsonism and a lack of typical lesions on imaging, with lymphomatosis cerebri confirmed at autopsy.
原发性中枢神经系统淋巴瘤是一种侵袭性非霍奇金淋巴瘤，发生于眼、脑膜、脊髓或脑。用大剂量化疗和自体干细胞移植联合治疗原发性中枢神经系统淋巴瘤已被证明有很高的缓解率，且缓解通常持续多年。原发性中枢神经系统淋巴瘤的复发通常表现为 MRI 上的一个或多个对比增强病变。在极少数情况下，淋巴瘤细胞可能在脑实质内弥漫性增殖而没有肿块形成，这种模式称为脑淋巴瘤病。脑淋巴瘤病提出了一个重大的诊断挑战，尚未有报道称其表现为帕金森病。在这里，我们报告了一例最初为肿块形成、对比增强的原发性中枢神经系统淋巴瘤，经化疗和自体干细胞移植后缓解，7 年后移植后复发，出现帕金森病症状，影像学上无典型病变，尸检证实为脑淋巴瘤病。
