Herpes simplex virus infections of the central nervous system

Herpes simplex virus (HSV) infections of the central nervous system (CNS) can occur within weeks after birth (neonatal HSV disease) or in childhood or adulthood [herpes simplex encephalitis (HSE)]. Most cases of neonatal HSV disease are caused by HSV type 2, whereas virtually all cases of HSE are caused by HSV type 1. Diagnostic advances made during the past decade include the application of polymerase chain reaction (PCR) technology to cerebrospinal fluid from patients with suspected HSV CNS disease to evaluate for the presence of HSV DNA. Although not foolproof, PCR is a powerful diagnostic tool that has supplanted brain biopsy as the modality of choice for diagnosing HSV CNS disease, in no small part because of the invasiveness of brain biopsy. PCR also can provide information regarding the therapeutic response to antiviral therapy. Efforts made during the past decade to improve the outcome of HSV CNS disease have focused on increased doses of intravenous acyclovir administered for longer durations of time. Although advances have been achieved, morbidity and mortality rates from neonatal HSV disease and HSE remain unacceptably high.     

Epstein-Barr virus related central nervous system lymphoma in a child after renal transplantation

In this report, we describe the development of a rapidly progressive Epstein-Barr virus (EBV) related cerebral lymphoma in an 11 year old girl, eight months after renal transplantation. No serological evidence for a persistent EBV infection was found, but Epstein-Barr nuclear antigen (EBNA) could be demonstrated in the tumor. The clinical course of our patient was different from EBV-related syndromes in renal transplanted patients described in previous reports. Furthermore, pathological investigations of the biopsy specimen and tumor cells obtained at necropsy revealed a discrepancy in light chain expression. The possibility that lymphoproliferative disorders represent multiclonal B cell lymphomas is discussed.     

Diffuse central nervous system protoplasmic astrocytoma

Protoplasmic astrocytoma is an extremely rare form of grade II low grade glioma which usually presents as a discrete mass lesion. We describe a 3‐year‐old female with diffuse protoplasmic astrocytoma with parenchymal involvement and leptomeningeal spread. This tumour proved extremely difficult to diagnose and followed a progressive course. Three superficial biopsies did not give the diagnosis and this was only confirmed 8 months from presentation from a larger fourth biopsy taken deeper from the cerebellum. To our knowledge this case represents the distinct presentation of protoplasmic astrocytoma presenting as extensive diffuse meningeal disease. Pediatr Blood Cancer 2010;54:768–769. © 2009 Wiley‐Liss, Inc.     

食源性中枢神经系统疾病

某些中枢神经系统疾病可由粪 口感染所致 ,或因致病因子污染食物所致 ,把好饮食这一关可预防许多中枢神经系统疾病。1　肠道病毒感染所致中枢神经系统感染　　经典肠道病毒含脊髓灰质炎病毒 (Ⅰ、Ⅱ、Ⅲ型 )、柯萨奇 (Ｃｏｘｓａｃｋｉｅ)病毒 (Ａ组 1～ 2 4型 ,Ｂ组 1～ 6型 )及埃可(Ｅｃｈｏ)病毒 (1～ 33型 ) ,总共 6 6型。 1976年以后还发现6 8～ 71型新型肠道病毒。肠道病毒有时可致中枢神经系统感染。1 1　急性弛缓性瘫痪 (ＡＦＰ)　肠道病毒可引起肢体不对称性下级神经元瘫痪 (小儿麻痹症 )及脑膜脑炎。由于其减毒活疫苗的广泛使用 …     

Lymphocytic vasculitis involving the central nervous system occurs in patients with X‐linked lymphoproliferative disease in the absence of Epstein–Barr virus infection

X‐linked lymphoproliferative disease (XLP) is an immunodeficiency caused by defects in the adaptor molecule SAP. The manifestations of XLP generally occur following Epstein–Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T‐cell‐mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T‐cell populations were observed, but neither demonstrated evidence of lymphoma. Thus, loss of SAP function can lead to dysregulated immune responses characterized by the uncontrolled expansion and activation of T cells independent of EBV infection. Pediatr Blood Cancer 2009;53:1120–1123. © 2009 Wiley‐Liss, Inc.     

Neuroimaging of central nervous system infections

During the past decade, advances have been made in the technology used to image the pediatric central nervous system. Although computed tomography (CT) remains the first line of imaging for the sick child admitted to the emergency room with fever and altered mental status, magnetic resonance imaging (MRI) offers superior soft tissue imaging of central nervous system (CNS) infections and advanced techniques. MRI also is the standard of care for imaging spinal infections. CT remains superior for the detection of calcification and bony detail. With the advent of new MRI sequences such as T2-weighted fluid attenuated inversion recovery (FLAIR), diffusion-weighted imaging (DWI), and magnetic resonance spectroscopy (MRS), we are able to detect early and subtle abnormalities such as the vasculitis accompanying a meningitis and to identify patterns of signal alteration that can help us be more specific about the diagnosis in lesions with similar appearances.     

儿童原发性中枢神经系统淋巴瘤

原发性中枢神经系统淋巴瘤(PCNSL)是指病灶局限于中枢神经系统(包括脑实质、脊髓、颅神经、眼和软脑膜)而无全身播散证据的结外非霍奇金淋巴瘤(NHL),是一种少见的儿童和青少年NHL亚型。先天性或获得性免疫缺陷患儿患此病风险更高。由于缺乏儿童前瞻性研究,现对近年儿童PCSNL的病例报道和成人前瞻性研究进行汇总,以帮助读...     

Herpes simplex virus central nervous system relapse during treatment of infantile spasms with corticotropin

Here we report an infant who had herpes simplex virus (HSV) encephalitis and sustained severe bilateral damage to the posterior frontal lobes, postcentral gyri, and the thalami despite intravenous acyclovir treatment. At 7 months of age, the patient developed infantile spasms and was treated with corticotropin injections. After 10 days of corticotropin treatment, she developed lethargy, fever, and opisthotonic posturing. Her cerebrospinal fluid again was positive for HSV DNA, indicating recurrent HSV encephalitis, and repeat MRI revealed new lesions of the right frontal, parietal, temporal, and occipital lobes. Immunosuppression by corticotropin may have led to the reactivation of the HSV encephalitis. Corticotropin should be relatively contraindicated for use when a patient has a history of HSV infection, or intravenous acyclovir should be administered concurrently.     

整合素在中枢神经系统中的作用

整合素是位于细胞表面的重要黏附分子,具有介导细胞与细胞外基质及细胞与细胞间的黏附,调节细胞生存、增殖、分化等多项功能.中枢神经系统内整合素分布广泛,通过其与周围基质和神经细胞的相互作用,传递信息,在中枢神经系统的发育和功能维持中起重要作用.对整合素的进一步研究,可能为防治中枢神经系统疾病提供新的切入点和新的临床思路.