1/41. poliomyelitis-like syndrome associated with Epstein-Barr virus infection.A 20-month-old male presented with an acute clinical syndrome resembling poliomyelitis, characterized by a flaccid monoplegia, areflexia of the involved limb, and preserved sensation. Electrophysiologic studies supported a neuronopathic localization involving the anterior horn cells. Although laboratory evidence for a poliovirus infection was absent, serologic and polymerase chain reaction studies documented an active central nervous system infection with Epstein-Barr virus, indicating that a poliomyelitis-like syndrome may be produced by infectious agents other than enteroviruses.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/41. Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy.A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV dna was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56 lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8 T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/41. Subacute sensory neuropathy associated with Epstein-Barr virus.A 35-year-old man experienced severe sensory loss, pseudoathetosis, and areflexia during recovery from a severe viral illness. Sensory nerve action potentials were absent, motor conduction velocities were mildly slowed, and blink reflexes were normal. magnetic resonance imaging (MRI) revealed abnormal signal within the central and dorsal aspects of the thoracic cord. Acute and convalescent Epstein-Barr virus (EBV) titers suggested EBV as the etiology. Subacute sensory neuropathy, with peripheral and central nervous system involvement, is a rare complication of EBV infection.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/41. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.- - - - - - - - - - ranking = 1.1805105125613keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
5/41. Brainstem encephalitis resulting from Epstein-Barr virus mimicking an infiltrating tumor in a child.A case of a child with subacute neurologic features and imaging findings consistent with a brainstem encephalitis that was discovered to be related to a primary central nervous system infection caused by Epstein-Barr virus is presented. A brainstem tumor was initially suspected, but a correct diagnosis was formulated on the basis of the favorable clinical course and the detection of positive Epstein-Barr virus serology. In contrast to a prompt recovery of neurologic signs the neuroimaging alterations persisted for a longer time. The present report emphasizes the possible role of Epstein-Barr virus in the pathogenesis of infectious neurologic disorders in childhood, underlining the unusual presentation of a brainstem encephalitis, and considers the discrepancy between the course of neurologic features and the evolution of imaging alterations.- - - - - - - - - - ranking = 1.1083063075368keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
6/41. central nervous system involvement of Epstein-Barr virus lymphoproliferative disease in a patient with acute lymphocytic leukemia.Epstein-Barr virus-related lymphoproliferative disease (EBV-LPD) is a serious and often fatal complication of a variety of immune-suppressed conditions. A 6-year-old boy undergoing chemotherapy for standard-risk acute lymphocytic leukemia experienced separate episodes of EBV-LPD in different organ systems. The patient experienced three separate episodes of EBV-LPD in the cervical lymph node, the central nervous system (CNS), and the liver occurring, respectively, in January 1992, February 1992, and November 1993 after the completion of chemotherapy in May 1993. The EBV presence was confirmed by in situ hybridization in the biopsy samples from each lesion. Several different treatment modalities, including acyclovir, intravenous gamma globulin, and surgery were used to combat the EBV-LPD. The patient has recovered completely, with normal CNS and liver function, and for the past 6 years has experienced leukemia remission while not receiving chemotherapy. Careful monitoring of patients and the use of new immune therapies offer the highest chance for successful outcomes in such patients.- - - - - - - - - - ranking = 1.9535364282703keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/41. Chronic parvovirus B-19 meningoencephalitis with additional detection of Epstein-Barr virus dna in the cerebrospinal fluid of an immunocompetent patient.parvovirus B19 dna was detected by polymerase chain reaction in the brain biopsy specimen from a 67-year-old immunocompetent woman with severe chronic lymphocytic meningoencephalitis. In addition to parvovirus B19, Epstein-Barr virus dna was identified in the CSF. Genomic material from Epstein-Barr virus was absent in the brain tissue. Clinical symptoms and CSF pleocytosis improved under long-term corticosteroid-treatment. The aetio-pathogenetic role of parvovirus B19 and the possible meaning of the additionally detected Epstein-Barr virus dna are discussed.- - - - - - - - - - ranking = 0.072204205024516keywords = brain (Clic here for more details about this article) |
8/41. Fatal lymphoproliferative disease as a complication of Evans syndrome.A 9-month-old boy had bruising and petechiae. Investigation revealed a Coombs-positive hemolytic anemia and immune-mediated thrombocytopenia. The infant was treated with intravenous immunoglobulin and steroids. The infant eventually had recurrent fevers, hepatosplenomegaly, pulmonary nodules, and parenchymal central nervous system (CNS) lesions develop. Results of a lung biopsy revealed a polyclonal lymphoproliferative disease. polymerase chain reaction analysis showed the presence of the Epstein-Barr (EB) viral genome in the lung nodules. The infant died from progressive lung disease 6 months after the initial symptoms of Evans syndrome. Lymphoproliferative disease is known to occur in a variety of settings after immunosuppression, especially in solid organ transplant recipients. We report a case of polyclonal lymphocyte proliferation in a patient with Evans syndrome.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/41. Epstein-Barr virus-targeted therapy for AIDS-related primary lymphoma of the central nervous system.Epstein-Barr virus (EBV) targeted therapeutic strategies for viral associated malignant diseases have received only perfunctory consideration, first, because latent herpesviruses have been intractable to antiviral chemotherapy and, second, because the role EBV has in maintenance of the malignant cell phenotype has been uncertain. Two patients with EBV related primary central nervous system lymphoma (PCNSL) in the setting of advanced AIDS, were treated with low dose hydroxyurea based on in vitro anti-EBV activity. The responses obtained here suggest the promise of antiviral approaches in select cancers.- - - - - - - - - - ranking = 5keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/41. Simultaneous multiorgan presence of human herpesvirus 8 and restricted lymphotropism of Epstein-Barr virus dna sequences in a human immunodeficiency virus-negative immunodeficient infant.Because a profound dysregulation of the immune system occurs in primary immunodeficiencies, viral infections are not uncommon. Human herpesvirus (HHV)-8 dna was detected by polymerase chain reaction (PCR) analysis, Southern blotting, and in situ hybridization (ISH) in peripheral blood mononuclear cells and lymphoid organs (bone marrow, spleen, and lymph nodes) and endothelial and epithelial cells and macrophages from several organs (skin, lung, esophagus, intestine, choroid plexus [but not in brain or cerebellum], heart, striated muscle, liver, and kidney) of a human immunodeficiency virus-negative infant with DiGeorge anomaly who died of disseminated infection. Epstein-Barr virus dna sequences were detected in the spleen and lymph nodes (by PCR and ISH) and in bone marrow (only by ISH) but not in blood or nonlymphoid organs. This report is believed to be the first of multiorgan dissemination of HHV-8 in a primary immunodeficiency.- - - - - - - - - - ranking = 0.036102102512258keywords = brain (Clic here for more details about this article) |
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