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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epstein-Barr virus (EBV) has been implicated in a variety of central nervous system syndromes. In a few well-studied patients, EBV has been detected by viral isolation or EBV DNA has been found by Southern hybridization analysis. Using polymerase chain reaction, we evaluated brain biopsy specimens from 24 patients for the presence of EBV genomes. EBV DNA was found in brain specimens from 18 patients in whom presence of the virus in the brain was suspected clinically or on the basis of serological tests. Six patients had acquired immunodeficiency syndrome; 2 were kidney transplant recipients. Brain specimens from 4 patients with encephalitis due to other herpes group viruses and from a patient with metabolic encephalopathy were negative for EBV DNA as determined by polymerase chain reaction. The findings indicate a need to evaluate the role of EBV in diverse neurological syndromes, especially those occurring in immunodeficient hosts.
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PMID:Detection of Epstein-Barr virus in the brain by the polymerase chain reaction. 132 32

A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of pregnancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage III encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite aggressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy. Southern blot analysis for Epstein-Barr virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care.
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PMID:Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation. 240 63

IFN-alpha was detected in cerebrospinal fluid and/or sera from 7 of 8 patients with a progressive familial encephalopathy associated with calcifications of the basal ganglia and white matter alterations. The secretion of IFN-alpha was prolonged, as shown by its presence at different times between birth and 5 years, and was not associated with IFN-gamma. Virological investigations excluded various congenital infections. In only 2 patients, high levels of Epstein-Barr virus antibodies were observed, indicating the possibility of an abnormal response to viral infection rather than a congenital infection. Further investigations are required for characterization of the recessive autosomal trait of this syndrome and its relation to the IFN system.
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PMID:Intrathecal synthesis of interferon-alpha in infants with progressive familial encephalopathy. 283 39

Three patients had status epilepticus appearing de novo as the presenting manifestation of Epstein-Barr virus (EBV) encephalopathy. Clinical findings of infectious mononucleosis were absent and EBV-specific serologic tests made or confirmed the diagnosis in each case. Epstein-Barr virus should be considered as a potential cause when status epilepticus occurs in the absence of previous seizures or an identified cause of seizures. The diagnosis may be made in some cases only with the use of EBV-specific serologic testing.
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PMID:Status epilepticus and Epstein-Barr virus encephalopathy. Diagnosis by modern serologic techniques. 299 28

We studied 51 consecutive pediatric patients for the frequency and morbidity of viral infections after liver transplantation. The incidence of primary (67%) and reactivation (48%) Epstein-Barr virus (EBV) infections and reactivation (88%) cytomegalovirus (CMV) infection was comparable to that seen in adult transplant recipients. However, fewer pediatric than adult transplant recipients experienced primary CMV infection (P less than .01). Five (38%) of 13 CMV infections were symptomatic and included hepatitis, pneumonitis, enteritis, and mononucleosis. Two of 14 patients with primary EBV infection subsequently developed, at two months and two years after initial infection, an EBV-associated lymphoproliferative syndrome, and one of 10 patients with reactivated EBV infection developed a possible EBV-associated febrile encephalopathy. Other viruses causing infection in these children included herpes simplex virus, varicella-zoster virus, adenovirus, parainfluenza virus, respiratory syncytial virus, and rotavirus.
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PMID:Epstein-Barr virus, cytomegalovirus, and other viral infections in children after liver transplantation. 303 64

We describe a patient who presented with an acute monophasic illness characterized by behavioral abnormalities, visual illusions, and a seizure, who had magnetic resonance- and brain biopsy-documented evidence of multifocal central nervous system demyelination. Serological studies were diagnostic of recent Epstein-Barr virus infection and included evidence of intrathecal synthesis of Epstein-Barr virus-specific IgG antibodies against the viral capsid antigen. Viral DNA could not be amplified from cerebrospinal fluid by polymerase chain reaction, and viral antigen and genome were not detected in the brain biopsy specimen. The patient's clinical course, diagnostic studies, and neuropathological findings all support the diagnosis of a postinfectious Epstein-Barr virus-mediated demyelinating encephalopathy.
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PMID:Acute arcuate fiber demyelinating encephalopathy following Epstein-Barr virus infection. 761 16

Encephalopathy, leukoencephalopathy, and secondary parkinsonism occurred in 3 children with refractory leukemia undergoing allogenic bone marrow transplantation (BMT) who were treated with high-dose amphotericin B for pulmonary aspergillosis or sinus aspergillosis that did not involve the nervous system. Treatment included high-dose cytosine arabinoside, cyclophosphamide, and total body irradiation prior to the BMT. The children developed a progressively worsening encephalopathy and parkinsonian features, characterized by resting tremor, cogwheel rigidity, and masklike facies. Neuroimaging studies showed cerebellar, cerebral, and basal ganglia atrophy, as well as frontal and temporal lobe white matter involvement. Two of the 3 patients recovered, although 1 has residual intellectual impairment. The third succumbed to non-central nervous system Epstein-Barr virus-lymphoproliferative disease and had autopsy-confirmed leukoenephalopathy.
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PMID:Encephalopathy with parkinsonian features in children following bone marrow transplantations and high-dose amphotericin B. 777 56

Human Immunodeficiency Virus type-1 (HIV-1)-associated neurologic disease occurs as the initial presenting clinical manifestation of acquired immunodeficiency syndrome (AIDS) in 3-7% of infected patients, but in up to 18% of children and adolescents (Janssen, 1992; Janssen et al., 1992; Scott et al., 1989; Mintz et al., 1989a; Epstein et al., 1986). The overall prevalence of dementia in adult AIDS patients is 7.3-11.3% (Janssen, 1992), but up to 30-60% of children with AIDS manifest an analogous progressive encephalopathy (Epstein et al., 1986; Belman et al., 1988; Mintz, 1992; The European Collaborative Study, 1990). As a result of both direct and indirect effects of HIV-1 infection of the central nervous system (CNS), a distinct clinical and pathologic picture has emerged of insidious and severe neurologic deterioration, termed "AIDS Dementia Complex" (ADC) in adults, and "HIV-1-associated Progressive Encephalopathy" (PE) in children (Working Group, 1991) (see Table 1). In the severe manifestations of this pariah, there is little dispute as to the necessity of CNS HIV-1 infection for precipitating the cascade of adverse neurologic symptoms, although the pathogenic mechanisms of neurologic dysfunction and destruction--whether a result of direct cellular infection of HIV, secondarily produced and upregulated cytotoxic cytokines, or co-infection with opportunistic pathogens--remains an area of active research (Epstein and Gendelman, 1993; Fiala et al., 1993; Wiley and Nelson, 1988; Saito et al., 1994; Koenig et al., 1986; Sharer, 1992). Furthermore, the existence of systemic immune deficiency renders the CNS susceptible to opportunistic infection (OI), particularly in adult patients, adding further to morbidity and mortality (Clifford and Campbell, 1992). With the introduction of antiretroviral nucleoside analogues, there have been reports of a decreasing incidence of ADC (Portegies et al., 1989; Day et al., 1992), and amelioration--at least temporarily--of PE in children (Pizzo et al., 1988; Mintz and Epstein, 1992; Brouwers et al., 1990; Mintz et al., 1990). This appends further evidence to the central precipitating role of CNS HIV-1 infection.
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PMID:Clinical comparison of adult and pediatric NeuroAIDS. 787 89

Rapidly progressive diffuse immunoblastic lymphoma is an uncommon but devastating complication of organ transplantation that typically occurs early in the postoperative period. The fulminant course is characterized by progressive encephalopathy and coagulopathy, with malignant B-cell infiltration in the graft and other sites. Both de novo infection with Epstein-Barr virus (EBV) and treatment with the monoclonal antibody OKT3 have been implicated in the development of this disorder. We report two patients who received renal transplants from the same cadaver donor, with transmission of EBV from the same source, in whom treatment with OKT3 for acute rejection triggered the simultaneous development of fulminant and fatal B-cell immunoblastic lymphoma. We suggest that antilymphocyte agents be used with caution in EBV-seronegative graft recipients who receive a transplant from an EBV-seropositive donor to minimize the risk of this lethal complication.
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PMID:Simultaneous development of diffuse immunoblastic lymphoma in recipients of renal transplants from a single cadaver donor: transmission of Epstein-Barr virus and triggering by OKT3. 828 88

Fulminant hepatic failure as a result of hepatitis A is a rarely diagnosed complication entity in developed countries. With the advent of specific serologic markers for acute hepatitis A virus infection, the incidence and pathology of fulminant hepatitis A can be more clearly defined. We describe four patients (one adult, three children; two males and two females, ages 2 1/2-58 years) referred to our institution for orthotopic liver transplantation subsequent to fulminant hepatic failure following hepatitis A infection. All of these patients had a history of residence in or travel to hepatitis A endemic areas. Hepatitis A infection was documented by the presence of serum IgM against hepatitis A virus prior to transplantation. Infection with hepatitis B virus, cytomegalovirus, Epstein-Barr virus, and herpes simplex virus was excluded by clinical and specific serologic examinations. All patients presented with varying degrees of encephalopathy, progressing to coma. Coagulopathy in the form of prolonged prothrombin time and partial thromboplastin time was present in all patients. Peak liver parenchymal enzymes increased to greater than ten times the upper limit of the normal range. Total and direct bilirubin levels increased to > 20 and 10 mg/dl, respectively. Histologic evaluation of the explanted livers showed a spectrum of changes ranging from periportal hepatocellular necrosis with focal parenchymal collapse and prominent bile duct proliferation to massive necrosis with complete loss of hepatic architecture.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Fulminant hepatic failure with massive necrosis as a result of hepatitis A infection. 840 20


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