Guduru Mounika, Bendi Venkata Sunil, Bebawy Mariana S, Bande Dinesh, Matta Abhishek
Radiology, Creighton University Medical Center, Omaha, USA.
Neurology, Creighton University Medical Center, Omaha, USA.
Cureus. 2020 Oct 10;12(10):e10888. doi: 10.7759/cureus.10888.
Progressive multifocal leukoencephalopathy (PML) is a rare fatal central nervous system disorder characterized by infection-induced demyelination of white matter due to the opportunistic reactivation of John Cunningham virus in an immunocompromised patient. PML is associated with many immune-mediated diseases, lymphoproliferative conditions, and immunosuppressive agents. In this case report, we present a 79-year-old female patient diagnosed with rheumatoid arthritis who developed posterior fossa PML while on rituximab. She presented with subacute cerebellar ataxia, dysarthria, and nystagmus, and her brain MRI showed right pontine and pontocerebellar lesion with diffusion restriction and heterogenous enhancement highly characteristic of PML. Though many cases of PML with rituximab were reported in the literature, our case describes a rare type of PML affecting the posterior fossa in an HIV-negative patient on rituximab.
进行性多灶性白质脑病(PML)是一种罕见的致命性中枢神经系统疾病,其特征是在免疫功能低下的患者中,因约翰·坎宁安病毒机会性再激活导致感染引起白质脱髓鞘。PML与许多免疫介导疾病、淋巴增殖性疾病和免疫抑制剂有关。在本病例报告中,我们介绍了一名79岁的女性患者,她被诊断为类风湿关节炎,在使用利妥昔单抗治疗期间发生了后颅窝PML。她表现为亚急性小脑共济失调、构音障碍和眼球震颤,脑部MRI显示右侧脑桥和脑桥小脑病变,具有扩散受限和不均匀强化,这是PML的高度特征性表现。尽管文献中报道了许多使用利妥昔单抗后发生PML的病例,但我们的病例描述了一种罕见类型的PML,发生在一名使用利妥昔单抗的HIV阴性患者的后颅窝。