Sinnecker Tim, Othman Jalal, Kühl Marc, Metz Imke, Niendorf Thoralf, Kunkel Annett, Paul Friedemann, Wuerfel Jens, Faiss Juergen
Department of Neurology, Asklepios Fachklinikum Teupitz, Teupitz, Germany; NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany; Department of Neurology, Universitätsspital Basel, Basel, Switzerland.
Department of Neurology, Asklepios Fachklinikum Teupitz, Teupitz, Germany.
Case Rep Neurol Med. 2016;2016:5876798. doi: 10.1155/2016/5876798. Epub 2016 Nov 22.
. Natalizumab- (NTZ-) associated progressive multifocal leukoencephalopathy (PML) is a severe and often disabling infectious central nervous system disease that can become evident in multiple sclerosis (MS) patients after NTZ discontinuation. Recently, novel diagnostic biomarkers for the assessment of PML risk in NTZ treated MS patients such as the anti-JC virus antibody index have been reported, and the clinical relevance of milky-way lesions detectable by MRI has been discussed. . We report a MS patient in whom PML was highly suspected solely based on MRI findings after switching from NTZ to fingolimod despite repeatedly negative (ultrasensitive) polymerase chain reaction (PCR) testing for JC virus DNA in cerebrospinal fluid. The PML diagnosis was histopathologically confirmed by brain biopsy. The occurrence of an immune reconstitution inflammatory syndrome (IRIS) during fingolimod therapy, elevated measures of JCV antibody indices, and the relevance of milky-way-like lesions detectable by (7 T) MRI are discussed.
那他珠单抗（NTZ）相关的进行性多灶性白质脑病（PML）是一种严重且常导致残疾的中枢神经系统感染性疾病，在NTZ停药后可在多发性硬化症（MS）患者中显现出来。最近，已报道了用于评估接受NTZ治疗的MS患者发生PML风险的新型诊断生物标志物，如抗JC病毒抗体指数，并且已讨论了MRI可检测到的“银河”样病变的临床相关性。我们报告了一名MS患者，尽管脑脊液中JC病毒DNA的（超灵敏）聚合酶链反应（PCR）检测多次呈阴性，但在从NTZ转换为芬戈莫德后，仅基于MRI表现就高度怀疑其患有PML。通过脑活检在组织病理学上证实了PML诊断。讨论了芬戈莫德治疗期间免疫重建炎症综合征（IRIS）的发生、JCV抗体指数的升高以及（7T）MRI可检测到的“银河”样病变的相关性。
