Neurology Unit, Department of Pathophysiology & Transplantation, 'Dino Ferrari' Center, University of Milan, Fondazione Cà Granda, IRCCS Ospedale Maggiore Policlinico, Milan, Italy.
Immunotherapy. 2014;6(1):23-8. doi: 10.2217/imt.13.155.
Progressive multifocal leukoencephalopathy (PML) is a rare and severe complication of natalizumab therapy in patients with multiple sclerosis and it may be accompanied by immune reconstitution inflammatory syndrome (IRIS). Here, we describe a case of abnormally severe IRIS, which occurred 2 months after natalizumab-associated PML in a 38-year-old woman affected by multiple sclerosis. The patient was John Cunningham virus-positive and was treated for 21 months when she developed PML. The subsequent IRIS diffusely afflicted the brain, producing edema and signs of intracranial hypertension, with a clinically severe form compromising the state of consciousness, requiring intensive care and high-dosage steroid treatment. Nevertheless, she survived and partially recovered. There is still difficulty in differentiating PML progression from IRIS onset and there is not a clear description in the literature about different clinical forms of IRIS, prognostic factors and guidelines to properly treat this complication in order to reduce the residual disability of the patient surviving this treatment complication.
进行性多灶性白质脑病（PML）是多发性硬化症患者接受那他珠单抗治疗的一种罕见且严重的并发症，且可能伴有免疫重建炎症综合征（IRIS）。在此，我们描述了一例异常严重的 IRIS 病例，该病例发生在一位 38 岁的多发性硬化症女性患者接受那他珠单抗相关 PML 治疗后 2 个月。该患者为 JCV 阳性，在发生 PML 时已接受了 21 个月的治疗。随后的 IRIS 弥漫性累及大脑，产生水肿和颅内压升高的迹象，临床表现严重，意识状态受损，需要重症监护和大剂量类固醇治疗。尽管如此，她还是幸存了下来，并部分恢复了健康。目前仍然难以区分 PML 进展和 IRIS 发病，而且文献中对于不同形式的 IRIS、预后因素和适当治疗这种并发症的指南也没有明确的描述，以降低患者在这种治疗并发症后的残疾程度。
