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首页> 外文期刊>The Internet Journal of Neurology >Progessive Multifocal Leukoencephalopathy Partial Weber's Syndrome and Global Cerebellar Syndrome in a HIV-Positive Patient: A Case Report
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Progessive Multifocal Leukoencephalopathy Partial Weber's Syndrome and Global Cerebellar Syndrome in a HIV-Positive Patient: A Case Report

机译：HIV阳性患者进行性多灶性白质脑病偏韦伯综合征和全脑小脑综合征：病例报告

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Introduction The polyomavirus JC (JCV) infects 85% of healthy individuals, and its reactivation in a limited number of inmuno-suppressed people causes progressive multifocal leukoencephalopathy (PML), a severe demyelinating disease of the central nervous system. Pasquier et al 1 hypothesized that JCV-specific cytotoxic T lymphocytes (CTLs) might control JCV replication in healthy individuals, blocking the evolution of PML and they determined that the frequency of JCV VP1 epitope-specific CTLs varied from less than 1/100,000 to 1/2,494 peripheral blood mononuclear cells. Therefore more individuals had JCV VP1-specific than cytomegalovirus-specific CTLs (8 of 11 subjects [73%] versus 2 of 10 subjects [20%], respectively). These results showed that a CD8+-T-cell response against JCV is commonly found in inmuno-competent people and suggest that these cells might protect against the development of PML.PML is described for the first time in 1958, at the Massachusetts General Hospital in Boston by Richardson and his colleagues2 in three patients who presented with multiple demyelinating lesions on the central nervous system (CNS), rapid fatal outcome and underlying chronic lymphocytic leukemia (CLL) and Hodgkin's disease. Last report about PML and CLL made by Rayid Abdulqawi et al3 is available on The Internet Journal of Neurology.The genus of polyomavirus belongs to the family of Papovavirus. There are 3 polyomaviruses, the JC, BK and SV40 viruses. They are all viruses with double-stranded DNA. In almost all cases of PML, the JC virus (JCV) is the aetiologic agent, although in isolated reports, BK and SV40 viruses have been implicated. Both JC and BK are initials of patients from whom the respective virus was first isolated. JCV is difficult to isolate, requiring long term cultures in glial cells. There are 4 genotypes numbered from 1 to 4, of which genotypes 1 and 2 are associated with the disease of PML.4 PML is a demyelinating disease of the central nervous system, resulting from infection of oligodendrocytes and astrocytes. The pathologic hallmark is the triad of discrete foci of demyelination, enlarged nuclei of oligodendrocytes, and bizarre-shaped astrocytes. This is consistent with the in vitro viral tropism to glial cells. Neurons are generally spared, but recently it was observed that the granule cell neuron in the cerebellum could also be infected in PML. In one case report, the granule cell neuron was infected exclusively.5,6 Weber's syndrome was first described in 1863 by the German physician Hermann Weber7 Weber's Syndrome occurs when a midbrain lesion gives rise to an ipsilateral third nerve palsy (resulting in an ipsilateral loss of pupillary light reflex and accommodation, ptosis, pupillary dilatation, and lateral deviation of the eye) plus a contralateral hemiplegia. Partial Weber's Syndrome has been described where pupillary reflexes remain intact through sparing of the Edinger-Westphal nucleus in the upper mid-brain8,9 However, to our knowledge this is the first case of a partial Weber's syndrome with sparing of the contralateral upper limb.Neurological disease associated with Human immunodeficiency virus -1 (HIV-1) infection is becoming more common as antiretroviral therapy improves patient survival10 Isolated cerebellar symptoms in HIV infection have been reported rarely11,12 and usually result from lesions due to neoplasm or opportunistic infections12. In H|IV dementia complex cerebellar syndrome has been reported as an early sign preceding cognitive decline in 30% of cases13 Primary cerebellar degenerations in the absence of and toxic, metabolic or nutritional abnormality or genetic predisposition is exceedingly rare11,12 Cerebellar syndrome as a direct effect of HIV infection has been postulated11. Animal studies have shown that rat cerebellar granular cells exposed to HIV coat protein gp 120, had a markedly increased rate of cell death14.The aim of this study is to report a HIV-positive patient with a partia
机译：引言多瘤病毒JC（JCV）感染了85％的健康个体，在少数受免疫抑制的人群中激活，会引起进行性多灶性白质脑病（PML），这是一种严重的中枢神经系统脱髓鞘疾病。 Pasquier等人1假设，JCV特异性细胞毒性T淋巴细胞（CTL）可能控制健康个体中的JCV复制，从而阻止了PML的进化，他们确定JCV VP1表位特异性CTL的频率从不到1 / 100,000到1 / 2,494个外周血单核细胞。因此，与巨细胞病毒特异性CTL相比，具有JCV VP1特异性的个体更多（分别为11名受试者中的8名[73％]与10名受试者中的2名[20％]）。这些结果表明，在有免疫能力的人群中普遍发现针对JCV的CD8 + -T细胞反应，提示这些细胞可能可以预防PML的发展.1958年，美国马萨诸塞州综合医院首次描述了PML。 Richardson和他的同事[2]在波士顿的3例患者中出现了中枢神经系统（CNS）的多脱髓鞘病变，快速致命的结果以及潜在的慢性淋巴细胞性白血病（CLL）和霍奇金病。 Rayid Abdulqawi等人[3]撰写的有关PML和CLL的最新报告可在Internet Journal of Neurology上找到。多瘤病毒属属于Papovavirus家族。有3种多瘤病毒，即JC，BK和SV40病毒。它们都是带有双链DNA的病毒。在几乎所有的PML病例中，JC病毒（JCV）是病原体，尽管在单独的报道中也涉及BK和SV40病毒。 JC和BK都是最初从中分离出相应病毒的患者的姓名缩写。 JCV很难分离，需要在神经胶质细胞中长期培养。有4种基因型，编号为1-4，其中基因型1和2与PML疾病有关。4PML是中枢神经系统的脱髓鞘疾病，由少突胶质细胞和星形胶质细胞的感染引起。病理特征是脱髓鞘病灶离散，少突胶质细胞核增大以及奇形星形胶质细胞的三联征。这与体外病毒对神经胶质细胞的向性一致。神经元通常可以幸免，但是最近发现小脑中的颗粒细胞神经元也可能被PML感染。在一个病例报告中，颗粒细胞神经元仅被感染。5,6韦伯综合症于1863年由德国医师赫尔曼·韦伯（Hermann Weber）首次描述。7韦伯综合症发生于中脑病变引起同侧第三神经麻痹（导致同侧损失）瞳孔的光反射和适应，上睑下垂，瞳孔扩张和眼睛的侧向偏斜）以及对侧偏瘫。已经描述了部分韦伯综合症，其中通过保留上部中脑的爱丁格-威斯特法尔核保留了完整的瞳孔反射8，9。然而，据我们所知，这是部分韦伯综合症伴有对侧上肢保留的首例病例。随着抗逆转录病毒疗法改善患者生存率，与人类免疫缺陷病毒-1（HIV-1）感染相关的神经系统疾病正变得越来越普遍10。据报道，很少有HIV感染中出现小脑症状[11,12]，通常是由于肿瘤或机会性感染引起的病变[12]。据报道，在H | IV型痴呆中，小脑综合症是在30％的患者出现认知功能下降之前的早期迹象13。在没有毒性，代谢或营养异常或遗传易感性的情况下，原发性小脑变性极为罕见11,12艾滋病毒感染的影响已被假定11。动物研究表明，暴露于HIV外壳蛋白gp 120的大鼠小脑颗粒细胞的细胞死亡率显着增加14.本研究的目的是报告一名HIV阳性的患者

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