艾滋病所致的进行性多灶性白质脑病(附1例报告)

目的　提高对艾滋病所致的进行性多灶性白质脑病的认识以及引起临床重视。方法　报告 1例证实的艾滋病所致进行性多灶性白质脑病患者的临床表现、实验室及影像学特点。结果　患者神经系统表现为高级神经活动障碍 ,双侧锥体束损害 ,左侧偏身感觉减退。血清抗HIV抗体阳性。脑脊液检查正常。MR双侧额叶、右侧颞叶深部白质内均见斑片状、指状信号影 ,T1W低信号 ,T2 W高信号 ,病灶无强化。结论　艾滋病可引致进行性多灶性白质脑病 ,临床易误诊漏诊 ,应及时行有关检查及时确诊     

拟诊人类免疫缺陷病毒相关进行性多灶性白质脑病四例分析

目的总结人类免疫缺陷病毒(HIV)相关进行性多灶性白质脑病的临床表现、实验室和影像学特点、治疗及预后。方法回顾分析4例拟诊HIV相关进行性多灶性白质脑病患者的临床资料。结果 4例患者临床主要表现为逐渐加重的神经功能缺损症状、肢体无力,1例伴言语困难、1例伴头晕,病程中症状逐渐加重。头部MRI均表现为脱髓鞘改变,T1WI呈低信号,T2WI和FLAIR成像呈高信号,DWI呈中心低信号、周围高信号,增强扫描病灶未见明显强化。2例行高效抗逆转录病毒疗法,最长生存期达20个月;未行规范抗HIV治疗者远期预后不佳。结论 HIV相关进行性多灶性白质脑病临床以进行性加重的神经功能缺损症状为主,影像学表现典型,早期并及时予高效抗逆转录病毒疗法可以部分恢复。     

神经系统副肿瘤综合征34例临床特点分析

目的:分析34例神经系统副肿瘤综合征患者的临床特点。方法:对1990年至2005年收治的34例神经系统副肿瘤综合征患者的临床资料进行回顾性分析。结果:副肿瘤综合征的临床类型有周围神经病15例、Lambert-Eaton肌无力综合征5例、多发性肌炎和皮肌炎3例、进行性小脑变性2例、运动神经元病3例、进行性多灶性白质脑病1例、亚急性坏死性脊髓病1例、脑干脑炎2例、边缘系统脑炎2例。结论:神经系统副肿瘤综合征临床表现形式多样,容易误诊,临床早期确诊对于隐匿肿瘤的发现和治疗非常重要。     

艾滋病与神经系统机会性感染

目的探讨艾滋病(AIDS)合并神经系统(NS)机会性感染的临床特征。方法报道6例人类免疫缺陷病毒(HIV)抗体阳性患并发NS机会性感染的临床资料，结合献复习研究AIDS与NS机会性感染的相关性。结果6例分别表现为进行性多灶性白质脑病(PML)、带状疱疹、结核性脑膜炎(2例)、结核性脑、脊髓膜炎和脑脓肿，考虑AIDS所致NS机会性感染。6例患中5例预后不良。结论HIV具有亲淋巴性的特点，可造成机体严重的细胞免疫缺陷和体液免疫缺陷，较易并发NS机会性感染，且多数预后不良。对NS感染久治无效时，应考虑AIDS可能。     

获得性免疫缺陷综合征神经系统损害临床分析

目的提高对获得性免疫缺陷综合征（艾滋病）合并神经系统损害临床特点的认识,以减少漏诊。方法对28例人类获得性免疫缺陷病毒（HIV）感染和（或）艾滋病患者中的12例合并神经系统损害患者的临床资料和机会感染性疾病情况进行回顾,并结合文献分析总结。结果12例患者分别诊断为艾滋病脑病（5例）、慢性脑膜炎（3例）、周围神经病（表现为四肢远端对称性多发性神经病和获得性脱髓鞘性神经病各1例）、脑梗死（1例）和肌肉病（1例）。艾滋病合并神经系统损害的发病率约为42．86％（t2／28）。至少合并1～2种以上机会性感染,以真菌最为多见,发病率为83．33％（10／12）;隐球菌性脑膜炎发病率为25％（7128）。结论HIV感染可累及神经系统多个部位及肌肉。艾滋病期患者常合并多系统混合感染,以真菌最为常见;临床以消瘦、间歇性发热、头痛、咳嗽、认知功能减退、脑膜刺激征阳性等症状与体征为主,表现复杂多样,容易误诊或漏诊。诊断与鉴别诊断需依靠脑脊液检查、肺部CT、头部CT和（或）MRI等辅助检查结果,临床工作中应注意筛查,尽早明确诊断。     

艾滋病神经系统损害的脑部及脊髓影像学改变探讨

目的探讨艾滋病神经系统损害的脑部及脊髓影像学改变。方法对25例艾滋病伴神经系统损害患者包括脑部和脊髓损害进行影像学(CT或MRI)检查,分析其影像学特点。结果 25例有神经系统损害的艾滋病中表现脑部损害较为多见,影像学表现为3例颅内多发占位性病变;4例表现为进行性多灶白质脑病;11例隐球菌性脑病中4例表现为基底节多发小点片状低密度灶,2例有脑膜强化表现;其他无异常表现;4例表现为脑梗死;3例脊髓损害患者,其中1例表现为脊髓炎症改变,2例为后索损害表现为脊髓变细萎缩。结论艾滋病可引起神经系统不同部位及不同性质损害,在影像学上显示不同的非特征性改变。     

神经系统副肿瘤综合征临床分析

目的 了解神经系统副肿瘤综合征患者的临床特点.方法 收集我院收治的神经系统副肿瘤综合征患者28例,对其临床资料进行回顾性分析.结果 患者多为慢性隐袭或亚急性起病,进行性加重,治疗后无明显缓解,3.8%患者在发现肿瘤后才出现神经症状;96.2%在出现副肿瘤症状后才发现肿瘤,副肿瘤综合征的临床类型有Lambert-Eaton肌无力综合征8例、周围神经病7例、多发性肌炎和皮肌炎4例、脑干脑炎3例、进行性小脑变性2例、边缘系统脑炎2例、运动神经元病1例、进行性多灶性白质脑病1例.结论 神经系统副肿瘤综合征临床表现形式多样,容易误诊,临床早期确诊对于隐匿肿瘤的发现和治疗非常重要.     

获得性免疫缺陷综合征神经系统损害临床分析

目的 提高对获得性免疫缺陷综合征(艾滋病)合并神经系统损害临床特点的认识,以减少漏诊.方法 对28例人类获得性免疫缺陷病毒(HIV)感染和(或)艾滋病患者中的12例合并神经系统损害患者的临床资料和机会感染性疾病情况进行回顾,并结合文献分析总结.结果 12例患者分别诊断为艾滋病脑病(5例)、慢性脑膜炎(3例)、周围神经病...     

Good综合征并发进行性多灶性白质脑病1例报告

Good 综合征渊Good syndrome, GS冤是一种罕见的合并胸腺瘤的成人原发性免疫缺陷病,典型特征为胸腺瘤、低丙种球蛋白血症合并免疫缺陷,多见于成年人,无性别差异,占胸腺瘤患者的10%。进行性多灶性白质脑病(progressive multifocal leukoencephalopath, PML)是一种由JC 多瘤病毒(John Cunningham polyomavirus, JCPy V)感染引起的中枢神经系统亚急性进行性脱髓鞘疾病,好发于机体免疫功能严重受到抑制的人群。本文报告1 例女性GS并发PML 患者,以期提高对PML 及GS 的认识。     

艾滋病合并中枢神经系统病变128例临床分析

目的 提高对艾滋病合并中枢神经系统病变的认识和重视,为艾滋病临床诊断与治疗提供参考依据.方法 对我院2009-01-2010-08收治128例艾滋病合并中枢神经系统病变的临床资料进行回顾性分析.结果 2009-01-2010-08我院共收治艾滋病患者903例,其中艾滋病合并中枢神经系统病变者128例,发病率14.18％.其中隐球菌脑膜炎34例(占26.56％),HIV相关性脑病30例(23.44％),弓形虫脑病20例(15.63％),结核性脑膜炎13例(10.16％),脑白质脱髓鞘3例(2.34％),进行性多灶性脑白质病4例(3.13％),其他脑白质病变7例(5.47％),明显脑萎缩者9例(7.03％),脊髓病变4例;其他真菌性脑炎2例,并发脑梗死7例(5.47％),同时合并2种以上中枢神经系听病变者46例(占35.94％).住院期间死亡17例(13.28％).结论 AIDS合并中枢神经系统病变临床表现复杂多样,诊断、治疗较困难,预后差,病死率高.107例CD4T淋巴细胞计数低于200/mm3,占83.59％.早期诊断、及时选择合理抗病毒治疗能改善预后,在临床中应提高警惕.     

Prevalence of neurological complications in Japanese patients with AIDS after the introduction of HAART.

We investigated trends in neurological complications of infection with human immunodeficiency virus (HIV) in Japan after the introduction of highly active antiretroviral therapy (HAART). Two questionnaire surveys were performed in hospitals treating acquired immunodeficiency syndrome (AIDS) to compare two periods: immediately after the introduction of HAART (1999-2001); and a few years later (2002-3). Neurological complications accompanied 15.9% in 1999-2001 and 9.8% in 2002-3. Neurological complications developed without HAART in about 80% of cases. Neurological complications developed as the first AIDS-defining disease for 8.3% of AIDS patients in 1999-2001 and for 5.4% in 2002-3. Prevalences of HIV encephalopathy and myelopathy decreased markedly over the study period, as reported in other developed nations. However, prevalences of cytomegalovirus encephalitis, PML and primary brain lymphoma did not decrease. PML and primary brain lymphoma occurred in patients who received HAART and whose CD4 counts were relatively high during the study period. This is probably related to the extended survival of HIV-infected individuals after the introduction of HAART as a worldwide therapy, and the reactivation of viremia or latent infection persisting within the central nervous system.     

Clinical epidemiology and survival of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy: Data from the Italian Registry Investigative Neuro AIDS (IRINA)

Human immunodeficiency virus (HIV)-associated progressive multifocal leukoencephalopathy (PML) remains a relevant clinical problem even in the era of highly active antiretroviral therapy (HAART). Aims of the study were to analyze clinical and treatment-related features and the survival probability of PML patients observed within the Italian Registry Investigative Neuro AIDS (IRINA) during a 29-month period of HAART. Intravenous drug use, the presence of focal signs, and the involvement of white matter at neuroradiology increased the risk of having PML. A reduced probability of PML was observed when meningeal signs were reported. Patients starting HAART at PML diagnosis and previously naïve for antiretrovirals showed significantly higher 1-year probability of survival (.58), compared to those continuing HAART (.24), or never receiving HAART (.00). Higher CD4 cell count were associated with a higher survival probability (.45). At multivariate analysis, a younger age, higher CD4, starting HAART at PML diagnosis, the absence of previous acquired immunodeficiency syndrome (AIDS)-defining events, and the absence of a severe neurologic impairment were all associated with a reduced hazard of death. The use of cidofovir showed a trend towards a reduced risk of death.     

Clinical epidemiology and survival of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy: data from the Italian Registry Investigative Neuro AIDS (IRINA)

Human immunodeficiency virus (HIV)-associated progressive multifocal leukoencephalopathy (PML) remains a relevant clinical problem even in the era of highly active antiretroviral therapy (HAART). Aims of the study were to analyze clinical and treatment-related features and the survival probability of PML patients observed within the Italian Registry Investigative Neuro AIDS (IRINA) during a 29-month period of HAART. Intravenous drug use, the presence of focal signs, and the involvement of white matter at neuroradiology increased the risk of having PML. A reduced probability of PML was observed when meningeal signs were reported. Patients starting HAART at PML diagnosis and previously na?ve for antiretrovirals showed significantly higher 1-year probability of survival (.58), compared to those continuing HAART (.24), or never receiving HAART (.00). Higher CD4 cell count were associated with a higher survival probability (.45). At multivariate analysis, a younger age, higher CD4, starting HAART at PML diagnosis, the absence of previous acquired immunodeficiency syndrome (AIDS)-defining events, and the absence of a severe neurologic impairment were all associated with a reduced hazard of death. The use of cidofovir showed a trend towards a reduced risk of death.     

Progressive multifocal leukoencephalopathy: clinical and radiographic features

Between April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma, and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate of clinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similar clinical, radiographic, and pathological features in the AIDS and non-AIDs patients. Of 79 AIDS patients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT-clinical dissociation occurs.     

Progressive multifocal leukoencephalopathy in persons infected with human immunodeficiency virus, San Francisco, 1981-1989.

Progressive multifocal leukoencephalopathy (PML), a rare neurological disease, has been sporadically reported in persons infected with human immunodeficiency virus (HIV), the causative agent of acquired immune deficiency syndrome (AIDS). From January 1981 through February 1989, in San Francisco, we identified 94 HIV-infected persons with PML, of whom 48 (51%) were pathologically confirmed (as required for AIDS case reporting). These 48 patients were significantly older when diagnosed with AIDS (20% older than 50 years) than patients with AIDS without PML. The remaining 46 (49%) patients, diagnosed clinically and by neuroimaging, did not differ significantly from definitive patients in demographic or survival characteristics after PML diagnosis. We detected antibodies to JC virus, the causative agent of PML, in 9 of 14 (64%) AIDS-related patients with PML, and in 9 of 14 (64%) matched control subjects, suggesting that determination of JC virus antibody status before AIDS diagnosis does not reliably indicate which patients will contract PML. Our study shows that the proportion of patients with AIDS who contracted PML remained stable between 1981 and 1988, but increased in the first 2 months of 1989. Our findings further indicate that PML in HIV-infected patients may be underestimated by as much as 50%.     

Predictive factors for prolonged survival in acquired immunodeficiency syndrome—associated progressive multifocal leukoencephalopathy

Progressive multifocal leukoencephalopathy (PML) complicating the acquired immunodeficiency syndrome (AIDS) is typically inexorably progressive with death usually occurring within 6 months of symptom onset. Occasional patients have been observed to survive longer than 1 year, often with remission of clinical features. In this study, we identify predictive factors for prolonged survival in patients with biopsy proven, AIDS-associated PML, by comparing 7 patients with survival exceeding 12 months from symptom onset with 45 patients with shorter survivals. PML was the presenting manifestation of AIDS in 5 (71.4%) of 7 long-term survivors compared with 8 (17.8%) of 45 short-term survivors. CD4 T-lymphocyte counts were substantially higher in the long-term survivors, with 3 (42.9%) of 7 having counts exceeding 300 cells/mm³ in comparison with only 1 (4.3%) of 23 short-term survivors. Contrast enhancement on radiographic imaging was observed in 3 (50%) of 6 long-term survivors in comparison with 4 (8.9%) of 45 short-term survivors. Neurological recovery and radiographic improvement were not observed in any short-term survivors but were seen in 5 (71.4%) long-term survivors. There was no association between treatment modalities and survival. Predictors of long-term survival in AIDS patients with PML include PML as the heralding manifestation of AIDS, high CD4 T-lymphocyte count at disease onset, lesion enhacement on computed tomographic scan or magnetic resonance imaging, and evidence of recovery of neurological function.     

人类获得性免疫缺陷综合征神经系统并发症临床分析

目的 探讨获得性免疫缺陷综合征（AIDS）患者神经系统并发症的临床特征。方法 对1992年1月至2001年5月间确诊的伴有神经系统并发症的5例AIDS患者进行临床分析。结果 5例中有1例空泡性脊髓病，1例空泡性脊髓病伴痴呆，1例颅内结核瘤伴痴呆，1例脑梗死伴继发性癫痫及三叉神经痛，1例多发性运动感觉神经病。其中2例空泡性脊髓病均为首发症状。结论 AIDS患者容易发生各种神经系统并发症，尤以空泡性脊髓病及痴呆多见，年轻突发痴呆患者尤其伴有机会性感染时需考虑该病的可能。对这些患者，应检查血抗人类免疫缺陷病毒（HIV）抗体。     

New features of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy and natalizumab

Over the past three decades, progressive multifocal leukoencephalopathy (PML) evolved from being a clinical rarity to become an important cause of neurological complications in acquired immunodeficiency syndrome (AIDS) patients. Recently this disease unexpectedly occurred in patients receiving the novel immunomodulatory medication natalizumab. These changes in the epidemiology of PML also brought new questions with regard to the pathogenesis of this disease. The authors review the current challenges in the diagnosis and management of patients with PML, based on the recent advances in the understanding of the JC virus biology and discuss potential methods to monitor disease evolution and predict outcome.     

Pathomorphological variations of the AIDS-associated progressive multifocal leukoencephalopathy

Pathological analysis of 20 cases of the progressive multifocal leukoencephalopathy (PML) appearing in the course of acquired immune deficiency syndrome (AIDS) is presented. PML occurred in 10% of all AIDS cases, collected in the period from 1987 to 1999. PML appeared either as the only brain pathology or accompanied HIV-related brain alterations isolated or concomitant with one or several opportunistic infections and/or neoplastic growth (malignant lymphoma). Basing on the pathomorphological picture and clinical symptomatology early, atypical and severe forms of the disease were distinguished. All of them were characterized by typical PML demyelination with oligodendroglial and astrocytic pathology. The group with early changes revealed widespread, multifocal myelin alterations of a moderate intensity with predominant oligodendroglial abnormalities and less advanced astrocytic changes. Atypical form of the disease was represented by cases with unifocal changes, although containing all key elements of PML pathology. The leading pathological feature of the severe form of the disease consisted in a particular intensity of the demyelination, resulting in tissue destruction often with its cavitation, with typical glial reaction and intense macrophage and lymphocytic infiltration. The other distinguishing feature consisted in strong topographic prevalence of the pathological process either to brain hemispheres or cerebellum. Differences of PML pathology in the course of AIDS as compared with non-AIDS cases are discussed. Due to the relatively high frequency of cases of isolated or strongly predominant involvement of cerebellum, separation of the cerebellar form of the disease has been suggested.     

Progressive multifocal leukoencephalopathy in a child with hyperimmunoglobulin E recurrent infection syndrome and review of the literature.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease due to infection with polyomavirus JC (JCV). PML occurs almost exclusively in immunocompromised patients, and although it has increased markedly in relation to AIDS, remains exceptional in children. We present the case of an immunocompromised child with hyperimmunoglobulin E recurrent infection syndrome (HIES) and pathologically-proven PML. HIES is a rare congenital immunodeficiency that to our knowledge has never before been reported in association with neurological complications. Following a recurrence of bronchopneumonia, the child's motor and cognitive functions deteriorated progressively in parallel with alterations on cerebral MRI. The neurological onset coincided with lymphocyte subset changes. PCR for JCV DNA did not detect the virus in CSF, and brain biopsy was required to secure the diagnosis. Antiviral treatment with cidofovir produced no benefit. Autopsy revealed the typical neuropathological findings of PML which were associated with inflammatory eosinophilic infiltrate (a marker of HIES). In accordance with the few pediatric PML cases reported and here reviewed, the child died five months after neurological onset.