Bialasiewicz Seweryn, Hart Gareth, Oliver Kimberly, Agnihotri Shruti P, Koralnik Igor J, Viscidi Raphael, Nissen Michael D, Sloots Theo P, Burke Michael T, Isbel Nicole M, Burke John
1 Queensland Paediatric Infectious Diseases Laboratory, The University of Queensland, Brisbane, Australia. 2 Department of Renal Medicine, Princess Alexandra Hospital, The University of Queensland, Brisbane, Australia. 3 Department of Anatomical Pathology, Princess Alexandra Hospital, Brisbane, Australia. 4 Division of Neuro Immunology and Center for Virology and Vaccine Research, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA. 5 Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
Transplantation. 2017 Jun;101(6):1461-1467. doi: 10.1097/TP.0000000000001275.
A number of cerebral manifestations are associated with JC polyomavirus (JCPyV) which are diagnosed by detection of JCPyV in cerebrospinal fluid (CSF), often with the support of cerebral imaging. Here we present an unusual case of a kidney transplant patient presenting with progressive neurological deterioration attributed to JCPyV encephalopathy.
Quantitative polymerase chain reaction JCPyV was used prospectively and retrospectively to track the viral load within the patient blood, urine, CSF, and kidney sections. A JCPyV VP1 enzyme-linked immunosorbent assay was used to measure patient and donor antibody titers. Immunohistochemical staining was used to identify active JCPyV infection within the kidney allograft.
JC polyomavirus was detected in the CSF at the time of presentation. JC polyomavirus was not detected in pretransplant serum, however viral loads increased with time, peaking during the height of the neurological symptoms (1.5E copies/mL). No parenchymal brain lesions were evident on imaging, but transient cerebral venous sinus thrombosis was present. Progressive decline in neurological function necessitated immunotherapy cessation and allograft removal, which led to decreasing serum viral loads and resolution of neurological symptoms. JC polyomavirus was detected within the graft's collecting duct cells using quantitative polymerase chain reaction and immunohistochemical staining. The patient was JCPyV naive pretransplant, but showed high antibody titers during the neurological symptoms, with the IgM decrease paralleling the viral load after graft removal.
We report a case of atypical JCPyV encephalopathy associated with cerebral venous sinus thrombosis and disseminated primary JCPyV infection originating from the kidney allograft. Clinical improvement followed removal of the allograft and cessation of immunosuppression.
多种脑部表现与JC多瘤病毒（JCPyV）相关，这些表现通过在脑脊液（CSF）中检测JCPyV来诊断，通常还需要脑部影像学检查的支持。在此，我们报告一例肾移植患者出现因JCPyV脑病导致的进行性神经功能恶化的不寻常病例。
前瞻性和回顾性地使用定量聚合酶链反应检测JCPyV，以追踪患者血液、尿液、脑脊液和肾组织切片中的病毒载量。使用JCPyV VP1酶联免疫吸附测定法测量患者和供体的抗体滴度。免疫组织化学染色用于鉴定肾移植受者体内的活跃JCPyV感染。
就诊时在脑脊液中检测到JC多瘤病毒。移植前血清中未检测到JC多瘤病毒，但病毒载量随时间增加，在神经症状高峰期达到峰值（1.5E拷贝/毫升）。影像学检查未发现实质性脑病变，但存在短暂性脑静脉窦血栓形成。神经功能的进行性下降使免疫治疗不得不停止并切除移植肾，这导致血清病毒载量下降和神经症状缓解。使用定量聚合酶链反应和免疫组织化学染色在移植肾的集合管细胞中检测到JC多瘤病毒。患者移植前未感染JCPyV，但在出现神经症状时抗体滴度较高，IgM水平在移植肾切除后随病毒载量下降。
我们报告一例非典型JCPyV脑病病例，该病例与脑静脉窦血栓形成以及源自肾移植的播散性原发性JCPyV感染相关。切除移植肾并停止免疫抑制后临床症状改善。
