Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature
Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature
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DOI：
10.1007/s00415-014-7530-5
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发表时间：
2015-01-01
影响因子：
6
通讯作者：
Wattjes, Mike P.
中科院分区：
文献类型：
--
作者：
Wijburg, Martijn T.;van Oosten, Bob W.;Wattjes, Mike P.
颗粒细胞神经病变（GCN）是免疫功能低下患者中一种罕见的JC病毒（JCV）相关疾病，以小脑颗粒细胞层溶解性感染为特征。为了实现早期诊断和干预，我们确定了GCN的特征，并描述了疾病异质性的可能方面。我们报告了两例新的艾滋病毒感染患者的GCN，我们回顾性地评估了临床和放射学资料。此外，我们对所有已发表的GCN病例的临床、放射学和组织病理学结果进行了文献检索和回顾。包括本文报道的2例新病例在内，本研究共纳入18例GCN病例。艾滋病毒感染是最常见的基础疾病，在12例病例中出现，其次是单克隆抗体治疗，在3例病例中出现。除2例外，其余均见小脑萎缩。在12例患者中，观察到小脑和脑干白质变化的不均匀分布模式。GCN的影像学表现非常不均匀；表现为小脑萎缩，以及白质病变，特别是相邻的幕下白质。这表明GCN与其他jcv相关疾病重叠，如进行性多灶性白质脑病。
Granule cell neuronopathy (GCN) is a rare JC virus (JCV)-related disease in immunocompromised patients, characterized by lytic infection of the cerebellar granule cell layer. To enable early diagnosis and intervention, we identify features of GCN and describe possible aspects of disease heterogeneity. We report on two new cases of GCN in HIV-infected patients of whom we retrospectively assessed clinical and radiologic data. In addition, we carried out a literature search and review of clinical, radiologic and histopathologic findings of all published GCN cases. Including the two new cases reported here, a total of 18 GCN cases were included in this study. HIV infection, present in 12 of the cases, was the most common underlying condition, followed by monoclonal antibody treatment which was present in three cases. Cerebellar atrophy was detected in all except two cases. In 12 patients a heterogeneous distribution pattern of white matter changes in the cerebellum and brainstem was observed. Imaging findings in GCN are remarkably heterogeneous; exhibiting cerebellar atrophy, as well as white matter pathology, particularly in the adjacent infratentorial white matter. This suggests an overlap of GCN with other JCV-related diseases, such as progressive multifocal leukoencephalopathy.