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免疫抑制的肾移植患者脑干进行性多灶性白质脑病的良好预后:早期检测和快速免疫重建的重要性

Good outcome of brain stem progressive multifocal leukoencephalopathy in an immunosuppressed renal transplant patient: Importance of early detection and rapid immune reconstitution.

作者信息

Sauer Roland, Gölitz Philipp, Jacobi Johannes, Schwab Stefan, Linker Ralf A, Lee De-Hyung

机构信息

Department of Neurology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.

Neuroradiology Section, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.

出版信息

J Neurol Sci. 2017 Apr 15;375:76-79. doi: 10.1016/j.jns.2017.01.046. Epub 2017 Jan 14.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic and often fatal disease of the CNS which may occur under immunosuppression in transplant patients. Brain stem PML is associated with a particularly bad prognosis. Here, we present a case of a renal transplant patient treated with mycophenolate mofetil (MMF) and tacrolimus who developed brain stem PML with limb ataxia, dysarthria and dysphagia. Diagnosis was established by typical MRI features and detection of JCV-DNA in the CSF. Immune reconstitution after stopping MMF and tacrolimus led to a complete and sustained remission of symptoms with improvement of the brain stem lesion over a follow-up over 20months. In summary, early detection of PML and consequent treatment may improve neurological outcomes even in brain stem disease with a notorious bad prognosis.

摘要

进行性多灶性白质脑病(PML)是一种罕见的中枢神经系统机会性疾病,常为致命性,可发生于移植患者免疫抑制状态下。脑干型PML预后尤其不良。在此,我们报告1例肾移植患者,该患者接受霉酚酸酯(MMF)和他克莫司治疗,发生了伴有肢体共济失调、构音障碍和吞咽困难的脑干型PML。通过典型的MRI特征及脑脊液中JCV-DNA的检测确诊。停用MMF和他克莫司后免疫重建使症状完全且持续缓解,脑干病变在20多个月的随访中得到改善。总之,即使是预后不佳的脑干疾病,早期发现PML并进行相应治疗也可能改善神经功能结局。

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