King Abdulaziz Medical City.
Department of Neuroscience, King Faisal Specialist Hospital & Research Centre.
Neurologist. 2022 Sep 1;27(5):271-275. doi: 10.1097/NRL.0000000000000398.
Progressive multifocal leukoencephalopathy (PML) is a rare infection of the central nervous system due to the reactivation of the John Cunningham polyomavirus. It is commonly a progressive fatal disease with worldwide distribution.
We describe a rare case of PML, which was misdiagnosed as neuropsychiatric systemic lupus erythematosus (SLE) with a catastrophic outcome due to delay in diagnosis with superadded cyclophosphamide therapy.
There are several lessons taught from our case. Firstly, in patients with autoimmune disorders who are strongly immunosuppressed, the new onset of cognitive impairment and seizures should alert the treating physician to look carefully for PML. Secondly, in cases of SLE where the diagnosis of the cause of cognitive impairment and seizures is not clear, we suggest that immunosuppression should not be intensified until PML has clearly been ruled out. Lastly, multidisciplinary care in patients with suspected neuropsychiatric SLE including a neurologist, an infectious diseases consultant, a neuroradiologist, and a rheumatologist is needed.
进行性多灶性白质脑病（PML）是一种罕见的中枢神经系统感染，由约翰·坎宁安多瘤病毒（John Cunningham polyomavirus）重新激活引起。它通常是一种进行性致命疾病，分布于世界各地。
我们描述了一例罕见的 PML 病例，由于诊断延误和叠加环磷酰胺治疗，该病例最初误诊为神经精神性系统性红斑狼疮（SLE），导致灾难性后果。
从我们的病例中可以吸取几个教训。首先，在自身免疫性疾病患者中，如果免疫抑制作用较强，新发认知障碍和癫痫发作应引起治疗医生警惕，仔细寻找 PML 的可能性。其次，对于 SLE 患者，如果认知障碍和癫痫发作的病因诊断不明确，我们建议在明确排除 PML 之前，不应加强免疫抑制治疗。最后，疑似神经精神性 SLE 患者需要多学科的治疗，包括神经科医生、传染病顾问、神经放射科医生和风湿病学家。
