Progressive multifocal leukoencephalopathy in a patient with Good's syndrome.
Progressive multifocal leukoencephalopathy in a patient with Good's syndrome.
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DOI：
10.1136/bcr-2013-009763
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发表时间：
2013-07-29
期刊：
影响因子：
0.9
通讯作者：
Herrman, Lars
中科院分区：
文献类型：
--
作者：
Sveinsson, Olafur;Matell, Henrik;Herrman, Lars
古德氏综合征（GS）是一种以胸腺瘤、低γ球蛋白血症和t细胞功能受损为特征的免疫缺陷。临床症状是由常见或机会性病原体引起的复发性或慢性感染和腹泻。脑炎罕见，多与巨细胞病毒有关。我们报告了一位65岁的女性，她在4个月的时间里出现了失明、运动缺陷和认知变化。脑MRI显示枕叶皮层下白质对称改变，首先被认为是后路可逆性脑病综合征。发现胸腺瘤并进行手术。患者无B细胞，免疫球蛋白低，CD4/CD8比值倒置。确诊为GS。脑脊液中发现100万个JC病毒拷贝/mL，重复MRI显示与进行性多灶性白质脑病（PML）相符。她的病造成了致命的后果。本病例是第二例报道的GS和PML之间的关联。
Good's syndrome (GS) is an immunodeficiency characterised by thymoma, hypogammaglobulinemia and impaired T-cell function. The clinical symptoms are recurrent or chronic infections from common or opportunistic pathogens and diarrhoea. Encephalitis is rare, mostly associated to cytomegalovirus. We present a 65-year-old woman who developed blindness, motor deficits and cognitive changes over a 4-month period. MRI of the brain showed symmetric subcortical white matter changes in the occipital lobes, first thought to correspond to posterior reversible encephalopathy syndrome. A thymoma was found and operated. The patient had no B cells, low immunoglobulins and an inverted CD4/CD8 ratio. GS was diagnosed. In the cerbrospinal fluid >1 million JC virus copies/mL were found and a repeat MRI now showed a picture compatible with progressive multifocal leucoencephalopathy (PML). Her disease had a fatal outcome. The present case is the second reported association between GS and PML.