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Sample records for anti-nmda receptor encephalitis

  1. [Anti-NMDA-receptor encephalitis].

    Science.gov (United States)

    Engen, Kristine; Agartz, Ingrid

    2016-06-01

    BACKGROUND In 2007 a clinical disease caused by autoantibodies directed against the N-methyl-D-aspartate (NMDA) receptor was described for the first time. Anti-NMDA-receptor encephalitis is a subacute, autoimmune neurological disorder with psychiatric manifestations. The disease is a form of limbic encephalitis and is often paraneoplastic. The condition is also treatable. In this review article we examine the development of the disease, clinical practice, diagnostics and treatment.MATERIAL AND METHOD The article is based on references retrieved from searches in PubMed, and a discretionary selection of articles from the authors' own literature archive.RESULTS The disease most frequently affects young women. It may initially be perceived as a psychiatric condition, as it usually presents in the form of delusions, hallucinations or mania. The diagnosis should be suspected in patients who later develop neurological symptoms such as various movement disorders, epileptic seizures and autonomic instability. Examination of serum or cerebrospinal fluid for NMDA receptor antibodies should be included in the assessment of patients with suspected encephalitis. MRI, EEG and assessment for tumours are important tools in diagnosing the condition and any underlying malignancy.INTERPRETATION If treatment is initiated early, the prognosis is good. Altogether 75 % of patients will fully recover or experience significant improvement. Apart from surgical resection of a possible tumour, the treatment consists of immunotherapy. Because of good possibilities for treatment, it is important that clinicians, particularly those in acute psychiatry, are aware of and alert to this condition.

  2. Anti-NMDA Receptor Encephalitis and Vaccination

    Science.gov (United States)

    Wang, Hsiuying

    2017-01-01

    Anti-N-methyl-d-aspartate (Anti-NMDA) receptor encephalitis is an acute autoimmune neurological disorder. The cause of this disease is often unknown, and previous studies revealed that it might be caused by a virus, vaccine or tumor. It occurs more often in females than in males. Several cases were reported to be related to vaccination such as the H1N1 vaccine and tetanus/diphtheria/pertussis and polio vaccines. In this study, we reported an anti-NMDA receptor encephalitis case that may be caused by Japanese encephalitis vaccination. To investigate the association between anti-NMDA receptor encephalitis and vaccination, we analyzed the phylogenetic relationship of the microRNAs, which significantly regulate these vaccine viruses or bacteria, and the phylogenetic relationship of these viruses and bacteria. This reveals that anti-NMDA receptor encephalitis may be caused by Japanese encephalitis vaccination, as well as H1N1 vaccination or tetanus/diphtheria/pertussis and polio vaccinations, from the phylogenetic viewpoint. PMID:28106787

  3. Anti-NMDA Receptor Encephalitis and Vaccination

    Directory of Open Access Journals (Sweden)

    Hsiuying Wang

    2017-01-01

    Full Text Available Anti-N-methyl-d-aspartate (Anti-NMDA receptor encephalitis is an acute autoimmune neurological disorder. The cause of this disease is often unknown, and previous studies revealed that it might be caused by a virus, vaccine or tumor. It occurs more often in females than in males. Several cases were reported to be related to vaccination such as the H1N1 vaccine and tetanus/diphtheria/pertussis and polio vaccines. In this study, we reported an anti-NMDA receptor encephalitis case that may be caused by Japanese encephalitis vaccination. To investigate the association between anti-NMDA receptor encephalitis and vaccination, we analyzed the phylogenetic relationship of the microRNAs, which significantly regulate these vaccine viruses or bacteria, and the phylogenetic relationship of these viruses and bacteria. This reveals that anti-NMDA receptor encephalitis may be caused by Japanese encephalitis vaccination, as well as H1N1 vaccination or tetanus/diphtheria/pertussis and polio vaccinations, from the phylogenetic viewpoint.

  4. Anti-NMDA Receptor Encephalitis and Vaccination.

    Science.gov (United States)

    Wang, Hsiuying

    2017-01-18

    Anti-N-methyl-d-aspartate (Anti-NMDA) receptor encephalitis is an acute autoimmune neurological disorder. The cause of this disease is often unknown, and previous studies revealed that it might be caused by a virus, vaccine or tumor. It occurs more often in females than in males. Several cases were reported to be related to vaccination such as the H1N1 vaccine and tetanus/diphtheria/pertussis and polio vaccines. In this study, we reported an anti-NMDA receptor encephalitis case that may be caused by Japanese encephalitis vaccination. To investigate the association between anti-NMDA receptor encephalitis and vaccination, we analyzed the phylogenetic relationship of the microRNAs, which significantly regulate these vaccine viruses or bacteria, and the phylogenetic relationship of these viruses and bacteria. This reveals that anti-NMDA receptor encephalitis may be caused by Japanese encephalitis vaccination, as well as H1N1 vaccination or tetanus/diphtheria/pertussis and polio vaccinations, from the phylogenetic viewpoint.

  5. Anti-NMDA Receptor Encephalitis in a Pregnant Woman

    OpenAIRE

    Kim, JiYoung; Park, Seung Ha; Jung, Yu Ri; Park, Soon Won; Jung, Dae Soo

    2015-01-01

    Anti N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common types of autoimmune synaptic encephalitis. Anti-NMDA receptor encephalitis commonly occurs in young women with ovarian teratoma. It has variable clinical manifestations and treatment responses. Sometimes it is misdiagnosed as a psychiatric disorder or viral encephalitis. To the best of our knowledge, anti-NMDA receptor encephalitis is a rare condition in pregnant women. We report a case of anti-NMDA receptor ence...

  6. Anti-NMDA Receptor Encephalitis in a Pregnant Woman.

    Science.gov (United States)

    Kim, Jiyoung; Park, Seung Ha; Jung, Yu Ri; Park, Soon Won; Jung, Dae Soo

    2015-06-01

    Anti N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common types of autoimmune synaptic encephalitis. Anti-NMDA receptor encephalitis commonly occurs in young women with ovarian teratoma. It has variable clinical manifestations and treatment responses. Sometimes it is misdiagnosed as a psychiatric disorder or viral encephalitis. To the best of our knowledge, anti-NMDA receptor encephalitis is a rare condition in pregnant women. We report a case of anti-NMDA receptor encephalitis in a pregnant woman who presented with abnormal behavior, epileptic seizure, and hypoventilation.

  7. Anti-NMDA-receptor antibody encephalitis in infants

    Directory of Open Access Journals (Sweden)

    Amr A. Matoq

    2015-01-01

    Conclusion: Infants with anti-NMDA-receptor antibody encephalitis can present with frank seizures or seizure mimics. Regardless, prompt recognition and aggressive treatment of anti-NMDA-receptor antibody encephalitis, while challenging, can quickly arrest deterioration and hasten recovery, thereby, limiting neurological morbidity.

  8. [Two cases of anti-NMDA receptor encephalitis].

    Science.gov (United States)

    Nakamura, Kazue; Takahashi, Tsutomu; Matsuoka, Tadasu; Kido, Mikio; Uehara, Takashi; Suzuki, Michio

    2011-01-01

    Anti-NMDA receptor encephalitis, reported by Dalmau et al., is a paraneoplastic encephalitis frequently associated with ovarian teratoma. After the manifestation of schizophrenia-like psychotic symptoms in the initial stage, serious neurological symptoms such as convulsions and central hypoventilation develop. We report two cases of 17-year-old girls with anti-NMDA receptor encephalitis who exhibited different clinical courses. Case 1 showed a typical course of anti-NMDA receptor encephalitis associated with sustained consciousness disturbance requiring long-term artificial respiration. Case 2 underwent surgery for an ovarian teratoma in the early stages of the disorder, did not show convulsions or central hypoventilation, and recovered without any sequelae. Early resection of the ovarian teratoma and the immune suppression therapy may have contributed to the rapid recovery and favorable outcome in case 2. Psychiatrists are the first to see a majority of patients with anti-NMDA receptor encephalitis because of psychiatric symptoms and behavioral changes observed in the initial stage. For successful treatment, psychiatrists need to cooperate with neurologists and gynecologists early in the course of this disorder. Psychiatrists' knowledge of the symptoms and clinical course of this form of encephalitis is essential for early detection and adequate treatment, which may be life-saving and contribute to good functional outcomes.

  9. [Anti-NMDA-receptor encephalitis. An interdisciplinary clinical picture].

    Science.gov (United States)

    Prüss, H; Dalmau, J; Arolt, V; Wandinger, K-P

    2010-04-01

    Anti-NMDA-receptor encephalitis is a severe and considerably underdiagnosed form of encephalitis with characteristic clinical features including psychiatric symptoms, decreased levels of consciousness, hypoventilation, epileptic seizures, autonomic dysfunction and dyskinesias. Most patients are primarily seen by psychiatrists, often on the assumption of a drug-induced psychosis. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in women without tumour, in men and in children. The diagnosis is based on the characteristic clinical picture, supporting findings of brain MRI, electroencephalogram and cerebrospinal fluid (CSF), and the presence of highly specific autoantibodies directed against the NR1 subunit of NMDA-type glutamate receptors in the serum or CSF. In particular, anti-NMDA-receptor encephalitis must be excluded in patients with 'encephalitis of unknown cause'. In principle, the prognosis is favourable and recovery from symptoms can be expected even after prolonged intensive care treatment and mechanical ventilation. However, improvement correlates with prompt identification of the disorder, early immunotherapy and - in the case of a malignancy - with complete tumour removal. Patient care requires an interdisciplinary approach including neurologists, psychiatrists, paediatricians, oncologists and gynaecologists.

  10. Seizures and Anti-NMDA-Receptor Encephalitis

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-12-01

    Full Text Available The clinical and immunological features of 100 patients with encephalitis associated with antibodies against NR1-NR2 heteromers of the NMDA receptor were analyzed in a study at the Children’s Hospital of Philadelphia, and University of Pennsylvania.

  11. Cardiac sympathetic dysfunction in anti-NMDA receptor encephalitis.

    Science.gov (United States)

    Byun, Jung-Ick; Lee, Soon-Tae; Moon, Jangsup; Jung, Keun-Hwa; Shin, Jung-Won; Sunwoo, Jun-Sang; Lim, Jung-Ah; Shin, Yong-Won; Kim, Tae-Joon; Lee, Keon-Joo; Park, Kyung-Il; Jung, Ki-Young; Lee, Sang Kun; Chu, Kon

    2015-12-01

    Patients with anti-NMDA receptor (anti-NMDAR) encephalitis frequently suffer from autonomic dysfunctions, which can cause substantial morbidity. This study assessed cardiac autonomic functions in patients with anti-NMDAR encephalitis using heart rate variability (HRV) analysis. This was a retrospective single-center case-control study. Eleven patients with anti-NMDAR encephalitis and 15 age- and sex-matched controls were included in this study. To ensure that autonomic dysfunction does not occur in any encephalitis, we additionally analyzed HRV of 9 patients with herpes encephalitis (HSE) and compared with that of NMDAR encephalitis patients and controls. Five minute resting stationary electrocardiogram was collected from each subject, and HRV was analyzed. Total power and low frequency (LF) power were lower in anti-NMDAR encephalitis patients than those in controls (p=0.005, 0.001 respectively), indicating cardiac autonomic dysfunction especially in sympathetic system. Patients with HSE showed no significant difference in HRV parameters compared with that of controls. Cardiac autonomic dysfunction was associated with 3 month functional outcome in anti-NMDAR encephalitis patients.

  12. Efficacies of treatments for anti-NMDA receptor encephalitis.

    Science.gov (United States)

    Wang, Hsiuying

    2016-01-01

    Treatments for anti-N-methyl-D-aspartate (NMDA) receptor encephalitis include immunotherapy with steroids, intravenous immunoglobulin, plasma exchange, or plasmapheresis as first-line treatments, immunotherapy with rituximab or cyclophosphamide as second-line treatments, and tumor removal. In this systematic review, we evaluated previous studies and examined the association between certain microRNAs and anti-NMDA receptor encephalitis to investigate the performance of different treatment combinations. The efficacies of different combinations of treatments classified into the following four categories were compared: (I) intravenous immunoglobulin administration, (II) plasmapheresis or plasma exchange, (III) treatment with rituximab or cyclophosphamide and (IV) tumor removal. Statistical analyses showed that treatment combinations including at least two of these categories resulted in higher efficacy rates than treatment with a single form of therapy. These findings suggest that if a patient is not recovering, converting to other therapies is more likely to result in early recovery than continuing on the original therapy.

  13. Anti-NMDA Receptor antibody encephalitis with concomitant detection of Varicella zoster virus.

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    Solís, Natalia; Salazar, Lucrecia; Hasbun, Rodrigo

    2016-10-01

    The typical presentation of anti-NMDA (N-Methyl-d-Aspartate) receptor encephalitis involves young women with psychiatric, neurologic and autonomic symptoms; it is often associated with mature ovarian teratomas. NMDA receptor encephalitis has been described following Herpes simplex virus (HSV) encephalitis. This case describes a classic presentation of anti-NMDA receptor encephalitis with the concomitant presence of Varicella zoster virus in the cerebrospinal fluid.

  14. Catatonic syndrome in anti-NMDA receptor encephalitis

    Directory of Open Access Journals (Sweden)

    Starlin Vijay Mythri

    2016-01-01

    Full Text Available Anti-N-methyl-D-aspartate (NMDA receptor encephalitis is a newly recognised autoimmune condition. With its typical clinical pattern, consistent association with the presence of auto antibodies and rapid improvement with immunotherapy, this condition is giving insights into the boundaries between psychiatry and other neurosciences, and is opening avenues for future research. In a young lady who presented with catatonia, we considered anti-NMDA receptor encephalitis, after ruling out other aetiologies. After a positive antibody test we treated her with immunotherapy. She showed gradual improvement in her psychotic and catatonic symptoms. Knowledge regarding the nature and function of NMDA receptors and pathophysiology of this particular encephalitis is important for psychiatric practice. The great opportunity for research in this area due to its association with psychotic disorders is evident but an appeal to temper the enthusiasm by considering the historical lessons learnt from Karl Jaspers′ critique of General Paresis of Insane, is in place. Catatonic syndrome has to be conceptualised broadly and should be recognised with a separate nosological position.

  15. Catatonic Syndrome in Anti-NMDA Receptor Encephalitis

    Science.gov (United States)

    Mythri, Starlin Vijay; Mathew, Vivek

    2016-01-01

    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a newly recognised autoimmune condition. With its typical clinical pattern, consistent association with the presence of auto antibodies and rapid improvement with immunotherapy, this condition is giving insights into the boundaries between psychiatry and other neurosciences, and is opening avenues for future research. In a young lady who presented with catatonia, we considered anti-NMDA receptor encephalitis, after ruling out other aetiologies. After a positive antibody test we treated her with immunotherapy. She showed gradual improvement in her psychotic and catatonic symptoms. Knowledge regarding the nature and function of NMDA receptors and pathophysiology of this particular encephalitis is important for psychiatric practice. The great opportunity for research in this area due to its association with psychotic disorders is evident but an appeal to temper the enthusiasm by considering the historical lessons learnt from Karl Jaspers’ critique of General Paresis of Insane, is in place. Catatonic syndrome has to be conceptualised broadly and should be recognised with a separate nosological position. PMID:27114630

  16. Catatonic Syndrome in Anti-NMDA Receptor Encephalitis.

    Science.gov (United States)

    Mythri, Starlin Vijay; Mathew, Vivek

    2016-01-01

    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a newly recognised autoimmune condition. With its typical clinical pattern, consistent association with the presence of auto antibodies and rapid improvement with immunotherapy, this condition is giving insights into the boundaries between psychiatry and other neurosciences, and is opening avenues for future research. In a young lady who presented with catatonia, we considered anti-NMDA receptor encephalitis, after ruling out other aetiologies. After a positive antibody test we treated her with immunotherapy. She showed gradual improvement in her psychotic and catatonic symptoms. Knowledge regarding the nature and function of NMDA receptors and pathophysiology of this particular encephalitis is important for psychiatric practice. The great opportunity for research in this area due to its association with psychotic disorders is evident but an appeal to temper the enthusiasm by considering the historical lessons learnt from Karl Jaspers' critique of General Paresis of Insane, is in place. Catatonic syndrome has to be conceptualised broadly and should be recognised with a separate nosological position.

  17. Anti-NMDA receptor encephalitis: an important differential diagnosis in psychosis.

    LENUS (Irish Health Repository)

    Barry, Helen

    2012-02-01

    We present four cases of confirmed anti-NMDA receptor encephalitis; three presented initially with serious psychiatric symptoms and the other developed significant psychiatric symptoms during the initial phase of illness. Brain biopsy findings of one patient are also described. Psychiatrists should consider anti-NMDA receptor encephalitis in patients presenting with psychosis and additional features of dyskinesias, seizures and catatonia, particularly where there is no previous history of psychiatric disorder.

  18. Anti-NMDA Receptor Encephalitis in the Polar Bear (Ursus maritimus) Knut.

    Science.gov (United States)

    Prüss, H; Leubner, J; Wenke, N K; Czirják, G Á; Szentiks, C A; Greenwood, A D

    2015-08-27

    Knut the polar bear of the Berlin Zoological Garden drowned in 2011 following seizures and was diagnosed as having suffered encephalitis of unknown etiology after exhaustive pathogen screening. Using the diagnostic criteria applied to human patients, we demonstrate that Knut's encephalitis is almost identical to anti-NMDA receptor encephalitis which is a severe autoimmune disease representing the most common non-infectious encephalitis in humans. High concentrations of antibodies specific against the NR1 subunit of the NMDA receptor were detected in Knut's cerebrospinal fluid. Histological examination demonstrated very similar patterns of plasma cell infiltration and minimal neuronal loss in affected brain areas. We conclude that Knut suffered anti-NMDA receptor encephalitis making his the first reported non-human case of this treatable disease. The results suggest that anti-NMDA receptor encephalitis may be a disease of broad relevance to mammals that until now has remained undiagnosed.

  19. [Clinical diagnosis and treatment of anti-NMDA (N-methyl-D-aspartate) receptor encephalitis].

    Science.gov (United States)

    Kamei, Satoshi

    2013-05-01

    Recent clinical management of anti-NMDA receptor encephalitis is reviewed. This illness is required the management of the neurological emergency. Typical symptoms of anti-NMDA receptor encephalitis develop in several stages that progresses from psychosis, memory deficits, seizures, and language disintegration into a state of unresponsiveness with catatonic features often associated with abnormal movements, and autonomic and respiratory instability. The diagnosis is depended on the detection of the NMDA receptor antibody in CSF or serum under the above characteristic symptoms of encephalitis. The disorder predominantly affects children and young adults, occurs with or without tumor association. The presence of a tumor (usually an ovarian teratoma) is dependent on age and sex, being more frequent in women older than 18 years. Anti-NMDA receptor encephalitis should be treated with tumor resection and immunotherapy (corticosteroids, intravenous immunoglobulin, or plasma exchange) responded faster to treatment and less frequently needed second-line immunotherapy (cyclophosphamide or rituximab, or both).

  20. Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

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    Benjumea-Cuartas, Vanessa; Eisermann, Monika; Simonnet, Hina; Hully, Marie; Nabbout, Rima; Desguerre, Isabelle; Kaminska, Anna

    2017-01-01

    Anti-NMDA receptor encephalitis is a treatable autoimmune disease characterized by cognitive, motor and psychiatric features that primarily affects young adults and children. We present a case of a 7-year-old boy with asymmetrical (mainly right hemibody) and abnormal polymorphic movements without concomitant scalpictal EEG changes but had background slowing predominating over the left hemisphere. This report illustrates previous descriptions of asymmetric presentation of abnormal movements in pediatric anti-NMDA receptor encephalitis and emphasizes the importance of video-EEG interpreted within the overall clinical context, to differentiate epileptic from non-epileptic abnormal movements in patients with autoimmune encephalitis.

  1. Anti-NMDA receptor encephalitis: a neurological disease in psychiatric disguise.

    Science.gov (United States)

    Sharma, Bhawna; Handa, Rahul; Prakash, Swayam; Nagpal, Kadam; Gupta, Pankaj

    2014-02-01

    Anti-NMDA receptor encephalitis was first described in 2005 when psychiatric features, memory loss and altered consciousness were found in four women with ovarian teratoma. We report a case of anti-NMDA receptor encephalitis in a 16-year-old female who presented with psychiatric features followed by autonomic dysfunction and orofacial dyskinesias that showed drastic improvement to intravenous immunoglobulin. As many patients of anti-NMDAR encephalitis initially present with psychiatric features, it is important for psychiatrists to have high index of suspicion for this disease and thus avoid the delay in diagnosing this treatable condition which may be otherwise fatal.

  2. Anti-NMDA-receptor encephalitis: a severe, multistage, treatable disorder presenting with psychosis.

    Science.gov (United States)

    Wandinger, Klaus-Peter; Saschenbrecker, Sandra; Stoecker, Winfried; Dalmau, Josep

    2011-02-01

    Anti-NMDA-receptor encephalitis is a severe, treatable and potentially reversible disorder presenting with memory deficits, psychiatric symptoms and seizures. Initially described in young patients with ovarian teratoma, the disease is meanwhile increasingly recognized also in women without tumours, in men and in children. The presence of anti-glutamate receptor (type NMDA) autoantibodies in serum or cerebrospinal fluid is specific for this novel and widely underdiagnosed disorder. Early recognition is crucial since prognosis largely depends on adequate immunotherapy and, in paraneoplastic cases, complete tumour removal. Indirect immunofluorescence using NMDA-type glutamate receptors recombinantly expressed in human cells is a highly competent method for diagnosing anti-NMDA-receptor encephalitis.

  3. Anti-NMDA receptor encephalitis: psychiatric presentation and diagnostic challenges from psychosomatic medicine perspective.

    Science.gov (United States)

    Gulyayeva, Nataliya A; Massie, Mary Jane; Duhamel, Katherine N

    2014-04-01

    We describe two cases of confirmed anti-NMDA receptor encephalitis; one patient initially presented with a clinical picture that resembled delirium and later appeared to present with a conversion reaction and the second patient presented with a first psychotic break followed by the clinical picture of neuroleptic malignant syndrome with catatonia. Neither patient had a previous history of psychiatric illness or recreational drug use. These cases illustrate the diagnostic and treatment challenges associated with this neuropsychiatric condition and underscore the role of psychosomatic medicine psychiatrists in diagnosing anti-NMDA receptor encephalitis.

  4. Frequent rhabdomyolysis in anti-NMDA receptor encephalitis.

    Science.gov (United States)

    Lim, Jung-Ah; Lee, Soon-Tae; Kim, Tae-Joon; Moon, Jangsup; Sunwoo, Jun-Sang; Byun, Jung-Ick; Jung, Keun-Hwa; Jung, Ki-Young; Chu, Kon; Lee, Sang Kun

    2016-09-15

    The aim of this study was to analyze the clinical presentation and provocation factors of rhabdomyolysis in anti-NMDAR encephalitis. Among the 16 patients with anti-NMDAR encephalitis in our institutional cohort, nine patients had elevated CK enzyme levels and clinical evidence of rhabdomyolysis. Rhabdomyolysis was more frequent after immunotherapy. The use of dopamine receptor blocker (DRB) increased the risk of rhabdomyolysis. None of the patients without rhabdomyolysis received DRBs. Rhabdomyolysis is a frequent complication in anti-NMDAR encephalitis and more common after immunotherapy and the use of DRBs increases the risk. Therefore, DRBs should be administered carefully in patients with anti-NMDAR encephalitis.

  5. Heterotopic ossification following anti-NMDA receptor encephalitis: a case report

    OpenAIRE

    Wang, Dongmei; Wang, Shengnan; Huang, Xiaoxian; Wang, Qun

    2016-01-01

    Background Heterotopic ossification (HO) is defined as the formation of true bone tissue in non-osseous tissues. HO may occur under several conditions such as soft tissue injury, central nervous system injury and many other diseases like arthopathies, and vasculopathies. The underlying mechanisms of HO are not well elucidated. Anti-NMDA receptor encephalitis is a newly recognized autoimmune mediated disease which is predominant in young female patients with ovarian teratomas. Encephalitis com...

  6. A Case of Anti-NMDA Receptor Encephalitis Treated with ECT.

    Science.gov (United States)

    Jones, Kristin C; Schwartz, Ann C; Hermida, Adriana P; Kahn, David A

    2015-09-01

    We describe the case of a 17-year-old male who presented with acute onset of seizures and malignant catatonia with psychosis, agitation, and hypermetabolism, who responded to electroconvulsive therapy (ECT). Soon after he began to respond, he was diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis and then given immunosuppressive therapy. Anti-NMDA receptor encephalitis is an increasingly recognized autoimmune disorder that often presents with neuropsychiatric symptoms. The mainstays for treatment have been early diagnosis, tumor work-up and removal if found, and initiation of immunosuppressive therapy. Treatment response is often slow and residual symptoms common. In this case, ECT produced clinical stabilization before the underlying diagnosis of anti-NMDA receptor encephalitis was made and standard treatment initiated. We suggest that ECT may be highly beneficial for stabilizing life-threatening neuropsychiatric symptoms in this syndrome and should be considered as a potentially additive treatment to immunotherapy when rapid relief is sought.

  7. Anti-NMDA receptor encephalitis presenting as atypical anorexia nervosa: an adolescent case report.

    Science.gov (United States)

    Mechelhoff, David; van Noort, Betteke Maria; Weschke, Bernhard; Bachmann, Christian J; Wagner, Christiane; Pfeiffer, Ernst; Winter, Sibylle

    2015-11-01

    Since 2007, more than 600 patients have been diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, with almost 40 % of those affected being children or adolescents. In early phases of the illness, this life-threatening disease is characterized by psychiatric symptoms, such as depression, anxiety, obsessions, hallucinations or delusions. Consequently, a high percentage of patients receive psychiatric diagnoses at first, hindering the crucial early diagnosis and treatment of the anti-NMDA receptor encephalitis. We report on a 15-year-old girl initially presenting with pathological eating behaviour and significant weight loss resulting in an (atypical) anorexia nervosa (AN) diagnosis. Her early course of illness, diagnostic process, treatment and short-term outcome are described. This case report aims to raise awareness about the association between anorectic behaviour and anti-NMDA receptor encephalitis and highlight the importance of multidisciplinary teams in child and adolescent services.

  8. [Two pediatric cases of anti-NMDA receptor antibody encephalitis].

    Science.gov (United States)

    Ben Azoun, M; Tatencloux, S; Deiva, K; Blanc, P

    2014-11-01

    Although less frequent than viral encephalitis, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a frequent form of acute pediatric encephalitis. After a prodromal phase of flu-like symptoms, psychiatric symptoms predominate - agitation, anxiety, hallucinations - and can make correct diagnosis more difficult. Also noted are abnormal dyskinesia and dystonia-like movements, partial seizures, difficulties talking or memorizing, and autonomic manifestations. The presentation of two cases of anti-NMDAR encephalitis illustrates the symptoms of this disease. Although the CSF abnormalities are not highly specific of this disease, and MRI most often normal, EEG shows more specific signs. These observations enable us to discuss different treatment options and understand the progression of this disease.

  9. Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis.

    Science.gov (United States)

    Sühs, Kurt-Wolfram; Wegner, Florian; Skripuletz, Thomas; Trebst, Corinna; Tayeb, Said Ben; Raab, Peter; Stangel, Martin

    2015-10-01

    Anti-N-methyl D-aspartate (NMDA) receptor encephalitis is the most common type of encephalitis in the spectrum of autoimmune encephalitis defined by antibodies targeting neuronal surface antigens. In the present study, the clinical spectrum of this disease is presented using instructive cases in correlation with the anti-NMDA receptor antibody titers in the cerebrospinal fluid (CSF) and serum. A total of 7 female patients admitted to the hospital of Hannover Medical School (Hannover, Germany) between 2008 and 2014 were diagnosed with anti-NMDA receptor encephalitis. Among these patients, 3 cases were selected to illustrate the range of similar and distinct clinical features across the spectrum of the disease and to compare anti-NMDA antibody levels throughout the disease course. All patients received immunosuppressive treatment with methylprednisolone, intravenous immunoglobulin and/or plasmapheresis, followed in the majority of patients by second-line therapy with rituximab and cyclophosphamide. The disease course correlated with NMDA receptor antibody titers, and to a greater extent with the ratio between antibody titer and protein concentration. A favorable clinical outcome with a modified Rankin Scale (mRS) score of ≤1 was achieved in 4 patients, 1 patient had an mRS score of 2 after 3 months of observation only, whereas 2 patients remained severely impaired (mRS score 4). Early and aggressive immunosuppressive treatment appears to support a good clinical outcome; however, the clinical signs and symptoms differ distinctively and treatment decisions have to be made on an individual basis.

  10. [Anti-NMDA Receptor Antibody-Related Encephalitis].

    Science.gov (United States)

    Nagayama, Shigemi; Tanaka, Keiko

    2016-09-01

    Recently, the search for diagnostic antibody markers has drawn considerable attention in relation to autoimmune encephalitis. Among the antibody markers, the most frequently detected is the anti-N-methyl-D-aspartate receptor (NMDAR)antibody. Patients with this antibody develop characteristic clinical features. This disease tends to affect young women, and starts with psychiatric symptoms followed by seizures, involuntary movements, autonomic failure, and respiratory failure. Nearly half of these female patients have ovarian teratoma. Some of the patients with anti-NMDAR antibody show atypical clinical features. Approximately 4% show only psychiatric symptoms, which might lead to a diagnosis of malignant catatonia. Other reports describe patients experiencing refractory seizures to have the anti-NMDAR antibody. Some of the antibody-positive patients are associated with demyelinating disorders, and some develop anti-NMDAR encephalitis after recovery from herpes simplex encephalitis. It is important to test the anti-NMDAR antibody in these groups since immunotherapy ameliorates their symptoms. The anti-NMDAR antibody binds to the constitutional epitope at the extracellular domain of GluN1 and disrupts its function. Early introduction of immunotherapy together with tumor resection will results in improvement of neurological symptoms.

  11. Characterising seizures in anti-NMDA-receptor encephalitis with dynamic causal modelling.

    Science.gov (United States)

    Cooray, Gerald K; Sengupta, Biswa; Douglas, Pamela; Englund, Marita; Wickstrom, Ronny; Friston, Karl

    2015-09-01

    We characterised the pathophysiology of seizure onset in terms of slow fluctuations in synaptic efficacy using EEG in patients with anti-N-methyl-d-aspartate receptor (NMDA-R) encephalitis. EEG recordings were obtained from two female patients with anti-NMDA-R encephalitis with recurrent partial seizures (ages 19 and 31). Focal electrographic seizure activity was localised using an empirical Bayes beamformer. The spectral density of reconstructed source activity was then characterised with dynamic causal modelling (DCM). Eight models were compared for each patient, to evaluate the relative contribution of changes in intrinsic (excitatory and inhibitory) connectivity and endogenous afferent input. Bayesian model comparison established a role for changes in both excitatory and inhibitory connectivity during seizure activity (in addition to changes in the exogenous input). Seizures in both patients were associated with a sequence of changes in inhibitory and excitatory connectivity; a transient increase in inhibitory connectivity followed by a transient increase in excitatory connectivity and a final peak of excitatory-inhibitory balance at seizure offset. These systematic fluctuations in excitatory and inhibitory gain may be characteristic of (anti NMDA-R encephalitis) seizures. We present these results as a case study and replication to motivate analyses of larger patient cohorts, to see whether our findings generalise and further characterise the mechanisms of seizure activity in anti-NMDA-R encephalitis.

  12. EXTREME DELTA BRUSH EEG PATTERN IN A CASE WITH ANTI-NMDA RECEPTOR ENCEPHALITIS.

    Science.gov (United States)

    Söylemez, Elif; Güveli, Betül Tekin; Atakli, Dilek; Yatmazoğlu, Merve; Atay, Turan; Dayan, Cengiz

    2015-09-30

    Anti-N-methyl-D-aspartate receptor NMDA-R encephalitis is caused by antibodies against the NMDA-R and characterized by a severe encephalopathy with psychosis, epileptic seizures and autonomic disturbances. This disorder is often accompanied with malignancies, especially ovarian teratoma. Some patients' EEGs show a different pattern similar to the waveforms of premature infants and this pattern is specifically named as extreme delta brush (EDB). We report a 24-year-old female having anti-NMDA receptor encephalitis and EDB patern.

  13. Malignant catatonia due to anti-NMDA-receptor encephalitis in a 17-year-old girl: case report

    Directory of Open Access Journals (Sweden)

    Vidailhet Marie

    2011-05-01

    Full Text Available Abstract Anti-NMDA-Receptor encephalitis is a severe form of encephalitis that was recently identified in the context of acute neuropsychiatric presentation. Here, we describe the case of a 17-year-old girl referred for an acute mania with psychotic features and a clinical picture deteriorated to a catatonic state. Positive diagnosis of anti-NMDA-receptor encephalitis suggested specific treatment. She improved after plasma exchange and immunosuppressive therapy. Post-cognitive sequelae (memory impairment disappeared within 2-year follow-up and intensive cognitive rehabilitation.

  14. Steroid unresponsive anti-NMDA receptor encephalitis during pregnancy successfully treated with plasmapheresis.

    Science.gov (United States)

    Shahani, Lokesh

    2015-04-29

    Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is an autoimmune disorder resulting in neurological and psychiatric symptoms. It is rare during pregnancy and treatment is extremely challenging as little data exist to guide management. A 26-year-old woman presented at 22 weeks of gestation with intermittent headache and an acute episode of bizarre behaviour and grandiose delusions resulting in hospitalisation. The patient was worked up for encephalitis and was found to have anti-NMDA receptor antibody in cerebrospinal fluid as well as in serum. She was initially treated with high-dose steroids but failed to improve clinically and serologically. She was then treated with plasmapheresis and showed clinical and serological response. She had a successful delivery at 37 weeks and the baby did not show serological evidence of disease. This case adds to the sparse literature of anti-NMDA receptor encephalitis during pregnancy and adds to the differential diagnosis of new onset psychiatric symptoms during pregnancy.

  15. Rhythmic delta activity represents a form of nonconvulsive status epilepticus in anti-NMDA receptor antibody encephalitis.

    Science.gov (United States)

    Kirkpatrick, McNeill P; Clarke, Charles D; Sonmezturk, Hasan H; Abou-Khalil, Bassel

    2011-02-01

    Anti-NMDA receptor antibody encephalitis is a limbic encephalitis with psychiatric manifestations, abnormal movements, coma, and seizures. The coma and abnormal movements are not typically attributed to seizure activity, and slow activity is the most common EEG finding. We report drug-resistant nonconvulsive status epilepticus as the basis for coma in a 19-year-old woman with anti-NMDA receptor antibodies and a mediastinal teratoma. The EEG showed generalized rhythmic delta activity, with evolution in morphology, frequency, and field typical of nonconvulsive status epilepticus. The status was refractory to antiepileptic drugs, repeated drug-induced coma, resection of the tumor, intravenous steroids, rituximab, and plasmapheresis. She awoke after the addition of felbamate, and the rhythmic delta activity ceased. The rhythmic delta activity described with coma in anti-NMDA receptor antibody encephalitis may represent a pattern of status epilepticus in some patients. Felbamate, which has NMDA receptor antagonist activity, should be studied as a therapeutic agent in this condition.

  16. Differences in treatment of anti-NMDA receptor encephalitis: results of a worldwide survey.

    Science.gov (United States)

    Bartolini, Luca; Muscal, Eyal

    2017-02-02

    The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology(®) Clinical Practice to appraise differences in decisions about first- and second-line treatment and timing for initiation of second-line treatment for anti-NMDA receptor encephalitis. 399 participants answered all questions of the survey and were included in the analysis. 261 (65%) were adult neurologists, 86 (22%) were neurologists treating children, and 52 (13%) were pediatric rheumatologists. 179 (45%) responders practiced in the US. The majority agreed on the use of steroids and/or IVIg for first-line therapy and rituximab alone as second line. Differences in initial treatment regimen based on specialty included increased use of plasma exchange by adult neurologists (27%) and rituximab by pediatric rheumatologists (29%) (χ (2)(4) = 27.43, p < 0.001). Trainees opted for plasma exchange (35%) and junior faculty picked rituximab (15%) more as part of first line (χ (2)(4) = 13.37, p = 0.010). There was greater usage of anti-metabolites for second-line therapy outside of the US (15%) (χ (2)(4) = 11.67, p = 0.020). US physicians also utilized second-line treatment earlier than their mostly European counterparts (14 vs. 23% used later than 2 weeks; χ (2)(1) = 4.96, p = 0.026). Although treatment patterns were similar, differences observed across specialties and geographic locations may guide the development of consensus-driven guidelines by multi-disciplinary task forces. These guidelines may promote treatment trials of immunomodulators in autoimmune encephalitides.

  17. Ovarectomy despite Negative Imaging in Anti-NMDA Receptor Encephalitis: Effective Even Late

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    Anna-Lena Boeck

    2013-01-01

    Full Text Available Anti-NMDA receptor (NMDAR encephalitis is an autoimmune antibody-mediated neuropsychiatric disorder. The disorder is known to be associated with ovarian teratoma and predominantly affects young women. Here, we report the case of a 34-year-old woman with anti-NMDAR encephalitis, in which detailed investigations gave no specific hint for an ovarian teratoma. Despite this, and due to a continuous severe clinical syndrome, an ovarectomy was performed and histological examination revealed an occult teratoma. The ovarectomy led to a remarkable improvement even with a long term intensive care treatment for 11 months. The most important lesson to be learned from this instructive case is that even though none of the investigations was indicative for an ovarian teratoma, including an explorative laparoscopy with biopsy, there still may be an occult ovarian teratoma. This shows that tumour search and diagnosis are extremely important in patients presenting with anti-NMDAR encephalitis, and a laparotomy and ovarectomy is justified. Furthermore, removal of the teratoma even 11 months after a very severe course is still therapeutically effective.

  18. Anti-NMDA (a-NMDAR) receptor encephalitis related to acute consumption of metamphetamine: Relevance of differential diagnosis.

    Science.gov (United States)

    Iriondo, O; Zaldibar-Gerrikagoitia, J; Rodríguez, T; García, J M; Aguilera, L

    2017-03-01

    A 19-year-old male came to the Emergency Room of our hospital due to an episode of dystonic movements and disorientation 4 days after consuming methamphetamine, which evolved to a catatonic frank syndrome and eventually to status epilepticus. Definitive diagnosis was anti-NMDA receptor encephalitis, an acute inflammation of the limbic area of autoimmune origin in which early diagnosis and treatment are key elements for the final outcome. In this case, initial normal tests and previous methamphetamine poisoning delayed diagnosis, because inhaled-methamphetamine poisoning causes similar clinical symptoms to anti-NMDA receptor encephalitis. Methamphetamine poisoning may have caused an immune response in the patient, bringing on the progress of the pathology.

  19. Approach to the Management of Pediatric-Onset Anti-N-Methyl-d-Aspartate (Anti-NMDA) Receptor Encephalitis: A Case Series.

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    Brenton, J Nicholas; Kim, Joshua; Schwartz, Richard H

    2016-08-01

    Anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis is a treatable cause of autoimmune encephalitis. It remains unclear if the natural history of this disease is altered by choice of acute therapy or the employment of chronic immunotherapy. Chart review was undertaken for pediatric patients diagnosed with anti-NMDA receptor encephalitis. Data obtained included patient demographics, disease manifestations, treatment course, and clinical outcomes. Ten patients with anti-NMDA receptor encephalitis were identified. All patients were treated with immunotherapy in the acute period, and all patients experienced good recovery. Neurologic relapse did not occur in any patient. All patients received varied forms of chronic immunosuppression to prevent relapses. Complications of chronic immunotherapy occurred in 50% of patients. The benefits of chronic immunotherapy and the duration of use should be carefully weighed against the risks. Complications from immunotherapy are not uncommon and can be serious. Clinical trials assessing the benefit of long-term immunotherapy in this population are needed.

  20. [First occurrence of an organic manic schizophreniform syndrome followed by catatonia induced by anti-NMDA-receptor encephalitis].

    Science.gov (United States)

    Fousse, M; Becker, C; Faßbender, K; Reith, W; Körner, H; Alexandrou, M; Spiegel, J

    2013-04-01

    We report on a 39-year-old female patient who developed catatonia after there had been schizomanic symptoms in the six months before. At admission the patient exhibited catatonia, a tetraspastic syndrome and focal epileptic seizures. The cranial MRI revealed bilateral subcortical hyperintense lesions which took up contrast agent. Examination of the cerebrospinal fluid disclosed a lymphocytic pleocytosis and autochthone oligoclonal bands. In the serum autoantibodies against the NMDA-NR-1 receptor were reproducibly detected. A detailed search for a tumour was negative. In detail, we could exclude a neoplasm of the ovaries which is often present in the paraneoplastic type of anti-NMDA-receptor encephalitis. Therefore we assume an autoimmune, not paraneoplastic, encephalitis in our patient. The symptoms improved significantly after an immunosuppressive therapy - initially with glucocorticoids followed by rituximab - had been initiated. This case illustrates that an autoimmune encephalitis should be looked for when first psychotic symptoms occur.

  1. Longitudinal electroencephalographic (EEG) findings in pediatric anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis: the Padua experience.

    Science.gov (United States)

    Nosadini, Margherita; Boniver, Clementina; Zuliani, Luigi; de Palma, Luca; Cainelli, Elisa; Battistella, Pier Antonio; Toldo, Irene; Suppiej, Agnese; Sartori, Stefano

    2015-02-01

    To contribute to characterize electroencephalographic (EEG) activity in pediatric anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis, we reviewed electroclinical data of 5 children with anti-NMDA receptor encephalitis diagnosed in our department. We identified 4 longitudinal electroencephalographic phases: in the early phase, background activity was normal, with intermixed nonreactive slow waves; in the florid phase, background activity deteriorated with appearance of sequences of peculiar rhythmic theta and/or delta activity unrelated to clinical changes, unresponsive to stimuli and antiepileptic medications; in the recovery phase, these sequences decreased and reactive posterior rhythm re-emerged; electroencephalogram normalized 2 to 5 months after onset. In conclusion, in the presence of evocative clinical history, recognizing a characteristic longitudinal electroencephalographic activity could provide ancillary aspects addressing the diagnosis and the overall management of children with anti-N-methyl-d-aspartate receptor encephalitis; in particular, knowing that peculiar and recurrent paroxysmal nonepileptic rhythmic theta-delta patterns can occur in these patients could help distinguish paroxysmal epileptic and nonepileptic electroencephalographic activity.

  2. Anti-NMDA Receptor Encephalitis in a Patient with Previous Psychosis and Neurological Abnormalities: A Diagnostic Challenge

    Directory of Open Access Journals (Sweden)

    R. David Heekin

    2015-01-01

    Full Text Available Anti-N-methyl-D-aspartate (NMDA receptor encephalitis is an autoimmune disorder characterized by IgG autoantibodies directed against the NR1 subunit of the NMDA glutamate receptor. Psychiatric symptoms are common and include psychosis, mania, depressed mood, aggression, and speech abnormalities. Neurological symptoms such as seizures, decreased responsiveness, dyskinesias, and other movement abnormalities and/or autonomic instability are frequently seen as well. We present the case of a woman who was followed up at our facility for over 14 years for the treatment of multiple neuropsychiatric symptoms. Initially, she presented with paresthesias, memory loss, and manic symptoms. Nine years later, she presented to our facility again, this time with left sided numbness, left eyelid droop, and word finding difficulties. Finally, five years later, she presented with manic symptoms, hallucinations, and memory impairment. During her hospitalization, she subsequently developed catatonic symptoms and seizures. During her stay, it was discovered that she was positive for anti-NMDA receptor antibodies and her symptoms responded well to appropriate therapy. This case demonstrates that it may be useful for clinicians to consider screening for anti-NMDA receptor antibodies in long-term patients with neuropsychiatric symptoms that have not adequately responded to therapy.

  3. Clinical analysis of anti NMDA receptor encephalitis%抗NMDA受体脑炎的临床分析

    Institute of Scientific and Technical Information of China (English)

    吕爱红; 陈运转; 程巧玫

    2016-01-01

    Objective To investigate the clinical features EEG,imaging characteristics and treatment of patients with anti-NMDA receptor encephalitis,in order to guide clinical work.Methods Retrospectively analyzed the clinical features,electroencephalogram(EEG),cerebrospinal fluid,imaging characteristics and therapeutic effect of 8 patients with anti-NMDA receptor encephalitis.Results Seven cases(87.5%) had mental symptoms,6 cases(75.0%) had memory drops,4 cases(50.0%) had epileptic seizure,and 2 cases (25.0%) appeared Dyskineia; 6 cases of abnormal Electroencephalogram (EEG) were abnormal; 8 cases of cranial Magnetic resonance imaging(MRI) were abnormal; 5 cases of abnormal cerebrospinal fluid.Immune treatment of 8 cases achieved obvious curative effect.Conclusions Anti-NMDA receptor encephalitis is autoimmune reaction resistance encephalitis and clinical manifestations lack specificity,so timely electroencephalography(EEG),cranial magnetic resonance imaging and cerebrospinal fluid examination is helpful to early diagnosis,and early immune treatment can obtain exact curative effect.%目的 探讨抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患者的临床特征、脑电图、影像学特征及治疗情况,进一步指导临床工作.方法 回顾性分析8例抗NMDA受体脑炎患者的临床特征、脑电图、脑脊液及影像学特征和治疗效果.结果 7例(87.5%)出现精神症状,6例(75.0%)出现记忆力下降,4例(50.0%)出现癫痫发作,2例(25.0%)出现异动征;6例脑电图异常;8例头颅MRI均有异常;5例脑脊液异常.8例患者应用免疫治疗均取得了明显的疗效.结论 抗NMDA受体脑炎是自身免疫反应性脑炎,临床表现缺乏特异性,及时行脑电图、头颅MRI及脑脊液检查有助于早期诊断,早期免疫治疗可获得确切疗效.

  4. Anti-N-methyl-D-aspartate (anti-NMDA) receptor antibody encephalitis in a male adolescent with a large mediastinal teratoma.

    Science.gov (United States)

    Sommeling, Charlotte; Santens, Patrick

    2014-05-01

    We present a case of a 16-year-old boy with Klinefelter syndrome who presented with a syndrome of impaired alertness, orofacial dyskinesias, choreiform movements, epileptic seizures, and autonomic instability, pointing to a diagnosis of anti-N-methyl-Daspartate (anti-NMDA) receptor antibody encephalitis.

  5. Antecedent anti-NMDA receptor encephalitis in two patients with multiple sclerosis.

    Science.gov (United States)

    Baheerathan, A; Brownlee, W J; Chard, D T; Shields, K; Gregory, R; Trip, S A

    2017-02-01

    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD). In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders.

  6. Anti-NMDA receptor encephalitis: an easily missed diagnosis in older patients.

    Science.gov (United States)

    Rainey, Katie; Gholkar, Bethan; Cheesman, Mark

    2014-09-01

    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an important, treatable cause of encephalitis which remains under-recognised despite a growing body of the literature [1]. It is an immune-mediated syndrome which presents with a variety of neurological symptoms including headache, fever, personality change and seizures. Most case reports to date are of young adults, it is much less frequently reported in older adults. The syndrome has been associated with ovarian teratomas. The prognosis is good with early recognition and treatment, though may relapse. We present a case of NMDA encephalitis in an elderly patient who responded well to immunosuppressive therapy.

  7. A case of anti-NMDA receptor encephalitis with ADEM-like clinical/MR ifndings

    Institute of Scientific and Technical Information of China (English)

    Jia Liu; Huan Yi; Li Xu; Min Li; Xuan Wang; Fu-Hua Peng

    2016-01-01

    In recent years, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis overlapping with demyelinating disorders has attracted more and more attention. The case is about a 52-year-old woman with anti-NMDAR encephalitis presenting acute disseminated encephalomyelitis (ADEM)-like clinical/magnetic resonance (MR) ifndings. Here, the authors report this case and brielfy review her MR evolution and the conditions of her prognosis. The recognition that patients with anti-NMDAR encephalitis may have demyelinating disorders, simultaneously or sequentially, is important. Otherwise, a high dose of steroid treatment with several courses could obtain good effect, even if given in the late phase.

  8. Anti-NMDA Receptor Encephalitis Presenting as an Acute Psychotic Episode in a Young Woman: An Underdiagnosed yet Treatable Disorder

    Directory of Open Access Journals (Sweden)

    Shikma Keller

    2014-01-01

    Full Text Available Anti-NMDA receptor (NMDAR encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. In female patients an ovarian teratoma is often identified. We describe a 32-year-old woman who presented with acute psychosis. Shortly after admission, she developed generalized seizures and deteriorated into a catatonic state. Although ancillary tests including MRI, electroencephalogram, and cerebrospinal fluid (CSF analysis were unremarkable, the presentation of acute psychosis in combination with recurrent seizures and a relentless course suggested autoimmune encephalitis. The patient underwent pelvic ultrasound which disclosed a dermoid cyst and which led to an urgent cystectomy. Plasmapheresis was then initiated, yielding partial response over the next two weeks. Following the detection of high titers of anti-NMDAR antibodies in the CSF, the patient ultimately received second line immunosuppressive treatment with rituximab. Over several months of cognitive rehabilitation a profound improvement was eventually noted, although minor anterograde memory deficits remained. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.

  9. Anti-NMDA receptor encephalitis associated with transient cerebral dyschromatopsia, prosopagnosia, and lack of stereopsis.

    Science.gov (United States)

    Sawamura, Hiromasa; Yamamoto, Tomotaka; Ohtomo, Ryo; Bannai, Taro; Wakakura, Masato; Tsuji, Shoji

    2014-06-01

    A 20-year-old woman suffered from anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and was treated with removal of an ovarian teratoma and retroperitoneal ganglioneuroma in addition to immunotherapy. She was incapable of face recognition, had difficulty with object recognition, and lacked color sensation and stereo perception during recovery. These symptoms were transient and completely resolved over 4 months. Our report documents additional aspects of visual impairment associated with anti-NMDAR encephalitis and suggests that the disease can lead to diffuse cerebral dysfunction including the cortical visual system.

  10. Anti-NMDA-receptor antibody detected in encephalitis, schizophrenia, and narcolepsy with psychotic features

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    Tsutsui Ko

    2012-05-01

    Full Text Available Abstract Background Causative role of encephalitis in major psychotic features, dyskinesias (particularly orofacial, seizures, and autonomic and respiratory changes has been recently emphasized. These symptoms often occur in young females with ovarian teratomas and are frequently associated with serum and CSF autoantibodies to the NMDA receptor (NMDAR. Methods The study included a total of 61 patients from age 15 to 61 and was carried out between January 1, 2005, and Dec 31, 2010. The patients were divided into the following three clinical groups for comparison. Group A; Patients with typical clinical characteristics of anti-NMDAR encephalitis. Group B; Patients with narcolepsy with severe psychosis. Group C; Patients with schizophrenia or schizo-affective disorders. Results Ten out of 61 cases were anti-NMDAR antibody positive in typical encephalitis cases (group A: 3 of 5 cases and cases in a broader range of psychiatric disorders including narcolepsy (group B: 3 of 5 cases and schizophrenia (group C: 4 of 51 cases. Conclusion In addition to 3 typical cases, we found 7 cases with anti-NMDAR antibody associated with various psychotic and sleep symptoms, which lack any noticeable clinical signs of encephalitis (seizures and autonomic symptoms throughout the course of the disease episodes; this result suggest that further discussion on the nosology and pathophysiology of autoimmune-mediated atypical psychosis and sleep disorders is required.

  11. Anti-NMDA receptor encephalitis: a cause of acute psychosis and catatonia.

    Science.gov (United States)

    Ryan, Stephen A; Costello, Daniel J; Cassidy, Eugene M; Brown, Gemma; Harrington, Hugh J; Markx, Sander

    2013-03-01

    Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a newly described form of encephalitis associated with prominent psychiatric symptoms at onset. Recognition of the symptom complex is the key to diagnosis. Most patients with anti-NMDAR encephalitis develop a multistage illness that progresses from initial psychiatric symptoms to memory disturbance, seizures, dyskinesia, and catatonia. Psychiatric manifestations include anxiety, mania, social withdrawal, and psychosis (i.e., delusions, hallucinations, disorganized behavior). The disorder is more common in females (80%), in approximately half of whom it is associated with an underlying ovarian teratoma. Treatment involves immunosuppression, with steroids and intravenous immunoglobulin considered first line. The disorder is particularly relevant to psychiatrists, because most patients are initially seen by psychiatric services. Psychiatrists should consider anti-NMDAR encephalitis in patients presenting with psychosis as well as dyskinesia, seizures, and/or catatonia, especially if there is no history of a psychiatric disorder. We present the case of a 37-year-old woman who demonstrated many of the key clinical features of this potentially treatable disorder.

  12. EEG Findings May Serve as a Potential Biomarker for Anti-NMDA Receptor Encephalitis.

    Science.gov (United States)

    Foff, Erin Pennock; Taplinger, David; Suski, Joanna; Lopes, M Beatriz S; Quigg, Mark

    2017-01-01

    Objective To determine if an electroencephalographic (EEG) characteristic, beta:delta power ratio (BDPR), is significantly higher for N-methyl-d-aspartate receptor encephalitis (NMDARE) patients than for non-NMDARE patients on presenting EEG. Identification of an additional EEG biomarker with significant specificity for NMDARE (in the absence of frank delta brush) could potentially allow for early identification of at-risk patients. Methods Single center retrospective comparison of NMDARE and non-NMDARE consecutive cases of encephalitis, collated over a 6-year period (from 2008 to 2014). Results None of the 10 NMDARE patients displayed the extreme delta brush pattern on EEG previously described, but the ratio of BDPR was significantly higher for NMDARE patients (P < .005). There was no significant relationship between BDPR and the time of recording from symptom onset. Additional analysis of clinical characteristics also indicated that the patients with NMDARE (median age 19.5 years) were younger than the 5 patients with non-NMDARE (median age 36 years). Encephalopathy, seizure, and psychiatric complaints were the most common diagnoses at time of first health care presentation and did not favor a single etiology, though the latter was present only in the NMDARE population (50% at T0). Prodromal illness featuring headache was more common in the non-NMDARE population. Outcomes, as measured by the Modified Rankin Scale, were globally better in the NMDARE group. Conclusions Patients with NMDARE had a significantly higher BDPR on EEG when compared with non-NMDARE patients even in the absence of extreme delta brush. This suggests that early EEG characteristics may be helpful in distinguishing NMDARE from non-NMDARE.

  13. Anti-NMDA-R encephalitis: Follow-up of 24 months

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    Emilia Maria Veloso Soares

    Full Text Available ABSTRACT Anti-N-methyl-D-aspartate receptor (anti-NMDA-R encephalitis is the second-most-common cause of autoimmune encephalitis, based on epidemiological studies. It has been predominantly described in young females, with prominent psychiatric symptoms, memory loss, decrease in level of consciousness, epilepsy, and central hypoventilation. The condition is commonly associated with mature ovarian teratomas. We describe a video report with a classic presentation of anti-NMDA-R encephalitis in a young patient with no identifiable tumor. Anti-NMDA encephalitis is a recognizable and treatable illness. The prognosis of patients depends on early diagnosis, implementation of appropriate immunomodulatory therapy and, in paraneoplastic cases, complete tumor removal. Clinicians should be wary of this condition, especially when assessing patients with recent onset of psychiatric symptoms unresponsive to antipsychotic treatment.

  14. Stereotactic injection of cerebrospinal fluid from anti-NMDA receptor encephalitis into rat dentate gyrus impairs NMDA receptor function.

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    Würdemann, Till; Kersten, Maxi; Tokay, Tursonjan; Guli, Xiati; Kober, Maria; Rohde, Marco; Porath, Katrin; Sellmann, Tina; Bien, Christian G; Köhling, Rüdiger; Kirschstein, Timo

    2016-02-15

    Autoimmune encephalitis is increasingly recognized in patients with otherwise unexplained encephalopathy with epilepsy. Among these, patients with anti-N-methyl D-aspartate receptor (NMDAR) encephalitis present epileptic seizures, memory deficits, and psychiatric symptoms. However, the functional consequences of such autoantibodies are poorly understood. In order to investigate the pathophysiology of this disease, we stereotactically injected either cerebrospinal fluid (CSF) from three anti-NMDAR encephalitis patients or commercially available anti-NMDAR1 into the dentate gyrus of adult female rats. Control animals were injected with either CSF obtained from three epilepsy patients (ganglioglioma, posttraumatic epilepsy, focal cortical dysplasia) lacking anti-NMDAR or saline. Intracellular recordings from dentate gyrus granule cells showed a significant reduction of the NMDAR-evoked excitatory postsynaptic potentials (NMDAR-EPSPs) in animals treated with anti-NMDAR. As a consequence of this, action potential firing in these cells by NMDAR-EPSPs was significantly impaired. Long-term potentiation in the dentate gyrus was also significantly reduced in rats injected with anti-NMDAR as compared to control animals. This was accompanied by a significantly impaired learning performance in the Morris water maze hidden platform task when the animals had been injected with anti-NMDAR antibody-containing CSF. Our findings suggest that anti-NMDAR lead to reduced NMDAR function in vivo which could contribute to the memory impairment found in patients with anti-NMDAR encephalitis.

  15. Early and persistent ‘extreme delta brush’ in a patient with anti-NMDA receptor encephalitis

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    Stephen VanHaerents

    2014-01-01

    Full Text Available Since its original description in 2007, anti-N-methyl-d-aspartate receptor (anti-NMDAR encephalitis associated with an ovarian teratoma is an increasingly recognized etiology of previously unexplained encephalopathy and encephalitis. Extreme delta brush (EDB is a novel electroencephalogram (EEG finding seen in many patients with anti-NMDAR encephalitis. The presence of this pattern is associated with a more prolonged illness, although the specificity of this pattern is unclear. Additionally, the frequency and sensitivity of EDB in anti-NMDAR encephalitis and its implications for outcome have yet to be determined. We report a patient with early evidence of extreme delta brush and persistence of this pattern 17.5 weeks later with little clinical improvement.

  16. A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea.

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    Kim, Eun-Hee; Kim, Yeo Jin; Ko, Tae-Sung; Yum, Mi-Sun; Lee, Jun Hwa

    2016-11-01

    Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

  17. Acute psychosis due to non-paraneoplastic anti-NMDA-receptor encephalitis in a teenage girl: Case report.

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    Kramina, Sandra; Kevere, Laura; Bezborodovs, Nikita; Purvina, Santa; Rozentals, Guntis; Strautmanis, Jurgis; Viksna, Zane

    2015-12-01

    Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a disease occurring when antibodies produced by the body's own immune system attack NMDA-type glutamate receptors in the brain. Most anti-NMDAR encephalitis cases are associated with paraneoplastic syndrome. We analyze the case of a 15-year-old girl who was hospitalized in a child psychiatry clinic in Riga, Latvia, with de novo acute polymorphic psychotic disorder gradually progressing to a catatonic state. The patient received antipsychotic and electroconvulsive therapy with no beneficial effect. The council of doctors discussed differential diagnoses of schizophrenia-induced catatonia and the autoimmune limbic encephalitis-induced catatonic condition. When the diagnosis of anti-NMDAR autoimmune encephalitis was finally confirmed by repeated immunological assays (specific immunoglobulin [Ig] G and IgM in her blood serum and cerebrospinal fluid), and a paraneoplastic process was ruled out, she was started on immunomodulating therapy (methylprednisolone, Ig, plasmapheresis, rituximab), which changed the course of her disease. On immunomodulating treatment, her physical and mental health have gradually improved to almost complete reconvalescence. Psychiatrists should consider anti-NMDAR encephalitis as a differential diagnosis in first-episode psychosis patients presenting with disorientation, disturbed consciousness, pronounced cognitive deficits, movement disorder, dysautonomia, or rapid deterioration, and test for specific IgG NR1 autoantibodies, even if there are no specific findings on routine neuroimaging, electroencephalography (EEG), or cerebrospinal fluid tests.

  18. Anti-NMDA-receptor encephalitis presenting with catatonia and neuroleptic malignant syndrome in patients with intellectual disability and autism.

    Science.gov (United States)

    Kiani, Reza; Lawden, Mark; Eames, Penelope; Critchley, Peter; Bhaumik, Sabyasachi; Odedra, Sunita; Gumber, Rohit

    2015-02-01

    We report anti-N-methyl-d-aspartate (NMDA) receptor encephalitis in two patients with autism and intellectual disability presenting with neuropsychiatric symptoms of catatonia and neuroleptic malignant syndrome. Case reports such as these help raise awareness of this clinical issue. By paving the way for earlier diagnoses they ultimately maximise the potential for curative treatments and prevention of long-term complications.

  19. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: A retrospective study

    NARCIS (Netherlands)

    N. Gresa-Arribas (Nuria); M.J. Titulaer (Maarten); A. Torrents (Abiguei); H. Aguilar (Helena); L. McCracken (Lindsey); F. Leypoldt (Frank); A.J. Gleichman (Amy); R. Balice-Gordon (Rita); M.R. Rosenfeld (Myrna); D. Lynch (David); F. Graus (Francesc); J. Dalmau (Josep)

    2014-01-01

    textabstractBackground: Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a severe but treatable autoimmune disorder which diagnosis depends on sensitive and specific antibody testing. We aimed to assess the sensitivity and specificity of serum and CSF antibody testing in patients with anti-

  20. An Atypical Case of Anti-NMDA Receptor Encephalitis: Predominant Parkinsonism and Persisting Micrographia without Oro-facial Dyskinesia.

    Science.gov (United States)

    Kadoya, Masato; Kadoya, Akiko; Onoue, Hiroyuki; Ikewaki, Katsunori; Kaida, Kenichi

    2015-01-01

    We describe the case of a 46-year-old man with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with prominent parkinsonism. The patient presented with psychiatric symptoms followed by epileptic seizure and parkinsonism including micrographia. Magnetic resonance imaging (MRI) revealed lesions in the bilateral medial temporal lobes and basal ganglia on fluid-attenuated inversion recovery images. His symptoms and MRI findings were ameliorated by immunotherapy but then relapsed. After retreatment, his parkinsonism gradually improved except for the micrographia. This is an atypical case of anti-NMDAR encephalitis in that the patient showed prominent and refractory parkinsonism, thus indicating that the clinical diversity of anti-NMDAR encephalitis is greater than expected.

  1. In vivo effects of antibodies from patients with anti-NMDA receptor encephalitis: further evidence of synaptic glutamatergic dysfunction

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    Manto Mario

    2010-11-01

    Full Text Available Abstract Background A severe encephalitis that associates with auto-antibodies to the NR1 subunit of the NMDA receptor (NMDA-R was recently reported. Patients' antibodies cause a decrease of the density of NMDA-R and synaptic mediated currents, but the in vivo effects on the extracellular glutamate and glutamatergic transmission are unknown. Methods We investigated the acute metabolic effects of patients' CSF and purified IgG injected in vivo. Injections were performed in CA1 area of Ammon's horn and in premotor cortex in rats. Results Patient's CSF increased the concentrations of glutamate in the extracellular space. The increase was dose-dependent and was dramatic with purified IgG. Patients' CSF impaired both the NMDA- and the AMPA-mediated synaptic regulation of glutamate, and did not affect the glial transport of glutamate. Blockade of GABA-A receptors was associated with a marked elevation of extra-cellular levels of glutamate following a pretreatment with patients' CSF. Conclusion These results support a direct role of NMDA-R antibodies upon altering glutamatergic transmission. Furthermore, we provide additional evidence in vivo that NMDA-R antibodies deregulate the glutamatergic pathways and that the encephalitis associated with these antibodies is an auto-immune synaptic disorder.

  2. VNS terminating refractory nonconvulsive SE secondary to anti-NMDA encephalitis: A case report

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    Taoufik Alsaadi

    2015-01-01

    Full Text Available Anti-NMDA receptor encephalitis (ANRE has been previously reported as a rare cause of nonconvulsive status epilepticus (NCSE. Vagus nerve stimulation (VNS is generally considered as a palliative treatment for patients with drug-resistant partial-onset epilepsy. Here, we report a case of refractory NCSE that was terminated after vagus nerve stimulator implantation. To our knowledge, similar cases have not been reported previously.

  3. Misdiagnosis Analysis of Anti-NMDA Receptor Encephalitis (30 Cases)%抗NMDA受体脑炎30例误诊分析

    Institute of Scientific and Technical Information of China (English)

    滕丽华; 王仲

    2015-01-01

    involuntary movement, fever, headache, consciousness. Expet 1 confirmed case by PUMC hospital, 29 cases were misdiagnosed at other local hospitals as viral encephali-tis, antiviral treatment, mental illness and epilepsy, or poor epilepsy therapy effect, or disease progression positive test of anti NMDA antibody confirmed diagnosis, and the first visit misdiagnosis rate was 100%, and the diagnosis time was (270. 15 ± 114. 97)d. Corticosteroids and gamma globulin and immunosuppressant therapy was given, and in case of merger teratoma, sur-gical resection was applied. There was a certain correlation between prognosis and progression. Conclusion Clinicians should be aware of common early clinical manifestations of NMDA receptor encephalitis, early NMDA receptor antibody screening is rec-ommended in high-risk groups for differential diagnosis as the conventional detection method in order to give early diagnosis and early treatment for patients for better prognosis.

  4. Anti-NMDA-R encephalitis: Should we consider extreme delta brush as electrical status epilepticus?

    Science.gov (United States)

    Chanson, Eve; Bicilli, Élodie; Lauxerois, Michel; Kauffmann, Sophie; Chabanne, Russell; Ducray, François; Honnorat, Jérome; Clavelou, Pierre; Rosenberg, Sarah

    2016-02-01

    Seizures are common clinical manifestations in anti-N-methyl-d-aspartate receptor (anti-NMDA-R) encephalitis, among other neurological and psychiatric symptoms. During the course of the disease, some specific EEG patterns have been described: generalized rhythmic delta activity (GRDA) and extreme delta brush (EDB). In comatose patients, the association of these EEG abnormalities with subtle motor manifestations can suggest ongoing non-convulsive status epilepticus (NCSE). We report the case of a 28-year-old woman admitted for a clinical presentation typical of anti-NMDA-R encephalitis, which was confirmed by CSF analysis. She was rapidly intubated because of severe dysautonomia and disturbed consciousness. Clinical examination revealed subtle paroxysmal and intermittent myoclonic and tonic movements, correlated on video-EEG with GRDA and/or EDB. NCSE was then suspected, but electroclinical manifestations persisted despite many anti-epileptic drugs combinations, or reappeared when barbiturate anesthesia was decreased. In order to confirm or dismiss the diagnosis, intracranial pressure (ICP) and surface video-EEG monitoring were performed simultaneously and revealed no ICP increase, thus being strongly against a diagnosis of seizures. Sedation was progressively weaned, and clinical condition as well as EEG appearance progressively improved. Literature review revealed 11 similar cases, including 2 with focal NCSE. Of the nine other cases, NCSE diagnosis was finally excluded in 5 cases. NCSE diagnosis in association with anti-NMDA-R encephalitis is sometimes very difficult and its occurrence might be overestimated. Video-EEG is highly recommended and more invasive techniques may sometimes be necessary.

  5. Safety of plasma exchange therapy in patients with anti-NMDA receptor encephalitis%血浆置换治疗抗N-甲基-M-天冬氨酸受体脑炎的安全性分析

    Institute of Scientific and Technical Information of China (English)

    张艳; 高岱佺; 叶红; 宿英英

    2015-01-01

    目的 探讨血浆置换(PE)治疗抗N-甲基-M-天冬氨酸(NMDA)受体脑炎的安全性和可操作性.方法 前瞻性观察研究2012年1月至2015年1月收入宣武医院神经内科监护病房的抗NMDA受体脑炎患者PE治疗的不良反应和疗效.结果 共纳入9例患者接受PE治疗54次,全部(100%)患者经过PE治疗后CSF和(或)血清中NMDA受体抗体滴度均有减低或转为阴性,共5例(55.5%)患者临床好转.PE不良反应依次为:不自主运动增加、低血压、PE管路凝血、体温升高、过敏反应和深静脉置管处管口周围渗血.无致死的严重不良反应.结论 PE可有效治疗抗NMDA受体脑炎,并且安全可行.%Objective To explore the safety of plasma exchange (PE) therapy in patients with anti-NMDA receptor encephalitis.Methods Prospective reviews were conducted for the adverse events and therapeutic efficacies of PE therapy in 9 patients with anti-NMDA receptor encephalitis admitted into neurointensive care unit from January 2012 to January 2015.Results A total of 54 PE procedures were performed.The NMDA receptor antibody titer of cerebrospinal fluid (CSF) and/or sera in 9 patients (100%) decreased or turned negative.Five patients (55.5%) demonstrated clinical improvement after PE therapy.And clinical complications included frequent involuntary movements,hypotension,coagulation of PE channel,febricity,allergic response and errhysis around peripheral venous access.Conclusion PE is effective,safe and well-tolerated in patients with anti-NMDA receptor encephalitis.

  6. 畸胎瘤相关性抗NMDA受体脑炎文献复习--附1例报告%Report of one cases of anti-NMDA receptor encephalitis associated with the teratoid tumors and literatures review

    Institute of Scientific and Technical Information of China (English)

    冷慧; 梁乐; 付静; 刘潜

    2016-01-01

    Objective: To study the clinicopathological features, pathogenesis and the main points of diagnosis of the NMDA receptor encephalitis with teratoma.Methods: 1 case of anti-NMDA receptor encephalitis with teratoma of ovary was collected clinical data of patient from Beijing Haidian Hospital in 2014, using immunohistochemical EnVision method and reviewing the pertinent literatures and assembling detailed clinical pathological dates of 11 cases the NMDA receptor encephalitis with teratoma analyze the common pathological characteristics of 12 cases.Results:All 12 cases, 11 youngers, were female who accompanied with abnormal nerve behavior and epileptic seizure, and 6 cases of the teratoid tumors contain the nerve. hTe antibody of anti-NMDA receptor in cerebrospinal lfuid and serum were positive in 11 cases. EEG and MRI detection had no speciifcities. hTese patients were treated with tumor resection and immunoglobulin therapy, 9 showed a good prognosis, 1 recovered, and 1 died.Conclusion:Anti-NMDA receptor encephalitis associated with teratoma was autoimmune encephalitis induced by teratoma, which may be related with neural elements. Positive detection of the antibody of anti-NMDA receptor in cerebrospinal fluid and serum can be diagnosed. Early detection and removal of the tumor can help the patient to cure.%目的:探讨畸胎瘤相关性抗N-甲基-D天冬氨酸(N-methyl-D-aspartate,NMDA)受体脑炎的临床病理特征、发病机制及诊断要点。方法:收集2014年北京市海淀医院病理科卵巢畸胎瘤相关性抗NMDA受体脑炎1例,结合HE形态及免疫组织化学染色,分析畸胎瘤内组织成分,复习国内相关文献,收集其中有全面临床病理资料的畸胎瘤相关性抗NMDA受体脑炎共11例,研究所有12例的临床病理学共性。结果:12例病人均为女性,11例为青年,临床表现均出现精神行为异常及癫痫发作,6例畸胎瘤中含有神经成分,11例脑脊液或血清中检测

  7. A patient with a long history of relapsing psychosis and mania presenting with anti-NMDA receptor encephalitis ten years after first episode

    Directory of Open Access Journals (Sweden)

    Mateus Mistieri Simabukuro

    Full Text Available Anti-N-methyl- D-aspartate receptor (NMDAR encephalitis is a recently discovered autoimmune disorder, in which antibodies target NMDARs in the brain, leading to their removal from synapses. Early in the disease course, patients often present with marked psychosis and mood disturbances (i.e. mania, depression, explaining why most of these patients are first seen by psychiatrists. Hence, autoimmune encephalitis is receiving growing attention from psychiatry, mainly owing to concerns over misdiagnosing immunomediated and potentially curable disorders as primary psychiatric disorders, such as schizophrenia or major depressive disorder. Although anti-NMDAR encephalitis occurs in the context of new-onset psychiatric symptoms, there is a lack of information on differential diagnosis and treatment of this disorder after a long-term diagnostic history of functional psychiatric disorders. We report a case of a patient with a long history of bipolar affective disorder evolving with anti-NMDAR encephalitis, initially misdiagnosed as non-organic psychosis.

  8. [Anti-Ma2, anti-NMDA-receptor and anti-GluRε2 limbic encephalitis with testicular seminoma: short-term memory disturbance].

    Science.gov (United States)

    Kubota, Akihiro; Tajima, Takashi; Narukawa, Shinya; Yamazato, Masamizu; Fukaura, Hikoaki; Takahashi, Yukitoshi; Tanaka, Keiko; Shimizu, Jun; Nomura, Kyoichi

    2012-01-01

    A 36-year-old man presented with cognitive impairment and disturbance of short-term memory functions with character change. Cerebrospinal fluid analysis revealed no abnormalities; however, brain MRI revealed high-signal intensity from bilateral hippocampus lesions on fluid attenuated inversion recovery (FLAIR) images and T(2) weighted images. The 18F-fluorodeoxyglucose PET demonstrated high glucose uptake in the bilateral hippocampus lesions. He was diagnosed as limbic encephalitis, and was administered high-dose intravenous methylprednisolone and immune adsorption plasma therapy followed by intravenous immunoglobulin therapy. MRI abnormalities improved after treatment but recent memory disturbance remained. Ma2 antibody, NMDA-receptor antibody, and GluRε2 antibody were positive. Eleven months atter the onset of disease, the tumor was identified in left testicle by ultrasound and removed the tumor. The pathological findings were seminoma. We experienced a case of paraneoplastic limbic encephalitis associated with seminoma with short-term memory disturbance. The occurrence of paraneoplastic limbic encephalitis with antibodies against cell membrane (NMDA-receptor antibody and GluRε2 antibody) and intracellular (Ma2 antibody) is rare even in the literature.

  9. Successful treatment of anti-NMDA receptor encephalitis with a prompt ovarian tumour removal and prolonged course of plasmapheresis: A case report

    Science.gov (United States)

    Rypulak, Elzbieta; Borys, Michal; Piwowarczyk, Pawel; Fijalkowska, Magdalena; Potrec, Beata; Sysiak, Justyna; Spustek, Janusz; Bartkowska-Sniatkowska, Alicja; Kotarski, Jan; Turski, Waldemar A.; Rejdak, Konrad; Czuczwar, Miroslaw

    2016-01-01

    Anti-N-methyl-d-aspartate-receptor (NMDAR) encephalitis is an uncommon autoimmune disorder with a wide spectrum of neuropsychiatric symptoms. There is a great requirement to emphasize the importance of a multidisciplinary team approach in the process of diagnosis and treatment of the potentially fatal condition, including psychiatrists, neurologists, gynaecologists and intensivists. Physicians must be aware that psychiatric and neurological disorders, which are typical features for NMDAR encephalitis in young women with ovarian tumours, may progress into status epilepticus and respiratory insufficiency. This disease can only be successfully treated with prompt surgical intervention and an early implementation of a wide array of immunosuppressive therapies. Optimal timing of initiation of therapeutic plasma exchange, as well as duration of treatment necessary to achieve desirable outcomes in patients with NMDAR remains unknown. The present case report aims to raise awareness about the importance of early implementation of this potentially life-saving therapy and continuing the treatment courses until full subsidence of symptoms. PMID:28101360

  10. [Review of the psychiatric aspects of anti-NMDA (N-methyl-D-aspartic acid) receptor encephalitis, case report, and our plans for a future study].

    Science.gov (United States)

    Herman, Levente; Zsigmond, Ildiko Reka; Peter, Laszlo; Rethelyi, Janos M

    2016-12-01

    Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. In most of the cases, typical neurological symptoms appear later. Besides the clinical picture and typical symptoms, verifying presence of IgG antibodies in the serum or CSF is necessary to set up the diagnosis. Other diagnostic tools, including laboratory tests, MRI, lumbar puncture or EEG are neither specific, nor sensitive enough. Therapy is based on supportive care, plasma exchange and immune suppression, intensive care administration can be necessary. If there is an underlying tumor, tumor removal is the first-line treatment. The disease can cause fatal complications in the acute phase but with adequate therapy long-term prognosis is good, although rehabilitation can last for months. In the past few years besides the typical clinical picture and illness course an increasing number of case reports described no typical neurological symptoms, only psychiatric symptoms, including psychosis, disorganized behavior, and catatonic symptoms. Immune suppressive treatment was still effective in most of these cases. Such cases present a difficult diagnostic challenge. These patients may receive unnecessary antipsychotic treatment because of the suspected schizophrenia, although they often suffer from serious extrapyramidal side effects. A few years ago there was a hypothesis that a small part of the patients who are treated with therapy-resistant schizophrenia may suffer from anti-NMDAR encephalitis, so they require a different kind of medication. Evidence from the latest publications did not confirm this hypothesis, although the connection between anti-NMDAR antibodies and

  11. Prospective study of 20 children with anti-NMDA receptor encephalitis%20例儿童抗N-甲基-D-门冬氨酸受体脑炎的前瞻性研究

    Institute of Scientific and Technical Information of China (English)

    王昕; 杨健; 李尔珍; 王立文; 王珺; 彭晓音; 陈倩; 宋国维

    2016-01-01

    目的 探讨儿童抗N-甲基-D-门冬氨酸(anti-N-methyl-D-aspartate receptor encephalitis,NMDA)受体脑炎的临床特点、最佳治疗方案和预后.方法 对20例抗NMDA受体脑炎的患者进行临床队列观察,总结其临床表现、对治疗的反应和临床预后特点.结果 入组20例患儿中,男患儿8例,女患儿12例,年龄1岁11个月~13岁4个月.观察时间8个月~3年6个月,至病情稳定,6个月内无改善/进展.患者均以神经系统症状为首发表现,其中以癫癇发作最为突出,90%的患者均有癫癇发作;其次为语言障碍、认知行为异常和运动异常.偏瘫和小脑共济失调在6岁以下患者中出现频率较高.50%患者一线免疫治疗(IVIG、皮质激素)有效,一线治疗失败者予二线免疫(利妥昔单抗)治疗后,80%患儿有明显好转.部分患儿恢复缓慢,在8 ~12月的治疗中逐渐好转.结论 抗NMDA受体脑炎在儿童脑炎患者中并不少见,免疫治疗有效.一线免疫治疗失败后应给予二线免疫治疗.%Objective To assess the presentation of Anti-NMDA receptor (NMDAR)encephalitis,the spectrum of symptoms,immunotherapies used,timing of improvement,and long-term outcome.Methods In this observational study,we tested the presence of NMDAR antibodies in serum or CSF samples of patients with encephalitis from Dec 1,2011,to Dec 1,2014.All patients who tested positive for NMDAR antibodies were included in the study;patients were assessed at symptom onset and at months 4,8,12,18,24,and 36 by use of the modified Rankin scale(mRS).Treatment included first-line immunotherapy(steroids,intravenous immunoglobulin),second-line immunotherapy(rituximab).Results We enrolled 20 patients(range 1 year 11 months to 13 years 4 months),observation time range 8 months to 3 years 6 months.All Patients presented with acute neurology symptoms:90% with seizures.Speech problems,memory deficit,dyskinesias are quite popular in these children.Cerebellar ataxia and

  12. Stereotypic Movements in Case of Sporadic Creutzfeldt-Jakob Disease: Possible Role of Anti-NMDA Receptor Antibodies

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    Michelle Molina

    2012-12-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD and anti-NMDA receptor antibody encephalitis (NMDAE can both produce a rapidly progressive dementia with resulting state of catatonia or akinetic mutism. Both are associated with movement disorders. In published case series, myoclonus appears to be the most frequent movement disorder in sCJD, while stereotypic, synchronized, one-cycle-per-second movements such as arm or leg elevation, jaw opening, grimacing, head turning, and eye deviation are seen in NMDAE. We report a case of a 59-year-old woman with rapidly worsening cognitive disturbance leading to a nearly catatonic state interrupted by stereotypic movements. sCJD was diagnosed via periodic sharp wave complexes on EEG as well as cerebrospinal fluid (CSF 14-3-3 and tau protein elevation. Characteristic movement disorder of NMDAE was present in absence of ovarian mass or CSF pleiocytosis. Given prior case reports of presence of anti-NMDA receptor antibodies in sCJD, we propose that the movement disorder in this case was caused by anti-NMDA receptor antibodies whose formation was secondary to neuronal damage from prion disease. It is important to consider sCJD even in cases that have some clinical features suggestive of NMDAE.

  13. Anti-N-methyl-D-aspartate receptor encephalitis: a new autoimmune encephalitis

    Directory of Open Access Journals (Sweden)

    LI Xiang

    2013-01-01

    Full Text Available Anti-N-methyl-D-aspartate (anti-NMDA receptor encephalitis is a new category of treatable encephalitis associated with anti-NMDA receptor antibody, which attracts more and more attention recently. It is clinically characterized by prodromal fever, schizophrenia-like psychiatric symptoms, seizures, disturbance of consciousness, dyskinesia (particularly orofacial, and autonomic dysfunction, which often occur in young females with ovarian teratomas. Autoantibodies to the anti-NMDA receptor in serum and cerebrospinal fluid are positive. Electroencephalogram (EEG often reveals diffuse δ slowing without paroxysmal discharges, on which " δ rush" is considered as specific characteristic in some patients. Combined therapy including tumor resection and immunotherapy is recommended. The updates in mechanisms, clinical manifestations and diagnostic examinations associated with anti-NMDA receptor encephalitis will be discussed in this review.

  14. Analysis of Anti NMDA Encephalitis in 2 Cases%抗NMDA脑炎2例治疗分析

    Institute of Scientific and Technical Information of China (English)

    李俊

    2015-01-01

    Anti-N-methyl-D-aspartate receptor(NMDAR) encephalitis is a treatable new limbic encephalitis associated with anti-NMDAR receptor antibaody predominantly affecting young women which was identified in 2007.It is clinically characterized Psychiatric and Neurologic syndromes, including obvious mental and behavior disorder, seizures, movement disorders, autonomic dysfunction.Particulary orofaeial dyskinesia and central Magnetic resonance(MRI)imaging and Electroencephalography(ECG)test show no speciifcity, the disease is conifrmed by the presence of NMDAR NRl receptor antibodies in cerebrospinal lfuid or serum.The patients repond well to tumor resection and immunotherapies, including cortieosteroids, intravenous immunoglobulin, plasma exchange and so on.Clinical manifestations and mechanisms associated with anti-NMDA receptor encephalitis will be discussed in this review and aim to improve the cognition of the diease.%抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor)脑炎是一种自2007年确认的由抗NMDA受体抗体介导的可治性新型边缘叶脑炎,好发于年轻女性,临床上主要表现为发展迅速的神经和精神障碍综合征,包括突出的精神行为异常、癫痫发作、异常运动、自主神经功能紊乱等,其中口面颌肌张力障碍和中枢性通气不足是该病的相对特征性表现。MRI检查无特异性改变,脑电图常提示弥漫性的异常,常规脑脊液检查无特异性,血和脑脊液中的NMDAR亚单位NRl抗体阳性可以明确诊断。对糖皮质激素、免疫球蛋白、血浆置换等免疫治疗及肿瘤切除反应良好。为更好地认识该疾病,本文对其临床特点及发病机制等做一综述。

  15. Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.

    Science.gov (United States)

    Levite, Mia

    2014-08-01

    pathological effects: they activate glutamate/AMPA receptors, kill neurons by 'Excitotoxicity', and/or by complement activation modulated by complement regulatory proteins, cause multiple brain damage, aggravate chemoconvulsant-induced seizures, and also induce behavioral/motor impairments. Some patients with 'Autoimmune Epilepsy' that have anti-AMPA-GluR3B antibodies respond well (although sometimes transiently) to immunotherapy, and thanks to that have reduced seizures and overall improved neurological functions. (2) Anti-NMDA-NR1 antibodies are present in patients with autoimmune 'Anti-NMDA-receptor Encephalitis'. In humans, in animal models and in vitro the anti-NMDA-NR1 antibodies can be very pathogenic since they can cause a pronounced decrease of surface NMDA receptors expressed in hippocampal neurons, and also decrease the cluster density and synaptic localization of the NMDA receptors. The anti-NMDA-NR1 antibodies induce these effects by crosslinking and internalization of the NMDA receptors. Such changes can impair glutamate signaling via the NMDA receptors and lead to various neuronal/behavior/cognitive/psychiatric abnormalities. Anti-NMDA-NR1 antibodies are frequently present in high levels in the CSF of the patients with 'Anti-NMDA-receptor encephalitis' due to their intrathecal production. Many patients with 'Anti-NMDA receptor Encephalitis' respond well to several modes of immunotherapy. (3) Anti-NMDA-NR2A/B antibodies are present in a substantial number of patients with Systemic Lupus Erythematosus (SLE) with or without neuropsychiatric problems. The exact percentage of SLE patients having anti-NMDA-NR2A/B antibodies varies in different studies from 14 to 35%, and in one study such antibodies were found in 81% of patients with diffuse 'Neuropshychiatric SLE', and in 44% of patients with focal 'Neuropshychiatric SLE'. Anti-NMDA-NR2A/B antibodies are also present in subpopulations of patients with Epilepsy of several types, Encephalitis of several types (e

  16. Non-tumor-Associated Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis in Chinese Girls With Positive Anti-thyroid Antibodies.

    Science.gov (United States)

    Guan, Wenjuan; Fu, Zhenqiang; Zhang, Hui; Jing, Lijun; Lu, Jingjing; Zhang, Jing; Lu, Hong; Teng, Junfang; Jia, Yanjie

    2015-10-01

    Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a new category of autoimmune encephalitis associated with anti-NMDA receptor antibodies. The disease was first described in 2007, and it predominantly affects young women with or without ovarian teratomas. Most patients typically present with seizures, a decreased consciousness level, dyskinesia, autonomic dysfunction, and psychiatric symptoms. The presence of anti-thyroid antibodies in non-tumor-associated anti-NMDA receptor encephalitis was first described in 2010. Additionally, anti-thyroid antibodies were found in teratoma-associated anti-NMDA receptor encephalitis. We report the cases of 3 Chinese girls with non-tumor-associated anti-NMDA receptor encephalitis with positive anti-thyroid antibodies. We followed up the details of their titers and suggest that anti-thyroid antibodies were an indicator of autoimmune predisposition in the development of non-tumor-associated anti-NMDA receptor encephalitis.

  17. [Anti-NMDA encephalitis in psychiatry; malignant catatonia, atypical psychosis and ECT].

    Science.gov (United States)

    Kanbayashi, Takashi; Tsutsui, Ko; Tanaka, Keiko; Omori, Yuki; Takaki, Manabu; Omokawa, Mayu; Mori, Akane; Kusanagi, Hiroaki; Nishino, Seiji; Shimizu, Tetsuo

    2014-01-01

    The symptoms of malignant (lethal) catatonia has been reported similar to initial symptoms of anti-NMDAR encephalitis. Subsequently, this autoimmune limbic encephalitis has been noticed in many psychiatrists. We have experienced several cases with malignant catatonia having anti-NMDAR antibody without clinical signs of encephalitis. Thereafter, we have also found anti-NMDAR antibody positive patients of young females with acute florid psychiatric symptoms without clinical signs of encephalitis. The features of these patients mirror-those of "Atypical psychosis" proposed by Mitsuda in Japan, a notion derived from "Cycloid psychosis" conceptualized by German psychiatrist, Leonhard. Both cycloid and atypical psychosis have coinciding features of acute onset, emotional disturbances, psychomotor disturbances, alternations of consciousness, high prevalence in women and oriented premorbid personality. Both malignant catatonia and atypical psychosis have been known to be effectively treated with modified electro convulsion therapy (m-ECT). Our 5 cases with anti-NMDAR antibody, m-ECT treatments were effective. Infectious encephalitis is contra indication of m-ECT, but this autoimmune encephalitis would be careful indication. Schizophrenia is a common, heterogeneous, and complex disorder with unknown etiology. There is established evidence of NMDAR hypofunction as a central component of the functional disconnectivity; this is one of the most accepted models for schizophrenia. Moreover, autoimmune mechanisms have been proposed to be involved, at least in subgroups of schizophrenia patients. Further research of anti-NMDAR antibody and encephalitis would be important clues for the investigation of schizophrenia, catatonia and atypical psychosis.

  18. [Anti-NMDA receptor encephalitis: two paediatric cases].

    Science.gov (United States)

    González-Toro, M Cristina; Jadraque-Rodríguez, Rocío; Sempere-Pérez, Ángela; Martínez-Pastor, Pedro; Jover-Cerdá, Jenaro; Gómez-Gosálvez, Francisco

    2013-12-01

    Introduccion. La encefalitis asociada a anticuerpos antirreceptores de N-metil-D-aspartato (NMDA) es una patologia neurologica autoinmune documentada en la poblacion pediatrica de manera creciente en los ultimos años. Se presentan dos casos de nuestra experiencia con clinica similar. Casos clinicos. Caso 1: niña de 5 años que inicia un cuadro de convulsiones y alteracion de conciencia, asociando trastornos del movimiento y regresion de habilidades previamente adquiridas que evoluciona a autismo. Caso 2: niña de 13 años que presenta hemiparesia izquierda, movimientos anomalos, trastorno de conducta y disautonomia. En ambos casos se obtienen anticuerpos antirreceptores de NMDA positivos en el liquido cefalorraquideo y se diagnostican de encefalitis antirreceptor de NMDA. En el primer caso se inicia el tratamiento con perfusion intravenosa de corticoides e inmunoglobulinas y es necesario asociar rituximab. En el segundo, corticoides e inmunoglobulinas. La evolucion fue favorable en ambas pacientes, con una leve alteracion del lenguaje como secuela en el primer caso y una recaida en el segundo caso, con resolucion completa. Conclusion. La encefalitis antirreceptor de NMDA es un trastorno tratable y es importante el diagnostico y tratamiento precoz, ya que mejora el pronostico y disminuye las recaidas.

  19. Anti-NMDA receptor antibodies in patients with a first episode of schizophrenia

    Directory of Open Access Journals (Sweden)

    Masopust J

    2015-03-01

    Full Text Available Jirí Masopust,1,2 Ctirad Andrýs,3 Jan Bažant,1 Oldrich Vyšata,4 Kamil Kuca,5 Martin Vališ4 1Department of Psychiatry, Faculty of Medicine in Hradec Kralové, Charles University in Prague, University Hospital Hradec Králové, Hradec Králové, Czech Republic; 2National Institute of Mental Health, Klecany, Czech Republic; 3Institute of Clinical Immunology and Allergology, Charles University in Prague, Faculty of Medicine in Hradec Králové, and University Hospital Hradec Králové, Hradec Králové, Czech Republic; 4Department of Neurology, Charles University in Prague, Faculty of Medicine in Hradec Králové, and University Hospital Hradec Králové, Hradec Králové, Czech Republic; 5Biomedical Research Centrum, University Hospital Hradec Králové, Hradec Králové, Czech Republic Background: Encephalitis with antibodies against N-methyl-D-aspartate receptor (NMDA-R is classified as an autoimmune disorder with psychotic symptoms, which are frequently dominant. However, it remains unclear how frequently NMDA-R antibodies lead to a condition that mimics psychosis and first-episode schizophrenia. In our work, we investigated the presence of antibodies against NMDA-R in patients with first-episode psychosis (FEP in comparison with healthy volunteers.Methods: This study included 50 antipsychotic-naïve patients with FEP (including 21 women and 50 healthy volunteers (including 21 women. The mean age of the patients was 27.4 (±7.4 years and that of the healthy controls was 27.0 (±7.3 years. Antibodies against NMDA-R in the serum were detected by immunofluorescence.Results: None of the investigated patients with an FEP and none of the healthy controls showed positive antibodies against NMDA-Rs.Conclusion: According to results of studies, a small proportion of patients with an FEP possess antibodies against NMDA-R. However, the extent to which this finding contributes to the etiopathogenesis of the response to antipsychotic medication and

  20. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis mimicking a primary psychiatric disorder in an adolescent.

    Science.gov (United States)

    Lebon, Sébastien; Mayor-Dubois, Claire; Popea, Irina; Poloni, Claudia; Selvadoray, Nalini; Gumy, Alain; Roulet-Perez, Eliane

    2012-12-01

    Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis likely has a wider clinical spectrum than previously recognized. This article reports a previously healthy 16-year-old girl who was diagnosed with anti-NMDA receptor encephalitis 3 months after onset of severe depression with psychotic features. She had no neurological manifestations, and cerebral magnetic resonance imaging (MRI) was normal. Slow background on electroencephalogram and an oligoclonal band in the cerebrospinal fluid prompted the search for anti-NMDA receptor antibodies. She markedly improved over time but remained with mild neuropsychological sequelae after a trial of late immunotherapy. Only a high index of suspicion enables recognition of the milder forms of the disease masquerading as primary psychiatric disorders.

  1. Profound Autonomic Instability Complicated by Multiple Episodes of Cardiac Asystole and Refractory Bradycardia in a Patient with Anti-NMDA Encephalitis

    Directory of Open Access Journals (Sweden)

    Stephanie R. Mehr

    2016-01-01

    Full Text Available Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE is autoimmune encephalitis primarily affecting young adults and children. First described about a decade ago, it frequently manifests as a syndrome that includes progressive behavioral changes, psychosis, central hypoventilation, seizures, and autonomic instability. Although cardiac arrhythmias often accompany anti-NMDARE, the need for long-term electrophysiological support is rare. We describe the case of NMDARE whose ICU course was complicated by progressively worsening episodes of tachyarrhythmia-bradyarrhythmia and episodes of asystole from which she was successfully resuscitated. Her life-threatening episodes of autonomic instability were successfully controlled only after the placement of a permanent pacemaker during her ICU stay. She made a clinical recovery and was discharged to a skilled nursing facility after a protracted hospital course.

  2. A young woman presenting with psychotic and mood symptoms from anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis: an emerging diagnosis.

    Science.gov (United States)

    Yuan, Neal; Glezer, Anna

    2013-01-01

    Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis, first characterized in 2005, is a neurological disease with prominent psychiatric features that frequently involves the consultation of psychiatrists. Since its discovery, the rate of diagnosis of new cases has increased rapidly and several epidemiological studies now confirm that NMDA-R encephalitis may be as common as many other prominent infectious etiologies of encephalitis. We describe a case of a young woman presenting initially with psychotic and mood symptoms who was found to have anti-NMDA-R encephalitis. We further provide details of her treatment and prolonged recovery process after hospital discharge with a review of the literature and discussion of the epidemiology, symptomology, diagnosis, and management of both the neurologic and psychiatric manifestations of this condition. Last, we contextualize the importance of anti-NMDA-R encephalitis for psychiatrists, highlighting the role for psychiatrists in establishing the initial diagnosis as well as in providing ongoing psychiatric care.

  3. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis: an unusual cause of autistic regression in a toddler.

    Science.gov (United States)

    Scott, Ori; Richer, Lawrence; Forbes, Karen; Sonnenberg, Lyn; Currie, Angela; Eliyashevska, Myroslava; Goez, Helly R

    2014-05-01

    Anti N-methyl-d-aspartate (NMDA) receptor encephalitis in children is associated with psychiatric changes, seizures, and dyskinesias. We present the first report of autistic regression in a toddler caused by this entity. A 33-month-old boy presented with decreased appetite, irritability, and insomnia following an upper respiratory tract infection. Over the next few weeks he lost language and social skills, and abnormal movements of his hand developed. Within a month, this patient came to fit the diagnostic criteria for autistic spectrum disorder. Upon investigation, anti-NMDA receptor antibodies were found in the boy's cerebrospinal fluid. He was treated with intravenous immunoglobulins and steroids, resulting in reacquisition of language and social skills and resolution of movements. Our case emphasizes the significance of suspecting anti-NMDA receptor encephalitis as the cause of autistic regression, even in an age group where the diagnosis of autistic spectrum disorder is typically made, and especially when presentation follows a febrile illness.

  4. [Reversible cortical atrophy secondary to anti-NMDA receptor antibody encephalitis].

    Science.gov (United States)

    Bravo-Oro, Antonio; Acosta-Yebra, Danae; Grimaldo-Zapata, Ilse P; Reyes-Vaca, Guillermo

    2015-05-16

    Introduccion. La encefalitis por anticuerpos antirreceptor de N-metil-D-aspartato (NMDA) inicialmente se describio como un sindrome paraneoplasico asociado a teratoma de ovario, pero cada vez con mas frecuencia se han ido publicando casos en mujeres jovenes y niños como un cuadro encefalopatico autoinmune secundario en el 40-50% de los casos a un proceso viral. Clinicamente, se caracteriza por un cuadro progresivo de manifestaciones psiquiatricas, crisis convulsivas, discinesias y disautonomias. Un hallazgo neurorradiologico poco comunicado es la atrofia cortical reversible, de la cual se desconoce su mecanismo. Caso clinico. Niña que a los 6 años comenzo con crisis convulsivas focales, con electroencefalograma epileptogeno y tomografia de craneo inicial normal. Se inicio tratamiento anticonvulsionante. A las tres semanas aparecieron nuevas crisis convulsivas, manifestaciones psiquiatricas y alteraciones en el ciclo de sueño-vigilia. Ante la sospecha de encefalitis por anticuerpos antirreceptor de NMDA, estos se determinaron en el suero y el liquido cefalorraquideo con resultado positivo. Resonancia magnetica durante el ingreso con atrofia cortical generalizada. Oncologia Pediatrica descarto asociacion a tumores. A los dos años del cuadro, con la paciente libre de crisis convulsivas, una valoracion neuropsicologica mostro la afectacion de funciones ejecutivas y una resonancia magnetica de control evidencio la recuperacion de la atrofia cortical. Conclusion. El mecanismo de la atrofia cortical reversible se desconoce, pero en pacientes con encefalitis por anticuerpos antirreceptor de NMDA podria ser directamente proporcional a la cantidad de anticuerpos circulantes y el tiempo de exposicion a estos en la corteza cerebral. Es muy importante el diagnostico temprano y el inicio de inmunomodulacion.

  5. In vitro neuronal network activity in NMDA receptor encephalitis

    Directory of Open Access Journals (Sweden)

    Jantzen Sabine U

    2013-02-01

    Full Text Available Abstract Background Anti-NMDA-encephalitis is caused by antibodies against the N-methyl-D-aspartate receptor (NMDAR and characterized by a severe encephalopathy with psychosis, epileptic seizures and autonomic disturbances. It predominantly occurs in young women and is associated in 59% with an ovarian teratoma. Results We describe effects of cerebrospinal fluid (CSF from an anti-N-methyl-D-aspartate receptor (NMDAR encephalitis patient on in vitro neuronal network activity (ivNNA. In vitro NNA of dissociated primary rat cortical populations was recorded by the microelectrode array (MEA system. The 23-year old patient was severely affected but showed an excellent recovery following multimodal immunomodulatory therapy and removal of an ovarian teratoma. Patient CSF (pCSF taken during the initial weeks after disease onset suppressed global spike- and burst rates of ivNNA in contrast to pCSF sampled after clinical recovery and decrease of NMDAR antibody titers. The synchrony of pCSF-affected ivNNA remained unaltered during the course of the disease. Conclusion Patient CSF directly suppresses global activity of neuronal networks recorded by the MEA system. In contrast, pCSF did not regulate the synchrony of ivNNA suggesting that NMDAR antibodies selectively regulate distinct parameters of ivNNA while sparing their functional connectivity. Thus, assessing ivNNA could represent a new technique to evaluate functional consequences of autoimmune encephalitis-related CSF changes.

  6. Autoimmune NMDA receptor encephalitis.

    Science.gov (United States)

    Lazar-Molnar, Eszter; Tebo, Anne E

    2015-01-01

    Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a treatable autoimmune disease of the central nervous system (CNS) with prominent neurologic and psychiatric features at disease onset. The disease is associated with the production of autoantibodies to NMDAR, a protein involved in memory function and synaptic plasticity. Affected patients develop a multistage progressive illness with symptoms ranging from memory deficits, seizures and psychosis, to potentially lethal catatonia, and autonomic and breathing instability. The outcome can be much improved with accurate diagnosis and early treatment using adequate immunosuppressive therapy. However, since the neurological and psychiatric symptoms as well as the clinical examination results can be non-specific, the disease is probably under-recognized. Reliable and accurate clinical testing for the identification of NMDAR autoantibodies is crucial for diagnosis, timely treatment selection, and monitoring. Recently, a cell-based indirect immunofluorescent antibody test for the detection of IgG antibodies to NMDAR has become available for diagnostic use. This review highlights the progress and challenges of laboratory testing in the evaluation and management anti-NMDAR encephalitis, and perspectives for the future.

  7. 抗N-甲基-D-天冬氨酸受体脑炎:一种新型自身免疫性脑炎%Anti-N-methyl-D-aspartate receptor encephalitis: a new autoimmune encephalitis

    Institute of Scientific and Technical Information of China (English)

    陈向军; 李翔

    2013-01-01

    Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is a new category of treatable encephalitis associated with anti-NMDA receptor antibody, which attracts more and more attention recently. It is clinically characterized by prodromal fever, schizophrenia - like psychiatric symptoms, seizures, disturbance of consciousness, dyskinesia (particularly orofacial), and autonomic dysfunction, which often occur in young females with ovarian teratomas. Autoantibodies to the anti-NMDA receptor in serum and cerebrospinal fluid are positive. Electroencephalogram (EEG) often reveals diffuse δ slowing without paroxysmal discharges, on which " δ rush" is considered as specific characteristic in some patients. Combined therapy including tumor resection and immunotherapy is recommended. The updates in mechanisms, clinical manifestations and diagnostic examinations associated with anti - NMDA receptor encephalitis will be discussed in this review.%抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是目前较受关注的自身免疫性脑炎,可伴或不伴卵巢畸胎瘤.临床表现包括显著前驱高热、精神症状、癫发作、意识障碍、口手运动障碍及自主神经功能障碍等;脑脊液和血清抗NMDAR 抗体检测呈阳性反应,脑电图呈特异性"δ刷"表现.早期施行肿瘤切除和免疫抑制剂治疗有效.本文拟就抗NMDAR 脑炎发病机制、临床表现、辅助检查等研究进展进行概述.

  8. [NMDA receptor encephalitis in the course of recurrent CNS demyelinating disorders: a case report].

    Science.gov (United States)

    Yamamoto, Masanari; Kokubun, Norito; Watanabe, Yuka; Okabe, Ryuta; Nakamura, Toshiki; Hirata, Koichi

    2013-01-01

    We present the case of a 31-year-old woman who developed N-methyl-d-aspartate (NMDA) receptor encephalitis during the course of relapsing and remitting multiple brain lesions. The patient developed a tingling sensation in the left upper and lower extremities, and was first admitted to our hospital at age 27. She was tentatively diagnosed with multiple sclerosis on the basis of multiple lesions with Gd-enhancement in the brainstem, and 2 separate clinical relapses by age 28. At age 31, she developed a headache and pyrexia, followed by confusion and abnormal behavior. Her symptoms acutely progressed to stupor, and subsequently, she developed oral dyskinesia and athetosis-like involuntary movement of the left arm. The stupor state continued over 2 months. However, she had completely recovered by 3 months after the onset of psychiatric symptoms. Her serum and CSF samples tested positive for anti-NMDA receptor antibodies, and she was diagnosed with NMDA receptor encephalitis. Her serum was negative for anti-AQP4 antibody, but showed weak positivity for antinuclear antibody. Between ages 32 and 34, she experienced 2 clinical relapses, including right-hand clumsiness, confusion, aphasia, and dysphagia. FLAIR images showed a high-intensity area in the brain stem, thalamus, and subcortical white matter. No tumors were found throughout the course. A clinical entity of NMDA receptor encephalitis can include various neurologic disorders, such as the development of recurrent demyelinating brain lesions. Further investigation is required to clarify the pathophysiological role of anti-NMDA receptor antibody in our patient.

  9. Rehabilitation for a child with recalcitrant anti-N-methyl-D-aspartate receptor encephalitis: case report and literature review

    Directory of Open Access Journals (Sweden)

    Guo YH

    2014-11-01

    Full Text Available Yao-Hong Guo,1 Ta-Shen Kuan,1,2 Pei-Chun Hsieh,1 Wei-Chih Lien,1 Chun-Kai Chang,1 Yu-Ching Lin1–3 1Department of Physical Medicine and Rehabilitation, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; 2Department of Physical Medicine and Rehabilitation, College of Medicine, National Cheng Kung University, Tainan, Taiwan; 3Medical Device Innovation Center, National Cheng Kung University, Tainan, Taiwan Abstract: Anti-N-methyl-d-aspartate (anti-NMDA receptor encephalitis is a newly recognized, potentially fatal, but treatable autoimmune disease. Good outcome predictors include milder severity of symptoms, no need for intensive care unit admission, early aggressive immunotherapy, and prompt tumor removal. We report a case of a young girl aged 3 years 2 months and diagnosed as recalcitrant anti-NMDA receptor encephalitis without any underlying neoplasm. The patient had initial symptoms of behavioral changes that progressed to generalized choreoathetosis and orofacial dyskinesia, which resulted in 6 months of hospitalization in the pediatric intensive care unit. One year after initial onset of the disease, she had only achieved the developmental age of an infant aged 6–8 months in terms of gross and fine motor skills, but she resumed total independence in activities of daily living after receiving extensive immunotherapy and 28 months of rehabilitation. Our brief review will help clinical practitioners become more familiar with this disease and the unique rehabilitation programs. Keywords: anti-NMDA receptor encephalitis, autoimmune encephalitis, rehabilitation, cognition deficits

  10. 抗 NMDA 受体脑炎患者11例临床资料分析%Clinical Features of 11 Patients with Anti-N-methyl-D-aspartate Receptor Encephalitis

    Institute of Scientific and Technical Information of China (English)

    李庭毅; 卢祖能

    2015-01-01

    目的:探讨抗 NMDA 受体脑炎的临床表现、磁共振(MRI)图像及脑脊液特点。方法:回顾性分析我院11例抗 NMDA 受体脑炎患者的临床资料。结果:11例患者主要表现为精神症状、癫痫发作、运动障碍等;头部 MRI 检查无特异性;2例女性患者腹部 CT 发现畸胎瘤;11例患者脑脊液抗 NMDA 抗体(+)。结论:以精神行为异常、癫痫发作、运动障碍为主要表现的青年脑炎患者,要警惕抗 NMDA 受体脑炎的可能,需及时行脑脊液抗 NMDA 抗体检测。%Objective: To explore the characteristics of clinical presentations, magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) of Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. Methods: The clinical data of 11 patients with anti-NMDA receptor encephalitis, were retrospectively analyzed. Results: Eleven patients presented with psychiatric symptoms, epilepsy and movement disorders. MRI did not show specific changes. Two female patients suffered from teratoma. All patients were diagnosed by the presence of antibodies to the anti-NMDA receptor in CSF. Conclusion: The young cases with encephalitis, who presented with psychiatric symptoms, epilepsy and movement disorders, may hint at anti-NMDA receptor encephalitis. Timely autoantibody detection is necessary.

  11. 抗N-甲基-D-天冬氨酸受体脑炎1例报告%The Clinical Characteristics of Anti-N-methyl-D-aspartate-receptor Encephalitis:One Case Report

    Institute of Scientific and Technical Information of China (English)

    刘文钰; 田林郁; 郭佳南; 陈佳妮; 周东

    2015-01-01

    Aim To help the physician understand anti-N-methyl-D-aspartate-receptor encephalitis (anti-NMDA encephalitis). Methods With the description of one young female patient with anti-NMDA encephalitis, the treatment especially tumor search of anti-NMDA encephalitis combined with the literatures were discussed. Results A 18 year-old young girl had positive reaction to NMDAR antibody but tumor detection was negative twice. Then she received the first and second line immunotherapy, ventilator breathing and antiepileptic drugs with somehow improvement. About 5 months later, a teratoma was found near her cyst. Then she accepted laparoscopic resection. Pathologic analysis conifrmed teratoma. Advanced nerval function recovered quickly after the operation. Conclusion Anti-NMDA encephalitis may impact on the quality of life of the patients, especially for young women. Efforts for tumor search should always be encouraged.%目的:提高临床医生对抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的认识。方法收集1例18岁女性抗NMDAR脑炎患者的临床资料,对抗NMDAR脑炎的治疗及肿瘤搜索结合文献进行探讨。结果患者因头痛发热9 d,言语行为异常5 d入院,入院后频繁癫发作。肿瘤标记物、免疫、TORCH检查和影像学肿瘤搜索2次检查结果均为阴性,但抗NMDAR抗体阳性,诊断抗NMDAR脑炎、癫持续状态。经呼吸机辅助呼吸、抗癫药和免疫治疗后症状好转。病程约5个月后发现卵巢肿瘤,接受腹腔镜肿瘤切除,病理检查提示畸胎瘤。术后患者高级神经功能迅速恢复。结论抗NMDAR脑炎严重影响患者生活质量,尤其对于年轻女性应积极进行肿瘤搜索,切除肿瘤后患者恢复更快。

  12. [Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis: experience with six pediatric patients. Potential efficacy of methotrexate].

    Science.gov (United States)

    Bravo-Oro, Antonio; Abud-Mendoza, Carlos; Quezada-Corona, Arturo; Dalmau, Josep; Campos-Guevara, Verónica

    2013-11-01

    INTRODUCTION. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a condition that is increasingly more frequently diagnosed in the paediatric age. Unlike adults, in many cases it is not associated to tumours and the most common initial manifestations in children are seizures and movement disorders, while in adults there is a predominance of psychiatric alterations. CASE REPORTS. We present six confirmed paediatric cases with antibodies against the subunit NR1 of the NMDA receptor in serum and cerebrospinal fluid. Five of the cases began with seizures as the initial clinical symptom prior to the development of the classical clinical features of this condition. In all cases, steroids were used as the first line of treatment, although these only brought about control over the manifestations in one of them; the other patients therefore required second-line immunomodulators. All the patients received methotrexate as immunomodulator treatment to prevent relapses, and in all cases there was an improvement in the patients' situation. CONCLUSIONS. In our series of patients with anti-NMDA receptor encephalitis, none were associated with tumours. All of them were given methotrexate for at least one year and no adverse clinical or analytical events were observed; likewise, there were no neurological sequelae or relapses during treatment. Although it is a small series and it would be advisable to increase the number and time to progression, we see methotrexate as an excellent alternative immunomodulator treatment for this pathology.

  13. Anti-N-methyl-D-aspartic acid receptor encephalitis: clinical analysis of 7 cases%抗NMDA受体脑炎七例临床分析

    Institute of Scientific and Technical Information of China (English)

    宋璐; 刘爱华

    2013-01-01

    目的 总结抗NMDA受体脑炎的临床及辅助检查的特点、提高对该病的认识.方法 分析2010年1月至2012年12月在宣武医院神经内科就诊的7例抗NMDA受体脑炎患者的临床特征、辅助检查,总结不同治疗方案的预后.结果 7例患者表现为显著的精神症状、癫痫、运动障碍,部分患者伴随自主神经功能异常,MRI显示边缘系统异常信号;CSF及血清NMDA受体抗体阳性,5例患者预后良好.结论 及时的诊断及治疗有助于抗NMDA受体脑炎患者的恢复.%Objective To summarize the clinical manifestations,immunotherapeutic response and prognosis of anti-N-methyl-D-aspartic acid (NMDA) receptor encephalitis.Methods We analyzed the clinical features,tumor markers,serum/cerebrospinal fluid (CSF) antibodies of seven cases with antiNMDA receptor encephalitis.And the prognosis of different treatments was observed.Results Seven patients presented with significant psychiatric symptoms,memory loss,epilepsy,movement disorders and decrease consciousness.And some patients required assisted ventilation because of dysautonomia.All electroencephalographs showed slow wave activity without epileptic form discharges.Magnetic resonance imaging was normal or showed T2W and Flair-phase high signal in limbic system,cortex,thalamus.And CSF and serum anti-NMDA receptor antibodieswere positive (n =6).Conclusion Anti-NMDA receptor encephalitis is a kind of fatal but treatable condition.And timely diagnosis and treatment may yield a favorable prognosis.

  14. Anti-N-methyl-D-aspartate-receptor encephalitis: diagnosis, optimal management, and challenges

    Directory of Open Access Journals (Sweden)

    Mann AP

    2014-07-01

    psychiatrists, neurologists, oncologists, neuro-oncologists, immunologists, and intensivists to become familiar with this disorder and its potential complications. Remission can be optimized with prompt detection and aggressive, collaborative treatment within a multidisciplinary team.Keywords: anti-NMDA receptor, encephalitis, management, treatment, complications, paraneoplastic

  15. 抗N-甲基-D-天冬氨酸受体脑炎%Anti-N-Methyl-D-Aspartate Receptor Encephalitis

    Institute of Scientific and Technical Information of China (English)

    杨爱君; 崔红; 魏田力

    2011-01-01

    IN - Methyl - D - Aspartate (NMDA) receptors Eire ion channels that contain binding sites of glutamate and glycine. They play a pivotal role in the regulation of synaptic transmission and synaptic plasticity in the central nervous system. There are 3 NMDA receptor subtypes demonstrated, which have own distribution and function. Anti - NMDA receptor encephalitis is a treatable disorder predominantly affecting young women which was recently identified. It is characterized by a combination of psychotic encephalopathy, seizures, and abnormal movements of the trunk and face, particularly in the form of jaw dystonia. The anti - NMDA receptor encephalitis of children increases recently. Accordingly, enhanced awareness of the typical presentation can increase the index of suspicion and facilitate initiation of appropriate treatment.%N-甲基-D-天冬氨酸(NMDA)受体是包含谷氨酸和甘氨酸结合位点的离子通道,在中枢神经系统的突触传递和突触可塑性调节中起重要作用.已证实的3种NMDA亚基,在体内有着不同的分布和功能.抗NMDA受体脑炎是近年才被确认的好发于年轻女性的可治疗的疾病,其临床特点为精神病性脑病、癫(癎)样发作以及躯干和面部的异常运动,特别是下颌关节肌张力异常.近年儿童抗NMDA受体脑炎有逐渐增多趋势.因此,增加对典型临床表现的认识,可提高对本病的早期识别并给予适当的治疗.

  16. 抗NMDA受体脑炎的临床和脑电图特点%Clinical features of anti-NMDA receptor encephalitis

    Institute of Scientific and Technical Information of China (English)

    胡湘蜀; 欧阳梅; 李花; 李淡芳; 窦文波; 伍新颜; 陈威; 许绍强

    2014-01-01

    目的:研究抗N-甲基-M-天冬氨酸(NMDA)受体脑炎患者的临床特点,以提高对这种自身免疫性疾病的认识.方法:对3例经血清(其中1例并作脑脊液)抗NMDA受体抗体检测诊为抗NM-DA受体脑炎的患者,对病史、认知功能、脑脊液、头颅影像学、EEG、妇科B超、治疗方案等资料进行详细分析,并随访3~6个月.结果:3例患者均为女性,起病年龄13岁(2例)和25岁,以精神异常和癫(癎)发作为首发症状,病程中表现出意识障碍、运动障碍、语言障碍和认知障碍.脑脊液、头颅MRI和EEG表现为非特异改变,未发现伴发肿瘤.病程3~6个月.结论:抗NMDA受体脑炎临床表现易与病毒性脑炎混淆,对于不明原因的脑炎,尤其病程较长者,应尽早进行抗NMDA受体抗体检测.

  17. 10例抗NMDA受体脑炎患者的护理%Nursing care of ten patients with anti-NMDA receptor encephalitis

    Institute of Scientific and Technical Information of China (English)

    叶松岩; 吴颖颖

    2015-01-01

    目的 总结10例抗NMDA受体脑炎的护理经验,提高对此病的临床认识和护理水平.方法 10例患者均给予免疫治疗和对症支持护理.治疗过程中,8例出现癫痫发作,6例出现精神症状,6例出现意识障碍,4例出现运动障碍,2例出现自主神经功能障碍.结果 10例患者平均住院19d出院.2例患者完全恢复,8例患者遗留不同程度的认知功能、自主神经功能、运动功能等功能障碍.电话随访1~18个月,3例完全恢复,7例遗留不同程度的后遗症.结论 抗NMDA受体脑炎临床表现复杂,无特异性,多表现为癫痫发作、精神症状、意识障碍、运动障碍、自主神经功能障碍等,密切观察病情变化并给予相应的症状护理有利于促进患者康复.

  18. 抗NMDA受体脑炎纠误挽治回顾分析%Retrospective Analysis of Anti-N-methyl-D-aspartate Receptor Encephalitis

    Institute of Scientific and Technical Information of China (English)

    刘佳福; 于洋; 王树云; 陈向军; 潘曙明

    2015-01-01

    -tive anti-NMDA receptor antibody in her cerebral spinal fluid and serum confirmed the diagnosis. Intravenous immunoglobulin treatment and methylprednisolone plus teratoma resection were performed. The patient recovered later. Conclusion We should have a high suspicion for anti-NMDA receptor encephalitis in young patients with abrupt mental status changes with conscious and movement disorder, especially with ovarian teratoma. The NMDA receptor encephalitis diagnosis should be based on test of anti-NMDA receptor antibody in order to confirm the diagnosis.

  19. Anti-N-methyl-D-aspartate receptor encephalitis: an adolescent with ovarian teratoma%抗N-甲基-D-天冬氨酸受体脑炎一例

    Institute of Scientific and Technical Information of China (English)

    许春伶; 郝增平; 冀晓俊; 赵伟秦; 李继梅; 王佳伟; 王淑辉; 王得新; 刘美云; 乔衫衫; 靳家玉

    2010-01-01

    Objective To investigate the clinical presentation,diagnosis,and surgical management of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis.Methods One case of anti-NMDA receptor encephalitis in a previously healthy 17-year-old female related to the development of NMDA receptor autoantibodies triggered by an ovarian teratoma was reported.The related literature was reviewed and the clinical feature was summarized.Results Removal of the ovarian teratoma combined with intravenous immuneglobulin and corticosteroid proved curative with eventual resolution of the paraneoplastic disease process and associated psychiatric symptoms.Conclusion Increasingly,reports of anti-NMDA receptor encephalitis associated with ovarian teratomas,as well as a novel assay to measure these antibodies suggest an etiology for this disease process that may be amenable to prompt surgical excision.%目的 探讨抗N-甲基-M-天冬氨酸(NMDA)受体脑炎的临床特点、诊断及治疗.方法报道1例伴卵巢畸胎瘤的抗NMDA受体脑炎患者的临床表现及诊治过程,并总结其临床特征.结果患者为青年女性,表现为精神行为异常及抽搐,意识障碍及通气不足.早期发现畸胎瘤,脑脊液中抗NMDA受体抗体阳性.行畸胎瘤手术切除,联合静脉用免疫球蛋白和激素冲击治疗,患者临床痊愈.结论 抗NMDA受体脑炎是一种自身免疫性的、能用血清学方法诊断的疾病.早期明确诊断和给予及时治疗,预后良好.

  20. 儿童抗N-甲基-D-天冬氨酸受体脑炎的临床护理%Clinical nursing care of children with anti N-methyl-D-aspartate receptor encephalitis

    Institute of Scientific and Technical Information of China (English)

    潘月瑢

    2016-01-01

    目的:总结抗N-甲基-D-天冬氨酸受体脑炎的临床护理方法。方法:选取神经内科明确诊断的10例抗NMDA受体脑炎患儿,实施病情观察、安全护理、皮肤护理、康复护理及心理护理等护理措施,对所采用的护理方法及效果进行总结。结果:经积极治疗及有效护理,10例患儿中6例基本恢复,3例遗留轻微肢体功能障碍,1例存在语言障碍,无1例护理并发症及死亡病例。结论:对抗N-甲基-D-天冬氨酸受体脑炎的患儿采取积极有效的护理方法,能够减少并发症的发生,促进疾病的恢复。%Objective:To summarize the clinical nursing methods of anti NMDA receptor encephalitis.Method:Select the Department of internal medicine in our hospital.10 cases of anti NMDA receptor encephalitis,the implementation of patient observation,safety nursing, skin nursing,rehabilitation nursing and psychological nursing, the nursing method and effect of the summary.Result:After active treatment and effective nursing,10 cases in 6 cases recovered, 3 cases of minor residual limb dysfunction,1 case there is a language barrier,no 1 example nursing complications and deaths.Conclusion:Against anti NMDA receptor encephalitis of children to take positive and effective nursing method, can reduce the complication the occurrence,promote disease recovery.

  1. Cortical hypometabolism demonstrated by PET in relapsing NMDA receptor encephalitis.

    Science.gov (United States)

    Pillai, Sekhar C; Gill, Deepak; Webster, Richard; Howman-Giles, Robert; Dale, Russell C

    2010-09-01

    N-methyl-d-aspartate (NMDA) receptor encephalitis is a newly defined type of autoimmune encephalitis. Two girls (age 3 years, case 1, and 7 years, case 2) with relapsing NMDA receptor encephalitis each had the classic clinical features of encephalopathy, movement disorders, psychiatric symptoms, seizures, insomnia, and mild autonomic dysfunction. Both patients had persistent neuropsychiatric disability, despite immune therapies. Positron emission tomography (PET) scans were performed during clinical relapse at 6 weeks (case 1) and 5 months (case 2). In both cases, the scans demonstrated reduced fluorodeoxyglucose metabolism in the cerebral cortex, with the temporal regions being most affected. PET imaging was more sensitive than magnetic resonance imaging in these patients. In contrast, the one previous report of acute NMDA receptor encephalitis indicated cortical hypermetabolism. Thus, NMDA receptor encephalitis may be associated with variable PET findings, possibly dependent upon the timing of the study, or other factors. Future studies should investigate whether cortical hypometabolism is associated with a relapsing course, and whether it is predictive of a poorer outcome in NMDA receptor encephalitis.

  2. Encephalitis

    Science.gov (United States)

    ... with anyone who has encephalitis. Controlling mosquitoes (a mosquito bite can transmit some viruses) may reduce the chance of some infections that can lead to encephalitis. Apply an insect repellant containing the chemical, DEET when you go outside ( ...

  3. Severity anti-N-methyl-D-aspartate receptor encephalitis:review of clinical presentation,imaging and electroencephalogram%重症抗NMDAR脑炎临床、影像学及脑电图特征研究

    Institute of Scientific and Technical Information of China (English)

    陈萍; 杜芳; 刘之荣; 王津存; 吴中亮; 赵钢

    2016-01-01

    Objective To analysis the clinical presentation,imaging and electroencephalogram of severity anti NMDA receptor encephalitis.Method Twenty three patients were confirmed to have serum or cerebrospinal fluid (CSF) antibodies to the NMDA receptor,Modified Rankin Scale(MRS) were 4-5 scores.To analysis the charactor of white blood cells in CSF,severity of disturbance of consciousness,failure of respiration, head MRI and 24 hour long-term video electroencephalogram(VEEG).Results In the twenty three patients with severity anti NMDA receptor encephalitis,nineteen patients showed psychiatric disorder(82.6%),twenty patients showed disturbance of consciousness(87%).White blood cells in CSF of severe anti NMDA receptor encephalitis had no different with that of in mild anti NMDA receptor encephalitis(P<0.05). Seventeen cases of seizure(73.9%)including thirteen cases of status epileptics(56.5%).By 24hVEEG ,fifteen patients showed wide slow wave including twelve cases of θ wave ,five cases of δ wave. Two patients showed 10-20 HZ fast wave background.Six patients showed spike wave,sharp wave,spike slow wave.Only one case was normal VEEG.Fourteen patients were abnormal signals in MRI including six cases of cortex of frontal , temporal,parietal and occipital, five cases of subcortical white matter,five cases of hippocampus,two cases of brain stem, MRI showed normal in 9 cases. In ten cases with respiratory failure (43.5%), six cases were type I and four cases were type Ⅱ respiratory failure.Conclusion Severity ,white blood cells in CSF and degree of disturbance of consciousness has nothing to do with the titer of anti NMDA receptor antibody ,the status of epilepsy is easy to occur and the respiratory failure caused by central ventilation is insufficient in severity anti NMDA receptor encephalitis.%目的:分析重症抗NMDAR脑炎临床,影像学及脑电图(EEG)特征。方法23例血清和/或脑脊液(CSF)抗NMDAR抗体阳性、MRS评分4-5分的重症抗NMDAR

  4. Encephalitis

    Science.gov (United States)

    ... to be common, such as measles, mumps, and German measles. But because many countries immunize against them, ... problems like seizures, difficulties with muscle coordination, and learning disabilities. Can I Prevent Encephalitis? The best way ...

  5. Clinical and imaging characteristics of 16 patients with autoimmune neuronal synaptic encephalitis

    Directory of Open Access Journals (Sweden)

    N Kamble

    2015-01-01

    Conclusions: All patients with anti-VGKC, anti-NMDA and anti-TPO antibody positivity presented with a triad of behavioral changes, impaired cognition and seizures. Mutism was a predominant symptom in patients with an anti-NMDA antibody positivity, which may help in the early identification of this disorder. MRI brain showed changes restricted to limbic structures in anti-NMDA and anti-VGKC antibody positivity. An early diagnosis and treatment of autoimmune encephalitis is essential for a better outcome and for prevention of long-term sequel.

  6. 抗N-甲基-D-天门冬氨酸受体脑炎11例临床分析%Anti-N-methyl-D-aspartate receptor encephalitis: clinical analysis of 11 cases

    Institute of Scientific and Technical Information of China (English)

    吴颖颖; 时英英; 张弥兰; 刘建锋; 周瑶; 黄月; 刘慧勤; 马明明; 张杰文

    2015-01-01

    目的 分析抗N-甲基-D-天门冬氨酸(NMDA)受体脑炎的临床表现、实验室及影像学资料,以提高对该病的认识.方法 对2013年1月至2015年2月在郑州大学人民医院神经内科就诊的11例抗NMDA受体脑炎患者的临床表现、实验室及影像学资料进行回顾性分析.结果 主要临床表现为癫痫9例,精神症状7例,运动障碍5例,自主神经功能障碍3例.11例脑脊液抗NMDA受体抗体均阳性,其中5例同时出现血清抗NMDA受体抗体阳性.1例合并肺癌.11例患者均接受免疫治疗,出院时4例基本恢复正常,7例存在不同程度的功能障碍.电话随访1 ~18个月,1例失访,10例随访患者中3例完全恢复,7例遗留不同程度的后遗症.结论 无精神病史的患者出现不明原因的精神症状伴痫性发作、记忆障碍、意识障碍、运动障碍、自主神经功能障碍等表现,应尽早做血清及脑脊液抗NMDA受体抗体检测,以便于早诊断、早治疗,改善预后.%Objective To analyze the clinical manifestation,laboratory findings,radiological data of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis.Methods The clinical manifestation,laboratory findings,radiological data of eleven patients with anti-NMDA receptor encephalitis were analyzed.Results Main symptoms included epilepsy presented in 9 cases,psychiatric symptom in 7 cases,dyskinesia in 5 cases,autonomic dysfunction in 3 cases.The anti-NMDA receptor antibody was found in all the patients' cerebrospinal fluid (CSF).No teratoma was detected in any of the patients,and 1 case had lung cancer.All the patients received immune therapy.And at discharge,4 cases recuperated generally,7 cases had different degrees of dysfunction.After telephone follow-up of 1-18 months,1 case was lost,3 cases had complete recovery,and 7 cases had different degrees of sequela.Conclusion When Patients without mental illness history presents with unexplained mental symptoms accompanied by seizures

  7. A successful case of anti-NMDAR encephalitis without tumor treated with a prolonged regimen of plasmapheresis

    Directory of Open Access Journals (Sweden)

    Mateus Mistieri Simabukuro

    Full Text Available ABSTRACT Anti-NMDA receptor encephalitis is a severe but treatable autoimmune disease of the CNS. However, the use of immunotherapy and long-term outcomes have yet to be defined for this disease. We describe a case of an 18-year-old male diagnosed with anti-NMDAR encephalitis not associated with tumor, which did not respond to initial treatment with immunoglobulin, followed by corticosteroids, cyclophosphamide and evolved with significant clinical improvement after a prolonged course of plasmapheresis. Although it is not possible to affirm the good outcome was due solely to the prolonged plasmapheresis regimen, recently published data shows that improvement may take weeks or months to occur. This case discloses another therapeutic possibility for patients with refractory disease who fail to respond to recommended first-line and second-line therapy.

  8. Neuroleptic intolerance in patients with anti-NMDAR encephalitis

    Science.gov (United States)

    Lejuste, Florian; Thomas, Laure; Picard, Géraldine; Desestret, Virginie; Ducray, François; Rogemond, Veronique; Psimaras, Dimitri; Antoine, Jean-Christophe; Delattre, Jean-Yves; Groc, Laurent; Leboyer, Marion

    2016-01-01

    Objective: To precisely describe the initial psychiatric presentation of patients with anti-NMDA receptor (NMDAR) antibodies encephalitis (anti-NMDAR encephalitis) to identify potential clues enhancing its early diagnosis. Methods: We retrospectively studied the French Reference Centre medical records of every adult patient with anti-NMDAR encephalitis to specify the patients' initial psychiatric symptoms leading to hospitalization in a psychiatric department and the reasons underlying the diagnosis of anti-NMDAR encephalitis. Results: The medical records of 111 adult patients were reviewed. Psychiatric features were the initial presentation in 65 patients (59%). Among them, several psychiatric manifestations were observed, including visual and auditory hallucinations (n = 26, 40%), depression (n = 15, 23%), mania (n = 5, 8%), acute schizoaffective episode (n = 15, 23%), and eating disorder or addiction (n = 4; 6%). Forty-five patients (40% of total cohort) were first hospitalized in a psychiatric institution (91% women), with a median duration of stay of 9 days (range 0.25–239 days). Among them, 24 patients (53%) had associated discreet neurologic signs at the first evaluation, while 17 additional patients (38%) developed neurologic signs within a few days. Twenty-one patients (47%) were transferred to a medical unit for a suspicion of antipsychotic intolerance characterized by high temperature, muscle rigidity, mutism or coma, and biological results suggesting rhabdomyolysis. Conclusions: Several psychiatric presentations were observed in patients with anti-NMDAR encephalitis, although none was specific; however, patients, mostly women, also had discreet neurologic signs that should be carefully assessed as well as signs of antipsychotic intolerance that should raise suspicion for anti-NMDAR encephalitis. PMID:27606355

  9. Anti-N-Methyl-D-Aspartate Receptor Encephalitis in HIV Infection

    Directory of Open Access Journals (Sweden)

    Eunice Patarata

    2016-12-01

    Full Text Available Anti-N-methyl-D-aspartate receptor (anti-NMDAR encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Her mental state progressively deteriorated. Without evidence of infection, the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid clinched the diagnosis of autoimmune encephalitis. No evidence of neoplastic disease was found, and the beneficial response to immunosuppressive therapy was exceptional. This is the first report of anti-NMDAR encephalitis in an HIV-infected individual, reminding us that autoimmune encephalitis should be included in the differential diagnosis of a young patient presenting in an acute confusional state.

  10. Anti-N-Methyl-D-Aspartate Receptor Encephalitis in HIV Infection

    Science.gov (United States)

    Patarata, Eunice; Bernardino, Vera; Martins, Ana; Pereira, Rui; Loureiro, Conceição; Moraes-Fontes, Maria Francisca

    2016-01-01

    Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Her mental state progressively deteriorated. Without evidence of infection, the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid clinched the diagnosis of autoimmune encephalitis. No evidence of neoplastic disease was found, and the beneficial response to immunosuppressive therapy was exceptional. This is the first report of anti-NMDAR encephalitis in an HIV-infected individual, reminding us that autoimmune encephalitis should be included in the differential diagnosis of a young patient presenting in an acute confusional state. PMID:28101036

  11. 抗NMDA受体脑炎患者卵巢囊肿剔除术的麻醉处理%Anesthetic management for ovarian cystectomy in patients with anti-N-methyl-D-aspartate receptor encephalitis undergoing general anesthesia

    Institute of Scientific and Technical Information of China (English)

    陈雯; 桑诺尔; 罗爱伦; 黄宇光

    2014-01-01

    Objective Anti-N-methyl-D-Aspartate (NMDA) receptor encephalitis is a rare disease,recently described as autoimmune disorder of paraneoplastic limbic encephalitis,which is related to the NMDA receptor antibodies and frequently develops in young women with ovarian teratoma.The disease is usually accompanied by symptoms of psychosis and abnormal behaviors,autonomic nervous system dysfunction,central hypoventilation,and hyperthermia.During induction and maintenance of general anesthesia,we should be aware of adverse reactions such as cardiovascular events,hyperthermia and respiratory insufficiency.In order to maintain vital signs stable,pharmacological agents including vasopressors,β-blockers,antihypertensives,and anticholinergics should be prepared before the surgery.Invasive monitoring for blood pressure can be set if necessary.This study described the method for anesthetic management of 3 patients with anti-NMDA receptor encephalitis undergoing resection of ovarian teratoma under general anesthesia.Preoperative treatment included antipsychotic,anti-infective and immune therapy.General anesthesia was induced with propofol,rocuronium,fentanyl and midazolam to facilitate tracheal intubation and was maintained with inhalation of sevoflurane (mixed with oxygen and air) and intermittent iv boluses of fentanyl and rocuronium during the surgery.All the drugs mentioned above had no interaction or had slight indirect action on anti-NMDA receptors to avoid NMDA-related adverse reactions.In conclusion,the adequate preparation for the surgery should be done in this kind of patients,we should avoid using anesthetics having NMDA receptor antagonism (such as ketamine,N2O,methadone,dextromethorphan,phencyclidine) or other anesthetics acting indirectly (such as pentobarbital) on the NMDA receptors during anesthetic management in the patients with anti-NMDA receptor encephalitis.%目的 抗NMDA受体脑炎是一种与抗NMDA受体抗体相关的、自身免疫性副肿瘤边缘叶

  12. Psychotic symptoms in anti-N-methyl-d-aspartate (NMDA) receptor encephalitis: A case report and challenges.

    Science.gov (United States)

    Sharma, Pawan; Sagar, Rajesh; Patra, Bichitrananda; Saini, Lokesh; Gulati, Sheffali; Chakrabarty, Biswaroop

    2016-08-01

    Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis, only recently first described, is an increasingly well-recognized inflammatory encephalitis that is seen in children and adults. An 11-year old girl admitted to the psychiatry ward with a presentation of acute psychosis was diagnosed with NMDA receptor encephalitis following neurology referral and was treated accordingly. This case highlights psychiatric manifestations in encephalitis and the need for the psychiatrist to have high index of suspicion when atypical symptoms (e.g., dyskinesia, seizure, fever etc.) present in acutely psychotic patients.

  13. N-Methyl-D-Aspartate Receptor Antibodies in Herpes Simplex Encephalitis

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    J Gordon Millichap

    2013-02-01

    Full Text Available Researchers at Charite University Medicine Berlin, and other centers in Germany, Spain and the US performed a retrospective analysis of 44 patients with polymerase chain reaction-proven herpes simplex encephalitis (HSE for the presence of onconeuronal and synaptic receptor antibodies.

  14. Analysis of EEG features of neuronal surface antibody associated encephalitis

    Directory of Open Access Journals (Sweden)

    Lu-hua WEI

    2016-09-01

    Full Text Available Objective To summarize the clinical manifestations, EEG and head MRI features of neuronal surface antibody associated encephalitis, and to investigate the role of EEG in determining the relapse or fluctuation of this disease, characteristics of EEG corresponding to head MRI, and EEG features in different clinical stages. Methods A total of 23 patients with neuronal surface antibody associated encephalitis were divided into ascent, climax, descent and recovery stage according to their clinical course. The relation between EEG background activity, distribution of slow wave, epileptiform discharge, extreme delta brush (EDB and relapse or fluctuation of the disease was analyzed. The relation between EEG features and head MRI abnormalities, and also EEG features in different stages were analyzed. Results There were 19 anti-N-methyl-D-aspartate (NMDA receptor encephalitis patients, 3 anti-leucine-rich glioma-inactivated 1 (LGI1 antibody associated encephalitis and one anti-γ-aminobutyric acid B receptor (GABABR antibody associated encephalitis. The frequencies of clinical presentations were psychological or cognitive dysfunction, epileptic seizure, conscious disturbance, speech dysfunction and movement disorder in descending order. Within 30.50 d from onset, 6 patients demonstrated slow wave background, of whom 2 relapsed or fluctuated; 5 patients had α rhythm background and none of them relapsed or fluctuated. In patients with anti-NMDA receptor encephalitis, the difference in first hospital stay (Z = -0.785, P = 0.433 and relapse or fluctuation (Fisher's exact probability: P = 0.155 between EDB group and non-EDB group was not significant. There was no apparent correlation between EEG background activities and head MRI abnormalities in different stages. In ascent and climax stage, EEG background activities were predominantly slow wave, and the distribution of slow wave was relatively broader. EEG background changed to α rhythm from descent stage

  15. Anti-N-methyl-d-aspartate receptor encephalitis in a patient with neuromyelitis optica spectrum disorders.

    Science.gov (United States)

    Luo, Jing-Jing; Lv, He; Sun, Wei; Zhao, Juan; Hao, Hong-Jun; Gao, Feng; Huang, Yi-Ning

    2016-07-01

    We described a female patient with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis occurring sequentially with neuromyelitis optica spectrum disorders (NMOSD). The 19-year-old patient initially presented a diencephalic syndrome with aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) and brain lesions which involving bilateral medial temporal lobes and periependymal surfaces of the third ventricle on magnetic resonance imaging (MRI). Ten months later, the patient developed cognitive impairment, psychiatric symptoms and dyskinesia with left basal ganglia lesions on brain MRI. Meanwhile, the anti-NMDAR antibodies were positive in the patient's serum and cerebrospinal fluid, while the screening tests for an ovarian teratoma and other tumors were all negative. Hence, the patient was diagnosed NMOSD and anti-NMDAR encephalitis followed by low-dose rituximab treatment with a good response. This case was another evidence for demyelinating syndromes overlapping anti-NMDAR encephalitis in Chinese patients.

  16. First Bahraini adolescent with anti-NMDAR-Ab encephalitis.

    Science.gov (United States)

    Almuslamani, Ahood; Mahmood, Fatima

    2015-01-01

    Anti-N-methyl-D-aspartate-receptor (NMDA-R) encephalitis is a new autoimmune, often paraneoplastic disorder that presents with complex neuropsychiatric symptoms. It was first described in 2007 by Dalmau et al. Our patient presented with headache, behavioral changes and then seizures with hallucinations. She was initially misdiagnosed to have schizophrenia and was prescribed antipsychotics. She deteriorated and developed further seizures with hypoventilation and choreoathetosis. Her blood investigations were positive for mycoplasma IGM. Her CSF studies showed high white cell counts, predominantly lymphocytes, and high anti-NMDA-R titre. Her brain MRI scans showed high tbl2 and FLAIR intensities in the grey and white matter of the left cerebellar hemisphere suggestive of acute disseminated encephalomyelitis. She responded to treatment with antibiotics, multiple antiepileptics, steroids and needed five sessions of plasmapheresis. There was no underlying malignancy on repeated scanning of the abdomen. She needed around one year for full recovery with intensive rehabilitation. The objective of this paper was to highlight the occurrence of this fairly new, challenging, easily missed, not-so-rare form of encephalitis often occurring in the absence of fever.

  17. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis in a young Lebanese girl.

    Science.gov (United States)

    Safadieh, Layal; Dabbagh, Omar

    2013-10-01

    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently recognized autoimmune neurologic disorder that presents with severe neuropsychiatric symptoms in previously healthy children. A 4-year-old Lebanese girl presented with new-onset behavioral changes, orofacial dyskinesias, fluctuation in consciousness, inability to walk, and mutism. Antibodies directed against NMDA receptors were detected in the patient's serum and cerebrospinal fluid. Prompt treatment with a single course of intravenous immunoglobulin resulted in early complete recovery. This is the first case report of a Middle Eastern child affected with this condition.

  18. Anti-N-methyl-D-aspartate receptor encephalitis presenting with acute psychosis in a preteenage girl: a case report

    Directory of Open Access Journals (Sweden)

    Maggina Paraskevi

    2012-07-01

    Full Text Available Abstract Introduction Anti-N-methyl-D-aspartate receptor (anti-NMDAR encephalitis is a rare, newly defined autoimmune clinical entity that presents with atypical clinical manifestations. Most patients with anti-N-methyl-D-aspartate receptor encephalitis develop a progressive illness from psychosis into a state of unresponsiveness, with catatonic features often associated with abnormal movements and autonomic instability. This is the first report of anti-N-methyl-D-aspartate receptor encephalitis in a Greek pediatric hospital. Case presentation An 11-year-old Greek girl presented with clinical manifestations of acute psychosis. The differential diagnosis included viral encephalitis. The presence of a tumor usually an ovarian teratoma, a common clinical finding in many patients, was excluded. Early diagnosis and prompt immunotherapy resulted in full recovery up to one year after the initial diagnosis. Conclusion Acute psychosis is a rare psychiatric presentation in children, diagnosed only after possible organic syndromes that mimic acute psychosis are excluded, including anti-N-methyl-D-aspartate receptor receptor encephalitis. Pediatricians, neurologists and psychiatrists should consider this rare clinical syndrome, in order to make an early diagnosis and instigate appropriate treatment to maximize neurological recovery.

  19. Anti-AMPA-Receptor Encephalitis Presenting as a Rapid-Cycling Bipolar Disorder in a Young Woman with Turner Syndrome

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    Giuseppe Quaranta

    2015-01-01

    Full Text Available Background. Autoimmune encephalitis is a disorder characterised by the subacute onset of seizures, short-term memory loss, and psychiatric and behavioural symptoms. Initially, it was recognised as a paraneoplastic disorder, but recently a subgroup of patients without systemic cancer was identified. Case Description. We describe a 20-year-old woman with Turner syndrome presenting with a treatment-resistant rapid cycling bipolar disorder with cognitive impairment. She was diagnosed with anti-AMPA-receptor encephalitis. She showed marked improvement after starting memantine and valproic acid. Conclusion. This case description emphasises the importance of timely recognition of autoimmune limbic encephalitis in patients with psychiatric manifestations and a possible predisposition to autoimmune conditions, in order to rule out malignancy and to quickly initiate treatment.

  20. Young girl with abnormal behavior: Anti-N-Methyl-D-Aspartate receptor immune encephalitis

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    Vinit Suri

    2013-01-01

    Full Text Available Anti N Methyl D Aspartate receptor immune encephalitis (Anti NMDARE is a recently defined, under-recognized and often misdiagnosed disease, which typically occurs in young females and may be associated with an underlying tumor, usually ovarian teratoma. If diagnosed early, initiation of immunotherapy and tumor removal (if present may result in recovery. We report a case of a 17 years old girl with Anti NMDARE who was initially misdiagnosed as Functional psychosis, Neuroleptic Malignant Syndrome and Sepsis syndrome. To the best of our knowledge, this is only the second case of anti NMDARE being reported from India. This case report underscores the need for a greater awareness of this entity across multiple specialties, e.g., general medicine, psychiatry and neurology, to ensure a heightened diagnostic suspicion, which can lead to timely diagnosis and adequate therapy of this treatable disease.

  1. Ketamine Infusion Associated with Improved Neurology in a Patient with NMDA Receptor Encephalitis

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    Michael MacMahon

    2013-01-01

    Full Text Available A young lady was ventilated on intensive care for a prolonged period with NMDA receptor encephalitis. She had undergone steroid, immunoglobulin, and plasmapheresis with no evidence of recovery. Her main management issue was the control of severe orofacial and limb dyskinesia. Large doses of sedating agents had been used to control the dystonia but were ineffective, unless she was fully anaesthetised. The introduction of a ketamine infusion was associated with a dramatic improvement in her symptoms such that it was possible to remove her tracheostomy two days after commencement. She was discharged shortly after that and is making a good recovery. The successful use of ketamine has not previously been described in this context, and we hope this case report will provide some insight into the management of this rare but serious condition.

  2. A case of non-paraneoplastic anti-N-methyl d-aspartate receptor encephalitis presenting as a neuropsychiatric disorder

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    Bindu Yoga

    2014-11-01

    Full Text Available N-methyl d-aspartate receptor antibody encephalitis can often be a paraneoplastic manifestation of occult malignancy such as ovarian teratoma and rarely teratoma of mediastinum or testis and small cell lung carcinoma. We report a case of non-paraneoplastic anti-N-methyl d-aspartate receptor antibody–positive autoimmune encephalitis in a young patient who presented with neuropsychiatric features and made a very good recovery following treatment with intravenous immunoglobulin and steroids. The case highlights the need for increased vigilance for the condition in young females with or without a previous psychiatric history and emphasises the need for a multidisciplinary approach in the management of this challenging disorder with a good prognosis.

  3. A case of non-paraneoplastic anti-N-methyl d-aspartate receptor encephalitis presenting as a neuropsychiatric disorder.

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    Yoga, Bindu; Kunc, Marek; Ahmed, Fayyaz

    2014-01-01

    N-methyl d-aspartate receptor antibody encephalitis can often be a paraneoplastic manifestation of occult malignancy such as ovarian teratoma and rarely teratoma of mediastinum or testis and small cell lung carcinoma. We report a case of non-paraneoplastic anti-N-methyl d-aspartate receptor antibody-positive autoimmune encephalitis in a young patient who presented with neuropsychiatric features and made a very good recovery following treatment with intravenous immunoglobulin and steroids. The case highlights the need for increased vigilance for the condition in young females with or without a previous psychiatric history and emphasises the need for a multidisciplinary approach in the management of this challenging disorder with a good prognosis.

  4. Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis.

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    Leypoldt, Frank; Gelderblom, Mathias; Schöttle, Daniel; Hoffmann, Sascha; Wandinger, Klaus-Peter

    2013-04-01

    Encephalitis associated with antibodies against N-methyl-d-aspartic acid (NMDA) receptor is characterized by severe memory deficits, decreased consciousness, epileptic seizures and movement disorders and occurs most commonly in young women. Recovery is mostly good but little is known about the disease course in patients whose treatment has been delayed severely. We present a 16-year-old girl with a 36-month follow-up. A single course of methylprednisolone attenuated some symptoms but severe and incapacitating frontotemporal syndrome remained. Second-line treatment with rituximab was initiated 12months after the onset of symptoms. A surprising recovery occurred 18months after treatment and 30months after onset. Recovery in NMDA receptor antibody-associated encephalitis can be severely delayed and does not have to be linear. Whether delayed therapy contributed to recovery in this patient cannot be answered with certainty. Spontaneous recovery independent of therapy is possible, as it has been observed previously as late as 3years after onset. Although serum antibodies disappeared with recovery in this patient, previous cases have shown serum antibodies to be unreliable markers of disease activity. Second-line treatment, especially with substances as well tolerated as rituximab, should at least be considered in NMDA receptor encephalitis with persistent neuropsychiatric syndromes after first-line therapy.

  5. 抗 NMDA 受体脑炎45例特点和临床转归分析%The clinical characteristics and outcomes of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis:a study of 45 cases

    Institute of Scientific and Technical Information of China (English)

    冯雪丹; 胡永强; 冯文良; 程家玲; 张隆辉; 邓欢; 王琳

    2016-01-01

    Objective To investigate the clinical characteristics of anti-N-methyl-D-aspartate receptor (NMDAR)encephalitis in the group. Methods The clinical data of 45 cases with anti-NMDAR encephalitis at neurology department of Beijing Youanmen Hospital and Beijing Xuanwu hospital from October 2012 to April 2015 were collected. The clinical features and followed-up outcomes were analyzed retrospectively. Results In the study of 45 patients, the outbreak age was between 14-61 years old, the mean age was 32.2±13.24 years. Twenty-seven patients were male, three of who(11%)had an underlying tumors. Eighteen were female, five of who(28%)had ovarian teratomas. Prodiomal symptoms were noted in 23(51%)of patients. The main clinical manifestations include psychiatric symptoms(91%), seizures(76%), involuntary movement(42%), hypoventilation(24%), decreased level of consciousness(47%)and autonomic instability(40%).The CSF routine test and biochemistry panel were abnormal in 89% patients and test for anti-NMDA receptor antibodies in CSF were positive in all patients. The EEG records main show generalized or frontal to temporal slow waves wild mild degree(21%)to middle degree(59%) abnormal. Brain CT or magnetic resonance imaging(MRI)were abnormal in 53% of patients and majority of them showing that T2 or FLAIR hyperintensities within frontal lobes and temporal lobes. All patients were treated with a combination of corticosteroids and IVIg .Six patients were received immunosuppressive agent(Clclophosphamide or Mycophenolate Mofetil). Except one was lost to follow-up, 86% experienced complete or near-complete recovery (modified rankin scale[MRS]0-2). Conclusion The incidence of anti-NMDAR encephalitis is not uncommon in men, and this disease is less associated with tumor. Anti-NMDAR encephalitis is characterized by psychiatric-behavior abnormality, seizures and anti-NMDAR antibody positive in CSF. 79% patients received a substantial recovery by early first-line immunosuppressive

  6. N-methyl-D-aspartate receptor encephalitis mediates loss of intrinsic activity measured by functional MRI.

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    Brier, Matthew R; Day, Gregory S; Snyder, Abraham Z; Tanenbaum, Aaron B; Ances, Beau M

    2016-06-01

    Spontaneous brain activity is required for the development and maintenance of normal brain function. Many disease processes disrupt the organization of intrinsic brain activity, but few pervasively reduce the amplitude of resting state blood oxygen level dependent (BOLD) fMRI fluctuations. We report the case of a female with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, longitudinally studied during the course of her illness to determine the contribution of NMDAR signaling to spontaneous brain activity. Resting state BOLD fMRI was measured at the height of her illness and 18 weeks following discharge from hospital. Conventional resting state networks were defined using established methods. Correlation and covariance matrices were calculated by extracting the BOLD time series from regions of interest and calculating either the correlation or covariance quantity. The intrinsic activity was compared between visits, and to expected activity from 45 similarly aged healthy individuals. Near the height of the illness, the patient exhibited profound loss of consciousness, high-amplitude slowing of the electroencephalogram, and a severe reduction in the amplitude of spontaneous BOLD fMRI fluctuations. The patient's neurological status and measures of intrinsic activity improved following treatment. We conclude that NMDAR-mediated signaling plays a critical role in the mechanisms that give rise to organized spontaneous brain activity. Loss of intrinsic activity is associated with profound disruptions of consciousness and cognition.

  7. The clinical significance of EEG in early diagnosis and prognosis of Anti-NMDA receptor encephalitis in children%脑电图监测对儿童抗N-甲基-D-天冬氨酸受体脑炎早期诊断和预后判定的意义

    Institute of Scientific and Technical Information of China (English)

    李华; 王晓慧; 方方; 吕俊兰; 肖静; 陈春红; 丁昌红; 任晓暾; 伍妘

    2015-01-01

    目的:探讨脑电图(EEG)对儿童抗N-甲基-D-天冬氨酸(NMDA)受体脑炎早期诊断和预后判定的意义.方法:对2011年8月~2013年9月收治的49例抗NMDA受体脑炎患儿按照改良的Rankin表评估病情轻重,分为病情轻组、中重组,并于疾病急性期、恢复期行视频脑电图(V-EEG)监测,根据改良的Rankin表评价患儿转归,患儿恢复期后进行神经心理学测试,评价预后.结果:疾病急性期:49例中EEG正常3例(6%),轻度异常5例(10%)(均为病情轻组),中重度异常41例(84%),其中6例为病情轻组,余35例为病情中重组.46例异常EEG表现为非特异性改变41(89%),特异性改变-δ刷状波5(10%);病情恢复期:EEG异常者病情轻组仅有2例(4%),而病情中重组18例(39%),病情轻组于恢复期EEG改善率高.结论:EEG异常程度与临床病情严重程度具有一定的相关性.抗NMDA受体脑炎的预后差率随EEG评判分级的增加而增高.EEG可作为抗NMDA受体脑炎早期诊断及预后判定的有效、无创、可重复的检查手段.

  8. A clinical and neurobiological case of IgM NMDA receptor antibody associated encephalitis mimicking bipolar disorder.

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    Choe, Chi-Un; Karamatskos, Evangelos; Schattling, Benjamin; Leypoldt, Frank; Liuzzi, Gianpiero; Gerloff, Christian; Friese, Manuel A; Mulert, Christoph

    2013-07-30

    Autoimmune encephalitis associated with IgG antibodies to the N-methyl-d-aspartic acid receptor subunit NR1 (NMDAR) presents with neurological symptoms, such as seizures, and especially psychiatric symptoms, such as hallucinations, psychosis, agitation and anxiety. To date, however, the pathological relevance of IgM NMDAR antibodies remains elusive. Here, we describe clinical, neuroradiological and neurobiological findings of a 28-year-old male presenting with IgM NMDAR antibodies coincident with autoimmune encephalitis characterized by symptoms of bipolar disorder. After repeated steroid treatment, cognitive and psychiatric abnormalities improved and no NMDAR antibody was detectable. Using primary neuronal cultures, we demonstrate that patient's serum containing IgM NMDAR antibodies reduced the detection of NMDAR on neuronal cells and decreased cell survival. Although NMDAR encephalitis with IgG antibodies is increasingly recognized and diagnosed, atypical presentations with NMDAR antibodies with immunoglobulin subclasses other than IgG pose a diagnostic and therapeutic challenge. Further clinical and neurobiological studies are needed to study the pathophysiological relevance of IgM NMDAR antibodies.

  9. Analysis of efficacy and safety of intravenous immunoglobulin for treatment of anti-N-methyl-D-aspartate receptor encephalitis%人免疫球蛋白治疗抗N-甲基-D-门冬氨酸受体脑炎的疗效和安全性分析

    Institute of Scientific and Technical Information of China (English)

    张艳; 宿英英; 叶红; 高岱佺

    2015-01-01

    目的 探讨静脉输注人免疫球蛋白(WIG)治疗抗N-甲基-D-门冬氨酸(NMDA)受体脑炎的疗效和安全性.方法 对2012年1月至2014年12月收入首都医科大学宣武医院神经内科的抗NMDA受体脑炎、首次接受IVIG治疗[0.4g/(b·d),1次/d,连续5d]患者进行回顾性研究.在IVIG治疗期间和用药结束后1周内记录患者不良反应发生情况.IVIG治疗结束1个月后,采用改良mRS评分评估临床疗效,将患者按疗效分为有效组和无效组. 结果 共纳入患者26例,男性11例,女性15例,年龄16~ 57岁,平均(25±10)岁.患者发病至开始IVIG治疗的时间为8~68 d(中位数26 d).治疗1个月后,有效14例(显效6例、好转8例),有效率为53.8%.IVIG治疗至临床症状改善的患者时间为7 ~30 d,平均17 d.有效组男性占比高于无效组[64.3%(9/14)比16.7%(2/12),P<0.05].男性患者有效率高于女性患者[81.8%(9/11)比33.3%(5/15),P<0.05].观察期内出现发热3例、肝功能异常2例、皮疹1例,经减慢IVIG输注速度并给予保肝、抗过敏治疗,患者均恢复正常. 结论 IVIG治疗抗NMDA受体脑炎有效且相对安全,可作为一线治疗方案.%Objective To evaluate the efficacy and safety of intravenous immunoglobulin (IVIG) for treatment of anti-N-methyl-D-aspartatc (NMDA) receptor encephalitis.Methods Data of patients with anti-NMDA receptor encephalitis seen in Xuanwu Hospital, Capital Medical University from January 2012 to December 2014 were collected and a retrospective study was conducted.All patients received the first course of treatment with IVIG 0.4 g/(kg · d) , once daily for 5 consecutive days.The adverse reactions of IVIG during the observational period of IVIG administration and one week thereafter were recorded.The therapeutic effect of IVIG was assessed by using modified Rankin Scale one month post IVIG.According to the therapeutic effect of IVIG, patients were divided into effective group and ineffective group

  10. Anti-N-methyl-d-aspartate receptor encephalitis in a patient with a 7-year history of being diagnosed as schizophrenia: complexities in diagnosis and treatment

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    Huang C

    2015-06-01

    Full Text Available Chaohua Huang,1,2,4,* Yukun Kang,1,* Bo Zhang,1 Bin Li,1 Changjian Qiu,1 Shanming Liu,1 Hongyan Ren,1,2 Yanchun Yang,1 Xiehe Liu,1 Tao Li,1–3 Wanjun Guo1,21Mental Health Center, West China Hospital, Sichuan University, Chengdu, Sichuan, People’s Republic of China; 2State Key Laboratory of Biotherapy, Psychiatric Laboratory, West China Hospital, Sichuan University, Chengdu, Sichuan, People’s Republic of China; 3Mental Health Education Center, Sichuan University, Chengdu, Sichuan, People’s Republic of China; 4Mental Health Center, Affiliated Hospital of Luzhou Medical College, Luzhou, People’s Republic of China*These authors contributed equally to this workAbstract: Anti-N-methyl-d-aspartate receptor (NMDAR encephalitis is a form of autoimmune encephalitis associated with antibodies against the NR1 subunits of NMDARs. Although new-onset acute prominent psychotic syndromes in patients with NMDAR encephalitis have been well documented, there is a lack of case studies on differential diagnosis and treatment of anti-NMDAR encephalitis after a long-term diagnostic history of functional psychotic disorders. The present study reports an unusual case of anti-NMDAR encephalitis. The patient had been diagnosed with schizophrenia 7 years earlier, and was currently hospitalized for acute-onset psychiatric symptoms. The diagnosis became unclear when the initial psychosis was confounded with considerations of other neurotoxicities (such as neuroleptic malignant syndrome. Finally, identification of specific immunoglobulin G NR1 autoantibodies in the cerebrospinal fluid and greater effectiveness of immunotherapy over antipsychotics alone (which has been well documented in anti-NMDAR encephalitis indicated the diagnosis of anti-NMDAR encephalitis in this case. Based on the available evidence, however, the relationship between the newly diagnosed anti-NMDAR encephalitis and the seemingly clear, long-term history of schizophrenia in the preceding 7

  11. Anti-N-Methyl-D-Aspartate Receptor Encephalitis Causing a Prolonged Depressive Disorder Evolving to Inflammatory Brain Disease

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    Sara Mariotto

    2014-02-01

    Full Text Available Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR encephalitis is a rapidly evolving condition that combines psychiatric and neurologic manifestations. Much remains unclear about its clinical onset and subsequent course. Although successful treatment depends on diagnosing the disorder early and therefore minimizing long-term complications, this is a difficult task owing to the atypical onset of this condition and the prolonged clinical course that has been observed in some patients. This report, illustrating a patient with slowly progressing psychiatric manifestations, unusual imaging and electrophysiological features, extends the information on varied clinical phenotypes. Case Report: A 32-year-old woman suffered from an isolated depressive disorder for 4 months before undergoing psychiatric evaluation. During the following 5 months, she manifested hypersexuality, dysarthria, imbalance, dyskinesias and decreased word output. Brain magnetic resonance imaging (MRI showed multifocal hyperintense T2/FLAIR lesions, a few contrast-enhanced, involving the corona radiata, the periventricular white matter, the deep gray nuclei, the optic nerves and the brainstem. MRI spectroscopy disclosed confluent bilateral demyelination and focal optic nerve involvement suggesting widespread encephalitis. Visual evoked potential studies indicated a demyelinating disorder. Serological screening and total body positron-emission tomography yielded negative findings for malignancies. Cerebrospinal fluid examination disclosed IgG oligoclonal bands and anti-NMDAR antibodies. Corticosteroids and intravenous immunoglobulin provided only slight improvement, whereas switching to cyclophosphamide markedly improved her neurological status. Conclusion: In patients with a prolonged clinical course, including psychiatric and neurological symptoms, the differential diagnosis should be anti-NMDAR encephalitis. This report expands the known disease phenotypes in this emerging

  12. Viral encephalitis

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    Marcus Tulius T Silva

    2013-09-01

    Full Text Available While systemic viral infections are exceptionally common, symptomatic viral infections of the brain parenchyma itself are very rare, but a serious neurologic condition. It is estimated that viral encephalitis occurs at a rate of 1.4 cases per 100.000 inhabitants. Geography is a major determinant of encephalitis caused by vector-borne pathogens. A diagnosis of viral encephalitis could be a challenge to the clinician, since almost 70% of viral encephalitis cases are left without an etiologic agent identified. In this review, the most common viral encephalitis will be discussed, with focus on ecology, diagnosis, and clinical management.

  13. Limbic Encephalitis Associated with Anti-γ-aminobutyric Acid B Receptor Antibodies: A Case Series from China

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    Hong-Zhi Guan; Hai-Tao Ren; Xun-Zhe Yang; Qiang Lu; Bin Peng; Yi-Cheng Zhu; Xiao-Qiu Shao

    2015-01-01

    Background: Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABABR) in patients with limbic encephalitis (LE) was first described in 2010.We present a series of Han Chinese patients for further clinical refinement.Methods: Serum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence.Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed.Results: All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram (EEG) or neuroimaging evidence fulfilled the diagnostic criteria of LE.Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSE Seventeen (17/18) patients presented with new-onset refractory seizure or status epileptics.Twelve (12/18) patients had memory deficits, 11 (11/18) patients had personality change, 7 (7/18) patients had disturbance of consciousness, and 3 (3/18) patients showed cerebellar dysfunction.One patient with LE had progressive motor and sensory polyneuropathy.Lung cancer was detected in 6 (6/18) patients.Ten (10/18) patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging.Ten (10/18) patients had temporal lobe epileptic activity with or without general slowing on EEG.Seventeen patients received immunotherapy and 15 of them showed neurological improvement.Four patients with lung cancer died within 1-12 months due to neoplastic complications.Conclusions: Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy.Patients with underlying lung tumor have a relatively poor prognosis

  14. 儿童抗N-甲基-D-天冬氨酸受体脑炎七例%Anti-N-methyl-D-aspartate receptor encephalitis in seven children

    Institute of Scientific and Technical Information of China (English)

    王晓慧; 任海涛; 许春伶; 方方; 丁昌红; 吕俊兰; 韩彤立; 刘丽英; 李久伟; 伍妘; 崔丽英

    2012-01-01

    Objective To study the clinical and laboratory features and diagnosis of the patient with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children.Method The data of clinical feature,laboratory findings,and radiological manifestation were reviewed and analyzed.Result Of the 7 patients,4 were female and 3 were male.The age of onset was from 6.6 to 15.5 years (average 9.5 years).The onset of 4 cases started with convulsion.Six cases had seizures which was difficult to control by antiepileptic drugs.All patients had psychiatric symptoms and speech disorder.Six cases had different levels of decreased consciousness and dyskinesias.6 cases had autonomic nerve instability,and 7 cases developed sleep disorders.The results of MRI examination were normal in all patients.The EEG of most patients showed focal or diffuse slow waves.Six cases had oligoclonal bands.All cases were confirmed to have the disease by detection of anti-NMDA receptor antibodies.No tumor was detected in any of the patients.All patients received immunotherapy.Conclusion Anti-NMDAR encephalitis is a severe but treatable disorder that frequently affects children and adolescents.Pediatric patients had clinical manifestations similar to those of adult patients.But children have a lower incidence of tumors and hypoventilation also occurs less frequently in children.Most of children had a good prognosis.%目的 探讨儿童抗N-甲基-D-天冬氨酸(NMDA)受体脑炎临床特点和诊断,提高对该病的认识.方法 对确诊为抗NMDA受体脑炎患儿的临床表现、实验室及影像学资料进行回顾性分析.结果 本组7例,女4例,男3例,年龄6岁8个月至15岁6个月,平均年龄9岁6个月.4例以惊厥起病,6例病程中存在抗癫(癎)药物难以控制的惊厥发作;7例均有精神症状及语言障碍;6例具有不同程度意识障碍、锥体外系不自主运动,其中口舌面异常运动6例、舞蹈和(或)手足徐动5例、姿势异常5例;6例

  15. Role of Autoantibodies to N-Methyl-d-Aspartate (NMDA) Receptor in Relapsing Herpes Simplex Encephalitis: A Retrospective, One-Center Experience.

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    Sutcu, Murat; Akturk, Hacer; Somer, Ayper; Tatli, Burak; Torun, Selda Hancerli; Yıldız, Edibe Pembegul; Şık, Guntulu; Citak, Agop; Agacfidan, Ali; Salman, Nuran

    2016-03-01

    Post-herpes simplex virus encephalitis relapses have been recently associated with autoimmunity driven by autoantibodies against N-methyl-d-aspartate (NMDA) receptors. Because it offers different treatment options, determination of this condition is important. Between 2011 and 2014, 7 children with proven diagnosis of herpes simplex virus encephalitis were identified in a university hospital of Istanbul. Two patients had neurologic relapse characterized mainly by movement disorders 2 to 3 weeks after initial encephalitis. The first patient received a second 14 days of acyclovir treatment together with antiepileptic drugs and left with severe neurologic sequelae. The second patient was found to be NMDA receptors antibody positive in the cerebrospinal fluid. She was treated with intravenous immunoglobulin and prednisolone. She showed substantial improvement, gradually regaining lost neurologic abilities. Post-herpes simplex virus encephalitis relapses may frequently be immune-mediated rather than a viral reactivation, particularly in children displaying movement disorders like choreoathetosis. Immunotherapy may provide benefit for this potentially devastating condition, like the case described in this report.

  16. Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis: A nationwide study in Taiwan.

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    Duan, Bi-Chun; Weng, Wen-Chin; Lin, Kuang-Lin; Wong, Lee Chin; Li, Sung-Tse; Hsu, Mei-Hsin; Lin, Jainn-Jim; Fan, Pi-Chaun; Lin, Ming-I; Chiu, Nan-Chang; Lin, Yu-Ching; Wang, Huei-Shyong; Hung, Kun-Long; Lee, Wang-Tso

    2016-09-01

    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years.Total 28 patients (20 females and 8 males) with age ranging from 8 months to 38 years were enrolled. Nearly all patients (n = 27/28, 96%) presented with at least 2 types of disorders, including orofacial-lingual dyskinesia (OFLD; n = 20), catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathethosis (n = 9), and ballism (n = 3). Only 1 patient below 10 years presented with isolated periodic choreoathethosis without other movement disorders. OFLD was common in all age groups. Choreoathetosis was most common in patients aged ≤10 years, while catatonia was most common in patients aged >10 years (P = 0.001 and 0.020, respectively). Bradykinesia was also more common in patients aged >10 years (P = 0.020). The clinical presentations of movement disorders were not significantly different in the age of 10 to 18 years and those >18 years. Neither patient ≤10 years old nor male patients had associated tumors. All patients' movement disorders were improved after treatment, while female patients with tumors had worse short-term outcome (P = 0.014). Compared with other disorders, choreoathetosis persisted significantly longer in patients ≤10 years (P = 0.038), while OFLD and catatonia last longer in patients >10 years (P = 0.047 and 0.002, respectively).Our study shows that

  17. Pediatric anti-N-methyl-D-aspartate (NMDA) receptor encephalitis: experience of a tertiary care teaching center from north India.

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    Chakrabarty, Biswaroop; Tripathi, Manjari; Gulati, Sheffali; Yoganathan, Sangeetha; Pandit, Awadh Kishore; Sinha, Aditi; Rathi, Bhim Singh

    2014-11-01

    Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is characterized by acute- or subacute-onset encephalopathy with extrapyramidal, psychiatric, and epileptic manifestations. Diagnosis is confirmed by positive antibodies to NMDA receptor in cerebrospinal fluid and serum. Eleven pediatric cases presented over a 2-year period at a tertiary care teaching hospital in North India. The average age at presentation was 9 years (range: 2.5 to 18 years, median: 10 years) with a slight female predominance (1.2:1). The common modes of presentation were progressive extrapyramidal syndrome with global neuroregression in 45% (5 of 11), epileptiform encephalopathy in 27% (3 of 11), and an overlap between the 2 in 27% (3 of 11). Fifty-eight percent showed significant response to steroids and intravenous immunoglobulin. This entity should be considered in an acute- or subacute-onset encephalopathy if common infectious etiologies are ruled out and there are specific clinical pointers. Early diagnosis and treatment significantly improves the outcome.

  18. Acanthamoeba encephalitis

    Directory of Open Access Journals (Sweden)

    Kaushal V

    2008-01-01

    Full Text Available Central nervous system infection with free-living amoebae is rare. We present a fatal case of Acanthamoeba encephalitis in a 63-year-old female from India where acanthamoebae were demonstrated and cultured from CSF. In spite of treatment with amphotericin B, fluconazole and rifampicin the patient did not survive. Amoebic infection should be suspected in a patient of encephalitis of unexplained aetiology as timely diagnosis can lead to a favourable outcome.

  19. Electroencephalographic and fluorodeoxyglucose-positron emission tomography correlates in anti-N-methyl-d-aspartate receptor autoimmune encephalitis

    Directory of Open Access Journals (Sweden)

    John C. Probasco

    2014-01-01

    Conclusions and relevance: This case of anti-NMDAR autoimmune encephalitis illustrates the challenges of distinguishing prolonged LE from LSE. We discuss the parallels between these two conditions and propose a management paradigm to optimize evaluation and treatment.

  20. Encephalitis and GABAB receptor antibodies: Novel findings in a new case series of 20 patients

    NARCIS (Netherlands)

    R. Höftberger (Romana); M.J. Titulaer (Maarten); L. Sabater (Lidia); J.S. Dome (Jeffrey); A. Rózsás (Anita); B. Hegedus (Balazs); M.A. Hoda (Mir Alireza); V. Laszlo (Viktoria); H.J. Ankersmit (Hendrik Jan); L. Harms (Lutz); S. Boyero (Sabas); A. de Felipe (Alicia); A. Saiz (Albert Abe); J. Dalmau (Josep); F. Graus (Francesc)

    2013-01-01

    textabstractObjective: To report the clinical features of 20 newly diagnosed patients with GABAB receptor (GABABR) antibodies and determine the frequency of associated tumors and concurrent neuronal autoantibodies. Methods: Clinical data were retrospectively obtained and evaluated. Serum and CSF sam

  1. Clinical analysis of five cases positive for anti-N-methyl-D-aspartic acid (NMDA) receptor antibodies%抗NMDA受体抗体检测在抗NMDA受体脑炎诊断中的意义

    Institute of Scientific and Technical Information of China (English)

    刘媛媛; 曹秉振; 唐吉刚; 胡怀强; 李传芬

    2015-01-01

    目的:总结间接免疫荧光法检测抗NMDA受体抗体阳性患者的临床特点及诊疗思路。方法回顾性分析2012年4月到2014年11月在济南军区总医院神经内科住院期间检测抗NMDA受体抗体阳性的5例患者的诊断、临床特点、治疗及预后。结果5例患者中4例临床确诊为抗NMDA受体脑炎,1例诊断为单纯疱疹病毒性脑炎(herpes simplex virus encephalitis, HSE)。5例患者发病相似,3例有明确的流感样前驱感染史,3例以精神症状首次发病,4例病情发展到一定阶段出现癫痫、呼吸衰竭入住NICU。4例抗NMDA受体脑炎患者病情后期均出现突出的运动障碍等症状。患者颅脑MRI、脑电图存在不同程度及范围异常,4例合并病毒学及免疫学指标异常。抗NMDA受体脑炎患者经激素、免疫球蛋白等治疗病情好转;HSE患者经抗病毒等治疗后病情改善。随访1年后2例抗NMDA受体脑炎患者出现肿瘤,1例死亡。结论间接免疫荧光检测抗NMDA受体抗体阳性的患者多数为抗NMDA受体脑炎,少数可为HSE等,诊断要综合患者病情及各项检查分析,防止误诊、漏诊,以正确指导治疗。%Objective To summarize and analysis the clinical features, diagnosis and treatment of the cases which were positive for anti-N-methyl-D-aspartic acid (NMDA) receptor antibodies by indirect immunofluorescence assay (IFA). Methods We analyzed the disease process, clinical characteristics, auxiliary examination , diagnosis, treat-ment, and prognosis of five cases positive for anti NMDA receptor antibodies in their serum and cerebrospinal fluid (CSF). Results Four of the five cases positive for anti-N-methyl-D-aspartic acid (NMDA) receptor antibodies were di-agnosed with anti-NMDA receptor encephalitis and one was diagnosed with Herpes Simplex Virus Encephalitis(HSE). The five cases had a similar disease presentation including prodromal flu-like symptoms in three cases and

  2. Encephalitis (For Parents)

    Science.gov (United States)

    ... West Nile encephalitis, St. Louis encephalitis, and Western Equine encephalitis. Over the last several years in the ... to prevent further swelling of the brain. Because antibiotics aren't effective against viruses, they aren't ...

  3. Anti-N-methyl-D-aspartate receptor encephalitis:A new limbic encephalitis%抗NMDAR脑炎:一种新型边缘叶脑炎

    Institute of Scientific and Technical Information of China (English)

    周晨光; 王建平

    2014-01-01

    抗N-甲基-M-天冬氨酸受体(N-methyl-D-aspartate receptor)脑炎是一种自2007年确认的由抗NMDA受体抗体介导的可治性新型边缘叶脑炎,好发于年轻女性,临床上主要表现为发展迅速的神经和精神障碍综合征,包括突出的精神行为异常、癫痫发作、异常运动、自主神经功能紊乱等,其中口面颌肌张力障碍和中枢性通气不足是该病的相对特征性表现.MRI检查无特异性改变,脑电图常提示弥漫性的异常,常规脑脊液检查无特异性,血和脑脊液中的NMDAR亚单位NR1抗体阳性可以明确诊断.对糖皮质激素、免疫球蛋白、血浆置换等免疫治疗及肿瘤切除反应良好.为更好地认识该疾病,本文对其临床特点及发病机制等做一综述.

  4. The calcitonin receptor gene is a candidate for regulation of susceptibility to herpes simplex type 1 neuronal infection leading to encephalitis in rat.

    Directory of Open Access Journals (Sweden)

    Nada Abdelmagid

    Full Text Available Herpes simplex encephalitis (HSE is a fatal infection of the central nervous system (CNS predominantly caused by Herpes simplex virus type 1. Factors regulating the susceptibility to HSE are still largely unknown. To identify host gene(s regulating HSE susceptibility we performed a genome-wide linkage scan in an intercross between the susceptible DA and the resistant PVG rat. We found one major quantitative trait locus (QTL, Hse1, on rat chromosome 4 (confidence interval 24.3-31 Mb; LOD score 29.5 governing disease susceptibility. Fine mapping of Hse1 using recombinants, haplotype mapping and sequencing, as well as expression analysis of all genes in the interval identified the calcitonin receptor gene (Calcr as the main candidate, which also is supported by functional studies. Thus, using unbiased genetic approach variability in Calcr was identified as potentially critical for infection and viral spread to the CNS and subsequent HSE development.

  5. 儿童抗N-甲基-D-天冬氨酸受体脑炎%Anti-N-methyl-D-aspartate receptor encephalitis in children

    Institute of Scientific and Technical Information of China (English)

    陈晨

    2013-01-01

    抗N-甲基-D-天冬氨酸受体(N-methyl-D-asparate receptor,NMDAR)脑炎是免疫介导的中枢神经系统综合征,主要临床表现有精神症状、运动障碍、抽搐发作等,常被误诊为病毒性脑炎,与肿瘤尤其卵巢畸胎瘤关系密切;儿童患病率高,抗NMDAR抗体是其较特异的检查;治疗主要为免疫抑制,一线治疗为激素、免疫球蛋白、血浆置换,二线治疗主要包括生物制剂、细胞毒药物;早期诊断及治疗对神经系统的恢复至关重要,总体预后良好.%Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune syndrome of central nervous system.The main clinical manifestations include psychiatric symptoms,movement disorders and seizures.It is often misdiagnosed as viral encephalitis.Tumors,especially the ovarian teratoma,play an important role in the disease.The prevalence in children is beyond imagination.Anti-NMDAR antibodies are specific diagnostic tests.Immune suppression is the main treatment.The first-line treatments include corticosteroids,intravenous immunoglobulin,and plasmapheresis.The second-line treatments include biological agents and cytotoxic drugs.Early diagnosis and treatment are essential for neurologic recovery.Overall prognosis is good.

  6. Nuclear receptor REV-ERBα mediates circadian sensitivity to mortality in murine vesicular stomatitis virus-induced encephalitis.

    Science.gov (United States)

    Gagnidze, Khatuna; Hajdarovic, Kaitlyn H; Moskalenko, Marina; Karatsoreos, Ilia N; McEwen, Bruce S; Bulloch, Karen

    2016-05-17

    Certain components and functions of the immune system, most notably cytokine production and immune cell migration, are under circadian regulation. Such regulation suggests that circadian rhythms may have an effect on disease onset, progression, and resolution. In the vesicular stomatitis virus (VSV)-induced encephalitis model, the replication, caudal penetration, and survivability of intranasally applied VSV depends on both innate and adaptive immune mechanisms. In the current study, we investigated the effect of circadian time of infection on the progression and outcome of VSV-induced encephalitis and demonstrated a significant decrease in the survival rate in mice infected at the start of the rest cycle, zeitgeber time 0 (ZT0). The lower survival rate in these mice was associated with higher levels of circulating chemokine (C-C motif) ligand 2 (CCL2), a greater number of peripherally derived immune cells accumulating in the olfactory bulb (OB), and increased production of proinflammatory cytokines, indicating an immune-mediated pathology. We also found that the acrophase of molecular circadian clock component REV-ERBα mRNA expression in the OB coincides with the start of the active cycle, ZT12, when VSV infection results in a more favorable outcome. This result led us to hypothesize that REV-ERBα may mediate the circadian effect on survival following VSV infection. Blocking REV-ERBα activity before VSV administration resulted in a significant increase in the expression of CCL2 and decreased survival in mice infected at the start of the active cycle. These data demonstrate that REV-ERBα-mediated inhibition of CCL2 expression during viral-induced encephalitis may have a protective effect.

  7. Antiglutamate Receptor Antibodies and Cognitive Impairment in Primary Antiphospholipid Syndrome and Systemic Lupus Erythematosus

    Science.gov (United States)

    Gerosa, Maria; Poletti, Barbara; Pregnolato, Francesca; Castellino, Gabriella; Lafronza, Annalisa; Silani, Vincenzo; Riboldi, Piersandro; Meroni, Pier Luigi; Merrill, Joan T.

    2016-01-01

    Systemic lupus erythematosus (SLE) and antiphospholipid syndrome have an increased risk to develop cognitive impairment. A possible role for antiphospholipid antibodies (aPL) and antiglutamate receptor (anti-NMDA) antibodies in the pathogenesis of neurological manifestations of these two conditions, have been suggested. In particular, the role of anti-NMDA antibodies in the pathogenesis of neuropsychiatric SLE is supported by several experimental studies in animal models and by the finding of a correlation between anti-NMDA positivity in cerebrospinal fluid and neurological manifestations of SLE. However, data from the literature are controversial, as several studies have reported a correlation of these antibodies with mild cognitive impairment in SLE, but more recent studies have not confirmed this finding. The synergism between anti-NMDA and other concomitant autoantibodies, such as aPL, can be hypothesized to play a role in inducing the tissue damage and eventually the functional abnormalities. In line with this hypothesis, we have found a high incidence of at least one impaired cognitive domain in a small cohort of patients with primary APS (PAPS) and SLE. Interestingly, aPL were associated with low scoring for language ability and attention while anti-NMDA titers and mini-mental state examination scoring were inversely correlated. However, when patients were stratified according to the presence/absence of aPL, the correlation was confirmed in aPL positive patients only. Should those findings be confirmed, the etiology of the prevalent defects found in PAPS patients as well as the synergism between aPL and anti-NMDA antibodies would need to be explored. PMID:26870034

  8. Japanese Encephalitis: Frequently Asked Questions

    Science.gov (United States)

    ... the vaccine, what should I do? What is Japanese encephalitis? Japanese encephalitis (JE) is a potentially severe ... cause inflammation of the brain (encephalitis). Where does Japanese encephalitis occur? JE occurs in Asia and parts ...

  9. Meningitis and Encephalitis

    Science.gov (United States)

    ... for viral encephalitis or other severe viral infections.Anticonvulsants are used to prevent or treat seizures. Corticosteroidd ... for viral encephalitis or other severe viral infections.Anticonvulsants are used to prevent or treat seizures. Corticosteroidd ...

  10. Eastern Equine Encephalitis

    Science.gov (United States)

    ... bite of an infected mosquito. Eastern equine encephalitis (EEE) is a rare illness in humans, and only ... EEEV have no apparent illness. Severe cases of EEE (involving encephalitis, an inflammation of the brain) begin ...

  11. [Autoimmune Associated Encephalitis and Dementia].

    Science.gov (United States)

    Watanabe, Osamu

    2016-04-01

    Antibodies against various neural surface antigens induce cognitive impairments. Anti-VGKC (voltage gated potassium channel) complex antibodies are well known as one of the causative autoantibodies. An anti-VGKC antibody was identified as the autoantibody in acquired neuromyotonia (Isaacs' syndrome), which causes muscle cramps and difficulty in opening the palm of the hands. However, this antibody also tests positive in autoimmune limbic encephalitis, which has a subacute progress and causes poor memory or epilepsy attacks. Typical cases have a distinctive adult-onset, frequent, brief dystonic seizure semiology that predominantly affects the arms and ipsilateral face. It has now been termed faciobrachial dystonic seizures. In recent years, the true target antigens of the anti-VGKC antibody of this VGKC limbic encephalitis have been recognized as leucine rich glioma inactivated protein (LGI)-1 and others. These antibodies to amnesia-related LGI-1 in limbic encephalitis neutralize the LGI-1-ADAM22 (an anchor protein) interaction and reduce synaptic AMPA receptors. There have been reports of limbic encephalitis associated with anti-VGKC complex antibodies mimicking Creutzfeldt-Jakob disease (CJD). Less than 2% of the patients with sporadic CJD (sCJD) develop serum anti-VGKC complex antibodies and, when positive, only at low titres. Low titres of these antibodies occur only rarely in suspected patients with sCJD, and when present, should be interpreted with caution.

  12. 抗NMDAR抗体脑炎研究进展%Research on the advances of anti-N-methyl-D-aspartate receptor (NMDAR)encephalitis

    Institute of Scientific and Technical Information of China (English)

    周晶; 秦新月

    2011-01-01

    抗N-甲基-M-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDA)脑炎是近年来新发现的一类副肿瘤性边缘叶脑炎(limbic encephalitis,IE),病因及机制不详.该病发病率低,常发生于伴有卵巢畸胎瘤的年轻女性,临床表现多样,以初期的上感症状发展到精神异常、意识障碍、异常运动和自主神经功能紊乱、癫痫发作、中枢性通气功能障碍为常见表现,脑脊液常为炎性改变.MRI在大脑皮质或小脑可表现短暂异常或颞叶内侧高信号影改变.脑电图多为非特异性改变.血及脑脊液抗NMDA受体抗体阳性可确诊.包括肿瘤切除和免疫治疗、重症监护和物理康复在内的联合治疗为最佳治疗方案.本文就抗NMDA受体脑炎的研究进展做一综述.

  13. LIMBIC ENCEPHALITIS OF HERPESVIRUS ETIOLOGY

    Directory of Open Access Journals (Sweden)

    E. V. Simonova

    2014-01-01

    Full Text Available Based on the literature data and our personal observations in the article discusses the types of variants of the course of nervous system caused by herpesviruses. In the description of a clinical case demonstrated a classic example of limbic encephalitis infectious etiology. The study involved 36 children with various neurological and infectious diseases: 19 children with the diagnosis - convulsions, 8 children with the diagnosis — epilepsy, 5 children with acute viral encephalitis, 3 children with neuropathies. It was established that in the genesis of diseases such as epilepsy, convulsive syndrome, limbic encephalitis, neuropathy peripheral nerves leading role belongs to of herpes virus infection, in which the dominant role belongs HHV-6 infection. Pathogenetically proved the impact of the virus on the receptor apparatus of glia with the disorder of the functional state of mitochondria of these cells. According to our own observations provided data that result in persistence of HHV-6 variant-in mono or in combination with other herpesviruses growing threat of epilepsy and other neurological disorders. 

  14. Anti-NMDAR encephalitis misdiagnosed as Hashimoto's encephalopathy.

    Science.gov (United States)

    Mirabelli-Badenier, M; Biancheri, R; Morana, G; Fornarino, S; Siri, L; Celle, M E; Veneselli, E; Vincent, A; Gaggero, R; Mancardi, M M

    2014-01-01

    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a well-defined autoimmune disorder. Hashimoto's encephalopathy (HE) is a still controversial entity, lacking definite diagnostic criteria. We described a 14-year-old-girl presenting with a clinical picture consistent with the diagnosis of anti-NMDAR encephalitis, confirmed by NMDAR antibody testing. Four years earlier, she had presented a similar episode of acute encephalopathy diagnosed as HE. Anti-NMDAR encephalitis and HE share similar clinical features so that the differential diagnosis can be difficult if specific antibodies are not tested. The correct diagnosis of anti-NMDAR encephalitis is crucial to plan the appropriate management and follow-up, namely in term of oncological screening, since it can be paraneoplastic in origin. We suggest to re-evaluate the clinical history of all subjects with previous HE diagnosis in order to evaluate the possible diagnosis of anti-NMDAR encephalitis and plan the appropriate management of these patients.

  15. Anti-GABAB receptor encephalitis: clinical analysis of 5 cases%抗γ-氨基丁酸B型受体脑炎患者五例临床分析

    Institute of Scientific and Technical Information of China (English)

    吴颖颖; 杨辉丽; 雷高凡; 孙新艳; 邵文君; 陈帅; 刘慧勤; 时英英; 徐长水

    2016-01-01

    目的 探讨抗r氨基丁酸B型(GABA;)受体脑炎患者的临床表现、辅助检查特点以及治疗和预后. 方法 对5例在郑州大学人民医院神经内科就诊的抗GABAB受体脑炎患者的临床资料进行回顾性分析. 结果 所有患者首发症状为癫痫发作,4例病程中出现精神行为异常,3例病程中出现认知功能障碍,1例有自主神经功能障碍,1例出现中枢性低通气不足和睡眠异常.3例患者头部MRI示边缘系统受累.3例患者脑脊液示淋巴细胞增多.所有患者血清和脑脊液抗GABABR抗体均阳性.4例患者胸部CT提示肺部占位,其中2例活检证实为小细胞肺癌.所有患者在明确抗GABAB受体脑炎诊断后均接受免疫治疗,对免疫治疗反应较好.1例患者未发现肿瘤,随访完全恢复;其余4例患者均遗留不同程度的后遗症. 结论 抗GABA;受体脑炎对免疫治疗有良好反应,早识别、早诊断、早治疗对改善患者的预后很重要.%Objective To investigate the clinical manifestation,auxiliary examination characteristics,therapeutic intervention and prognosis of anti-GABAB receptor encephalitis.Methods Clinical data of 5 patients diagnosed as anti-GABAB receptor encephalitis in Neurology Department of Zhengzhou University People's Hospital were retrospectively analyzed.Results The initial symptom was seizures in all of the 5 patients with anti-GABAB receptor encephalitis.During the course of disease,mental and behavior disorders were found in 4 patients,cognitive disorder in 3 patients,autonomic dysfunction in 1 patient,central hypoventilation and parasomnia in 1 patient.Brain MRI showed limbic system lesions in 3 patients.Cerebrospinal fluid (CSF) test indicated lymphocytosis in three patients.The anti-GABABR antibody was positive in both serum and CSF in all patients.Chest CT showed lung occupying lesions in 4 patients,of which 2 lesions were diagnosed as small cell lung cancer by pathological examination.All patients

  16. Viral meningitis and encephalitis.

    Science.gov (United States)

    Tuppeny, Misti

    2013-09-01

    Meningitis is an inflammation of the meninges, whereas encephalitis is inflammation of the parenchymal brain tissue. The single distinguishing element between the 2 diagnoses is the altered state of consciousness, focal deficits, and seizures found in encephalitis. Consequently meningoencephalitis is a term used when both findings are present in the patient. Viral meningitis is not necessarily reported as it is often underdiagnosed, whereas encephalitis cases are on the increase in various areas of North America. Improved imaging and viral diagnostics, as well as enhanced neurocritical care management, have improved patient outcomes to date.

  17. Autoimmune encephalitis: Clinical diagnosis versus antibody confirmation

    Directory of Open Access Journals (Sweden)

    Asha Caroline Cyril

    2015-01-01

    Full Text Available Context: Autoimmune encephalitis is a heterogeneous disorder which is being diagnosed with increasing frequency. The diagnosis of these disorders is based on the detection of autoantibodies and characteristic clinical profiles. Aims: We aimed to study the antibody profile in encephalitis patients with suspected autoimmune etiology presenting to a tertiary care center. Settings and Design: The subjects were selected by screening all patients with clinical profile suggesting autoimmune encephalitis admitted in the neuromedical intensive care unit (ICU of a tertiary care center in South India. Materials and Methods: Patients who fulfilled modified Zuliani et al.′s, criteria for autoimmune encephalitis were identified during the period December 2009-June 2013. Blood samples from these subjects were screened for six neuronal antibodies. Statistical analysis used: Chi-square test was applied to compare the antibody positive and negative patients. Results: Out of 1,227 patients screened, 39 subjects (14 males: 25 females were identified with a mean age of 15.95 years and 19 cases were assessed in the acute and 20 in the convalescent phase of the illness. Seizure (87.8 % was the most common presenting symptom; status epilepticus occurred in 23 (60.5% patients during the course of the illness. Fourteen (35.9% patients were N-methyl-D-aspartate receptor (NMDAR antibody-positive and all were negative for the other antibodies tested. Conclusions: One-third of patients presenting with acute noninfective encephalitis would be positive for NMDAR antibodies with the remaining two-thirds with clinically suspected autoimmune encephalitis being antibody-negative. There are few markers in the clinical and investigative profiles to distinguish antibody-positive and -negative patients.

  18. Tick-Borne Encephalitis (TBE)

    Science.gov (United States)

    ... Search The CDC Cancel Submit Search The CDC Tick-borne Encephalitis (TBE) Note: Javascript is disabled or ... CDC.gov . Recommend on Facebook Tweet Share Compartir Tick-borne encephalitis, or TBE, is a human viral ...

  19. Treatment of viral encephalitis.

    Science.gov (United States)

    Domingues, Renan Barros

    2009-03-01

    Several viruses may cause central nervous system diseases with a broad range of clinical manifestations. The time course of the viral encephalitis can be acute, subacute, or chronic. Pathologically there are encephalitis with direct viral entry into the CNS in which brain parenchyma exhibits neuronal damaging and viral antigens and there are postinfectious autoimmune encephalitis associated with systemic viral infections with brain tissue presenting perivascular aggregation of immune cells and myelin damaging. Some virus affect previously healthy individuals while others produce encephalitis among imunocompromised ones. Factors such evolving lifestyles and ecological changes have had a considerable impact on the epidemiology of some viral encephalitis [e.g. West-Nile virus, and Japanese B virus]. Citomegalovirus and JC virus are examples of infections of the brain that have been seen more frequently because they occur in immunocompromised patients. In the other hand many scientific achievements in neuroimaging, molecular diagnosis, antiviral therapy, immunomodulatory treatments, and neurointensive care have allowed more precise and earlier diagnoses and more efficient treatments, resulting in improved outcomes. In this article, we will present the current drug options in the management of the main acute and chronic viral infection of the central nervous system of immunocompetent and immunocompromised adults, focusing on drugs mechanisms of action, efficacy, and side effects. The early diagnosis and correct management of such diseases can reduce mortality and neurological sequelae; however, even with recent treatment advances, potentially devastating outcomes are still possible.

  20. Whose failure? Encephalitis kills!

    Directory of Open Access Journals (Sweden)

    Nagabhushana Rao Potharaju

    2014-01-01

    Full Text Available Encephalitis continues to be one of the most dreaded diagnoses because a high rate of morbidity and mortality are accepted even before starting the treatment. Most encephalitis cases occur in rural areas due to poor environmental sanitation, high-vector density, shortage of protected water supplies and lack of health education. Vaccination, environmental sanitation, vector control, health education and attention to prompt diagnosis and treatment in rural hospitals are the four essential pillars for reducing case fatality rate (CFR of encephalitis. Frequently, virulence of the virus, immunological state of the host, unavailability of antiviral drugs and lack of enough tertiary care hospitals (TCH are not responsible for the high CFR. Basic supportive care is not being practiced meticulously in Primary and Secondary Care Hospitals (PSCH, and their services are not being utilized fully. Main causes of high mortality and morbidity rates are hypoxia and ischemia of brain and other organs precipitated by preventable, controllable or treatable complications due to lack of basic medical and nursing care during transport to the TCH. Undiagnosed Rickettsial infections are suspected to be partly responsible for the high CFR in some areas. Improving rural hospitals and their ambulance services are the most economical way to reduce CFR. "Treatment facilities must be made available at places where cases occur." The best way to reduce CFR of encephalitis in developing and underdeveloped countries is to increase and improve PSCH and sensitize politicians, administrators, medical/nursing professionals and more importantly to impress and convince the public to utilize them.

  1. [Hashimoto encephalitis and depression].

    Science.gov (United States)

    Veltman, E M; Rhebergen, D; van Exel, E; Stek, M L

    2015-01-01

    Hashimoto encephalitis (he) is an auto-immune disease, with 40-50% of patients developing psychopathology. This could require targeted treatment. HE and prednison could both cloud the identification of a concurrent depressive disorder. We saw a 78-year-old woman with he and a severe depression, and treated her succesfully with ect.

  2. Raccoon roundworm encephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Mehta, Pareen; Boyd, Zachary [University of Missouri, Kansas City School of Medicine, Kansas City, MO (United States); Cully, Brent [University of Missouri, Kansas City School of Medicine, Kansas City, MO (United States); Children' s Mercy Hospital and Clinics, Department of Radiology, Kansas City, MO (United States)

    2010-11-15

    Raccoon roundworm encephalitis is a rare but devastating infection characterized by progressive neurological decline despite attempted therapy. Patients present with deteriorating neurological function, eosinophilia, and history of pica or geophagia resulting in ingestion of the parasite. Neuroimaging studies demonstrate nonspecific findings of progressive white matter inflammation and cortical atrophy. (orig.)

  3. Encephalitis in primary HIV infection

    DEFF Research Database (Denmark)

    Helleberg, M; Kirk, O

    2013-01-01

    We report a case of primary HIV encephalitis, which initially presented as acute psychosis. Magnetic resonance imaging of the brain was suggestive of vasculitis and multiple infarctions, whereas a brain biopsy after six weeks of symptoms showed HIV encephalitis with microglial nodules, but no signs...... of vasculitis. We review previous reported cases and radiological findings in HIV encephalitis and discuss the role of antiretroviral therapy and steroids in its management....

  4. Moyamoya Disease Mimicking Encephalitis

    Directory of Open Access Journals (Sweden)

    Maryam Khalesi

    2014-09-01

    Full Text Available Moyamoya disease is a rare vaso-occlusive illness with an unknown etiology characterized by stenosis of the internal carotid arteries with spontaneous development of a collateral vascular network. A 15-month-old girl was referred to the emergency ward of Imam Reza Hospital due to decreased level of consciousness, focal seizures and fever during the previous 24 hours with an impression of encephalitis. Physical examination revealed left side hemiparesis; however brain CT-Scan did not show any significant lesions. Initial therapy with vancomycin, ceftriaxone and acyclovir was administered. CSF analysis did not show any abnormality and the blood as well as CSF cultures results were negative. Brain MRI showed hyperintensity at right frontal and parietal regions, suggesting vascular lesion. Magnetic resonance angiography (MRA showed bilaterally multiple torsions in vessels at the basal ganglia consistent with moyamoya vessels. In all children exhibiting encephalitis, vascular events such as moyamoya disease should be considered. Brain MRI is a critical tool for this purpose. Common causes of encephalitis such as herpes simplex should also be ruled out.

  5. Interleukin-12 Receptor β1 or Interleukin-12 Receptor β2 deficiency in mice indicates that Interleukin-12 and Interleukin-23 are not essential for host recovery from viral encephalitis.1

    OpenAIRE

    Ireland, Derek D. C.; Palian, Beth M.; Reiss, Carol Shoshkes

    2005-01-01

    Vesicular Stomatitis Virus (VSV), a negative sense, single-stranded RNA Rhabdovirus causes acute viral encephalitis when administered intranasally to mice. Interleukin-12 (IL-12) is a key pro-inflammatory cytokine, produced largely by the antigen presenting cells (APC) that bridges the innate and acquired immune responses. IL-12 is efficacious in enhancing recovery from VSV infection of the murine CNS. This effect is mediated by nitric oxide (NO) produced by the neuronal isoform of nitric oxi...

  6. MRI Findings In Dengue Encephalitis

    Directory of Open Access Journals (Sweden)

    Ashraf V.V

    2004-01-01

    Full Text Available Neurological manifestations are rare in dengue fever. Two cases with encephalopathy and systemic features of dengue fever with abnormal CSF and MR imaging are reported. Striking MRI finding was bilateral symmetrical thalamic lesions similar to those reported in Japanese encephalitis. This report highlights that MRI findings can be similar in dengue and Japanese encephalitis.

  7. Multiphasic presentation of Rasmussen's encephalitis

    NARCIS (Netherlands)

    A. Avbersek; A. Miserocchi; A.W. McEvoy; A.V. Patel; E. Aronica; I. Blumcke; T.S. Jacques; J. Acheson; M. Thom; S.M. Sisodiya

    2015-01-01

    Rasmussen's encephalitis is a rare, chronic inflammatory disorder of unknown cause, characterised by drug-resistant focal epilepsy that may rarely present in adolescence or adulthood. We present a case of Rasmussen's encephalitis with prominent recurrent fluctuation in symptoms and well-documented f

  8. Dengue encephalitis -a case report

    Institute of Scientific and Technical Information of China (English)

    P.C.Bhattacharyya; Jagdish Prasad Agarwal

    2009-01-01

    Encephalitis is an uncommon manifestation of dengue fever.Here we present a 4 years old female child from Northeast Region of India who suffered from dengue encephalitis.To our knowledge,this is probably the first diagnosed case of dengue fever from this region.

  9. [Herpetic encephalitis: a clinical case].

    Science.gov (United States)

    Dryhant, L P; Sereda, V H; Kushpiĭ, O V; Tkachenko, V V; Kravchuk, N A; Inhula, N I; Sizina, A V; Sachko, Iu Iu; Andrusenko, A S; Tytenko, Iu I; Babirad, A M

    2012-01-01

    An example of diagnostics and treatment of patient is in-process made with herpetic encephalitis. It is well-proven in researches, that a herpetic encephalitis is 11.5% among sharp encephalitises. Morbidity is sporadic, some researchers specify on an increase its spring. An infection can be passed tiny and pin a way. Seasonal vibrations are not incident to the herpetic encephalitis. Two peaks of morbidity are on 5-30 years and age more senior 50 years. More than in 95% cases the virus of simple herpes of type serves as an exciter of herpetic encephalitis 1. A characteristic triad of herpetic encephalitis is the sharp feverish beginning, development of cramps of dzheksonovskogo type and violation of consciousness, developing usually after a brief respirator infection. Sometimes sudden development of cramps and loss of consciousness is preceded a fever. Example of such development of disease is made an in our work.

  10. KEGG DISEASE / Acute encephalitis [KEGG DISEASE

    Lifescience Database Archive (English)

    Full Text Available DISEASE: H01417 Entry H01417Disease Name Acute encephalitis Description Acute encep...ns Infections caused by dsDNA viruses H01417Acute encephalitis Human diseases in ICD-10 classification [BR:b...of the central nervous system G04Encephalitis, myelitis and encephalomyelitis H01417Acute encephalitis Patho...elines for management. Journal Eur J Neurol 12:331-43 (2005) KEGG DISEASE / Acute encephalitis ...

  11. Encefalitis carcinomatosa Carcinomatous encephalitis

    Directory of Open Access Journals (Sweden)

    Flavio Sánchez

    2004-12-01

    Full Text Available Se comunica un caso de encefalitis carcinomatosa, en un paciente con diagnóstico de adenocarcinoma de pulmón y síndrome confusional. Esta entidad es poco conocida en relación a otras afecciones neurológicas relacionadas al cáncer. Se discute su presentación clínica, se destaca la necesidad de sospecharla aun luego de obtener una tomografía computada normal y sus típicas imágenes miliares en resonancia magnética.We report a case of carcinomatous encephalitis in a patient with lung adenocarcinoma and confusional syndrome. This is a rare form of brain metastases. We discuss its clinical picture, the importance of suspecting it after a normal computed tomography scan and the miliary typical images at magnetic resonance.

  12. 抗γ-氨基丁酸B受体脑炎患者的临床特征分析%The characteristics of clinical manifestations in patients with anti-γ-aminobutyric acid B receptor encephalitis

    Institute of Scientific and Technical Information of China (English)

    张隽; 张阵; 樊春秋; 吉训明; 黄小钦

    2016-01-01

    Objective To analyze the clinical features and prognosis in adult Chinese patients with anti-γ-aminobutyric acid B receptor (GABA-BR) encephalitis.Methods We reviewed the clinical manifestations,cerebrospinal fluid (CSF) examinations,brain magnetic resonance imaging and prognosis of 12 patients who were diagnosed as anti-GABA-BR encephalitis in Capital Medical University Xuanwu Hospital from March 2013 to December 2015.Results The major clinical features of anti-GABA-BR encephalitis patients included seizures (12/12),cognitive disorder (10/12),psychiatric symptoms (10/12),sleep disorders (3/12),visual hallucination (2/12),involuntary movements (2/12),decreased consciousness (3/12),cerebellar signs (2/12),fever (2/12).GABA-BR-antibody was positive in CSF and serum of all the patients.Electroencephalogram revealed epileptic discharges in 4/12 patients.Brain MRI showed abnormal signal in up to 9/12 patients,located in the hippocampus,temporal lobes,thalamus and periventricular area.PET or SPECT indicated hypometabolism in 5/6 patients.After the average of fourteen months follow-up,9/12 patients had good prognosis,3 patients did not.Of 3 patients with poor outcome,2 had small cell lung cancer.Conclusions The predominant clinical features of the anti-GABA-BR encephalitis are seizures,cognitive disorder and psychiatric symptoms.The lesions are not only located in limbic system.Early diagnosis and immune modulation may provide a good outcome.%目的 分析我国成年人抗γ-氨基丁酸B(GABA-B)受体脑炎患者的临床特征和预后特点.方法 收集2013年3月至2015年12月首都医科大学宣武医院收治的12例抗GABA-B受体脑炎患者,总结其临床症状、实验室检查、脑电图检查、影像学资料,并进行分析.结果 12例抗GABA-B受体脑炎患者主要临床表现为癫痫发作12例,认知障碍10例,精神行为异常10例,睡眠障碍3例,视幻觉2例,不自主运动2例,意识障碍3例,小脑体征2例,发热2例.12例患者脑

  13. Clinical and electroencephalographic analysis of anti-N-methyl-D-aspartate receptor encephalitis in children%儿童抗N-甲基-D-天冬氨酸受体脑炎临床与脑电图分析

    Institute of Scientific and Technical Information of China (English)

    李华; 王晓慧; 方方; 丁昌红; 吕俊兰; 陈春红; 韩彤立; 伍妘; 张炜华

    2016-01-01

    目的 总结儿童抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床与脑电图特征.方法 回顾性分析2011年8月至2015年3月在首都医科大学附属北京儿童医院神经内科确诊并住院治疗的105例抗NMDA受体脑炎患儿的临床与脑电图资料.105例患儿中男38例、女67例,发病年龄为6月龄至15岁8个月,平均(8±4)岁;确诊时间4~850 d,中位数24.5 d.根据改良的Rankin量表对患儿进行病情评估,同时进行持续脑电图监测,对监测结果进行分析总结.结果 105例患儿中病情轻、中、重度者分别为12、65和28例.91例(86.7%)患儿脑电图异常,其中背景活动减慢28例(26.7%),广泛性或弥漫性慢波25例(23.8%),限局性慢波33例(31.4%),癫痫样波形41例(39.O%).10例患儿(9.5%)在睡眠非快速眼运动(NREM)期呈现单侧或弥漫性α-θ频段节律,7例(6.7%)存在极度δ刷状波(EDB).病情轻、中和重3组间脑电图异常分别为5例、58例(89.2%)和28例(100.0%),7例EDB现象患儿均为重度组,10例异常α-θ节律患儿均为中度组.结论 儿童抗NMDA受体脑炎脑电图表现符合一般脑炎的脑电图改变,且脑电图异常程度与病情严重程度成正比.脑电图出现EDB现象和异常α-θ节律对本病诊断及病情的严重性均有提示作用.%Objective To study the clinical and electroencephalographic (EEG) characteristics of anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARencephalitis) in children.Method Retrospective analysis was performed on the clinical and EEG data of 105 patients with anti-NMDAR encephalitis treated in Beijing Children's Hospital (August 2011-March 2015).Of the 105 patients,38 were male and 67 were female.The age of onset was from 6 months and 26 days to 15 years and 8 months (average (8±4)years).The time for confirmed diagnosis was from 4 days to 850 days (median 24.5 days).According to the modified Rankin scales,the patient's clinical conditions were assessed and

  14. Prospects for cannabinoid therapies in viral encephalitis.

    Science.gov (United States)

    Solbrig, Marylou V; Fan, Yijun; Hazelton, Paul

    2013-11-06

    Cannabinoids are promising therapies to support neurogenesis and decelerate disease progression in neuroinflammatory and degenerative disorders. Whether neuroprotective effects of cannabinoids are sustainable during persistent viral infection of the CNS is not known. Using a rodent model of chronic viral encephalitis based on Borna Disease (BD) virus, in which 1 week treatment with the general cannabinoid WIN 55,212-2 has been shown to be neuroprotective (Solbrig et al., 2010), we examine longer term (2 week treatment) effects of a general (CB1 and CB2) cannabinoid receptor agonist WIN55,212-2 (1mg/kg ip twice per day) or a specific (CB2) cannabinoid receptor agonist HU-308 (5mg/kg ip once daily) on histopathology, measures of frontostriatal neurogenesis and gliogenesis, and viral load. We find that WIN and HU-308 differ in their ability to protect new BrdU(+) cells. The selective CB2 agonist HU increases BrdU(+) cells in prefrontal cortex (PFC), significantly increases BrdU(+) cells in striatum, differentially regulates polydendrocytes vs. microglia/macrophages, and reduces immune activation at a time WIN-treated rats appear tolerant to the anti-inflammatory effect of their cannabinoid treatment. WIN and HU had little direct viral effect in PFC and striatum, yet reduced viral signal in hippocampus. Thus, HU-308 action on CB2 receptors, receptors known to be renewed during microglia proliferation and action, is a nontolerizing mechanism of controlling CNS inflammation during viral encephalitis by reducing microglia activation, as well as partially limiting viral infection, and uses a nonpsychotropic cannabinoid agonist.

  15. Protection against Japanese encephalitis by inactivated vaccines.

    Science.gov (United States)

    Hoke, C H; Nisalak, A; Sangawhipa, N; Jatanasen, S; Laorakapongse, T; Innis, B L; Kotchasenee, S; Gingrich, J B; Latendresse, J; Fukai, K

    1988-09-01

    Encephalitis caused by Japanese encephalitis virus occurs in annual epidemics throughout Asia, making it the principal cause of epidemic viral encephalitis in the world. No currently available vaccine has demonstrated efficacy in preventing this disease in a controlled trial. We performed a placebo-controlled, blinded, randomized trial in a northern Thai province, with two doses of monovalent (Nakayama strain) or bivalent (Nakayama plus Beijing strains) inactivated, purified Japanese encephalitis vaccine made from whole virus derived from mouse brain. We examined the effect of these vaccines on the incidence and severity of Japanese encephalitis and dengue hemorrhagic fever, a disease caused by a closely related flavivirus. Between November 1984 and March 1985, 65,224 children received two doses of monovalent Japanese encephalitis vaccine (n = 21,628), bivalent Japanese encephalitis vaccine (n = 22,080), or tetanus toxoid placebo (n = 21,516), with only minor side effects. The cumulative attack rate for encephalitis due to Japanese encephalitis virus was 51 per 100,000 in the placebo group and 5 per 100,000 in each vaccine group. The efficacy in both vaccine groups combined was 91 percent (95 percent confidence interval, 70 to 97 percent). Attack rates for dengue hemorrhagic fever declined, but not significantly. The severity of cases of dengue was also reduced. We conclude that two doses of inactivated Japanese encephalitis vaccine, either monovalent or bivalent, protect against encephalitis due to Japanese encephalitis virus and may have a limited beneficial effect on the severity of dengue hemorrhagic fever.

  16. 儿童抗N-甲基-D-天冬氨酸受体脑炎的脑电图特征%Electroencephalographic features of anti-N-methyl-D-aspartate receptor encephalitis in children

    Institute of Scientific and Technical Information of China (English)

    高鑫; 杨志仙; 薛姣; 季涛云; 张尧; 刘晓燕; 吴晔; 张月华; 包新华

    2016-01-01

    Objective To investigate electroencephalographic (EEG) characteristics of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in children.Method Clinical data of 28 children diagnosed as anti-NMDAR encephalitis were retrospectively analyzed for EEG characteristics in different periods and severity of disease and outcome.Result Among the 28 patients with anti-NMDAR encephalitis,15 were males and 13 were females.Their age at disease onset ranged from 1 year 3 months to 12 years 4 months.Patients were divided into mild group (5 cases) and severe group (23 cases).In the different stage of the disease,occipital background activity of the EEG was preserved in more than half of patients.Accompanied by the evolution of disease course,the occipital background activity and slow waves gradually recovered to normal.In the peak phase of disease,occipital background activity in the awake state was preserved in 4/5 patients of the mild group and 9/17 patients of the severe group.Alpha and theta band rhythms in non-rapid eye movement (NREM) sleep existed in 77% (17/22) patients.EEG monitoring showed delta brushes in 2 cases,and the delta brushes were mixed with background fast waves in one case;71% (20/28) patients had epileptiform discharges in EEG during the course,and among them,6 patients had secondary epilepsy.Conclusion The background activity in the awake state and abnormal diffuse slow waves of EEG were evolved and gradually recovered during the course of the disease.Regardless milder or severe illness condition,occipital background activity was still preserved during different stages in most patients.Alpha and theta rhythms in NREM sleep might represent a relatively ovcrt EEG characteristic.The presence of delta brush in EEG was rare,and sometimes they were difficult to be distinguished from fast wave activities caused by drugs.The presence of epileptiform discharges in EEG suggested the possibility of secondary epilepsy.%目的 探讨儿童抗N-甲基-D-天

  17. Vaccines for preventing Japanese encephalitis

    DEFF Research Database (Denmark)

    Schiøler, Karin Linda; Samuel, Miny; Wai, Kim Lay

    2007-01-01

    BACKGROUND: Vaccination is recognized as the only practical measure for preventing Japanese encephalitis. Production shortage, costs, and issues of licensure impair vaccination programmes in many affected countries. Concerns over vaccine effectiveness and safety also have a negative impact...... on acceptance and uptake. OBJECTIVES: To evaluate vaccines for preventing Japanese encephalitis in terms of effectiveness, adverse events, and immunogenicity. SEARCH STRATEGY: In March 2007, we searched the Cochrane Infectious Diseases Group Specialized Register, CENTRAL (The Cochrane Library 2007, Issue 1......), MEDLINE, EMBASE, LILACS, BIOSIS, and reference lists. We also attempted to contact corresponding authors and vaccine companies. SELECTION CRITERIA: Randomized controlled trials (RCTs), including cluster-RCTs, comparing Japanese encephalitis vaccines with placebo (inert agent or unrelated vaccine...

  18. Present status of rasmussen encephalitis

    Institute of Scientific and Technical Information of China (English)

    Yuguang Guan; Tianfu Li; Guoming Luan

    2014-01-01

    Rasmussen's encephalitis is a rare progressive encephalitis that results in intractable seizures,cognitive decline, and hemiparesis.The affected hemispheres of RE patients are typically unilateral.There is no conclusive evidence elucidating RE etiology.To date,no antiepileptic drug has been demonstrated to be effective for controlling the disease or stopping its pro-gression.Immunoglobulins and high doses of steroids may be transiently helpful in some patients at an early stage.Only hemi-spherectomy are useful methods to control seizures in RE patients.

  19. 三例不同类型的抗神经元表面抗原抗体阳性的边缘性脑炎患者的临床分析及比较%Clinical analysis and comparison of three patients with limbic encephalitis of different kinds of antibodies against neuronal cell surface

    Institute of Scientific and Technical Information of China (English)

    刘丽萍; 周玉颖; 张淼; 张惠红; 陆达

    2016-01-01

    impairment,psychiatric symptoms,seizures,abnormal movements and increased T2-weighted imaging or fluid-attenuated inversion-recovery signal on MR imaging;however,mediated by different antibodies,each patient possessed specific features.The young girl with anti-NMDA receptor encephalitis presented oro-facial dyskinesias,hypersalivation and hypoventilation,and had to get tracheotomy and mechanical ventilation,and ovarian teratoma was found through CT.The patient with anti-LGI1 antibodies manifested like faciobrachial dystonic seizure.The patient with anti-GABAB receptor antibody got the disease after nephrectomy due to renal clear cell carcinoma.With or without tumor,all the three patients responded well to immunotherapy.Conclusion Different kinds of limbic encephalitis with different antibody against neuronal cell surface have different clinical features,whereas,all of them are sensitive to immunotherapy.

  20. Electroconvulsive therapy and/or plasmapheresis in autoimmune encephalitis?

    Science.gov (United States)

    Gough, Jessica L; Coebergh, Jan; Chandra, Brunda; Nilforooshan, Ramin

    2016-01-01

    Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy (ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies. PMID

  1. Clinical analysis of patients with anti-N-methyl-D-aspartate receptor encephalitis: a report of 3 cases%抗N-甲基D-天冬氨酸受体脑炎临床分析(附三例报道)

    Institute of Scientific and Technical Information of China (English)

    黄曜纬; 颜振兴; 贺荣霓; 谢惠芳

    2015-01-01

    Objective To explore the clinical features,laboratory test results,diagnosis,treatment and prognosis of patients with anti-N-methyl-D-aspartic acid receptor (NMDAR) encephalitis.Methods A retrospectives analysis of clinical characteristics,laboratory test,treatment and prognosis of 3 patients with NMDRA encephalitis,admitted to our hospital from February 2012 to November 2014,was performed in our study.Results The most predominant symptoms of 3 patients with anti-NMDAR encephalitis included fever,psychiatric behavioral disturbances,seizures,orofacial dyskinesias and movement disorders and autonomic dysfunction.One patient had immature ovarian teratoma and one had mature mediastinal teratoma.Anti-NMDAR antibody was positive in serum and cerebrospinal fluid of two patients with teratoma,and anti-NMDAR antibody was positive in cerebrospinal fluid and negative in serum of one patient without tumour;all three patients showed abnormal electroencephalography,and 2 had increased signal on MR imaging T2 sequences or fluid-attenuated inversion.Patients were responsive to early immunotherapy and tumour resection.Conclusion The patient with NMDAR encephalitis presented with characteristic clinical manifestations;detection of anti-NMDAR antibody in serum and cerebrospinal fluid may be important for early diagnosis;early immunotherapy and tumour resection are effective for patients with NMDAR encephalitis.%目的 分析抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点、实验室检查结果及诊断治疗方法与预后. 方法 收集自南方医科大学珠江医院神经内科自2012年2月至2014年11月收治的3例NMDAR脑炎患者的临床资料,回顾性分析其临床症状特点、实验室检查、治疗及预后. 结果 3例患者临床以发热、精神行为异常、抽搐、口面部不自主运动、自主神经功能紊乱为主要特征,其中1例伴有卵巢未成熟型畸胎瘤,l例伴有纵膈成熟型畸胎瘤,2例血及脑脊

  2. The characteristics of clinical manifestations, brain MRI and cerebrospinal fluid findings in patients with anti-N-methyl-D-aspartate receptor encephalitis%抗N-甲基-D-天冬氨酸受体脑炎患者的临床、磁共振成像和脑脊液特征分析

    Institute of Scientific and Technical Information of China (English)

    贺菲菲; 叶静; 董恺; 赵筱玲

    2014-01-01

    目的 分析我国成年人抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患者的临床、头MRI和脑脊液特点.方法 收集29例抗NMDA受体脑炎患者的临床、MRI和脑脊液资料.结果 29例抗NMDA受体脑炎中,主要临床表现为精神异常占86%(25例),癫痫83%(24例),意识障碍55%(16例),不自主运动55%(16例),中枢性通气障碍34%(10例),大量唾液分泌17%(5例),尚有自主神经紊乱、偏瘫、失语等.14%(4例)伴有畸胎瘤.头MRI异常者占62%(18例),病灶分布于颞叶、海马、丘脑、脑干、扣带回、额顶叶、胼胝体、内囊/基底节/脑室旁.83%腰椎穿刺结果异常,其中脑脊液寡克隆区带阳性率为95% (19/20).3年内复发率为31% (9/29).结论 抗NMDA受体脑炎患者主要临床表现为精神异常、癫痫,特征性临床表现有不自主运动、中枢性通气障碍、大量唾液分泌;MRI病灶分布不仅局限于边缘叶;是复发率较高但可治疗的疾病.%Objective To analyze the clinical manifestations and the features of brain MRI and cerebrospinal fluid (CSF) findings in adult Chinese patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods We reviewed the clinical manifestations,brain MRI and CSF examinations of 29 patients who were diagnosed as anti-NMDAR encephalitis.Results The major clinical features of anti-NMDAR encephalitis patients included psychiatric symptoms (86%,25/29),seizures (83%,24/29),decreased consciousness (55%,16/29),involuntary movements (55%,16/29),central hypoventilation (34%,10/29),and hypersalivation (17%,5/29).Some patients also experienced autonomic instability,hemiplegia and aphasia.Underlying ovarian teratoma was identified in 14% of affected patients(4/29).Brain MRI was found abnormal in up to 62% patients (18/29),located in the temporal lobes,hippocampus,thalamus,brain stem,cingulate gyrus,frontal and parietal cortex,corpus callosum,internal capsule,basal ganglia and

  3. Clinical study on antibody-associated limbic encephalitis

    Directory of Open Access Journals (Sweden)

    WANG Jia-wei

    2013-01-01

    Full Text Available In recent years, the antibody-associated limbic encephalitis (LE has attracted attentions of more and more clinicians. The associated antibodies mainly act on neuronal cell surface antigens, including the N-methyl-D-aspartate (NMDA receptor, the α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA receptor, the γ-aminobutyric acid B (GABAB receptor, leucine-rich glioma-inactivated 1 (LGI1 and contactin-associated protein-like 2 (Caspr2 and so on. The clinical manifestation is primarily defined by the subacute onset of short-term memory loss, seizures, confusion and psychiatric symptoms suggesting the involvement of the limbic system. These severe and protracted disorders can affect children and young adults, occurring with or without tumor association. Routine detection of serum and cerebrospinal fluid (CSF and imaging tests show no specificity, but associated antibodies can be detected in serum and (or CSF. The patients respond well to tumor resection and immunotherapies, including corticosteroids, intravenous immunoglobulin (IVIg, plasma exchange or combination of them, but may relapse. This article aims to study the clinical features and treatment of antibody-associated limbic encephalitis and to improve the diagnosis and prognosis of these diseases.

  4. Herpes simplex virus encephalitis in hamadan, iran.

    Directory of Open Access Journals (Sweden)

    Masoud Sabouri Ghannad

    2013-09-01

    Full Text Available Encephalitis can cause a severe public health problem. The main aim of this research was to evaluate the medical laboratory results of patients with Herpes Simplex Virus (HSV encephalitis.Diagnosis of encephalitis for these patients was firstly based on a clinical profile for Herpes Simplex Encephalitis (HSE, plus either a detected HSV1&2-DNA by PCR in CSF or brain neuro-imaging results.Molecular testing on CSF showed that 15 patients (15% had HSV infection, 5 patients (5% had Varicella Zoster Virus (VZV and one case was positive for Human Immunodeficiency Virus (HIV-RNA in CSF. The cause of encephalitis in 79 out of 100 patients (79% was unknown. The comparison of CSF analysis in HSV positives and negatives showed a significant increase of glucose and protein levels in HSV positives than negatives. The mortality rate was 46.6% (7/15 in patients with HSV encephalitis compared to 11.4% (10/85 in non-HSV encephalitis (P = 0.003.In the current study, 15% of cases were diagnosed as having HSV.

  5. CCL2, but not its receptor, is essential to restrict immune privileged central nervous system-invasion of Japanese encephalitis virus via regulating accumulation of CD11b(+) Ly-6C(hi) monocytes.

    Science.gov (United States)

    Kim, Jin Hyoung; Patil, Ajit Mahadev; Choi, Jin Young; Kim, Seong Bum; Uyangaa, Erdenebileg; Hossain, Ferdaus Mohd Altaf; Park, Sang-Youel; Lee, John Hwa; Kim, Koanhoi; Eo, Seong Kug

    2016-10-01

    Japanese encephalitis virus (JEV) is a re-emerging zoonotic flavivirus that poses an increasing threat to global health and welfare due to rapid changes in climate and demography. Although the CCR2-CCL2 axis plays an important role in trafficking CD11b(+) Ly-6C(hi) monocytes to regulate immunopathological diseases, little is known about their role in monocyte trafficking during viral encephalitis caused by JEV infection. Here, we explored the role of CCR2 and its ligand CCL2 in JE caused by JEV infection using CCR2- and CCL2-ablated murine models. Somewhat surprisingly, the ablation of CCR2 and CCL2 resulted in starkly contrasting susceptibility to JE. CCR2 ablation induced enhanced resistance to JE, whereas CCL2 ablation highly increased susceptibility to JE. This contrasting regulation of JE progression by CCR2 and CCL2 was coupled to central nervous system (CNS) infiltration of Ly-6C(hi) monocytes and Ly-6G(hi) granulocytes. There was also enhanced expression of CC and CXC chemokines in the CNS of CCL2-ablated mice, which appeared to induce CNS infiltration of these cell populations. However, our data revealed that contrasting regulation of JE in CCR2- and CCL2-ablated mice was unlikely to be mediated by innate natural killer and adaptive T-cell responses. Furthermore, CCL2 produced by haematopoietic stem cell-derived leucocytes played a dominant role in CNS accumulation of Ly-6C(hi) monocytes in infected bone marrow chimeric models, thereby exacerbating JE progression. Collectively, our data indicate that CCL2 plays an essential role in conferring protection against JE caused by JEV infection. In addition, blockage of CCR2, but not CCL2, will aid in the development of strategies for prophylactics and therapeutics of JE.

  6. Prostate cancer may trigger paraneoplastic limbic encephalitis

    DEFF Research Database (Denmark)

    Jakobsen, Jakob Kristian; Zakharia, Elias Raja; Boysen, Anders Kindberg Fossø

    2013-01-01

    We present a case of a previously healthy and active 64-year-old man who experienced a rapid neuropsychiatric decline. All tests for metabolic causes, neuroinfection, intracranial infarction or tumor were negative. By the means of magnetic resonance imaging, electroencephalography and the anti......-Hu antibody test the patient was diagnosed with paraneoplastic limbic encephalitis related to prostate cancer. The patient died within 6 months. We review the literature on prostate cancer-related paraneoplastic limbic encephalitis. High-risk prostate cancer can trigger paraneoplastic limbic encephalitis...

  7. Cerebrospinal Fluid Biomarkers of Simian Immunodeficiency Virus Encephalitis : CSF Biomarkers of SIV Encephalitis.

    Science.gov (United States)

    Bissel, Stephanie J; Kofler, Julia; Nyaundi, Julia; Murphey-Corb, Michael; Wisniewski, Stephen R; Wiley, Clayton A

    2016-06-01

    Antiretroviral therapy has led to increased survival of HIV-infected patients but also increased prevalence of HIV-associated neurocognitive disorders. We previously identified YKL40 as a potential cerebrospinal fluid (CSF) biomarker of lentiviral central nervous system (CNS) disease in HIV-infected patients and in the macaque model of HIV encephalitis. The aim of this study was to define the specificity and sensitivity along with the predictive value of YKL40 as a biomarker of encephalitis and to assess its relationship to CSF viral load. CSF YKL40 and SIV RNA concentrations were analyzed over the course of infection in 19 SIV-infected pigtailed macaques and statistical analyses were performed to evaluate the relationship to encephalitis. Using these relationships, CSF alterations of 31 neuroimmune markers were studied pre-infection, during acute and asymptomatic infection, at the onset of encephalitis, and at necropsy. YKL40 CSF concentrations above 1122 ng/ml were found to be a specific and sensitive biomarker for the presence of encephalitis and were highly correlated with CSF viral load. Macaques that developed encephalitis had evidence of chronic CNS immune activation during early, asymptomatic, and end stages of infection. At the onset of encephalitis, CSF demonstrated a rise of neuroimmune markers associated with macrophage recruitment, activation and interferon response. CSF YKL40 concentration and viral load are valuable biomarkers to define the onset of encephalitis. Chronic CNS immune activation precedes the development of encephalitis while some responses suggest protection from CNS lentiviral disease.

  8. Anti-N-methyl-D-aspartate Receptor Encephalitis Mimicking Herpes Simplex Encephalitis Onset%发病酷似单纯疱疹病毒脑炎的抗N-甲基-D-天冬氨酸受体脑炎

    Institute of Scientific and Technical Information of China (English)

    王瑞金; 齐冬; 王淑辉; 张拥波

    2015-01-01

    目的 提高对发病酷似单纯疱疹病毒脑炎(Herpes simplex encephalitis,HSE)的抗N-甲基-D-天冬氨酸(N-methyl-D-aspartate,NMDA)受体脑炎的认识.方法 报道2例发病酷似单纯疱疹病毒脑炎的抗NMDA受体脑炎,并复习相关文献.结果 2例患者均急性起病,表现为发热、抽搐、认知功能减退、精神行为异常等.脑MRI均未见异常.病例1、病例2脑脊液白细胞最高分别为20×106/L、21×106/L.脑脊液蛋白、葡萄糖以及氯化物水平均正常.2例患者早期均被诊断为HSE,并接受阿昔洛韦抗病毒治疗,但病情仍进展.抗NMDA受体抗体检测显示例1脑脊液及血液均阳性,例2脑脊液阳性,从而明确了抗NMDA受体脑炎的诊断.结论 对脑MRI无异常,脑脊液白细胞无明显升高的边缘系统脑炎,应想到抗NMDA受体脑炎可能.

  9. Limbic encephalitis associated with elevated antithyroid antibodies.

    Science.gov (United States)

    Hacohen, Yael; Joseph, Sonia; Kneen, Rachel; Eunson, Paul; Lin, Jean-Pierre; Vincent, Angela; Lim, Ming

    2014-06-01

    Immune-mediated limbic encephalitis affects both adults and children. Patients typically present with seizures, memory problems, and imaging changes in the medial temporal lobes. Both paraneoplastic and nonparaneoplastic forms have been described in which the antibody to the voltage-gated potassium channel-complex associated protein, leucine-rich glioma-inactivated 1, is most commonly reported. Elevated antithyroid antibodies have also been reported in a range of neurological syndromes with encephalopathy, such as limbic encephalitis, often collectively termed Hashimoto encephalopathy, a condition whereby corticosteroids responsiveness with a complete recovery is commonly observed. Here we describe 3 children presenting with limbic encephalitis with elevated thyroid antibodies that did not respond to corticosteroids alone and required more aggressive immunotherapy, mirroring the slower treatment response that is more frequently seen in other immune-mediated forms of limbic encephalitis.

  10. Emergency Neurologic Life Support: Meningitis and Encephalitis.

    Science.gov (United States)

    Gaieski, David F; Nathan, Barnett R; O'Brien, Nicole F

    2015-12-01

    Bacterial meningitis and viral encephalitis, particularly herpes simplex encephalitis, are severe neurological infections that, if not treated promptly and effectively, lead to poor neurological outcome or death. Because treatment is more effective if given early, the topic of meningitis and encephalitis was chosen as an Emergency Neurological Life Support protocol. This protocol provides a practical approach to recognition and urgent treatment of bacterial meningitis and encephalitis. Appropriate imaging, spinal fluid analysis, and early empiric treatment is discussed. Though uncommon in its full form, the typical clinical triad of headache, fever, and neck stiffness should alert the clinical practitioner to the possibility of a central nervous system infection. Early attention to the airway and maintaining normotension is crucial in treatment of these patients, as is rapid treatment with anti-infectives and, in some cases, corticosteroids.

  11. [Saint Louis encephalitis: case report].

    Science.gov (United States)

    Carballo, Carolina; Cabana, Magdalena; Ledezma, Francisca; Pascual, Carolina; Cazes, Claudia; Mistchenko, Alicia; López, Eduardo

    2016-08-01

    Saint Louis encephalitis is transmitted by Culex mosquitoes. In Argentina sporadic cases are registered. Symptomatic illness is unusual in children. We present a case of meningoencephalitis caused by an uncommon viral infection. The clinical signs and symptoms are unusual for pediatric patients and the bilateral thalamic compromise showed on magnetic resonance has not been described previously. An 8-year-old girl consulted due to fever, behavior disorders and ataxia. Clonus and neck stiffness were detected at physical exam. Cerebrospinal fluid revealed mononuclear leukocytosis; bilateral ischemic compromise was observed in thalamus by magnetic resonance. Saint Louis virus was confirmed by serology: serum and cerebrospinal fluid IgM were positive during the acute phase of the disease and serum IgG was positive four weeks later. Most of the signs and symptoms of the disease were resolved, however mild behavior disorders were observed as acute sequelae up to 45 days after hospital discharge.

  12. Cysticercal encephalitis with cortical blindness.

    Science.gov (United States)

    Prasad, Rajniti; Thakur, Neha; Mohanty, C; Singh, M K; Mishra, O P; Singh, Utpal Kant

    2010-10-21

    The authors report a 6-year-old boy, who had presented with low-grade fever, altered sensorium, headache and seizure for 5 days. On examination, he had features of raised intracranial pressure with left VI cranial-nerve palsy and bilateral extensor plantar response. CT scan showed multiple calcifications in cerebral cortex. MRI cranium showed multiple cysts involving whole of the brain. He was diagnosed as having cysticercal encephalitis, based on immunological and imaging study. He was managed with 20% mannitol, phenytoin and albendazole, and regained consciousness 7 days later, but had residual neurological deficit as left-lower-limb monoparesis and visual acuity of just projection of rays (PR+) and perception of light (PL+).

  13. Encephalitis

    Science.gov (United States)

    ... therapy depending on the type and severity of complications. This therapy may include: Physical therapy to improve strength, flexibility, balance, motor coordination and mobility Occupational therapy to develop everyday ...

  14. Herpes encephalitis preceded by ipsilateral vestibular neuronitis.

    Science.gov (United States)

    Philpot, Stephen J; Archer, John S

    2005-11-01

    A 74-year-old woman developed vertigo and jerk nystagmus to the left with normal cerebral imaging. Three days later she developed fever, altered mental state and left medial temporal lobe hypodensity, confirmed on lumbar puncture to be due to herpes simplex type 1 encephalitis. We propose that the patient had vestibular neuronitis caused by HSV-1 that progressed to ipsilateral temporal lobe encephalitis.

  15. Relevance of Neuroinflammation and Encephalitis in Autism

    Directory of Open Access Journals (Sweden)

    Janet eKern

    2016-01-01

    Full Text Available In recent years, many studies indicate that children with an autism spectrum disorder (ASD diagnosis have brain pathology suggestive of ongoing neuroinflammation or encephalitis in different regions of their brains. Evidence of neuroinflammation or encephalitis in ASD includes: microglial and astrocytic activation, a unique and elevated proinflammatory profile of cytokines, and aberrant expression of nuclear factor kappa-light-chain-enhancer of activated B cells. A conservative estimate based on the research suggests that at least 69% of individuals with an ASD diagnosis have microglial activation or neuroinflammation. Encephalitis, which is defined as inflammation of the brain, is medical diagnosis code G04.90 in the International Classification of Disease, 10th revision; however, children with an ASD diagnosis are not generally assessed for a possible medical diagnosis of encephalitis. This is unfortunate because if a child with ASD has neuroinflammation, then treating the underlying brain inflammation could lead to improved outcomes. The purpose of this review of the literature is to examine the evidence of neuroinflammation/encephalitis in those with an ASD diagnosis and to address how a medical diagnosis of encephalitis, when appropriate, could benefit these children by driving more immediate and targeted treatments.

  16. Clinical and electroencephalogram in different stage of resistance to N-methyl-D-aspartate receptor encephalitis%抗 N-甲基-D-天冬氨酸受体脑炎的临床特点及不同阶段的脑电图表现

    Institute of Scientific and Technical Information of China (English)

    王臖; 邓烨; 耿介立; 陆钦池

    2016-01-01

    目的:研究抗 N-甲基-D-天冬氨酸(NMDA)受体脑炎患者的临床特点及其不同阶段的脑电图(EEG)表现,提高对这种自身免疫性疾病的认识。方法对3例经血清及脑脊液抗NMDA受体抗体检测呈阳性的患者,对其病史、认知功能、脑脊液、头颅影像学、脑电图、妇科彩超、治疗方案等资料进行详细分析,诊断为抗 NMDA 受体脑炎,并随访2~3年。结果3例患者均为女性,发病年龄分别为22岁、27岁和30岁,以精神异常和癫痫发作为首发症状,病程中表现出意识障碍、运动障碍、语言障碍和认知障碍。脑脊液、头颅 MRI 检查表现无特异改变,EEG 显示呈刷状样波形,2例妇科彩超显示卵巢畸胎瘤,1例由于病情发展迅速,未行超声检查已死亡。病程为1~3个月。结论抗 NMDA 受体脑炎临床表现易与病毒性脑炎混淆,对于无精神病史的患者出现不明原因的精神症状伴痫性发作、记忆丧失、意识水平降低、运动障碍甚至出现中枢性通气不足,特别是伴有卵巢畸胎瘤者的年轻女性,在 EEG 检查中显示呈刷状样波形,应尽早进行抗 NMDA 受体抗体检测,及时诊断和治疗有助于抗 NMDA 受体脑炎患者的恢复。%Objective To discuss the clinical features of pantients with anti-N-methyl-D-aspar-tate receptor(NMDAR)encephalitis and the performance of electroencephalogram (EEG)in different phase and make a better understanding in this autoimmune disease.Methods 3 patients were diagnosed NMDAR encephalitis which were confirmed by antibody detection in serum or CSF.We recorded their clinical data,including disease history,CSF data,cranial MR images (MRI),EEG,mini-mental state examination,gynecological ultrasound and treatmentand followed up 2-3 years.Results 3 female patients with onset at age of 22,27 and 30 years old showed common manifestation of psychiatric symptoms and seizures as the initial signs

  17. Clinical analysis of 9 cases of anti-NMDAR encephalitis

    Directory of Open Access Journals (Sweden)

    Yun-xia CHEN

    2016-10-01

    Full Text Available Objective  To explore the pathogenesis and clinical features of anti-N-methyl-D-aspartate receptor (NMDAR encephalitis. Methods  The clinical characteristics of 9 patients with anti-NMDAR encephalitis in Department of Neurology, Army General Hospital of Chinese PLA from October 2013 to October 2015, were retrospectively analyzed. And by literatures review, the pathogenesis, clinical feature, diagnosis, treatments and prognosis of this disease were summarized. Results  The average age of the 9 patients (3 men and 6 women was 39 years, 5 of them had precursor nonspecific infection. The initial symptom of neurologic system was mental-behavior disorder in all the 6 females, or epilepsy in all the 3 males. Mental-behavior disorder existed in all the 9 cases during the disease course, including 7 cases of epilepsy, 8 cases of consciousness disturbance, 5 cases of involuntary movement, and 3 cases of inadequate ventilation. The anti-NMDAR antibodies were positively detected from the cerebrospinal fluid and blood of all the 9 cases. After gamma globulin and hormone therapy, 8 cases were cured or better, and only 1 case invalid. A six months follow-up found that 8 cases were independent with eusemia and 1 case got disability. Conclusions  The clinical characteristics of anti-NMDAR encephalitis are mental-behavior disorder, or epilepsy upon the basis of a precursor infection, with positive anti-NMDAR antibodies detected from the cerebrospinal fluid and blood. Gamma globulin and hormone therapy may help th e clinical recovery. DOI: 10.11855/j.issn.0577-7402.2016.09.13

  18. Encephalization of Australian and New Guinean marsupials.

    Science.gov (United States)

    Ashwell, K W S

    2008-01-01

    Encephalization of Australian marsupials was analyzed using the endocranial volume (ECV) of 52 species of Dasyuromorphia and Notoryctemorphia, 14 species of Peramelemorphia and 116 species of Diprotodontia from Australia and New Guinea and compared with 16 species of Ameridelphian marsupials and 3 species of native and recently introduced Australian eutherian carnivores (dingo, feral cat and feral fox). Linear regression analysis of the relationship between ECV and body weight for marsupials revealed that allometric parameters for these groups are different from those previously derived for samples of (mainly eutherian) mammals, with higher slopes for Dasyuromorphia and Diprotodontia and lower slopes for Ameridelphians and Peramelemorphia. Absolute ECV for small Australian and New Guinea marsupial carnivores (Antechinus and Sminthopsis) were found to be comparable to eutherians of similar body weight, but large marsupial carnivores such as the Tasmanian devil and thylacine had substantially smaller ECVs than eutherian carnivores of similar body weight. Similarly, members of some superfamilies within Diprotodontia (Burramyoidea, Petauroidea, Tarsipedoidea) had ECVs comparable to prosimians, whereas bandicoots, bilbies and many macropods were found to be poorly encephalized. When both encephalization quotient (EQ) and residuals from regression analysis were used to compare relative ECV of extinct/threatened species with common species there were no significant differences for any of the orders of Australian marsupials, suggesting that encephalization is not a major factor in the current extinction crisis for Australian marsupials. Similarly there were no consistent differences in relative ECV between marsupials from New Guinea and associated islands compared to Australia or between arid and non-arid Australian regions for any of the marsupial orders. The results indicate that marsupials are not uniformly poorly encephalized and that small marsupial carnivores and

  19. Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

    Directory of Open Access Journals (Sweden)

    Hongliang Zhang

    2013-01-01

    Full Text Available Paraneoplastic neurological syndromes (PNSs occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC, lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promoted the understanding of these syndromes and led to the current hypothesis of an autoimmune pathophysiology. Accumulating data strongly suggested direct pathogenicity of these antibodies. The field of PNS has expanded rapidly in the past few years with the discovery of limbic encephalitis associated with glutamic acid decarboxylase (GAD 65, the voltage (VGKC-gated potassium channel complex, the methyl (N-NMDA-D-aspartate, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA, and gamma aminobutyric acid (GABA (B receptors, and so forth. Despite this, the clinical spectrum of these diseases has not yet been fully investigated. The clinical importance of these conditions lies in their frequent response to immunotherapies and, less commonly, their association with distinctive tumors. This review provides an overview on the pathogenesis and diagnosis of PNS, with emphasis on the role of antibodies in limbic encephalitis.

  20. Multiple autoimmune antibody limbic encephalitis: a case in a pregnant woman

    Directory of Open Access Journals (Sweden)

    Meha Goyal

    2015-03-01

    Full Text Available Autoimmune limbic encephalitis is most commonly associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR, among other neuronal cell surface receptors. Here, a case of a pregnant female with limbic encephalitis in the presence of multiple additional autoimmune antibodies is described. The patient was a 36-year-old female who presented with 4 days of confusion, hallucinations, hypersexuality, disinhibition, and pressured speech. The patient's work-up detected the presence of anti-NMDAR antibodies, anti-glutamic acid decarboxylase antibodies, and a yet uncharacterized neuronal autoantibody. The patient was also found to be pregnant. No evidence of ovarian or other pelvic malignancy was discovered. Symptomatic control was achieved with plasma exchange.

  1. Putaminal involvement in Rasmussen encephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Rajesh, Bhagavatheeswaran; Ashalatha, Radhakrishnan [Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Neurology, Trivandrum, Kerala (India); Kesavadas, Chandrasekharan; Thomas, Bejoy [Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Imaging Sciences and Interventional Radiology, Trivandrum, Kerala (India)

    2006-08-15

    Rasmussen encephalitis (RE) is a rare devastating disease of childhood causing progressive neurological deficits and intractable seizures, typically affecting one hemisphere. Characteristic MRI features include progressive unihemispheric focal cortical atrophy and grey- or white-matter high-signal changes and basal ganglion involvement, particularly of the caudate nucleus. To analyse the pattern of involvement of different brain structures in a series of patients with RE and to attempt clinical correlation. We reviewed the medical records and neuroimaging data of 12 patients diagnosed with RE satisfying the European Consensus Statement diagnostic criteria. The disease manifested as seizures in all patients and was refractory; epilepsia partialis continua was a notable feature (nine patients). Hemiparesis of varying grades was noted in all but one patient; none had extrapyramidal signs. Neuroimaging showed cortical involvement in the insular/periinsular regions in 11 patients. Caudate atrophy was noted in ten patients. Putaminal atrophy was seen in nine patients, six of whom had additional hyperintense signal changes. Our study highlights frequent putaminal atrophy and signal changes in RE, which suggests a more extensive basal ganglion involvement than emphasized previously. Recognition of putaminal changes may be a useful additional tool in the radiological diagnosis of RE. (orig.)

  2. Microbial study of meningitis and encephalitis cases.

    Science.gov (United States)

    Selim, Heba S; El-Barrawy, Mohamed A; Rakha, Magda E; Yingst, Samuel L; Baskharoun, Magda F

    2007-01-01

    Meningitis and/or encephalitis can pose a serious public health problem especially during outbreaks. A rapid and accurate diagnosis is important for effective earlier treatment. This study aimed to identify the possible microbial causes of meningitis and/or encephalitis cases. CSF and serum samples were collected from 322 patients who had signs and symptoms suggestive of meningitis and/or encephalitis. Out of 250 cases with confirmed clinical diagnosis, 83 (33.2%) were definitely diagnosed as bacterial meningitis and/or encephalitis cases (by using CSF culture, biochemical tests, latex agglutination test, and CSF stain), 17 (6.8%) were definitely diagnosed as having viral causes ( by viral isolation on tissue culture, PCR and ELISA), and one (0.4%) was diagnosed as fungal meningitis case (by India ink stain, culture, and biochemical tests). Also, there was one encephalitis case with positive serum ELISA IgM antibodies against Sandfly scilian virus. N. meningitidis, S. pneumonia and M. tuberculosis were the most frequently detected bacterial agents, while Enteroviruses, herpes simplex viruses and varicella zoster viruses were the most common viral agents encountered. Further studies are needed to assess the role of different microbial agents in CNS infections and their effective methods of diagnosis.

  3. Neuropsychiatric autoimmune encephalitis without VGKC-complex, NMDAR, and GAD autoantibodies: case report and literature review.

    Science.gov (United States)

    Najjar, Souhel; Pearlman, Daniel; Devinsky, Orrin; Najjar, Amanda; Nadkarni, Siddhartha; Butler, Tracy; Zagzag, David

    2013-03-01

    We report a patient with a seronegative autoimmune panencephalitis, adding a subtype to the emerging spectrum of seronegative autoimmune encephalitis, and we review the sparse literature on isolated psychiatric presentations of autoimmune encephalitis. (A PubMed search for "seronegative autoimmune encephalitis," "nonvasculitic autoimmune inflammatory meningoencephalitis," and related terms revealed VGKC)-complex, N-methyl-D-aspartate receptor (NMDAR), and glutamic acid decarboxylase (GAD) autoantibodies. We excluded genetic, metabolic, paraneoplastic, degenerative, and infectious etiologies. The patient's symptoms remitted fully with immune therapy, but recurred in association with widespread bihemispheric brain lesions. Brain biopsy revealed mild nonvasculitic inflammation and prominent vascular hyalinization. Immune therapy with plasma exchanges cleared the MRI abnormalities but, 10 years after onset, the patient still suffers neuropsychiatric sequelae. We conclude that autoimmune panencephalitis seronegative for VGKC-complex, NMDAR, and GAD autoantibodies is a subtype of autoimmune encephalitis that can present with pure neuropsychiatric features and a normal brain MRI. Immunologic mechanisms may account for psychiatric symptoms in a subset of patients now diagnosed with classical psychotic disorders. Delay in starting immune therapy can lead to permanent neuropsychiatric sequelae. We propose a standardized classification system for the autoimmune encephalitides, integrating earlier pathology-oriented terms with more recently defined serologic and clinical phenotypes.

  4. Evaluation of Incidence and Clinical Features of Antibody-Associated Autoimmune Encephalitis Mimicking Dementia

    Directory of Open Access Journals (Sweden)

    Arzu Çoban

    2014-01-01

    Full Text Available Background. Anti-neuronal autoimmunity may cause cognitive impairment that meets the criteria for dementia. Objective. Our aim was to detect the incidence and clinical features of autoimmune encephalitis imitating clinical findings of primary dementia disorders and to delineate the validity of anti-neuronal antibody screening in dementia patients. Methods. Fifty consecutive patients fulfilling the clinical criteria for primary dementia, 130 control patients, and 50 healthy controls were included. Their sera were investigated for several ion channel and glutamic acid decarboxylase (GAD antibodies by a cell-based assay, radioimmunoassay, and ELISA, as required. Results. Sixteen patients satisfying dementia criteria had atypical findings or findings suggestive of autoimmune encephalitis. N-methyl-D-aspartate receptor (NMDAR antibody was detected in a patient with dementia, Parkinsonism, and REM sleep behavior disorder (RBD fulfilling the criteria for dementia with Lewy bodies (DLB. One control patient with bipolar disease displayed low anti-GAD antibody levels. Conclusions. Our study showed for the first time the presence of parkinsonism and RBD in an anti-NMDAR encephalitis patient mimicking DLB. Although autoimmune encephalitis patients may occasionally present with cognitive decline, most dementia patients do not exhibit anti-neuronal antibodies, suggesting that routine analysis of these antibodies in dementia is not mandatory, even though they display atypical features.

  5. 重症抗 N-甲基-D-天冬氨酸受体脑炎的临床特点及预后分析%Clinical characteristic and prognostic analysis of severe anti-N-methyl-D-aspartate receptor encephalitis

    Institute of Scientific and Technical Information of China (English)

    黄小钦; 樊春秋; 叶静

    2016-01-01

    目的:探讨重症抗N-甲基-D-天冬氨酸受体( NMDAR)脑炎的临床特点及预后。方法回顾性分析9例重症抗NMDAR脑炎患者的临床资料。结果9例患者(女7例,男2例)均入住ICU治疗;平均发病年龄27.7岁;平均病程22.4 d, ICU平均住院时间50.9 d。主要临床表现有发热(7例)、精神行为异常(9例)、癫痫发作(9例)、意识障碍(8例)、运动功能异常(7例)、自主神经功能障碍(9例)及低通气(6例)。3例合并畸胎瘤。所有患者CSF抗NMDAR抗体均阳性,6例血清抗NMDAR抗体阳性,7例CSF-IgA升高。3例患者头颅MRI示颞叶或海马异常信号。5例患者EEG示异常慢波。9例患者接受糖皮质激素、丙种球蛋白或血浆置换等免疫调节治疗。5例患者完全恢复,4例患者症状改善伴残留症状。结论重症抗NMDAR脑炎常表现为进展迅速的精神行为改变、癫痫等症状,还有意识障碍、运动障碍、自主神经功能障碍,合并肿瘤并不多见。多数重症抗NMDAR脑炎患者积极免疫治疗预后较好。%Objective To observe the clinical characteristic and prognostic analysis of severe anti-N-methyl-D-aspartate receptor ( anti-NMDAR) encephalitis.Methods The clinical data of 9 patients with severe anti-NMDAR encephalitis were retrospective analyzed.Results Nine patients ( two male and seven females ) were taken care at ICU.They were 27.7 years old at average.The median course was 22.4 d and median length of stay in ICU was 50. 9 d.The mainly clinical characteristics were fever(7 cases), psychiatric behavioral symptoms(9 cases), seizures(9 cases) , decreased consciousness ( 8 cases ) , abnormal movement ( 7 cases ) , automatic instability ( 9 cases ) and hypoventilation(6 cases) .Three cases had ovarian teratoma.Anti-NMDAR antibodies in CSF of all patients were testing positive, while in serum were 6 cases.CSF-IgA in 7 cases were increased.The brain MRI indicated

  6. A Practical Approach to Meningitis and Encephalitis.

    Science.gov (United States)

    Richie, Megan B; Josephson, S Andrew

    2015-12-01

    Meningitis is an inflammatory syndrome involving the meninges that classically manifests with headache and nuchal rigidity and is diagnosed by cerebrospinal fluid examination. In contrast, encephalitis refers to inflammation of the brain parenchyma itself and often results in focal neurologic deficits or seizures. In this article, the authors review the differential diagnosis of meningitis and encephalitis, with an emphasis on infectious etiologies. The recommended practical clinical approach focuses on early high-yield diagnostic testing and empiric antimicrobial administration, given the high morbidity associated with these diseases and the time-sensitive nature of treatment initiation. If the initial workup does not yield a diagnosis, further etiology-specific testing based upon risk factors and clinical characteristics should be pursued. Effective treatment is available for many causes of meningitis and encephalitis, and when possible should address both the primary disease process as well as potential complications.

  7. Benzalkonium Chloride Intoxication Mimicking Herpes Zoster Encephalitis

    Directory of Open Access Journals (Sweden)

    Ekrem Güler

    2011-06-01

    Full Text Available Benzalkonium chloride (BAC is a frequently used disinfectant and its most well-known side effect is contact dermatitis. In this report, two children who had vesicular dermatitis, headache, lethargy, fever and encephalopathy mimicking Herpes zoster encephalitis were presented. Their consciousness level improved on the second day. From the medical history it was understood that the mother had applied 20% BAC solution to the scalps of two children. The aim of the presentation of this report is to draw attention to the fact that BAC application to the scalp for treating pediculosis capitis may resemble the herpes encephalitis clinical picture.

  8. Mycotic encephalitis: predilection for grey matter

    Energy Technology Data Exchange (ETDEWEB)

    Knaap, M.S. van der (Dept. of Child Neurology, Free Univ. Hospital, Amsterdam (Netherlands)); Valk, J. (Dept. of Diagnostic Radiology, Free Univ. Hospital, Amsterdam (Netherlands)); Jansen, G.H. (Dept. of Pathology, Subdivision of Neuropathology, Univ. Hospital, Utrecht (Netherlands)); Kapelle, L.J. (Dept. of Neurology, Univ. Hospital, Utrecht (Netherlands)); Nieuwenhuizen, O. van (Dept. of Child Neurology, Wilhelmina Children' s Hospital, Utrecht (Netherlands))

    1993-10-01

    In mycotic infections of the brain three patterns of abnormality may be observed: meningitis, granuloma, and encephalitis. The first two, consisting of diffuse meningeal enhancement and mass lesion respectively, can easily be visualised by CT or MRI, but are nonspecific. The third pattern has been described histopathologically; as the clinical picture is nonspecific and the diagnosis is often unsuspected, especially in immunocompetent patients, acquaintance with the characteristic CT and MRI patterns of mycotic encephalitis may help in establishing the correct diagnosis, with important therapeutic consequences. (orig.)

  9. Necrotising encephalitis in a French bulldog.

    Science.gov (United States)

    Timmann, D; Konar, M; Howard, J; Vandevelde, M

    2007-06-01

    A 20-month-old, female French bulldog was presented with a three-month history of generalised seizures and progressive ataxia with occasional falling over on either side. Neurological examination revealed signs, suggesting a multifocal intracranial lesion. Magnetic resonance imaging of the brain revealed two connected lesions on the left side of the caudal brainstem and a further lesion in the cerebrum. The dog was euthanased, and histopathological examination revealed lesions which closely resembled those of necrotising encephalitis in Yorkshire terriers. This is the first case describing this type of necrotising encephalitis in a French bulldog.

  10. Clinical analysis of relapsing anti-N-methyl-D-aspartate receptor encephalitis%复发性抗N-甲基-D-天冬氨酸受体脑炎临床分析

    Institute of Scientific and Technical Information of China (English)

    关鸿志; 孔维泽; 彭斌; 黄颜; 卢强; 袁晶; 柳青; 沈航; 任海涛

    2015-01-01

    目的 探讨复发性抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床特点、诊断与诊疗.方法 回顾2011年6月至2014年11月在北京协和医院神经科收治的复发性抗NMDA受体脑炎16例,分析症状学、脑脊液学、神经影像学、免疫学和肿瘤学等特点及治疗方案.结果 男5例,女11例,平均21.2岁.首次发病的起病症状包括:精神症状10例,不自主运动2例,癫痫发作3例,近记忆力减退1例;主要症状数:2 ~8个(平均5.8个);入ICU救治43.8%;改良Rankin评分(mRS)平均4.56;1例于首次发病时发现卵巢畸胎瘤并行切.16例共发生复发32次,多次复发者8例,平均复发间隔5.0个月.复发时症状数1~7个,其中以单一症状复发者11例次,多症状复发者21例次;复发期平均症状数2.59个/例次,低于初次发病(P<0.05).复发期mRS评分1~5分,平均2.69,低于初次发病(P<0.05).2例复发时出现不同于首次发病时的新症状.复发期脑脊液抗NMDA受体抗体阳性率为100%,血清抗NMDA受体抗体阳性率为53.1%.复发期脑电图异常12例.头MRI首次发病检查中8例异常,复发时原有病灶均消失,7例见新病灶.5例于复发期行头PET,均有异常.2例于复发期发现卵巢畸胎瘤并手术切除.16例给予一线免疫治疗,12例加用二线免疫治疗.全部病例经免疫治疗后病情缓解,mRS评分0~2分,平均0.78分.结论 抗NMDA受体脑炎具有一次复发和多次复发的可能性,即使切除畸胎瘤仍有可能复发.多数患者在复发时症状较轻.症状学、脑脊液抗体检测和神经影像学等为判断和评估复发的重要方法.为预防复发,选择长程免疫治疗方案是必要的,并需要遵循个体化的原则.%Objective With the discovery of more patients with anti-N-methyl-D-aspartate (antiNMDAR) encephalitis,frequent clinical relapses pose a new challenge to neurologists.Methods Retrospective reviews were conducted for 16 hospitalized patients with relapsing

  11. Joint determination of biological encephalization, economic specialization

    NARCIS (Netherlands)

    Horan, R.D.; Shogren, J.F.; Bulte, E.H.

    2011-01-01

    In this paper, we develop a paleoeconomic model of the co-evolution of economic specialization and encephalization-the common physiological measure of intelligence as reflected by brain mass relative to total body mass. Our economic analysis links ecological and social intelligence theories of incre

  12. Early Diagnosis of Herpes Simplex Encephalitis

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-06-01

    Full Text Available Records of 38 patients, 23 boys and 15 girls (ages 3 months to 16 years [42% ages 3-12 months], seen between 1990 and 1997 with proven herpes simplex encephalitis (HSE, were reviewed retrospectively to determine the diagnostic reliability of polymerase chain reaction (PCR results, in a study at the Neuropediatric Service, Hopital Saint Vincent de Paul, Paris, France.

  13. Brainstem Encephalitis and ADEM Following Mumps

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-03-01

    Full Text Available Clinical manifestations of brainstem encephalitis (BSE with fever, decreased level of consciousness, and left facial and abducens paralysis developed 1 week after bilateral parotitis and mumps in a 4 year-old female child and were followed by symptoms of acute disseminated encephalomyelitis (ADEM within 20 days of recovery from BSE.

  14. [Meningitis and encephalitis in Poland in 2005].

    Science.gov (United States)

    Stefanoff, Paweł; Rosińska, Magdalena

    2007-01-01

    In Poland, 2 806 cases of neuroinfections were reported in 2005, of which 998 had bacterial aetiology, 1469 viral, and 339 cases had other or unknown origin. Incidence of bacterial neuroin-fections increased in 2003-2005, following a decreasing trend observed during the past decade. Etiological factor was determined in 486 (49%) cases of bacterial neuroinfections. Among them Neisseria meningitidis was found in 135 cases, Haemophilus influenzae in 59 cases and Streptococ-cus pneumoniae in 111 cases. Unlike previously in 2005 serogroup B was no longer the predominant type of N. meningitidis cultured from patients. Both types B and C constituted similar proportions of all strains serotyped in 2005. Viral neuroinfections incidence in 2005 remained on the same level as in 2004. Etiological factor of central nervous system aseptic infections were established only in minor proportion of cases--3% of meningitis and 20% of encephalitis. Among confirmed cases, there were 177 cases of tick-borne encephalitis and 13 cases of herpetic encephalitis. Tick borne encephalitis incidence decreased in 2005 (0.46), compared to 2003-2004. Most of the cases were reported from endemic areas of northeastern part of the country.

  15. [Meningitis and encephalitis in Poland in 2006].

    Science.gov (United States)

    Kicman-Gawłowska, Agnieszka; Chrześcijańska, Irena; Stefanoff, Paweł

    2008-01-01

    In Poland, 3 693 cases of neuroinfections were reported in 2006, of which 989 had bacterial aetiology, 1 874--viral aetiology, and 512--other or unknown origin. The etiological agent was determined in 455 (46%) cases of bacterial neuroinfections. Among them Neisseria meningitidis was found in 148 cases, Haemophilus influenzae type B (Hib) in 39 cases and Streptococcus pneumoniae in 119 cases. An increasing trend in meningococcal infections incidence has been observed in 2006, and a substantial decrease of Hib incidence, related to increasing vaccination coverage. Viral neuroinfections incidence in 2006 increased compared to year 2005. Etiological factors of central nervous system aseptic infections were established only in minor proportion of cases--3% of meningitis and 20% of encephalitis. Among confirmed cases, there were 317 cases of tick-borne encephalitis and 31 cases of herpetic encephalitis. Tick borne encephalitis incidence increased in 2006 (0.83), compared to 2004 - 2005. Most of the cases were reported from endemic areas of north-eastern part of the country.

  16. Natural course of LGI1 encephalitis

    DEFF Research Database (Denmark)

    Szots, Monika; Marton, Annamaria; Kover, Ferenc

    2014-01-01

    , respectively, and none of the patients received immunotherapy. LE showed characteristics of LGI1 encephalitis in both cases, including low sodium content in the sera; disorientation, hallucination, short-term memory loss; and epileptic seizures. One patient had faciobrachial tonic seizures. MRI indicated...

  17. Receptors for enterovirus 71

    OpenAIRE

    Yamayoshi, Seiya; Fujii, Ken; Koike, Satoshi

    2014-01-01

    Enterovirus 71 (EV71) is one of the major causative agents of hand, foot and mouth disease (HFMD). Occasionally, EV71 infection is associated with severe neurological diseases, such as acute encephalitis, acute flaccid paralysis and cardiopulmonary failure. Several molecules act as cell surface receptors that stimulate EV71 infection, including scavenger receptor B2 (SCARB2), P-selectin glycoprotein ligand-1 (PSGL-1), sialylated glycan, heparan sulfate and annexin II (Anx2). SCARB2 plays crit...

  18. Identification and isolation of Genotype-I Japanese Encephalitis virus from encephalitis patients

    Directory of Open Access Journals (Sweden)

    Gao Xiaoyan

    2010-11-01

    Full Text Available Abstract Historically, Japanese Encephalitis virus (JEV genotype III (GIII has been responsible for human diseases. In recent years, JEV genotype I (GI has been isolated from mosquitoes collected in numerous countries, but has not been isolated from patients with encephalitis. In this study, we report recovery of JEV GI live virus and identification of JEV GI RNA from cerebrospinal fluid (CSF of encephalitis patients in JE endemic areas of China. Whole-genome sequencing and molecular phylogenetic analysis of the JEV isolate from the CSF samples was performed. The isolate in this study is highly similar to other JEV GI strains which isolated from mosquitoes at both the nucleotide and deduced amino acid levels. Phylogenetic analysis based on the genomic sequence showed that the isolate belongs to JEV GI, which is consistent with the phylogenetic analysis based on the pre-membrane (PrM and Glycoprotein genes. As a conclusion, this is the first time to isolate JEV GI strain from CSF samples of encephalitis patients, so continuous survey and evaluate the infectivity and pathogenecity of JEV GI strains are necessary, especially for the JEV GI strains from encephalitis patients. With respect to the latter, because all current JEV vaccines (live and inactivated are derived from JEV GIII strains, future studies should be aimed at investigating and monitoring cross-protection of the human JEV GI isolates against widely used JEV vaccines.

  19. Dengue encephalitis-A rare manifestation of dengue fever

    OpenAIRE

    Madi, Deepak; Achappa, Basavaprabhu; Ramapuram, John T; Chowta, Nityananda; Laxman, Mridula; Mahalingam, Soundarya

    2014-01-01

    The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue. The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with...

  20. Acute encephalitis associated with measles: MRI features

    Energy Technology Data Exchange (ETDEWEB)

    Lee, K.Y.; Cho, W.H.; Kim, S.H. [Department of Radiology, Sanggye Paik Hospital, Inje University, 760-1 Sanggye-7 dong, Nowon-gu, Seoul 139707 (Korea); Kim, H.D. [Department of Paediatrics, Sanggye Paik Hospital, Inje University, 760-1 Sanggye-7 dong, Nowon-gu, Seoul 139707 (Korea); Kim, I.O. [Department of Radiology, Seoul National University Hospital, 28, Yongon-dong, Chongno-gu, Seoul 110744 (Korea)

    2003-02-01

    We document the MRI features in six patients aged 5-14 years with acute encephalitis following measles. The diagnosis was made on a characteristic morbiliform rash and detection of specific IgM and IgG antibodies. The symptoms of encephalitis occurred 1-11 days after the appearance of the rash. All patients underwent MRI within 1-4 days of the onset of neurological symptoms. Diffusion weighted images (DWI) were obtained in three patients. In all patients, T2-weighted images showed widely distributed, multifocal high signal in both cerebral hemispheres with swelling of the cortex, with bilateral, symmetrical involvement of the putamen and caudate nucleus. The lesions had showed low apparent diffusion coefficients. Three patients showed subacute gyriform haemorrhage, and asymmetrical gyriform contrast enhancement on follow-up MRI. (orig.)

  1. Decompressive craniectomy in herpes simplex encephalitis

    Directory of Open Access Journals (Sweden)

    Muhammed Jasim Abdul Jalal

    2015-01-01

    Full Text Available Intracranial hypertension is a common cause of morbidity in herpes simplex encephalitis (HSE. HSE is the most common form of acute viral encephalitis. Hereby we report a case of HSE in which decompressive craniectomy was performed to treat refractory intracranial hypertension. A 32-year-old male presented with headache, vomiting, fever, and focal seizures involving the right upper limb. Cerebrospinal fluid-meningoencephalitic profile was positive for herpes simplex. Magnetic resonance image of the brain showed swollen and edematous right temporal lobe with increased signal in gray matter and subcortical white matter with loss of gray, white differentiation in T2-weighted sequences. Decompressive craniectomy was performed in view of refractory intracranial hypertension. Decompressive surgery for HSE with refractory hypertension can positively affect patient survival, with good outcomes in terms of cognitive functions.

  2. Benzalkonium Chloride Intoxication Mimicking Herpes Zoster Encephalitis

    OpenAIRE

    Güler, Ekrem; Olgar, Şeref; Davutoğlu, Mehmet; Garipardıç, Mesut; Karabiber, Hamza

    2011-01-01

    Benzalkonium chloride (BAC) is a frequently used disinfectant and its most well-known side effect is contact dermatitis. In this report, two children who had vesicular dermatitis, headache, lethargy, fever and encephalopathy mimicking Herpes zoster encephalitis were presented. Their consciousness level improved on the second day. From the medical history it was understood that the mother had applied 20% BAC solution to the scalps of two children. The aim of the presentation of this report is ...

  3. Benzalkonium Chloride Intoxication Mimicking Herpes Zoster Encephalitis

    OpenAIRE

    Ekrem Güler; Şeref Olgar; Mehmet Davutoğlu; Mesut Garipardıç; Hamza Karabiber

    2014-01-01

    Benzalkonium chloride (BAC) is a frequently used disinfectant and its most well-known side effect is contact dermatitis. In this report, two children who had vesicular dermatitis, headache, lethargy, fever and encephalopathy mimicking Herpes zoster encephalitis were presented. Their consciousness level improved on the second day. From the medical history it was understood that the mother had applied 20% BAC solution to the scalps of two children. The aim of the presentation of this report is...

  4. Multiple Paths to Encephalization and Technical Civilizations

    Science.gov (United States)

    Schwartzman, David; Middendorf, George

    2011-12-01

    We propose consideration of at least two possible evolutionary paths for the emergence of intelligent life with the potential for technical civilization. The first is the path via encephalization of homeothermic animals; the second is the path to swarm intelligence of so-called superorganisms, in particular the social insects. The path to each appears to be facilitated by environmental change: homeothermic animals by decreased climatic temperature and for swarm intelligence by increased oxygen levels.

  5. Postinfectious encephalitis with multifocal white matter lesions.

    Science.gov (United States)

    Boulloche, J; Parain, D; Mallet, E; Tron, P

    1989-08-01

    Two cases of multifocal white matter lesions occurring after viral illness are reported. Evoked potentials study and cranial magnetic resonance imaging (T2-weighted image) showed early abnormalities while CT scan was initially normal. Patients improved dramatically with steroid therapy. It would seem that because of a considerable responsiveness to steroids this affection should be differentiated from other types of encephalitis. Relations with multiple sclerosis are discussed.

  6. An ion channel chip for diagnosis and prognosis of autoimmune neurological disorders.

    Science.gov (United States)

    Chatelain, Franck C; Mazzuca, Michel; Larroque, Marie-Madeleine; Rogemond, Veronique; Honnorat, Jerome; Lesage, Florian

    2013-12-01

    Autoantibodies directed against ion channels and ionotropic receptors are associated with neuromuscular and neurological disorders. Their detection has proven to be useful for diagnosis, prognosis and treatment of these autoimmune syndromes. We have designed an ion channel chip for the systematic and rapid screening of antibodies directed against tens of different ion channels. The chip has been validated by confirming the presence of autoantibodies in patients with anti-NMDA receptor encephalitis. Such a chip will be useful for the diagnosis of already documented disorders, but also to identify new targets of autoimmunity and classification of the corresponding diseases. The article presents some promising patents on the Ion Channel Chip.

  7. A REVIEW ARTICLE ON HERPES SIMPLEX ENCEPHALITIS

    Directory of Open Access Journals (Sweden)

    A. Karimi MD

    2009-01-01

    Full Text Available Abstract:Herpes Simplex encephalitis (HSE is a life threatening outcome of Herpes simplex virus (HSV infection of the central nervous system (CNS. HSVaccounts for 2-5 percent of all cases of encephalitis. One third of cases occur in those younger than 20 years old and one half in those older than 50 years old.Clinical diagnosis is recommended in the encephalopathic, febrile patients with focal neurological signs. However, the clinical findings are not pathogonomic because numerous other diseases of CNS can mimic HSE. Diagnosis should be confirmed based on medical history, analysis of cerebrospinal fluid (CSF for protein and glucose contents, the cellular analysis and identifying the pathogens by serology and Polymerase Chain Reaction (PCR amplification .The diagnostic gold standard is the detection of HSV DNA in the cerebrospinal fluid by PCR. But negative results need to be interpreted regarding thepatients clinical signs and symptoms and the time of CSF sampling. Spike and slow wave patterns is observed in Electroencephalogram (EEG.Neuroimaging, especially Magnetic Resonance Imaging (MRI is essential for evaluating the patients, which shows temporal lobe edema or hemorrhage.All patients with HSE should be treated by intravenous Acyclovir (10mg/kg q8hr for 14-21 days. After completing therapy, PCR of the CSF can confirmthe elimination of replicating virus, assisting further management of the patient.Keywords:Herpes Simplex Virus (HSV, Encephalitis, Children

  8. [Meningitis and encephalitis in Poland in 2004].

    Science.gov (United States)

    Stefanoff, Paweł; Rosińska, Magdalena

    2006-01-01

    In Poland, 2 725 cases of neuroinfections were reported in 2004, of which 945 had bacterial etiology, 1427 viral, and 353 cases had other or unknown origin. Incidence of bacterial neuroinfections increased in 2004, despite a decreasing trend observed during the past decade. Etiological factor was determined in 433 (46%) cases of bacterial neuroinfections. Among them Neisseria meningitidis was found in 119 cases, Haemophilus influenzae in 77 cases and Streptococcus pneumoniae in 107 cases. As in the previous years, serotype B was the predominant type of N. meningitidis cultured from patients, but type C appears to be systematically increasing, accounting for 27% of the strains serotyped in 2004. Viral neuroinfections were less common in 2004, compared to previous years. Etiological factor of central nervous system aseptic infections were established only in 17% of cases. Among confirmed cases, there were 262 cases of tick-borne encephalitis and 15 cases of herpetic encephalitis. Tick borne encephalitis incidence decreased in 2004 (0.7), compared to 2003 (339 cases, incidence 0.9). Most of the cases were reported from endemic areas of north-eastern part of the country.

  9. Successful Laparoscopic Resection of 7 mm Ovarian Mature Cystic Teratoma Associated with Anti-NMDAR Encephalitis

    Directory of Open Access Journals (Sweden)

    Masaru Hayashi

    2014-01-01

    Full Text Available Anti-NMDAR (N-methyl-D-aspartate receptor encephalitis is an immune-mediated encephalitis. It has been predominantly described in young women and is commonly associated with an ovarian teratoma. We report a case of anti-NMDAR encephalitis associated with a 7 mm ovarian teratoma that was completely resected by laparoscopic surgery. An 18-year-old woman suddenly presented with personality changes requiring her admission to the department of neurology. After that, she also showed involuntary movements, disturbance of consciousness, and central hypoventilation. As an abdominal image revealed the possibility of a right ovarian teratoma of 5×7 mm, a laparoscopic operation was performed. The macroscopic appearance of the right ovary did not show any abnormalities; nevertheless, we performed a partial resection of the right ovary, with reference to the image diagnosis, in order to spare the ovarian reserve. The 22×22 mm partially resected ovary contained an intact 5×7 mm cystic tumor. The pathological diagnosis was mature cystic teratoma with components of brain tissue. An anti-NMDAR-antibody test proved positive in both serum and cerebrospinal fluid 1 month after the surgery. From these results, she was diagnosed with anti-NMDAR encephalitis. By the administration of cyclophosphamide in addition to the operation, she recovered drastically without any of the symptoms shown before.

  10. D-cycloserin, a NMDA-agonist may be a treatment option for anti-NMDAR encephalitis

    Institute of Scientific and Technical Information of China (English)

    Hong-Zhi Guan; Tie-Kuan Du; Jin Xu; Xia Lv; Hua-Dong Zhu; Yi-Cheng Zhu; Bin Peng; Li-Ying Cui

    2016-01-01

    Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is caused by reversible neuron dysfunction associated an autoantibody-mediated decrease of NMDAR in the entire brain. A N-methyl-D-aspartate (NMDA) -agonist treatment for anti-NMDAR encephalitis might have a role considering its speciifc mechanism. The authors used D-cycloserine, a partial NMDA-agonist in a refractory case with prolonged intensive care unit duration. A 13-year-old female presented with headache, cognitive deterioration, generalized seizures, coma and hypoventilation with required mechanical ventilation. Anti-NMDAR antibodies were identiifed in cerebrospinal lfuid and serum conifrming anti-NMDAR encephalitis. The patient was refractory to ifrst-line and second-line immunotherapy and removal of ovary teratoma. D-cycloserine was then administered and her symptoms improved gradually and signiifcantly. This is the ifrst reported case in which D-cycloserine was applied to this disease. D-cycloserine might be a potential option as speciifc treatment in anti-NMDAR encephalitis.

  11. Epstein-Barr virus encephalitis and encephalomyelitis: MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Shian, W.J. [Department of Pediatrics, Tao-Yuan Veterans Hospital, No. 100, Sec 3, Cheng-Kung Rd, City of Tao-Yuan, Taiwan (Taiwan, Province of China); Chi, C.S. [Department of Pediatrics, Taichung Veterans General Hospital, Taichung, Taiwan (Taiwan, Province of China)

    1996-09-01

    The purpose of this project is to investigate the clinical and brain MR characteristics of Epstein-Barr virus (EBV) encephalitis and encephalomyelitis. Clinical and 30 MR findings of 29 patients with EBV encephalitis or encephalomyelitis were retrospectively reviewed. Patients included 24 with encephalitis, 3 with encephalomyelitis, and 2 with brain-stem encephalitis. Altered consciousness, seizures, visual hallucination, and acute psychotic reaction were the common presentations. Eight patients had positive MR findings. These included T2 prolongation over gray and white matter, periventricular leukomalacia, and brain atrophy. Transient T2 prolongation over gray and white matter was found in one patient. Our results indicate that EBV encephalitis and encephalomyelitis have a wide range of both clinical and MR findings. The MR lesions may disappear in a short period, so the timing for the MR scan may be critical. (orig.). With 5 figs., 2 tabs.

  12. Immunotherapy for anti-NMDAR encephalitis: A review of paraneoplastic, autoimmune encephalopathy

    Directory of Open Access Journals (Sweden)

    Ruu-Fen Tzang

    2017-03-01

    Full Text Available Paraneoplastic immune-mediated encephalopathy is a recently described disease. Dalmau et al first linked the presence of Anti-N-methyl-d-aspartate receptor (anti-NMDAR antibodies to paraneoplastic psychiatric and neurologic disease after encountering a case of anti-NMDAR encephalitis in a woman with ovarian teratoma in 2007. Nerve cells from a teratoma or previous viral infection could trigger autoantibodies, causing NMDA receptors to become dysfunctional in neurotransmission across synapses. Symptoms of anti-NMDAR encephalitis include prodromal symptoms, psychiatric symptoms, speech dysfunction, seizure, abnormal neurological movement, and autonomic dysfunction. This disease is reversible and treatable; however, early diagnosis and treatment are essential as they may prevent excess antibodies from causing severe or prolonged harm in the brain. First-line Immunotherapy includes intravenous high-dose steroids (methylprednisolone, intravenous immunoglobulin (IVIG, and/or plasmapheresis. Second-line immunotherapy includes targeted B-cell therapy with rituximab and cyclophosphamide. Taiwan is one of the countries where tests for detecting this disease, which are expensive, are not currently available. A cell-based indirect immunofluorescence test for the detection of IgG antibodies against the NMDA receptor should become more available for aiding diagnostics. Most importantly, early immune modulatory therapy including steroid, IVIG, and plasma exchange should become financially more feasible for use in treatment in Taiwan.

  13. Herpes Simplex Virus (HSV-1 Encephalitis Mimicking Glioblastoma: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Burke A. Cunha

    2014-12-01

    Full Text Available Glioblastoma multiforme (GBM often presents as a brain mass with encephalitis. In a patient with GBM, subsequent presentation with new onset encephalitis may be due to another GBM or Herpes simplex virus 1 (HSV-1 encephalitis. We present a case of HSV-1 encephalitis mimicking GBM in a patient with previous GBM.

  14. What should you know about limbic encephalitis?

    Directory of Open Access Journals (Sweden)

    Sara Machado

    2012-10-01

    Full Text Available Autoimmune encephalitis is an inflammatory disorder characterized by a subacute impairment of short-term memory, psychiatric features and seizures. It is often associated with a variety of other neurological symptoms, and its differential diagnosis is wide, leading to challenges in its recognition. It used to be regarded as a rare disease, usually paraneoplastic and with poor prognosis. However, with the recent recognition of membrane-surface directed antibodies, it is now known that in a substantial proportion of cases there is no association with any malignancy and there is a good prognosis if treated. Hence, early recognition and prompt initiation of immunotherapies are of great importance.

  15. Granulomatous Amoebic Encephalitis: Clinical Diagnosis and Management

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    Naveed A. Khan

    2005-01-01

    Full Text Available Granulomatous amoebic encephalitis (GAE is a serious human disease with fatal consequences. With the mortality rate of more than 90%, it is not surprising that the majority of GAE infections are identified at the post-mortem stage. The most distressing aspect is that the high level of mortality is attributed to lack of awareness. Early diagnosis with aggressive treatment can lead to successful prognosis for the patient. Here, we describe a brief overview of the current understanding of the pathophysiology of GAE, available diagnostic methods, possible therapeutic interventions and the causative agents.

  16. An SIRS epidemic model of Japanese Encephalitis

    OpenAIRE

    Mukhopadhyay, B. B.; Tapaswi, P. K.

    1994-01-01

    An epidemiological model of the dynamics of Japanese Encephalitis (J.E.) spread coupling the SIRS (Susceptible/Infected/Removal/Susceptible) models of J.E. spread in the reservoir population and in the human population has been proposed. The basic reproductive rate R(0) in the coupled system has been worked out. Using Aron's results (cf. [1] and [2]), it has been observed that the disease-free system is stable in this coupled system also, if R(0) is less than unity, and if R(0) is greater tha...

  17. Childhood epileptic seizures imitating migraine and encephalitis

    Directory of Open Access Journals (Sweden)

    Kravljanac Ružica

    2012-01-01

    Full Text Available Introduction. Paroxismal events can resemble epileptic seizures, however, some epileptic seizures, especially benign occipital childhood epilepsies can imitate migraine, cycling vomiting or encephalitis. Objective. The aim of this study was evaluation of clinical and electroencephalographic (EEG features and outcome in children with benign occipital childhood epilepsies. Methods. Investigation included 18 patients with benign occipital childhood epilepsies hospitalized in the period from 2007 to 2010. The diagnosis was based on clinical and EEG characteristics of seizures, while treatment included acute therapy for seizures and chronic antiepileptic drugs. Prognosis was analyzed in terms of neurological outcome and seizure recurrence rate. Results. Benign occipital childhood epilepsy with early onset was diagnosed in 15 children. Vegetative symptoms, mostly ictal vomiting (13, eye deviation and loss of consciousness (13 dominated in the clinical presentation. The most frequent EEG findings showed occipital epileptic discharges. Benign occipital childhood epilepsy with late onset was diagnosed in three cases. Seizures were manifested by visual hallucinations, headache and secondary generalized convulsions. All three patients were administered chronic antiepileptic drugs and had good outcome. Conclusion. In our patients, clinical manifestations of benign occipital epilepsies had some similarities with clinical features of migraine and encephalitis. It could explain misdiagnosis in some of them. Knowledge about main features and differences between each of these disorders is crucial for making appropriate diagnosis.

  18. A REVIEW ARTICLE ON HERPES SIMPLEX ENCEPHALITIS

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    A. Karimi MD,

    2007-02-01

    Full Text Available Herpes Simplex encephalitis (HSE is a life threatening outcome of Herpes simplex virus (HSV infection of the central nervous system (CNS. HSVaccounts for 2-5 percent of all cases of encephalitis. One third of cases occur in those younger than 20 years old and one half in those older than 50 years old.Clinical diagnosis is recommended in the encephalopathic, febrile patients with focal neurological signs. However, the clinical findings are not pathogonomic because numerous other diseases of CNS can mimic HSE. Diagnosis should be confirmed based on medical history, analysis of cerebrospinal fluid (CSF for protein and glucose contents, the cellular analysis and identifying the pathogens by serology and Polymerase Chain Reaction (PCR amplification .The diagnostic gold standard is the detection of HSV DNA in the cerebrospinal fluid by PCR. But negative results need to be interpreted regarding thepatients clinical signs and symptoms and the time of CSF sampling. Spike and slow wave patterns is observed in Electroencephalogram (EEG.Neuroimaging, especially Magnetic Resonance Imaging (MRI is essential for evaluating the patients, which shows temporal lobe edema or hemorrhage.All patients with HSE should be treated by intravenous Acyclovir (10mg/kg q8hr for 14-21 days. After completing therapy, PCR of the CSF can confirmthe elimination of replicating virus, assisting further management of the patient.

  19. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis.

    Science.gov (United States)

    Haider, Ali S; Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-07-05

    Hashimoto's encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient's paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto's encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids.

  20. Japanese Encephalitis: Estimating Future Trends in Asia

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    Julia Metelka

    2015-08-01

    Full Text Available Limited surveillance programs and lack of diagnostic laboratory testing capacity in many low and middle income Asian countries have made it difficult to validate epidemiological patterns and anticipate future changes in disease risk. In this study, we consider the case of Japanese Encephalitis in Asia and examine how populations of human hosts and animal reservoirs are expected to change over the next three decades. Growth was modelled at the sub-national level for rural and urban areas to estimate where high-density, susceptible populations will potentially overlap with populations of the virus' amplifying host. High-risk areas based on these projections were compared to the current distribution of Japanese Encephalitis, and known immunization activities in order to identify areas of highest priority for concern. Results indicated that mapping JE risk factors at the sub-national level is an effective way to contextualize and supplement JE surveillance data. New patterns of risk factor change occurring in Southeast Asia were identified, including around major urban areas experiencing both urbanization and growth in pig populations. A hotspot analysis of pig-to-population ratio found a significant spatial cluster extending northward through Southeast Asia and interior China. Mapping forecasted changes in risk factors for JE highlights regions vulnerable to emerging zoonoses and may be an important tool for developing effecting transnational health policies.

  1. Immunotherapy strategy for 35 cases of severe anti-N-methyl-D-aspartate receptor encephalitis%重症抗N-甲基-D-天冬氨酸受体脑炎35例免疫治疗分析

    Institute of Scientific and Technical Information of China (English)

    袁晶; 彭斌; 关鸿志; 黄颜; 卢强; 任海涛; 杨洵哲; 姜南; 朱以诚

    2016-01-01

    -N-methyl-D-aspartate receptor (NMDAR) encephalitis in China,by demonstrating the clinical characteristics and comparing the treatment strategy with that adopted in foreign countries.Methods A total of 35 hospitalized cases who met the diagnostic criteria for severe anti-NMDAR encephalitis were retrospectively analyzed.Demographic data,clinical history,past medical history,laboratory tests,imaging studies,treatment and the follow-up information were recorded using unified forms.Results Mental and behavioral abnormalities,seizures and consciousness disturbance occurred in all cases;involuntary movements,speech disorders,memory loss,central hypoventilation and autonomic dysfunction happened in 45%-65% of cases.Sixteen patients (45.71%) required mechanical ventilation.Modified Rankin score (mRS) arranged 4-5 (mean mRS 4.86).The percentage of patients with elevated intracranial pressure,white blood cell and protein in cerebrospinal fluid were 42.86%,60.00%,and 14.29%,respectively.Abnormal findings in brain magnetic resonance imaging scan happened in 31.43% cases,located in frontal lobe,temporal lobe,insular lobe,hippocampus,cingulate gyrus,corpus callosum,brain stem,and cerebellum.All cases received intravenous immunoglobulin,for one to maximum seven cycles,with an average of three cycles.91.43% of cases received glucocorticoid therapy,including 54.29% of cases received high-dose methylprednisolone.Two patients (5.71%) received plasma exchange.Five patients (14.29%) received second-line therapy including rituximab for 4 patients and intravenous cyclophosphamide (CTX) for one.Fifteen patients (42.86%) received long-term immunosuppression therapy.All cases acquired improvement after immunotherapy and were transferred out from ICU,the median ICU time was 46 days and median hospitalized duration was 72 days.The mRS were 5 for 2 cases,1-4 for the rest patients,and no patient died during hospitalization.During a median follow-up period of 17.6 months,30 of 35 patients (85.71

  2. Limbic Encephalitis Driven by a Pleural Mesothelioma: A Paraneoplastic Complication

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    Jacob O. Day

    2016-10-01

    Full Text Available Paraneoplastic neurological syndromes have only been described with pleural mesothelioma in five cases. We have described a 72-year-old man who developed anterograde amnesia 27 months after diagnosis of epithelioid pleural mesothelioma. Investigations revealed a limbic encephalitis with no alternative causes identified. Limbic encephalitis is a classical paraneoplastic syndrome and presentation within five years of a cancer with no other causes identified is sufficient to diagnose a paraneoplastic etiology. This is the first case of isolated paraneoplastic limbic encephalitis driven by a pleural mesothelioma.

  3. Case of Herpes encephalitis followed-up by CT

    Energy Technology Data Exchange (ETDEWEB)

    Fukui, Y.; Nagai, S.; Nishibayashi, Y.; Okamoto, H.; Goishi, J. (Matsuyama Red Cross Hospital, Ehime (Japan))

    1982-03-01

    A 9-month-old girl was admitted with lethargy, fever and convulsion. EGG showed localized slow waves in the right temporal region. CT showed a localized low density area accompanied by a hemorrhagic focus in the right frontal lobe. Herpes encephalitis was suspected, and cytosine arabinoside was administered. The antibody titers of the serum and cerebrospinal fluid against herpes simplex virus type I significantly rose. Clinically the patient recovered without serious sequelae. CT revealed marked cerebral atrophy and subdural hematoma which were surgically treated. The importance of CT in the diagnosis and prognosis of herpes encephalitis was argued, and CT findings of herpes encephalitis were discussed.

  4. Replicon particles of Venezuelan equine encephalitis virus as a reductionist murine model for encephalitis.

    Science.gov (United States)

    Schäfer, Alexandra; Whitmore, Alan C; Konopka, Jennifer L; Johnston, Robert E

    2009-05-01

    Venezuelan equine encephalitis virus (VEE) replicon particles (VRP) were used to model the initial phase of VEE-induced encephalitis in the mouse brain. VRP can target and infect cells as VEE, but VRP do not propagate beyond the first infected cell due to the absence of the structural genes. Direct intracranial inoculation of VRP into mice induced acute encephalitis with signs similar to the neuronal phase of wild-type VEE infection and other models of virus-induced encephalitis. Using the previously established VRP-mRNP tagging system, a new method to distinguish the host responses in infected cells from those in uninfected bystander cell populations, we detected a robust and rapid innate immune response in the central nervous system (CNS) by infected neurons and uninfected bystander cells. Moreover, this innate immune response in the CNS compromised blood-brain barrier integrity, created an inflammatory response, and directed an adaptive immune response characterized by proliferation and activation of microglia cells and infiltration of inflammatory monocytes, in addition to CD4(+) and CD8(+) T lymphocytes. Taken together, these data suggest that a naïve CNS has an intrinsic potential to induce an innate immune response that could be crucial to the outcome of the infection by determining the composition and dynamics of the adaptive immune response. Furthermore, these results establish a model for neurotropic virus infection to identify host and viral factors that contribute to invasion of the brain, the mechanism(s) whereby the adaptive immune response can clear the infection, and the role of the host innate response in these processes.

  5. Listeria monocytogenes encephalitis mimicking Herpes Simplex virus encephalitis: the differential diagnostic importance of cerebrospinal fluid lactic acid levels.

    Science.gov (United States)

    Cunha, Burke A; Fatehpuria, Ritu; Eisenstein, Lawrence E

    2007-01-01

    Listeria monocytogenes is a common cause of bacterial meningitis in elderly patients and in those with impaired cellular immunity. The most common central nervous system infection caused by L. monocytogenes is acute bacterial meningitis; meningoencephalitis is uncommon and encephalitis is rare. Early diagnosis of L. monocytogenes meningitis is difficult because only 50% of cerebrospinal fluid (CSF) Gram stains are negative. L. monocytogenes is one of the few central nervous system pathogens associated with red blood cells in the CSF. When L. monocytogenes presents as encephalitis with red blood cells in the CSF, the clinical presentation mimics most closely herpes simplex virus (HSV)-1 encephalitis. Because the therapies for L. monocytogenes and HSV-1 are different, early diagnostic differentiation is clinically important. The CSF lactic acid is the best way to rapidly differentiate between these two entities; the CSF lactic acid level is elevated in L. monocytogenes but is not elevated in HSV-1 encephalitis. The case presented is an elderly man with chronic lymphocytic leukemia who presented with encephalitis. Advanced age and chronic lymphocytic leukemia predispose him to a wide variety of pathogens, but the rapidity and severity of his clinical presentation made L. monocytogenes and HSV-1 encephalitis the most likely diagnostic possibilities. The CSF Gram stain was negative, but the elevated CSF lactic acid levels with encephalitis and red blood cells in the CSF indicated L. monocytogenes as the most likely pathogen. We present a case of L. monocytogenes encephalitis mimicking HSV-1 encephalitis. While receiving ampicillin therapy, the patient remained unresponsive for more than 1 week and then suddenly regained consciousness and recovered without neurologic sequelae.

  6. Incidence of Japanese Encephalitis among Acute Encephalitis Syndrome Cases in West Bengal, India

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    Bhaswati Bandyopadhyay

    2013-01-01

    Full Text Available Background and Objectives. Japanese encephalitis (JE is the most important cause of acute and epidemic viral encephalitis. Every year sporadic JE cases are reported from the various districts of West Bengal, indicating its endemicity in this state. JE vaccination programme has been undertaken by the State Health Department of West Bengal. This study was aimed at seeing the present scenario of JE among acute encephalitis syndrome (AES cases in West Bengal. Materials and Methods. Blood and/or CSF samples were referred from suspected AES cases to the referral virology laboratory of the Calcutta School of Tropical Medicine from different hospitals of Kolkata. IgM antibody capture ELISA was performed on the CSF and serum samples by JE virus MAC ELISA kit supplied by the National Institute of Virology, Pune. Results. The present study reveals that 22.76% and 5% of the AES cases were positive for JE IgM in 2011 and 2012, respectively. JE is mainly prevalent in children and adolescents below 20 years of age with no gender predilection. Although the percentages of JE positive cases were high in 2011, it sharply decreased thereafter possibly due to better awareness programs, due to mass vaccination, or simply due to natural epidemiological niche periodicity due to herd immunity.

  7. [The mechanism, diagnosis and treatment of HHV-6B encephalitis].

    Science.gov (United States)

    Yoshikawa, Tetsushi

    2012-01-01

    Human herpesvirus 6B (HHV-6B) is causative agent for exanthem subitum, which is common febrile illness in infant. This disease is generally benign and self-limited disease, however rarely causes several central nervous system complications. As various types of HHV-6B encephalitis has been demonstrated, pathophysiology of the disease would be complicate. Thus, different therapeutic strategies should be established for each type of HHV-6B encephalitis at the time of primary viral infection. Meanwhile, this virus can reactivate in transplant recipients and cause post-trasplant limbic encephalitis. It has been demonstrated that neuroimaging analysis particularly MRI image is useful for diagnosis of post-transplant HHV-6B encephalitis. As high copies of viral DNA are detected in patient's CSF, direct invasion of HHV-6B might play important role in causing the disease. Ganciclovir or foscarnet could be effective against HHV-6B based on in vitro analysis.

  8. Enterovirus 71 Brainstem Encephalitis and Cognitive and Motor Deficits

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-12-01

    Full Text Available Follow-up studies were conducted in 63 previously healthy children with enterovirus 71 brainstem encephalitis (49 stage II, 7 stage Ilia, and 7 stage Illb at National Cheng Kung University Hospital, Tainan, Taiwan.

  9. Dengue encephalitis-A rare manifestation of dengue fever

    Institute of Scientific and Technical Information of China (English)

    Deepak Madi; Basavaprabhu Achappa; John T Ramapuram; Nityananda Chowta; Mridula Laxman; Soundarya Mahalingam

    2014-01-01

    The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue. The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with fever, altered sensorium and seizures. Dengue NS-1 antigen test was reactive. Dengue IgM was also positive. CSF PCR was negative for herpes simplex 1 & 2. Dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant.

  10. EEG Abnormalities as Diagnostic and Prognostic Factor for Encephalitis

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    Gavrilovic Aleksandar

    2016-09-01

    Full Text Available The aim of the study is to examine whether EEG abnormalities in patients with encephalitis might be prognostic and diagnostic factors for final epilepsy outcome and/or be correlated with the severity of the disability.

  11. A rat model for embolic encephalitis

    DEFF Research Database (Denmark)

    Astrup, Lærke Boye; Rasmussen, Rune Skovgaard; Aalbæk, Bent;

    2011-01-01

    -brain-barrier. This provides our model with several advantages: minimized surgical intervention, bacteria gain access to the brain by the circulation and, no foreign materials are implated in the brain. We thereby mirror the human scenario in several ways: 1: Cerebral infarction by thrombosis or disseminated intravascular...... have recently shown that sepsis is a common cause of microabscesses in the brain, and that S. aureus is one of the most common organisms isolated from these abscesses. This raises the question whether the blood-brain barrier truly makes the brain an immune-privileged organ or not. This makes the brain...... it is difficult to obtain tissue for further examination. This puts a hard demand on animal models of brain lesions in sepsis. We hereby present a novel animal model of embolic encephalitis. Our model introduces bacteria by an embolus to an area of brain necrosis and damage to the blood...

  12. Milrinone in Enterovirus 71 Brain Stem Encephalitis

    Science.gov (United States)

    Wang, Shih-Min

    2016-01-01

    Enterovirus 71 (EV71) was implicated in a widespread outbreak of hand-foot-and-mouth disease (HFMD) across the Asia Pacific area since 1997 and has also been reported sporadically in patients with brain stem encephalitis. Neurogenic shock with pulmonary edema (PE) is a fatal complication of EV71 infection. Among inotropic agents, milrinone is selected as a therapeutic agent for EV71- induced PE due to its immunopathogenesis. Milrinone is a type III phosphodiesterase inhibitor that has both inotropic and vasodilator effects. Its clinical efficacy has been shown by modulating inflammation, reducing sympathetic over-activity, and improving survival in patients with EV71-associated PE. Milrinone exhibits immunoregulatory and anti-inflammatory effects in the management of systemic inflammatory responses in severe EV71 infection. PMID:27065870

  13. Endemic Venezuelan equine encephalitis in northern Peru.

    Science.gov (United States)

    Aguilar, Patricia V; Greene, Ivorlyne P; Coffey, Lark L; Medina, Gladys; Moncayo, Abelardo C; Anishchenko, Michael; Ludwig, George V; Turell, Michael J; O'Guinn, Monica L; Lee, John; Tesh, Robert B; Watts, Douglas M; Russell, Kevin L; Hice, Christine; Yanoviak, Stephen; Morrison, Amy C; Klein, Terry A; Dohm, David J; Guzman, Hilda; Travassos da Rosa, Amelia P A; Guevara, Carolina; Kochel, Tadeusz; Olson, James; Cabezas, Cesar; Weaver, Scott C

    2004-05-01

    Since Venezuelan equine encephalitis virus (VEEV) was isolated in Peru in 1942, >70 isolates have been obtained from mosquitoes, humans, and sylvatic mammals primarily in the Amazon region. To investigate genetic relationships among the Peru VEEV isolates and between the Peru isolates and other VEEV strains, a fragment of the PE2 gene was amplified and analyzed by single-stranded conformation polymorphism. Representatives of seven genotypes underwent sequencing and phylogenetic analysis. The results identified four VEE complex lineages that cocirculate in the Amazon region: subtypes ID (Panama and Colombia/Venezuela genotypes), IIIC, and a new, proposed subtype IIID, which was isolated from a febrile human, mosquitoes, and spiny rats. Both ID lineages and the IIID subtype are associated with febrile human illness. Most of the subtype ID isolates belonged to the Panama genotype, but the Colombia/Venezuela genotype, which is phylogenetically related to epizootic strains, also continues to circulate in the Amazon basin.

  14. [Meningitis and encephalitis in Poland in 2007].

    Science.gov (United States)

    Lankiewicz, Aleksandra; Polkowska, Aleksandra; Chrześcijańska, Irena; Kicman-Gawłowska, Agnieszka; Stefanoff, Paweł

    2009-01-01

    In Poland, 3,361 cases of neuroinfections were reported in 2007, of which 1,078 had bacterial etiology, 1,717--viral aetiology, and 566--other or unknown origin. The etiological agent was determined in 611 (57%) cases of bacterial neuroinfections. Among them N. meningitidis was found in 224 cases, H. influenzae type B (Hib) in 35 cases and S. pneumoniae in 161 cases. An increasing trend in meningococcal infections incidence has been observed in 2007, and a substantial decrease ofHib incidence, related to increasing vaccination coverage. Viral neuroinfections incidence in 2007 increased compared to year 2006. Among confirmed cases, there were 233 cases oftick-borne encephalitis. Most of the cases were reported from endemic areas of north-eastern part of the country.

  15. [Meningitis and encephalitis in Poland in 2009].

    Science.gov (United States)

    Polkowska, Aleksandra

    2011-01-01

    In Poland, 2 517 cases of neuroinfections were reported in 2009, of which 865 had bacterial aetiology, 1 244--viral aetiology, and 408-- other or unknown origin. The etiological agent was determined in 493 (57%) cases of bacterial neuroinfections. Among them Neisseria meningitidis was found in 190 cases, Haemophilus influenzae type B (Hib) in 13 cases and Streptococcus pneumoniae in 163 cases. An increasing trend in pneumococcal infections incidence has been observed since 2005, and a substantial decrease of Hib incidence, related to increasing vaccination coverage. Viral neuroinfections incidence in 2009 increased compared to year 2008. Among confirmed cases, there were 351 cases of tick-borne encephalitis. Most of the cases were reported from endemic areas of north-eastern part of the country.

  16. [Meningitis and encephalitis in Poland in 2008].

    Science.gov (United States)

    Turczyńska, Aleksandra; Polkowska, Aleksandra

    2010-01-01

    In Poland, 2 475 cases of neuroinfections were reported in 2008, of which 979 had bacterial aetiology, 1 122--viral aetiology, and 374--other or unknown origin. The etiological agent was determined in 555 (56%) cases of bacterial neuroinfections. Among them Neisseria meningitidis was found in 220 cases, Haemophilus influenzae type B (Hib) in 23 cases and Streptococcus pneumoniae in 151 cases. An increasing trend in meningococcal infections incidence has been observed in 2008, and a substantial decrease of Hib incidence, related to increasing vaccination coverage. Viral neuroinfections incidence in 2008 decreased compared to year 2007. Among confirmed cases, there were 202 cases of tick-borne encephalitis. Most of the cases were reported from endemic areas of north-eastern part of the country.

  17. Milrinone in Enterovirus 71 Brain Stem Encephalitis

    Directory of Open Access Journals (Sweden)

    SHIH-MIN eWANG

    2016-03-01

    Full Text Available Enterovirus 71 (EV71 was implicated in a widespread outbreak of hand-foot-and-mouth disease (HFMD across the Asia Pacific area since 1997 and has also been reported sporadically in patients with brain stem encephalitis. Neurogenic shock with pulmonary edema (PE is a fatal complication of EV71 infection. Among inotropic agents, milrinone is selected as a therapeutic agent for EV71- induced PE due to its immunopathogenesis. Milrinone is a type III phosphodiesterase inhibitor that has both inotropic and vasodilator effects. Its clinical efficacy has been shown by modulating inflammation, reducing sympathetic over-activity, and improving survival in patients with EV71-associated PE. Milrinone exhibits immunoregulatory and anti-inflammatory effects in the management of systemic inflammatory responses in severe EV71 infection.

  18. Development history of herpes simplex encephalitis

    Directory of Open Access Journals (Sweden)

    Jia-wei WANG

    2014-08-01

    Full Text Available Herpes simplex encephalitis (HSE is an acute central nervous system infection caused by herpes simplex virus (HSV. Early clinical manifestations mainly include fever, headache and unconsciousness; when progressing, psychiatric symptoms can occur. Death or serious neurological sequelae will happen if not treated. With the development of laboratory tests and imaging techniques, the early diagnosis of HSE is possible. Even though imaging with temporal lobe abnormal signal has the implication to HSE, the application of polymerase chain reaction (PCR in detecting HSV DNA in cerebrospinal fluid is currently the "gold standard" to diagnose HSE. Once diagnosed, acyclovir must be given as soon as possible, as delayed treatment will result in a poor outcome. doi: 10.3969/j.issn.1672-6731.2014.08.003

  19. Genetic variation of St. Louis encephalitis virus.

    Science.gov (United States)

    May, Fiona J; Li, Li; Zhang, Shuliu; Guzman, Hilda; Beasley, David W C; Tesh, Robert B; Higgs, Stephen; Raj, Pushker; Bueno, Rudy; Randle, Yvonne; Chandler, Laura; Barrett, Alan D T

    2008-08-01

    St. Louis encephalitis virus (SLEV) has been regularly isolated throughout the Americas since 1933. Previous phylogenetic studies involving 62 isolates have defined seven major lineages (I-VII), further divided into 14 clades. In this study, 28 strains isolated in Texas in 1991 and 2001-2003, and three older, previously unsequenced strains from Jamaica and California were sequenced over the envelope protein gene. The inclusion of these new sequences, and others published since 2001, has allowed better delineation of the previously published SLEV lineages, in particular the clades of lineage II. Phylogenetic analysis of 106 isolates identified 13 clades. All 1991 and 2001-2003 isolates from Nueces, Jefferson and Harris Counties (Texas Gulf Coast) group in clade IIB with other isolates from these counties isolated during the 1980s and 1990s. This lack of evidence for introduction of novel strains into the Texas Gulf Coast over a long period of time is consistent with overwintering of SLEV in this region. Two El Paso isolates, both from 2002, group in clade VA with recent Californian isolates from 1998-2001 and some South American strains with a broad temporal range. Overall, these data are consistent with multiple introductions of SLEV from South America into North America, and provide support for the hypothesis that in most situations, SLEV circulates within a locality, with occasional incursions from other areas. Finally, SLEV has much lower nucleotide (10.1 %) and amino acid variation (2.8 %) than other members of the Japanese encephalitis virus complex (maximum variation 24.6 % nucleotide and 11.8 % amino acid).

  20. receptores

    Directory of Open Access Journals (Sweden)

    Salete Regina Daronco Benetti

    2006-01-01

    Full Text Available Se trata de un estudio etnográfico, que tuvo lo objetivo de interpretar el sistema de conocimiento y del significado atribuidos a la sangre referente a la transfusión sanguínea por los donadores y receptores de un banco de sangre. Para la colecta de las informaciones se observaron los participantes y la entrevista etnográfica se realizó el análisis de dominio, taxonómicos y temáticos. Los dominios culturales fueron: la sangre es vida: fuente de vida y alimento valioso; creencias religiosas: fuentes simbólicas de apoyos; donación sanguínea: un gesto colaborador que exige cuidarse, gratifica y trae felicidad; donación sanguínea: fuente simbólica de inseguridad; estar enfermo es una condición para realizar transfusión sanguínea; transfusión sanguínea: esperanza de vida; Creencias populares: transfusión sanguínea como riesgo para la salud; donadores de sangre: personas benditas; donar y recibir sangre: como significado de felicidad. Temática: “líquido precioso que origina, sostiene, modifica la vida, provoca miedo e inseguridad”.

  1. [Acute encephalitis. Neuropsychiatric manifestations as expression of influenza virus infection].

    Science.gov (United States)

    Moreno-Flagge, Noris; Bayard, Vicente; Quirós, Evelia; Alonso, Tomás

    2009-01-01

    The aim is to review the encephalitis in infants and adolescents as well as its etiology, clinical manifestation, epidemiology, physiopathology, diagnostic methods and treatment, and the neuropsyquiatric signs appearing an influenza epidemy. Encephalitis is an inflammation of the central nervous system (CNS) which involves the brain. The clinical manifestations usually are: headache, fever and confusional stage. It could also be manifested as seizures, personality changes, or psiqyiatric symptoms. The clinical manifestations are related to the virus and the cell type affected in the brain. A meningitis or encephalopathy need to be ruled out. It could be present as an epidemic or isolated form, beeing this the most frequent form. It could be produced by a great variety of infections agents including virus, bacterias, fungal and parasitic. Viral causes are herpesvirus, arbovirus, rabies and enterovirus. Bacterias such as Borrelia burgdorferi, Rickettsia and Mycoplasma neumoniae. Some fungal causes are: Coccidioides immitis and Histoplasma capsulatum. More than 100 agents are related to encephalitis. The diagnosis of encephalitis is a challenge for the clinician and its infectious etiology is clear in only 40 to 70% of all cases. The diagnosis of encephalitis can be established with absolute certainty only by the microscopic examination of brain tissue. Epidemiology is related to age of the patients, geographic area, season, weather or the host immune system. Early intervention can reduce the mortality rate and sequels. We describe four patients with encephalitis and neuropsychiatric symptoms during an influenza epidemic.

  2. Autoantibodies to neuronal surface antigens in thyroid antibody-positive and -negative limbic encephalitis

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    Erdem Tuzun

    2011-01-01

    Full Text Available Background : Thyroid antibodies (Thy-Abs are frequently detected in various autoimmune disorders in coexistence with other systemic autoantibodies. In association with an encephalopathy, they are often taken as evidence of Hashimoto′s encephalitis (HE. However, the presence of Thy-Abs in a cohort of limbic encephalitis (LE patients and their association with anti-neuronal autoimmunity has not been explored. Patients and Methods : We investigated thyroid and anti-neuronal antibodies in the sera of 24 LE patients without identified tumors by cell-based assay and radioimmunoassay and evaluated their clinical features. Results : There was a female predominance in Thy-Ab-positive LE patients. Five of the eight Thy-Ab-positive patients and six of the 16 Thy-Ab-negative patients had antibodies to voltage-gated potassium channel (VGKC, N-methyl-D-aspartate receptor (NMDAR or undefined surface antigens on cultured hippocampal neurons. There were trends towards fewer VGKC antibodies (1/8 vs. 5/16, P = 0.159 and more NMDAR antibodies (2/8 vs. 1/16, P = 0.095 among the Thy-Ab-positive LE patients; antibodies to undefined surface antigens were only identified in Thy-Ab-positive patients (2/8 vs. 0/16, P = 0.018. There were no distinguishing clinical features between Thy-Ab-positive patients with and without neuronal antibodies. However, patients with anti-neuronal antibodies showed a better treatment response. Conclusion : Thy-Abs can be found in a high proportion of patients with non-paraneoplastic LE, often in association with antibodies to specific or as yet undefined neuronal surface antigens. These results suggest that acute idiopathic encephalitis patients with Thy-Abs should be closely monitored for ion-channel antibodies and it should not be assumed that they have HE.

  3. DMPD: Monocyte/macrophage traffic in HIV and SIV encephalitis. [Dynamic Macrophage Pathway CSML Database

    Lifescience Database Archive (English)

    Full Text Available 12960230 Monocyte/macrophage traffic in HIV and SIV encephalitis. Kim WK, Corey S, ...Show Monocyte/macrophage traffic in HIV and SIV encephalitis. PubmedID 12960230 Title Monocyte/macrophage traffic

  4. [Effects of blockade of ionotropic glutamate receptors on the development of pentylenetetrazole kindling in mice].

    Science.gov (United States)

    Lukomskaia, N Ia; Lavrent'eva, V V; Starshinova, L A; Zhabko, E P; Gorbunova, L V; Tikhonova, T B; Gmiro, V E; Magazanik, L G

    2005-11-01

    Effects of mono- and dicationic derivatives of adamantane and phenylcyclohexyl on the petyleneterazole-induced (35 mg/kg i. p.) kindling were studied in the experiments on mice. Monocationic derivative of phenylcyclohexyl IEM-1921, effectively retarded the development of kindling beginning the dose 0.0001 microM/kg. Memantine: derivative of adamantane (derivative of adamatane) produced the same effect with 100-fold increased dose. Dicationic derivative ofphenylcyclohexyl: IEM-1925, is able to block equally the open channels of both NMDA and subtype of Ca-permeable AMPA receptors. Its effect on kindling differed markedly from selective NMDA antagonists (IEM-1921 and memantine) in more complicated dose-dependence. The retardation of kindling IEM-1925 was induced at 0.001 microM/kg. On the contrary, a 10-time lower dose: 0.0001 microM/kg, facilitated the development of kindling. The observed difference in the activity of selective NMDA antagonists and the drugs combining anti-NMDA and anti-AMPA potency indicates that both types of ionotropic glutamate receptors are involved in the mechanism of petyleneterazole-induced kindling. The integral effect of channel blockade evoked by drugs seems to be dependent not only upon the ratio of the receptor types but on the kinetics of drug action, too.

  5. Development of a vaccine to prevent Japanese encephalitis: a brief review

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    Viroj Wiwanitkit

    2009-01-01

    Viroj WiwanitkitWiwanitkit House, Bangkhae, Bangkok, ThailandAbstract: Japanese encephalitis (ICD 10: A83.0) is an important specific viral encephalitis caused by the Japanese encephalitis virus, a virus of the Flavivirus group. Millions of people, especially those in endemic areas of developing countries in Asia, are at high risk from this infection. Therefore proper management to deal with this virus is essential. There is no specific treatment for Japanese encephalitis virus. Supportive an...

  6. Charles Bonnet syndrome after herpes simplex encephalitis.

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    Aydin, Ömer Faruk; Ince, Hülya; Taşdemir, Haydar Ali; Özyürek, Hamit

    2012-04-01

    Visual impairment associated with Charles Bonnet syndrome is rarely reported in childhood. We describe a child who presented with visual hallucinations and postinfectious bilateral retrobulbar optic neuritis. The patient had undergone acyclovir therapy for 3 weeks because of herpes encephalitis. Four days after therapy was completed, he experienced visual impairment in both eyes. He manifested a bilateral decrease in visual acuity, with normal funduscopic findings. The patient experienced visual hallucinations for about 1 week, and then experienced total loss of vision. During his hallucinations, the patient did not exhibit behavioral changes or cognitive impairment. The visual hallucinations included unfamiliar children hiding under his bed, and he spoke to someone whom he did not know. Magnetic resonance imaging indicated bilateral optic nerve hyperintensity on T(2)-weighted and contrast-enhanced images. The patient received corticosteroid therapy for his retrobulbar optic neuritis, and his vision returned to normal after 1 month. Although rare, visual impairment can be associated with complex visual hallucinations indicative of Charles Bonnet syndrome.

  7. Acute encephalitis syndrome following scrub typhus infection

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    Ayan Kar

    2014-01-01

    Full Text Available Objective: The aim was to find the incidence of acute encephalitis syndrome (AES secondary to scrub infection and to observe the clinical, biochemical, radiological profile, and outcomes in these patients. Materials and Methods: A total of 20 consecutive patients of AES were evaluated for scrub infection using scrub typhus immunoglobulin M enzyme linked immuno-sorbant assay positivity along with the presence or absence of an eschar. Clinical profile, routine laboratory tests, cerebrospinal fluid (CSF analysis, and neuroimaging were analyzed. Patients were treated with doxycycline and followed-up. Results: Among 20 consecutive patients with AES, 6 (30% were due to scrub infection. They presented with acute onset fever, altered sensorium, seizures. "Eschar" was seen in 50% of patients. CSF done in two of them was similar to consistent with viral meningitis. Magnetic resonance imaging brain revealed cerebral edema, bright lesions in the putamen and the thalamus on T2-weighted and fluid-attenuated inversion recovery sequences. Renal involvement was seen in all patients. All patients responded well to oral doxycycline. Conclusion: AES is not an uncommon neurological presentation following scrub typhus infection. It should be suspected in all patients with fever, altered sensorium, and renal involvement. Oral doxycycline should be started as early as possible for better outcomes.

  8. The Type I Interferon Response Determines Differences in Choroid Plexus Susceptibility between Newborns and Adults in Herpes Simplex Virus Encephalitis

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    Douglas R. Wilcox

    2016-04-01

    Full Text Available Newborns are significantly more susceptible to severe viral encephalitis than adults, with differences in the host response to infection implicated as a major factor. However, the specific host signaling pathways responsible for differences in susceptibility and neurologic morbidity have remained unknown. In a murine model of HSV encephalitis, we demonstrated that the choroid plexus (CP is susceptible to herpes simplex virus 1 (HSV-1 early in infection of the newborn but not the adult brain. We confirmed susceptibility of the CP to HSV infection in a human case of newborn HSV encephalitis. We investigated components of the type I interferon (IFN response in the murine brain that might account for differences in cell susceptibility and found that newborns have a dampened interferon response and significantly lower basal levels of the alpha/beta interferon (IFN-α/β receptor (IFNAR than do adults. To test the contribution of IFNAR to restricting infection from the CP, we infected IFNAR knockout (KO adult mice, which showed restored CP susceptibility to HSV-1 infection in the adult. Furthermore, reduced IFNAR levels did not account for differences we found in the basal levels of several other innate signaling proteins in the wild-type newborn and the adult, including protein kinase R (PKR, that suggested specific regulation of innate immunity in the developing brain. Viral targeting of the CP, a region of the brain that plays a critical role in neurodevelopment, provides a link between newborn susceptibility to HSV and long-term neurologic morbidity among survivors of newborn HSV encephalitis.

  9. Epidemiology of Japanese encephalitis in the Philippines: a systematic review.

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    Anna Lena Lopez

    2015-03-01

    Full Text Available Japanese encephalitis virus (JEV is an important cause of encephalitis in most of Asia, with high case fatality rates and often significant neurologic sequelae among survivors. The epidemiology of JE in the Philippines is not well defined. To support consideration of JE vaccine for introduction into the national schedule in the Philippines, we conducted a systematic literature review and summarized JE surveillance data from 2011 to 2014.We conducted searches on Japanese encephalitis and the Philippines in four databases and one library. Data from acute encephalitis syndrome (AES and JE surveillance and from the national reference laboratory from January 2011 to March 2014 were tabulated and mapped.We identified 29 published reports and presentations on JE in the Philippines, including 5 serologic surveys, 18 reports of clinical cases, and 8 animal studies (including two with both clinical cases and animal data. The 18 clinical studies reported 257 cases of laboratory-confirmed JE from 1972 to 2013. JE virus (JEV was the causative agent in 7% to 18% of cases of clinical meningitis and encephalitis combined, and 16% to 40% of clinical encephalitis cases. JE predominantly affected children under 15 years of age and 6% to 7% of cases resulted in death. Surveillance data from January 2011 to March 2014 identified 73 (15% laboratory-confirmed JE cases out of 497 cases tested.This comprehensive review demonstrates the endemicity and extensive geographic range of JE in the Philippines, and supports the use of JE vaccine in the country. Continued and improved surveillance with laboratory confirmation is needed to systematically quantify the burden of JE, to provide information that can guide prioritization of high risk areas in the country and determination of appropriate age and schedule of vaccine introduction, and to measure the impact of preventive measures including immunization against this important public health threat.

  10. Roseolovirus-associated encephalitis in immunocompetent and immunocompromised individuals.

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    Ongrádi, Joseph; Ablashi, Dharam V; Yoshikawa, Tetsushi; Stercz, Balázs; Ogata, Masao

    2017-02-01

    The roseoloviruses, human herpesvirus (HHV)-6A, HHV-6B, and HHV-7, can cause severe encephalitis or encephalopathy. In immunocompetent children, primary HHV-6B infection is occasionally accompanied by diverse clinical forms of encephalitis. Roseolovirus coinfections with heterologous viruses and delayed primary HHV-7 infection in immunocompetent adults result in very severe neurological and generalized symptoms. Recovery from neurological sequelae is slow and sometimes incomplete. In immunocompromised patients with underlying hematological malignancies and transplantation, frequent single or simultaneous reactivation of roseoloviruses elicit severe, lethal organ dysfunctions, including damages in the limbic system, brain stem, and hippocampus. Most cases have been due to HHV-6B with HHV-6A accounting for 2-3%. The most severe manifestation of HHV-6B reactivation is post-transplantation limbic encephalitis. Seizures, cognitive problems, and abnormal EEG are common. Major risk factors for HHV-6B-associated encephalitis include unrelated cord blood cell transplantation and repeated hematopoietic stem cell transplantation. Rare genetic disorders, male gender, certain HLA constellation, and immune tolerance to replicating HHV-6 in persons carrying chromosomally integrated HHV-6 might also predispose an individual to roseolovirus-associated brain damage. At this time, little is known about the risk factors for HHV-7-associated encephalitis. Intrathecal glial cell destruction due to virus replication, overexpression of proinflammatory cytokines, and viral mimicry of chemokines all contribute to brain dysfunction. High virus load in the cerebrospinal fluid, hippocampal astrogliosis, and viral protein expression in HHV-6B-associated cases and multiple microscopic neuronal degeneration in HHV-7-associated cases are typical laboratory findings. Early empirical therapy with ganciclovir or foscarnet might save the life of a patient with roseolovirus-associated encephalitis.

  11. Anterior opercular syndrome induced by Epstein-Barr virus encephalitis.

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    Matsushima, Takashi; Nishioka, Kenya; Tanaka, Ryota; Yokoyama, Kazumasa; Hattori, Nobutaka

    2016-01-01

    We report a 19-year-old female presenting with fever, drooling, anarthria, and voluntary facial movement disruption, characteristic of anterior opercular syndrome (AOS). Serological examination revealed Epstein-Barr virus (EBV) infection following acute encephalitis with severe ataxia. A single-photon emission computerized tomography (SPECT) examination indicated hypoperfusion in the left perisylvian region, bilateral thalamus, occipital lobe, and cerebellum. This is the first report of AOS related to EBV encephalitis. SPECT was a useful method for detecting the damaged region of the operculum. In addition, AOS is a clinically distinct entity that may help us understand the mechanisms of language circuits within the operculum.

  12. Toxoplasmic encephalitis associated with meningitis in a heart transplant recipient.

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    Baliu, C; Sanclemente, G; Cardona, M; Castel, M A; Perez-Villa, F; Moreno, A; Cervera, C

    2014-08-01

    Toxoplasma gondii is an opportunistic pathogen that causes neurologic and extraneurologic manifestations in immunosuppressed patients. Encephalitis and intracranial mass lesions are easily recognized as typical manifestations of toxoplasmosis. However, meningitis caused by T. gondii is a rare condition with very few cases described in the literature. We present the case of a heart transplant recipient who developed toxoplasmic encephalitis associated with meningitis. After an extensive review of the medical literature, we found only 1 case of meningitis in solid organ transplant recipients and meningitis in immunocompromised individuals.

  13. Encephalitis with Prolonged but Reversible Splenial Lesion

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    Alena Meleková

    2015-12-01

    Full Text Available Introduction: The splenium of the corpus callosum has a specific structure of blood supply with a tendency towards blood-brain barrier breakdown, intramyelinic edema, and damage due to hypoxia or toxins. Signs and symptoms of reversible syndrome of the splenium of the corpus callosum typically include disorientation, confusion, impaired consciousness, and epileptic seizures. Case report: A previously healthy 32-year-old man suffered from weakness, headache, and fever. Subsequently, he developed apathy, ataxia, and inability to walk, and therefore was admitted to the hospital. Cerebrospinal fluid showed protein elevation (0.9 g/l and pleocytosis (232/1 ul. A brain MRI showed hyperintense lesions in the middle of the corpus callosum. The patient was treated with antibiotics, and subsequently, in combination with steroids. Two months later, the hyperintense lesions in the splenium and the basal ganglia had disappeared. Almost seven months since his hospitalization in the Department of Neurology, the patient has returned to his previous employment. He now does not exhibit any mental changes, an optic edema and urological problems have improved. In addition, he is now actively engaged in sports. Conclusion: We have described a case of a 32-year-old man with confusion, ataxia, and inability to stand and walk. The man developed a febrile meningeal syndrome and a hyperintense lesion of the splenium, which lasted for two months. Neurological changes, optic nerve edema, and urinary retention have resolved over the course of seven months. We think that the prolonged but transient lesion of the splenium may have been caused by encephalitis of viral origin.

  14. Herpes simplex encephalitis and subsequent cytomegalovirus encephalitis after chemoradiotherapy for central nervous system lymphoma: a case report and literature review.

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    Suzuki, Hiroshi I; Hangaishi, Akira; Hosoya, Noriko; Watanabe, Takuro; Kanda, Yoshinobu; Motokura, Toru; Chiba, Shigeru; Kurokawa, Mineo

    2008-06-01

    Neurological complications during the treatment of hematological malignancies have a wide range of causes. Treatment-related leukoencephalopathy has been recognized as a major complication of combined chemotherapy and radiotherapy for central nervous system (CNS) lymphoma, and can complicate the diagnosis of CNS infection. Herein, we present a patient with diffuse large B-cell lymphoma who developed herpes simplex encephalitis (HSE) and subsequent cytomegalovirus encephalitis after chemoradiotherapy for CNS relapse. Although cerebrospinal fluid examination (CSF) showed no significant pleocytosis, brain magnetic resonance imaging and polymerase chain reaction analysis of the CSF were useful in the diagnosis. With a review of the literature on the association between HSE and radiotherapy for CNS malignancies, our case suggests that an awareness of viral encephalitis is important in the differential diagnosis of acute neurologic disturbance during chemoradiotherapy for CNS lymphoma.

  15. Comparative spatial dynamics of Japanese encephalitis and acute encephalitis syndrome in Nepal.

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    Colin Robertson

    Full Text Available Japanese Encephalitis (JE is a vector-borne disease of major importance in Asia. Recent increases in cases have spawned the development of more stringent JE surveillance. Due to the difficulty of making a clinical diagnosis, increased tracking of common symptoms associated with JE-generally classified as the umbrella term, acute encephalitis syndrome (AES has been developed in many countries. In Nepal, there is some debate as to what AES cases are, and how JE risk factors relate to AES risk. Three parts of this analysis included investigating the temporal pattern of cases, examining the age and vaccination status patterns among AES surveillance data, and then focusing on spatial patterns of risk factors. AES and JE cases from 2007-2011 reported at a district level (n = 75 were examined in relation to landscape risk factors. Landscape pattern indices were used to quantify landscape patterns associated with JE risk. The relative spatial distribution of landscape risk factors were compared using geographically weighted regression. Pattern indices describing the amount of irrigated land edge density and the degree of landscape mixing for irrigated areas were positively associated with JE and AES, while fragmented forest measured by the number of forest patches were negatively associated with AES and JE. For both JE and AES, the local GWR models outperformed global models, indicating spatial heterogeneity in risks. Temporally, the patterns of JE and AES risk were almost identical; suggesting the relative higher caseload of AES compared to JE could provide a valuable early-warning signal for JE surveillance and reduce diagnostic testing costs. Overall, the landscape variables associated with a high degree of landscape mixing and small scale irrigated agriculture were positively linked to JE and AES risk, highlighting the importance of integrating land management policies, disease prevention strategies and promoting healthy sustainable livelihoods

  16. Postencephalitic epilepsy and drug-resistant epilepsy after infectious and antibody-associated encephalitis in childhood: Clinical and etiologic risk factors.

    Science.gov (United States)

    Pillai, Sekhar C; Mohammad, Shekeeb S; Hacohen, Yael; Tantsis, Esther; Prelog, Kristina; Barnes, Elizabeth H; Gill, Deepak; Lim, Ming J; Brilot, Fabienne; Vincent, Angela; Dale, Russell C

    2016-01-01

    To define the risk factors for postencephalitic epilepsy (PE) and drug-resistant epilepsy (DRE) in childhood following infectious and autoimmune encephalitis, we included 147 acute encephalitis patients with a median follow-up of 7.3 years (range 2-15.8 years). PE was defined as the use of antiepileptic drugs (AEDs) for ≥24 months, and DRE was defined as the persistence of seizures despite ≥2 appropriate AEDs at final follow-up. PE and DRE were diagnosed in 31 (21%) and 15 (10%) of patients, respectively. The features during acute encephalitis predictive of DRE (presented as odds ratio [OR] with confidence intervals [CIs]) were status epilepticus (OR 10.8, CI 3.4-34.3), visual disturbance (6.4, 1.4-29.9), focal seizures (6.2, 1.9-20.6), magnetic resonance imaging (MRI) hippocampal/amygdala involvement (5.0, 1.7-15.4), intensive care admission (4.7, 1.4-15.4), use of >3 AEDs (4.5, 1.2-16.1), MRI gadolinium enhancement (4.1, 1.2-14.2), any seizure (3.9, 1.1-14.4), and electroencephalography (EEG) epileptiform discharges (3.9, 1.3-12.0). On multivariable regression analysis, only status epilepticus remained predictive of DRE in all models. DRE was common in herpes simplex virus (3/9, 33%) and unknown (8/40, 20%) encephalitis, but absent in acute disseminated encephalomyelitis (ADEM) (0/32, 0%), enterovirus (0/18), and anti-N-methyl-d-aspartate receptor-NMDAR encephalitis (0/9). We have identified risk factors for DRE and demonstrated "high-risk," and "low-risk" etiologies.

  17. Concurrent infection of Japanese encephalitis and mixed plasmodium infection

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    Girish Chandra Bhatt

    2012-01-01

    Full Text Available Japanese encephalitis (JE and malaria would coexist in the areas where both illnesses are endemic with overlapping clinical pictures, especially in a case of febrile encephalopathy with hepatosplenomegaly. However, there are no published data till date showing concurrent infection of these two agents despite both diseases being coendemic in many areas. We report a case of concurrent infection of JE and mixed plasmodium infection, where the case, initially diagnosed as cerebral malaria did not improve on antimalarials and alternative diagnosis of JEV encephalitis was thought which was confirmed by a serological test. To the best of our knowledge, this is the first case report of concurrent Japanese encephalitis with mixed plasmodium infection. We report a case of 3-year-old male child, who presented with febrile encephalopathy with hepatosplenomegaly. Based on a rapid diagnostic test and peripheral smear examination, a diagnosis of mixed P.Vivax and P.falciparum infection was made and the patient was treated with quinine and doxycycline. However, besides giving antimalarials the patient did not improve and an alternative diagnosis of JE was considered as the patient was from the endemic zone of Japanese encephalitis. Cerebrospinal fluid (CSF of the patient was sent for a virological study which came out to be positive for JE IgM in CSF, which is confirmatory of JE infection. In a patient with febrile encephalopathy with hepatosplenomegaly especially in areas coendemic for JE and malaria, the possibility of mixed infection should be kept in mind.

  18. Computed tomography in young children with herpes simplex virus encephalitis

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    Sugimoto, T.; Woo, M.; Okazaki, H.; Nishida, N.; Hara, T.; Yasuhara, A.; Kasahara, M.; Kobayashi, Y.

    1985-09-01

    Computed tomographic (CT) scans were obtained from eight infants and young children with herpes simplex virus encephalitis. In two cases the initial scan showed diffuse edematous changes as a mass effect without laterality. Unilateral localized low attenuation in the initial scan was evident 4 days after the onset in one patient, and high attenuation in the initial scan appeared on the 6th day in another patient, but in general, it was not possible to establish an early diagnosis of herpes simplex virus encephalitis from CT scan. In the longitudinal study the calcification with ventriculomegaly appeared in 3 of 5 survivors, and gyriform calcification in 2 of 3 patients, respectively. The appearance of multicystic encephalomalacia was evident in one patient 6 months after the onset of neonatal herpes simplex encephalitis. It is shown that the CT findings of neonates and young children with herpes simplex encephalitis are different from those of older children and adults, and the importance of longitudinal CT studies was stressed in clarifying the pathophysiology of the central nervous system involvement in survivors.

  19. Fulminant encephalitis associated with a vaccine strain of rubella virus.

    Science.gov (United States)

    Gualberto, Felipe Augusto Souza; de Oliveira, Maria Isabel; Alves, Venancio A F; Kanamura, Cristina T; Rosemberg, Sérgio; Sato, Helena Keico; Arantes, Benedito A F; Curti, Suely Pires; Figueiredo, Cristina Adelaide

    2013-12-01

    Involvement of the central nervous system is common in measles, but rare in rubella. However, rubella virus (RV) can cause a variety of central nervous system syndromes, including meningitis, encephalitis, Guillain-Barré syndrome and sub acute sclerosing panencephalitis. We report the occurrence of one fatal case of the encephalitis associated with measles-rubella (MR) vaccine during an immunization campaign in São Paulo, Brazil. A 31 year-old-man, previously in good health, was admitted at emergency room, with confusion, agitation, inability to stand and hold his head up. Ten days prior to admission, he was vaccinated with combined MR vaccine (Serum Institute of India) and three days later he developed 'flu-like' illness with fever, myalgia and headache. Results of clinical and laboratory exams were consistent with a pattern of viral encephalitis. During hospitalization, his condition deteriorated rapidly with tetraplegia and progression to coma. On the 3rd day of hospitalization he died. Histopathology confirmed encephalitis and immunohistochemistry was positive for RV on brain tissue. RV was also detected by qPCR and virus isolation in cerebrospinal fluid, brain and other clinical samples. The sequence obtained from the isolated virus was identical to that of the RA 27/3 vaccine strain.

  20. Acute measles encephalitis in partially vaccinated adults.

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    Annette Fox

    Full Text Available BACKGROUND: The pathogenesis of acute measles encephalitis (AME is poorly understood. Treatment with immune-modulators is based on theories that post-infectious autoimmune responses cause demyelination. The clinical course and immunological parameters of AME were examined during an outbreak in Vietnam. METHODS AND FINDINGS: Fifteen measles IgM-positive patients with confusion or Glasgow Coma Scale (GCS score below 13, and thirteen with uncomplicated measles were enrolled from 2008-2010. Standardized clinical exams were performed and blood collected for lymphocyte and measles- and auto-antibody analysis. The median age of AME patients was 21 years, similar to controls. Eleven reported receiving measles vaccination when aged one year. Confusion developed a median of 4 days after rash. Six patients had GCS <8 and four required mechanical ventilation. CSF showed pleocytosis (64% and proteinorrhachia (71% but measles virus RNA was not detected. MRI revealed bilateral lesions in the cerebellum and brain stem in some patients. Most received dexamethasone +/- IVIG within 4 days of admission but symptoms persisted for ≥3 weeks in five. The concentration of voltage gated calcium channel-complex-reactive antibodies was 900 pM in one patient, and declined to 609 pM ∼ 3 months later. Measles-reactive IgG antibody avidity was high in AME patients born after vaccine coverage exceeded 50% (∼ 25 years earlier. AME patients had low CD4 (218/µl, p = 0.029 and CD8 (200/µl, p = 0.012 T-cell counts compared to controls. CONCLUSION: Young adults presenting with AME in Vietnam reported a history of one prior measles immunization, and those aged <25 years had high measles-reactive IgG avidity indicative of prior vaccination. This suggests that one-dose measles immunization is not sufficient to prevent AME in young adults and reinforces the importance of maintaining high coverage with a two-dose measles immunization schedule. Treatment with

  1. Epstein-Barr Virus Encephalitis: A Case Report

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    Somayh HASHEMIAN

    2015-01-01

    Full Text Available How to Cite This Article: Hashemian S, Ashrafzadeh F, Akhondian J, Beiraghi Toosi M. Epstein-Barr Virus Encephalitis: A Case Report. Iran J Child Neurol. 2015 Winter;9(1:107-110.  Abstract Many neurologic manifestations of Epstein-Barr virus (EBV infection have been documented, including encephalitis, aseptic meningitis, transverse myelitis, and Guillain-Barre syndrome. These manifestations can occur alone or coincidentally with the clinical picture of infectious mononucleosis. EBV encephalitis is rare and is indicated as a wide range of clinical manifestations. We report a 10-year-old girl presented with fever, gait disturbance, and bizarre behavior for one week. The results of the physical examination were unremarkable. The diagnosis of EBV encephalitis was made by changes in titers of EBV specific antibodies and MRI findings. A cranial MRI demonstrated abnormal high signal intensities in the basal ganglia and the striatal body, especially in the putamen and caudate nucleus. EBV infection should be considered when lesions are localized to the basal ganglia.ReferencesFujimoto H, Asaoka K, Imiazumi T, Ayabe M, Shoji H, Kaji M. Epstein-Barr virus Infections of the Central Nervous System. Intern Med 2003; 42:33-40.Mathew AG, Parvez Y. Fulminant Epstein Barr virus encephalitis. Indian Pediatrics 2013; 50:418-419Kalita J, Maurya PK, Kumar B, Misra UK. Epstein Barr virus encephalitis: Clinical diversity and radiological similarity. Neurol India 2011; 59:605-7Baskin HJ, Hedlund G. Neuroimaging of Herpes Virus Infections in Children. Pediatr Radiol 2007; 37:949-63.Weinberg A, Li SH, Palmer M, Tyler K .Quantitative CSF PCR in Epstein-Barr Virus Infections of the Central Nervous System. Ann Neurol 2002; 52:543-8.Ono J, Shimizu K, Harada k, Mano T, Okada S. Characteristic MR Features of Encephalitis Caused by Epstein-Barr virus. Pediatr Radiol 1998; 28:569-70.Hausler M, Raamaekers T, Doenges M, Shweizer K ,Ritter K. Neurological Complications of Acute

  2. Effect of combinations of antiviral drugs on herpes simplex encephalitis

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    Bryan M Gebhardt

    2009-12-01

    Full Text Available Bryan M Gebhardt1, Federico Focher2, Richard Eberle3, Andrzej Manikowski4, George E Wright41LSU Eye Center, Department of Ophthalmology, Louisiana State University Health Sciences Center, New Orleans, LA, USA; 2Istituto di Genetica Molecolare, Consiglio Nazionale delle Ricerche, Pavia, Italy; 3Department of Veterinary Pathobiology, Center for Veterinary Health Sciences, Oklahoma State University, Stillwater, OK, USA; 4GLSynthesis Inc., Worcester, MA, USAAbstract: 2-Phenylamino-6-oxo-9-(4-hydroxybutylpurine (HBPG is a thymidine kinase inhibitor that prevents encephalitic death in mice caused by herpes simplex virus (HSV types 1 and 2, although its potency is somewhat less than that of acyclovir (ACV. The present study was undertaken to determine the effect of combinations of HBPG and either ACV, phosphonoformate (PFA, or cidofovir (CDF against HSV encephalitis. BALB/c mice were given ocular infections with HSV-1 or HSV-2, and treated twice daily intraperitoneally for five days with HBPG, alone or in combination with ACV, PFA, or CDF. Animals were observed daily for up to 30 days, and the day of death of each was recorded. All of the combinations showed additivity, and the combination of HBPG + ACV appeared to be synergistic, ie, protected more mice against HSV-1 encephalitis compared with each drug given alone. Delay of treatment with HBPG for up to two days was still effective in preventing HSV-2 encephalitis. The combination of the thymidine kinase inhibitor HBPG and the antiherpes drug ACV may have synergistic activity against HSV encephalitis. The development of a potent and safe combination therapy for the prevention and/or treatment of HSV infection of the central nervous system can improve the outcome of this infection in humans.Keywords: antivirals, herpetic encephalitis

  3. Analysis of behavior and trafficking of dendritic cells within the brain during toxoplasmic encephalitis.

    Science.gov (United States)

    John, Beena; Ricart, Brendon; Tait Wojno, Elia D; Harris, Tajie H; Randall, Louise M; Christian, David A; Gregg, Beth; De Almeida, Daniel Manzoni; Weninger, Wolfgang; Hammer, Daniel A; Hunter, Christopher A

    2011-09-01

    Under normal conditions the immune system has limited access to the brain; however, during toxoplasmic encephalitis (TE), large numbers of T cells and APCs accumulate within this site. A combination of real time imaging, transgenic reporter mice, and recombinant parasites allowed a comprehensive analysis of CD11c+ cells during TE. These studies reveal that the CNS CD11c+ cells consist of a mixture of microglia and dendritic cells (DCs) with distinct behavior associated with their ability to interact with parasites or effector T cells. The CNS DCs upregulated several chemokine receptors during TE, but none of these individual receptors tested was required for migration of DCs into the brain. However, this process was pertussis toxin sensitive and dependent on the integrin LFA-1, suggesting that the synergistic effect of signaling through multiple chemokine receptors, possibly leading to changes in the affinity of LFA-1, is involved in the recruitment/retention of DCs to the CNS and thus provides new insights into how the immune system accesses this unique site.

  4. Influence of the CCR-5/MIP-1 α axis in the pathogenesis of Rocio virus encephalitis in a mouse model.

    Science.gov (United States)

    Chávez, Juliana H; França, Rafael F O; Oliveira, Carlo J F; de Aquino, Maria T P; Farias, Kleber J S; Machado, Paula R L; de Oliveira, Thelma F M; Yokosawa, Jonny; Soares, Edson G; da Silva, João S; da Fonseca, Benedito A L; Figueiredo, Luiz T M

    2013-11-01

    Rocio virus (ROCV) caused an outbreak of human encephalitis during the 1970s in Brazil and its immunopathogenesis remains poorly understood. CC-chemokine receptor 5 (CCR5) is a chemokine receptor that binds to macrophage inflammatory protein (MIP-1 α). Both molecules are associated with inflammatory cells migration during infections. In this study, we demonstrated the importance of the CCR5 and MIP-1 α, in the outcome of viral encephalitis of ROCV-infected mice. CCR5 and MIP-1 α knockout mice survived longer than wild-type (WT) ROCV-infected animals. In addition, knockout mice had reduced inflammation in the brain. Assessment of brain viral load showed mice virus detection five days post-infection in wild-type and CCR5-/- mice, while MIP-1 α-/- mice had lower viral loads seven days post-infection. Knockout mice required a higher lethal dose than wild-type mice as well. The CCR5/MIP-1 α axis may contribute to migration of infected cells to the brain and consequently affect the pathogenesis during ROCV infection.

  5. Encephalitis due to antibodies to voltage gated potassium channel (VGKC with cerebellar involvement in a teenager

    Directory of Open Access Journals (Sweden)

    Megan M Langille

    2015-01-01

    Full Text Available Encephalitis due to antibodies to voltage gated potassium channel (VGKC typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. We describe a case of 13 year girl female with encephalitis due to antibodies to VGKC with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. These have never been reported previously in the literature. Our case expands the phenotypic spectrum of this rare condition.

  6. Encephalitis due to antibodies to voltage gated potassium channel (VGKC) with cerebellar involvement in a teenager.

    Science.gov (United States)

    Langille, Megan M; Desai, Jay

    2015-01-01

    Encephalitis due to antibodies to voltage gated potassium channel (VGKC) typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. We describe a case of 13 year girl female with encephalitis due to antibodies to VGKC with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. These have never been reported previously in the literature. Our case expands the phenotypic spectrum of this rare condition.

  7. Gene Knockdown of Venezuelan Equine Encephalitis Virus E2 Glycoprotein Using DNA-Directed RNA Interference

    Science.gov (United States)

    2006-12-01

    e _s~u~m mary - Introduction: Alphaviruses are a large family of RNA viruses that can cause acute infection resulting in arthritis and encephalitis...One of the important alphaviruses is the Venezuelan equine encephalitis virus. This virus has been linked to a number of outbreaks in both North and... replication of VEE virus in vitro. Bhogal, H.S., McLaws, L.J., and Jager, S.J. 2006. Gene Knockdown of Venezuelan Equine Encephalitis Virus E2

  8. Identification of linear human B-cell epitopes of tick-borne encephalitis virus

    OpenAIRE

    Kuivanen, Suvi; Hepojoki, Jussi; Vene, Sirkka; Vaheri, Antti; Vapalahti, Olli

    2014-01-01

    Background Tick-borne encephalitis (TBE) is a central nervous system infection transmitted to humans by ticks. The causative agent, tick-borne encephalitis virus (TBEV), belongs to the genus Flavivirus (family Flaviviridae), which includes globally important arthropod-borne viruses, such as dengue, Yellow fever, Japanese encephalitis and West Nile viruses. Flaviviruses are highly cross-reactive in serological tests that are currently based on viral envelope proteins. The envelope (E) protein ...

  9. Isolation and characterization of tick-borne encephalitis virus from Ixodes persulcatus in Mongolia in 2012

    OpenAIRE

    Muto, Memi; Bazartseren, Boldbaatar; Tsevel, Bazartseren; Dashzevge, Erdenechimeg; Yoshii, Kentaro; KARIWA, Hiroaki

    2015-01-01

    Tick-borne encephalitis virus (TBEV) is a zoonotic virus belonging to the genus Flavivirus, in the family Flaviviridae. The virus, which is endemic in Europe and northern parts of Asia, causes severe encephalitis. Tick-borne encephalitis (TBE) has been reported in Mongolia since the 1980s, but details about the biological characteristics of the endemic virus are lacking. In this study, 680 ticks (Ixodes persulcatus) were collected in Selenge aimag, northern Mongolia, in 2012. Nine Mongolian T...

  10. Effect of Antiepileptic Drugs for Acute and Chronic Seizures in Children with Encephalitis

    OpenAIRE

    Kuang-Lin Lin; Jainn-Jim Lin; Shao-Hsuan Hsia; Min-Liang Chou; Po-Cheng Hung; Huei-Shyong Wang

    2015-01-01

    Background Encephalitis presents with seizures in the acute phase and increases the risk of late unprovoked seizures and epilepsy. This study aimed to evaluate the effect of antiepileptic drugs in pediatric patients with acute seizures due to encephalitis and epilepsy. Patients and Methods Cases of acute pediatric encephalitis between January 2000 and December 2010 were reviewed. Clinical data, including onset at age, seizure type, seizure frequency, effects of antiepileptic drugs, and progno...

  11. Acanthamoeba encephalitis: A Case Report and Review of Therapy

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    A Zamora

    2014-01-01

    Full Text Available Background: Acanthamoeba is a rare cause of encephalitis yet is associated with high mortality. Treatment protocols vary greatly and generally include combination therapy across a wide spectrum of antiinfective classes. Case Description: A 63-year-old male who underwent renal transplantation presented 6 months after transplantation with depressed level of consciousness. Imaging of the head with computerized tomography showed an enhancing lesion suspicious for brain abscess. Biopsy of the lesion showed Acanthamoeba cysts. The patient was treated with sulfadiazine, fluconazole, flucytosine, azithromycin, and miltefosine but without success. We review recently published cases of Acanthamoeba encephalitis with an emphasis on treatment protocols and outcomes. Conclusion: Free-living protozoans such as Acanthamoeba are ubiquitous in the environment and should be suspected in immunosuppressed persons who present with central nervous system findings and brain abscess. Biopsy is critical to establish the etiology so that appropriate combination therapy can be deployed.

  12. Hypothermia in VGKC antibody-associated limbic encephalitis.

    Science.gov (United States)

    Jacob, S; Irani, S R; Rajabally, Y A; Grubneac, A; Walters, R J; Yazaki, M; Clover, L; Vincent, A

    2008-02-01

    Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders. Limbic encephalitis is typically characterised by a sub-acute onset of disorientation, amnesia and seizures, but the clinical spectrum is not yet fully defined and the syndrome could be under-diagnosed. We here describe the clinical profile of four patients with VGKC-Ab-associated LE who had intermittent, episodic hypothermia. One of the patients also described a prodrome of severe neuropathic pain preceding the development of limbic symptoms. Both of these novel symptoms responded well to immunosuppressive therapy, with concurrent amelioration of amnesia/seizures.

  13. Molecular mechanisms of neuroinflammation and injury during acute viral encephalitis.

    Science.gov (United States)

    Shives, Katherine D; Tyler, Kenneth L; Beckham, J David

    2017-03-11

    Viral infections in the central nervous system are a major cause of encephalitis. West Nile virus (WNV) and Herpes simplex virus (HSV) are the most common causes of viral encephalitis in the United States. We review the role of neuroinflammation in the pathogenesis of WNV and HSV infections in the central nervous system (CNS). We discuss the role of the innate and cell-mediated immune responses in peripheral control of viral infection, viral invasion of the CNS, and in inflammatory-mediated neuronal injury. By understanding the role of specific inflammatory responses to viral infections in the CNS, targeted therapeutic approaches can be developed to maximize control of acute viral infection while minimizing neuronal injury in the CNS.

  14. Case of herpes simplex encephalitis (HSE) with characteristic CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Imamura, Shigehiro (Kumamoto Rosai Hospital (Japan)); Nakayama, Toshio; Yamanaga, Hiroaki; Nakanishi, Ryoji; Ideta, Tooru

    1984-01-01

    CT scans of a 59-year-old woman, with serologically comfirmed herpes simplex encephalitis (HSE) were studied sequentially from 9 days after onset. The initial findings in CT scan were low density areas in insular cortex, claustrum and hippocampus. The low density areas, then, spread to the temporal lobe, rectal and cingulate gyri and occipital lobe, according to clinical progression of the disease. However, these low density areas disappeared and changed into isodensity areas in 25-35 days after oneset, which then returned to low density areas again in 51 days after onset. Thes characteristic phenomenon resembled a ''fogging effect,'' which is frequently seen during the second or third week after onset of ischemic cerebral infarction. HSE is characterized pathologically by acute hemorrhagic necrotizing encephalitis. Though cerebral angiography was not performed in this case, these characteristic CT findings suggested that HSE may have been associated with vascular involvement.

  15. Neuropathology of S. Paulo south coast epidemic encephalitis (Rocio flavivurus).

    Science.gov (United States)

    Rosemberg, S

    1980-02-01

    The neuropathology of 8 cases of S. Paulo south coast epidemic encephalitis (Rocio flavivirus), a new arbo B virus encephalitis, is described. The topographic pattern of the lesions appears to be almost specific. The gray matter is predominantly affected. Interstitial mononuclear infiltration, microglial proliferation and perivascular lymphocytic cuffing were seen. Neuronophagia was seldom seen except during the acute phases of the disease. Throughout the neuraxis, the gray matter was affected to a greater degree than white matter. The more damaged structures, in descending order, were as follows: thalamus, dentate nucleus, substantia inominata, brain stem, spinal cord and basal nuclei. Most of the cases exhibited thalamic inflammatory necrosis. Electron microscopy disclosed in one case virus-like particles, resembling those described in other arbo B viruses in the cytoplasm of thalamic neurons. In this case, virus was isolated from the brain and an immunofluorescence test also showed antigenic material in the thalamic neurons.

  16. Gerstmann's syndrome following an acute herpes simplex encephalitis.

    Science.gov (United States)

    Ilchevsky, S; Boev, I; Kazakova, T

    1998-01-01

    The authors present a rare clinical case of a woman who developed Gerstmann's syndrome following an acute Herpes simplex viral encephalitis. Clinical observation and laboratory evaluation were performed during the acute phase of the disease. After that the follow-up continued for one-year period. The localization of the pathologic process was determined by computerized tomography, conducted periodically. The characteristics of the clinical picture are interpreted in the context of the contemporary concepts of the topical diagnosis of Gerstmann's syndrome. The possibility of a sudden onset of acute Herpes simplex viral encephalitis without a preceding febrile-intoxication syndrome is worth noting. Conclusions are drawn stressing the need of an early etiologic treatment and the importance of the rehabilitation activities during the convalescence period.

  17. Anti-NMDAR-encephalitis in children%儿童抗N-甲基-D-天冬氨酸受体抗体脑炎

    Institute of Scientific and Technical Information of China (English)

    乔娜娜

    2013-01-01

    儿童抗N-甲基-D-天冬氨酸受体抗体脑炎(anti-N-methyl-D-aspartate receptor encephalitis,NMDAR)是一种抗NMDA受体抗体相关性自身免疫性脑炎.该病发病、临床表现具有儿童其自身特点.成年患者多与肿瘤相关,患儿肿瘤相关性低,多与感染、遗传因素有关.其发病机制主要是抗MDAR抗体选择性、可逆性的减少神经元突触后膜表面的NMDAR密度.临床主要表现为精神症状、语言障碍、运动障碍、睡眠障碍及自主神经功能紊乱等.治疗以肿瘤切除和免疫治疗为主.该病儿童病例报道少,易误诊,部分患儿病情重,易留下后遗症,故早期诊断、治疗具有重要意义.该文就儿童抗NMDAR脑炎发病机制、临床表现及诊断治疗作一综述.%Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDAR-encephalitis) in children is a type of autoimmune encephalitis associated with NMDA receptor.Its etiology and clinical manifestations are specific in children.This disease is associated with teratoma more in adult than in children.However,its etiology is more associated with infection and heredity in children.The pathogenesis is that the anti-NMDAR-antibody can combine with NMDAR of neurons selectively and reversibly.The clinical manifestations include psychiatric syndrome,language,motor,sleep and autonomic nervous disorders.Tumor resection and immtmotherapy are chief treatments.This disorder is often misdiagnosed as it is rarely reported in children.It is valuable to make early diagnosis and treatment.This paper reviews the mechanism,clinical features,diagnosis and treatment of antiNMDAR-encephalitis of children.

  18. West Nile Virus Encephalitis: The First Human Case Recorded in Brazil

    Science.gov (United States)

    Vieira, Marcelo A. C. S.; Romano, Alessandro P. M.; Borba, Amaríles S.; Silva, Eliana V. P.; Chiang, Jannifer O.; Eulálio, Kelsen D.; Azevedo, Raimunda S. S.; Rodrigues, Sueli G.; Almeida-Neto, Walfrido S.; Vasconcelos, Pedro F. C.

    2015-01-01

    A Brazilian ranch worker with encephalitis and flaccid paralysis was evaluated in the regional Acute Encephalitis Syndromic Surveillance Program. This was the first Brazilian patient who met the Centers for Disease Control and Prevention (CDC) confirmation criteria for West Nile virus disease. Owing to the overlapping of neurological manifestations attributable to several viral infections of the central nervous system, this report exemplifies the importance of human acute encephalitis surveillance. The syndromic approach to human encephalitis cases may enable early detection of the introduction of unusual virus or endemic occurrence of potentially alarming diseases within a region. PMID:26055749

  19. Cyst rupture as a pathogenic mechanism of toxoplasmic encephalitis.

    Science.gov (United States)

    Frenkel, J K; Escajadillo, A

    1987-05-01

    Seemingly intact cysts and sequential stages of disintegrating cysts of Toxoplasma were identified immunohistologically within developing microglial nodules in a Panamanian night monkey (Aotus lemurinus). This monkey had been successfully immunized and challenged 5 months earlier. This supports the hypothesis that glial nodules unassociated with Toxoplasma tachyzoites may represent the tombstone of a Toxoplasma cyst. Disintegration of cysts may give rise to clinical encephalitis in the presence of apparently adequate immunity.

  20. Ethical Implications of the Mild Encephalitis Hypothesis of Schizophrenia

    Science.gov (United States)

    Riedmüller, Rita; Müller, Sabine

    2017-01-01

    Schizophrenia is a serious mental disease with a high mortality rate and severe social consequences. Due to insufficient knowledge about its etiopathogenesis, curative treatments are not available. One of the most promising new research concepts is the mild encephalitis hypothesis of schizophrenia, developed mainly by Karl Bechter and Norbert Müller. According to this hypothesis, a significant subgroup of schizophrenia patients suffer from a mild, but chronic, form of encephalitis with markedly different etiologies ranging from viral infections, traumas to autoimmune diseases. This inflammatory process is thought to occur in the beginning or during the course of the disease. In this article, we investigate the consequences of the mild encephalitis hypothesis of schizophrenia for the scientific community, and evaluate these consequences ethically. The mild encephalitis hypothesis implies that schizophrenia would no longer be considered an incurable psychiatric disorder. Instead, it would be considered a chronic, but treatable, neurological disease. This paradigm shift would doubtlessly have significant consequences: (1) major reforms would be necessary in the theoretical conceptualization of schizophrenia, which would challenge the psychiatric diagnostic systems, Diagnostic and Statistical Manual of Mental Disorders version 5 and ICD-10. (2) Psychotic patients should be treated in interdisciplinary teams, optimally in neuropsychiatric units; additionally, specialists for endocrinology, diabetology, and cardiology should be consulted for the frequently occuring somatic comorbidities. (3) Current diagnostic procedures and (4) therapies would have to be modified significantly. (5) There might be repercussions for the pharmaceutical industry as well: first, because old drugs with expired patent protection could partly replace expensive drugs and, second, because there would be a demand for the development of new anti-inflammatory drugs. (6) Legal evaluation of

  1. Imported West Nile virus encephalitis in an Israeli tourist.

    Science.gov (United States)

    Rogers, Benjamin A; Hueston, Linda; Ratnam, Irani

    2009-08-17

    West Nile virus is an arbovirus that has caused large outbreaks of febrile illness, meningitis and encephalitis in Europe, North America and the Middle East. We describe the first laboratory-confirmed human case of West Nile virus infection in Australia, in a 58-year-old tourist who was almost certainly infected in Israel. The case is a reminder of the need to consider exotic pathogens in travellers and of the risk of introducing new pathogens into Australia.

  2. Improvement of advanced postvaccinal demyelinating encephalitis due to plasmapheresis

    Directory of Open Access Journals (Sweden)

    Andreas Rogalewski

    2007-01-01

    Full Text Available Andreas Rogalewski1, Jörg Kraus3, Martin Hasselblatt2, Christoffer Kraemer1, Wolf-Rüdiger Schäbitz11Department of Neurology; 2Institute of Neuropathology, University of Muenster, Germany, 3Paracelsus Private Medical University and Salzburger Landesklinken, Christian-Doppler-Klinik, Department of Neurology, Salzburg, AustriaAbstract: We report a case of acute demyelinating encephalitis that occurred after viral vaccination against hepatitis A-, hepatitis B-, and poliovirus and vaccination against bacterial toxins of diphtheria and tetanus. After different diagnosis had been excluded, we diagnosed postvaccinal demyelinating encephalitis and started treatment with high dose intravenous methylprednisolone, followed by peroral application in decreasing dosages for three weeks. A few days after the treatment with methylprednisolone had been finished, the patient’s medical condition deteriorated again. Thus, we initiated plasma exchange at an advanced state of illness, which led to significant continuous improvement. The role of plasma exchange is discussed controversially, in particular the issue of timing. We report a case that shows improvement due to plasmapheresis several weeks after symptom onset.Keywords: ADEM, vaccination, encephalitis, plasmapheresis, demyelination, plasma exchange

  3. Immunotherapy Responsive Autoimmune Subacute Encephalitis: A Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Manoj Mittal

    2010-01-01

    Full Text Available Objective. To describe the clinical characteristics and radiological findings in two patients with subacute encephalitis associated with elevated serum voltage-gated potassium channel antibody (VGKCAb and antithyroperoxidase (TPO antibody. Case Reports. Case 1: 63-year-old woman was admitted for altered mental status and possible seizure activity. MRI brain showed hyperintensity in the bilateral hippocampal areas. She was positive for VGKCAb and anti-TPO antibodies. She was treated with steroids, IVIG, plasma exchange and azathioprine. After 8 months, she had marked improvement in her memory and seizures. Case 2: 61-year-old woman was admitted for video EEG monitoring of unclassified seizure and cognitive function decline. MRI of the brain showed mild hyperintensity in bilateral hippocampal areas and significant atrophy in the frontotemporal lesion. Anti-TPO antibody and VGKCAb were positive. She was treated with steroids, plasma exchange and azathioprine. After 9 months, she had marked improvement in her memory and seizures. Conclusion. Autoimmune subacute encephalitis appears to be an underdiagnosed entity. It is important to screen patients with subacute encephalitis for anti-TPO antibody and VGKCAb, particularly in the presence of seizures. Immunosuppressive therapy appears to be effective in treating this entity.

  4. Epstein-Barr Virus Encephalitis in an Immunocompetent Child: A Case Report and Management of Epstein-Barr Virus Encephalitis

    Directory of Open Access Journals (Sweden)

    Gulsen Akkoc

    2016-01-01

    Full Text Available Epstein-Barr virus (EBV usually causes mild, asymptomatic, and self-limited infections in children and adults; however, it may occasionally lead to severe conditions such as neurological diseases, malignant diseases, hepatic failure, and myocarditis. Epstein-Barr virus-related neurological disorders include meningitis, encephalitis, and cranial or peripheral neuritis, which are mostly seen in immunocompromised patients. The therapeutic modalities for EBV-related severe organ damage including central nervous system manifestations are still uncertain. Herein, we describe a seven-year-old boy with EBV encephalitis who presented with prolonged fever, exudative pharyngitis, reduced consciousness, and neck stiffness. Cranial magnetic resonance imaging showed contrast enhancement in the bilateral insular cortex and the right hypothalamus. The diagnosis was made by EBV-DNA amplification in both the blood and cerebrospinal fluid samples. He was discharged with acyclovir therapy without any sequelae.

  5. Silent circulation of St. Louis encephalitis virus prior to an encephalitis outbreak in Cordoba, Argentina (2005.

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    Luis Adrian Díaz

    2012-01-01

    Full Text Available St. Louis encephalitis virus is a complex zoonoses. In 2005, 47 laboratory-confirmed and probable clinical cases of SLEV infection were reported in Córdoba, Argentina. Although the causes of 2005 outbreak remain unknown, they might be related not only to virological factors, but also to ecological and environmental conditions. We hypothesized that one of the factors for SLE reemergence in Córdoba, Argentina, was the introduction of a new SLEV genotype (SLEV genotype III, with no previous activity in the area. In order to evaluate this hypothesis we carried out a molecular characterization of SLEV detections from mosquitoes collected between 2001 and 2004 in Córdoba city. A total of 315 mosquito pools (11,002 individuals including 12 mosquitoes species were analyzed. Overall, 20 pools (8 mosquitoes species were positive for SLEV. During this study, genotypes II, V and VII were detected. No mosquito pool infected with genotype III was detected before the 2005 outbreak. Genotype V was found every year and in the 8 sampled sites. Genotypes II and VII showed limited temporal and spatial activities. We cannot dismiss the association of genotype II and V as etiological agents during the outbreak. However, the silent circulation of other SLEV strains in Córdoba city before the 2005 outbreak suggests that the introduction of genotype III was an important factor associated to this event. Not mutually exclusive, other factors such as changes in avian hosts and mosquitoes vectors communities, driven by climatic and environmental modifications, should also be taken into consideration in further studies.

  6. Leucine-rich glioma inactivated-1 and voltage gated potassium channel autoimmune encephalitis associated with ischemic stroke; A Case Report

    Directory of Open Access Journals (Sweden)

    Marisa Patryce McGinley

    2016-05-01

    Full Text Available Autoimmune encephalitis is associated with a wide variety of antibodies and clinical presentations. Voltage gated potassium channel (VGKC antibodies are a cause of autoimmune non-paraneoplastic encephalitis characterized by memory impairment, psychiatric symptoms, and seizures. We present a case of VGKC encephalitis likely preceding an ischemic stroke. Reports of autoimmune encephalitis associated with ischemic stroke are rare. Several hypothesizes linking these two disease processes are proposed.

  7. Red Flags: Clinical Signs for Identifying Autoimmune Encephalitis in Psychiatric Patients

    Science.gov (United States)

    Herken, Julia; Prüss, Harald

    2017-01-01

    Autoimmune mechanisms causing diverse psychiatric symptoms are increasingly recognized and brought about a paradigm shift in neuropsychiatry. Identification of underlying antibodies against neuronal ion channels or receptors led to the speculation that a number of patients go misdiagnosed with a primary psychiatric disease. However, there is no clear consensus which clinical signs in psychiatric patients should prompt further investigations including measurement of anti-neuronal autoantibodies. We therefore aimed to analyze the presenting symptoms in patients with autoimmune encephalitis and the time between symptom onset and initiation of antibody diagnostics. For this, we recruited 100 patients from the Charité Center for Autoimmune Encephalitis between May and October 2016, including all types of autoimmune encephalitides. Psychiatric abnormalities were the most common clinical symptoms and were the presenting sign in 60%. One-third of patients were initially hospitalized in a psychiatric ward. All patients positive for antibodies against the N-methyl-d-aspartate receptor showed behavioral changes, hallucinations, memory deficits, catatonia, or delusions. Patients positive for antibodies against other cell surface or intracellular antigens were often hospitalized with a psychosomatic diagnosis. The time between occurrence of first symptoms and antibody testing was often alarmingly prolonged. In patients with symptom onset between 2013 and 2016, the mean delay was 74 days, in cases diagnosed between 2007 and 2012 even 483 days, suggesting though that increased awareness of this novel disease group helped to expedite proper diagnosis and treatment. By analyzing the medical records in detail, we identified clinical signs that may help to assist in earlier diagnosis, including seizures, catatonia, autonomic instability, or hyperkinesia. Indeed, reanalyzing the whole cohort using these “red flags” led to a 58% reduction of time between symptom onset and

  8. Recurrent aseptic encephalitis in periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome.

    Science.gov (United States)

    Frye, Richard E

    2006-05-01

    An 11-year-old boy with episodes of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) is reported. Two PFAPA episodes were associated with aseptic encephalitis and seizures. Recurrent acute aseptic encephalitis or seizures have never been reported during the febrile episodes of PFAPA. This possible association is discussed within the context of the etiology of PFAPA.

  9. RECURRENT ASEPTIC ENCEPHALITIS IN PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITISAND ADENOPATHY (PFAPA) SYNDROME

    OpenAIRE

    Frye, Richard E.

    2006-01-01

    An 11-year-old boy with episodes of periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) is reported. Two PFAPA episodes were associated with aseptic encephalitis and seizures. Recurrent acute aseptic encephalitis or seizures have never been reported during the febrile episodes of PFAPA. This possible association is discussed within the context of the etiology of PFAPA.

  10. Catatonia in encephalitis and nonconvulsive seizures: a case report and review of the literature.

    Science.gov (United States)

    Sahaya, Kinshuk; Lardizabal, David

    2010-03-01

    A 20-year-old woman was admitted for psychosis. On further investigation, she was found to be have viral encephalitis and generalized nonconvulsive seizures. After the seizures were controlled, she remained in a prolonged catatonic state. Repeated intravenous benzodiazepine administration, improved her cognition dramatically. This case emphasizes that catatonia may occur after encephalitis and nonconvulsive seizures.

  11. Balamuthia mandrillaris and Acanthamoeba Amebic Encephalitis with Neurotoxoplasmosis Coinfection in a Patient with Advanced HIV Infection

    OpenAIRE

    Pietrucha-Dilanchian, Paula; Chan, Joseph C.; Castellano-Sanchez, Amilcar; Hirzel, Alicia; Laowansiri, Panthipa; Tuda, Claudio; Visvesvara, Govinda S.; Qvarnstrom, Yvonne; Ratzan, Kenneth R.

    2012-01-01

    We describe a patient with advanced HIV infection and Balamuthia mandrillaris and Acanthamoeba amebic encephalitis with Toxoplasma gondii coinfection. A multidisciplinary effort and state-of-the-art diagnostic techniques were required for diagnosis. Our patient is the first reported case of an HIV-infected person with dual Balamuthia mandrillaris and Acanthamoeba amebic encephalitis with neurotoxoplasmosis coinfection.

  12. Rabies direct fluorescent antibody test does not inactivate rabies or eastern equine encephalitis viruses.

    Science.gov (United States)

    Jarvis, Jodie A; Franke, Mary A; Davis, April D

    2016-08-01

    An examination using the routine rabies direct fluorescent antibody test was performed on rabies or Eastern equine encephalitis positive mammalian brain tissue to assess inactivation of the virus. Neither virus was inactivated with acetone fixation nor the routine test, thus laboratory employees should treat all samples as rabies and when appropriate Eastern equine encephalitis positive throughout the whole procedure.

  13. Japanese encephalitis in two children--United States, 2010.

    Science.gov (United States)

    2011-03-11

    Japanese encephalitis virus (JEV) is the leading cause of vaccine-preventable encephalitis in Asia and the western Pacific. JEV is maintained in an enzootic cycle involving mosquitoes and amplifying vertebrate hosts, mainly pigs and wading birds. The virus is transmitted to humans primarily by Culex mosquitoes, which breed in flooded rice fields and pools of stagnant water and most often feed outdoors during the evening and night. JEV transmission occurs mainly in rural agricultural areas, but occasional human cases occur in urban areas. Japanese encephalitis (JE) in persons who have traveled or lived overseas is diagnosed infrequently in the United States, with only four cases identified from 1992 (when a JE vaccine was first licensed in the United States) to 2008. This report describes the only cases diagnosed in the United States and reported to CDC since then. The first was a fatal case in a U.S. child who had visited relatives in the Philippines. The other occurred in a refugee who became ill while traveling from Thailand to the United States and whose diagnosis was complicated by concurrent neurocysticercosis. JE should be considered in the differential diagnosis for any patient with an acute neurologic infection who recently has been in a JE-endemic country. Travelers to JE-endemic countries should be advised of the risk for JE and the importance of personal protective measures to prevent mosquito bites. JE vaccine should be considered for travelers who might be at greater risk based on the season, location, and duration of their visit and their planned activities.

  14. Quantitative gait analysis following hemispherotomy for Rasmussen′s encephalitis

    Directory of Open Access Journals (Sweden)

    Santhosh George Thomas

    2007-01-01

    Full Text Available Peri-insular hemispherotomy is a form of disconnective hemispherectomy involving complete disconnection of all ascending / descending and commisural connections of one hemisphere. We report a case of a seven and a half year old child with intractable epilepsy due to Rasmussen′s encephalitis who underwent peri-insular hemispherotomy and achieved complete freedom from seizures. Quantitative gait analysis was used to describe the changes in the kinematic and kinetic parameters of gait with surface electromyographs 18 months after surgery. The focus of this paper is to highlight the utility of gait analysis following hemispherotomy with a view to directing postsurgical motor training and rehabilitation.

  15. Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica.

    Science.gov (United States)

    Moussawi, Khaled; Lin, David J; Matiello, Marcelo; Chew, Sheena; Morganstern, Daniel; Vaitkevicius, Henrikas

    2016-01-01

    The spectrum of disorders associated with anti-neuromyelitis optica (NMO) antibody is being extended to include infrequent instances associated with cancer. We describe a patient with brainstem and limbic encephalitis from NMO-immunoglobulin G in serum and cerebrospinal fluid in the context of newly diagnosed breast cancer. The neurological features markedly improved with excision of her breast cancer and immune suppressive therapy. This case further broadens the NMO spectrum disorders (NMOSD) by an association between NMOSD and cancer and raises the question of coincidental occurrence and the appropriate circumstances to search for a tumor in certain instances of NMO.

  16. The prM-independent packaging of pseudotyped Japanese encephalitis virus.

    Science.gov (United States)

    Lee, Hee Jung; Min, Kyung-Il; Lee, Jungeun; Kang, Sin-Hyung; Jeon, Wonkyung; Nam, Jae Hwan; Ju, Young Ran; Kim, Young Bong

    2009-07-30

    As noted in other flaviviruses, the envelope (E) protein of Japanese encephalitis virus (JEV) interacts with a cellular receptor and mediates membrane fusion to allow viral entry into target cells, thus eliciting neutralizing antibody response. The formation of the flavivirus prM/E complex is followed by the cleavage of precursor membrane (prM) and membrane (M) protein by a cellular signalase. To test the effect of prM in JEV biology, we constructed JEV-MuLV pseudotyped viruses that express the prM/E protein or E only. The infectivity and titers of JEV pseudotyped viruses were examined in several cell lines. We also analyzed the neutralizing capacities with anti-JEV sera from JEV-immunized mice. Even though prM is crucial for multiple stages of JEV biology, the JEV-pseudotyped viruses produced with prM/E or with E only showed similar infectivity and titers in several cell lines and similar neutralizing sensitivity. These results showed that JEV-MuLV pseudotyped viruses did not require prM for production of infectious pseudotyped viruses.

  17. The case for immunomodulatory approaches in treating HSV encephalitis.

    Science.gov (United States)

    Ramakrishna, Chandran; Openshaw, Harry; Cantin, Edouard M

    2013-03-01

    HSV encephalitis (HSE) is the most prevalent sporadic viral encephalitis. Although safe and effective antiviral therapies and greatly improved noninvasive diagnostic procedures have significantly improved outcomes, mortality (~20%) and debilitating neurological sequelae in survivors remain unacceptably high. An encouraging new development is that the focus is now shifting away from the virus exclusively, to include consideration of the host immune response to infection in the pathology underlying development of HSE. In this article, the authors discuss results from recent studies in experimental mouse models, as well as clinical reports that demonstrate a role for exaggerated host inflammatory responses in the brain in the development of HSE that is motivating researchers and clinicians to consider new therapeutic approaches for treating HSE. The authors also discuss results from a few studies that have shown that immunomodulatory drugs can be highly protective against HSE, which supports a role for deleterious host inflammatory responses in HSE. The impressive outcomes of some immunomodulatory approaches in mouse models of HSE emphasize the urgent need for clinical trials to rigorously evaluate combination antiviral and immunomodulatory therapy in comparison with standard antiviral therapy for treatment of HSE, and support for such an initiative is gaining momentum.

  18. [Diagnostic and therapeutic strategy for acyclovir-resistant herpes encephalitis].

    Science.gov (United States)

    Saijo, Masayuki

    2014-01-01

    Acyclovir (ACV), which inhibits the replication of herpes simplex virus, is the standard drug for the treatment of herpes simplex encephalitis. Thanks to the introduction of ACV, the morbidity and mortality of HSE patients have significantly improved. However, the disease is still the severe infection, because it makes some patients with HSE suffer from severe consequences. The sensitivity test of the etiological HSV to ACV is very difficult due to the inability of isolation of the virus from cerebrospinal fluid (CSF). The cases of the ACV treatment-resistant HSE patients have been reported. However, these cases were not virologically confirmed. The first case of encephalitis in newborn baby with HSE caused by an ACV-resistant HSV-1, which was virologically confirmed, was reported by our group. According to the sensitivity profile of the causative viruses to antiviral drugs, the drugs of choice for HSE should be properly considered. Strategy for diagnoses of HSE including antiviral sensitivity assessment and selection of drugs in HSE is reviewed.

  19. Cytokine Immunopathogenesis of Enterovirus 71 Brain Stem Encephalitis

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    Shih-Min Wang

    2012-01-01

    Full Text Available Enterovirus 71 (EV71 is one of the most important causes of herpangina and hand, foot, and mouth disease. It can also cause severe complications of the central nervous system (CNS. Brain stem encephalitis with pulmonary edema is the severe complication that can lead to death. EV71 replicates in leukocytes, endothelial cells, and dendritic cells resulting in the production of immune and inflammatory mediators that shape innate and acquired immune responses and the complications of disease. Cytokines, as a part of innate immunity, favor the development of antiviral and Th1 immune responses. Cytokines and chemokines play an important role in the pathogenesis EV71 brain stem encephalitis. Both the CNS and the systemic inflammatory responses to infection play important, but distinctly different, roles in the pathogenesis of EV71 pulmonary edema. Administration of intravenous immunoglobulin and milrinone, a phosphodiesterase inhibitor, has been shown to modulate inflammation, to reduce sympathetic overactivity, and to improve survival in patients with EV71 autonomic nervous system dysregulation and pulmonary edema.

  20. DETECTION OF A NEUTROPHIL CHEMOTACTIC FACTOR IN JAPANESE ENCEPHALITIS PATIENTS

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    Aditi Singh

    2012-12-01

    Full Text Available Japanese encephalitis (JE one of the most common cause of acute encephalitis in tropical regions, has generated much public anxiety in India. An early influx of macrophages followed by neutrophils at the site of injury in different organs in humans and mice has previously been reported. It correlated with production of a neutrophil chemotactic protein derived from macrophages. In the present study out of a total of 324 acute encephalitic patients, admitted in Gandhi memorial and associated hospitals, Lucknow, 121 patients with one or more indicators of JE virus infection were included. Significant pleocytosis (mean TLC value of 126+52 cells / mm3 in CSF and leucocytosis (>11,000 cells/mm3 in peripheral blood was observed at the time of admission. The leucocytosis increased significantly during second week in 67% of patients. The peripheral blood mononuclear cells culture done on alternate days was tested for chemotactic activity (hMDF, which was observed to be highest in second week of illness. The direct detection of hMDF in circulation by dot blot was positive in 92% of acute serum samples, with negligible (12.5% reactivity for convalescent sera. A correlation between the hMDF levels and severity of illness has also been observed.

  1. Medical image of the week: granulomatous amoebic encephalitis

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    Chaudhury A

    2015-06-01

    Full Text Available No abstract available. Article truncated at 150 words. A 64-year-old woman with history of deceased donor kidney transplantation presented with altered mental status. MRI of the brain showed new region of T2/FLAIR hyperintensity with restricted diffusion and slight cortical enhancement in the left middle temporal lobe (Figure 1, Panel A. Her neurological exam was notable for expressive aphasia and right-sided weakness. Initial diagnosis of ischemic stroke was further evaluated due to immunosuppressive status. Her CSF showed a WBC of 12 cells/microL with 80% lymphocytes, glucose 61 mg/dL, and protein 53 mg/dL. Follow up MRI showed progression of T2/FLAIR hyperintensity, intraparenchymal hemorrhage, and peripheral patchy enhancement in the left anterior/medial temporal lobe and inferior frontal lobe suspicious for encephalitis (Figure 1, Panel B. Left temporal lobe biopsy revealed granulomatous encephalitis with microorganisms morphologically consistent with amoeba (Figure 2, and tissue cultures grew MRSA. Acanthamoeba species was confirmed by the Centers for Disease Control and Prevention (CDC with antibody testing. ...

  2. Japanese Encephalitis Vaccines: WHO position paper, February 2015--Recommendations.

    Science.gov (United States)

    2016-01-12

    This article presents the World Health Organization's (WHO) recommendations on the use of Japanese Encephalitis (JE) vaccines excerpted from the WHO position paper on Japanese Encephalitis vaccines recently published in the Weekly Epidemiological Record [1]. This updated position paper on JE vaccines replaces the 2006 position paper on this subject [2]; it focuses on new information concerning the availability, safety, immunogenicity and effectiveness of JE vaccines and the duration of protection they confer. Recent data on global prevalence and burden of disease caused by JE and cost-effectiveness considerations regarding JE vaccination are also summarized. Footnotes to this paper provide a number of core references including references to grading tables that assess the quality of the scientific evidence. In accordance with its mandate to provide guidance to Member States on health policy matters, WHO issues a series of regularly updated position papers on vaccines and combinations of vaccines against diseases that have an international public health impact. These papers are concerned primarily with the use of vaccines in large-scale immunization programmes; they summarize essential background information on diseases and vaccines, and conclude with WHO's current position on the use of vaccines in the global context. This paper reflects the recommendations of WHO's Strategic Advisory Group of Experts (SAGE) on immunization. These recommendations were discussed by SAGE at its October 2014 meeting. Evidence presented at the meeting can be accessed at http://www.who.int/immunization/sage/previous/en/index.html.

  3. Sequential MRI, SPECT and PET in respiratory syncytial virus encephalitis

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    Hirayama, K.; Sakazaki, Hiromi; Murakami, Seiko; Yonezawa, Sumiko [Department of Paediatrics, Izumi Municipal Hospital, Osaka (Japan); Fujimoto, Keiji [Dept. of Radiology, Izumi Municipal Hospital, Osaka (Japan); Seto, Toshiyuki; Tanaka, Katsuji; Hattori, Hideji; Matsuoka, Osamu [Dept. of Paediatrics, Osaka City University Medical School, Osaka (Japan); Murata, Ryosuke [Children`s Medical Centre, Osaka City General Hospital, Osaka (Japan)

    1999-04-01

    We report on a 3-year-old girl with respiratory syncytial virus (RSV) encephalitis manifested by disturbance of consciousness, conjugate eye deviation, anuria, truncal ataxia and intention tremor. T2-weighted magnetic resonance imaging (MRI) showed hyperintense areas in the cerebellar cortex. No lesion was detected in the cerebral cortex, pons or spinal cord. The hyperintense areas in the cerebellar cortex diminished with recovery from the clinical manifestations and had resolved 2 months after onset. The MRI lesions in the cerebellum were considered to be due to oedema. SPECT and positron emission tomography (PET), performed 3 months after onset, disclosed areas of hypoperfusion and hypometabolism at the same sites. One year after onset, MRI showed mild atrophy of the cerebellum. Hypoperfusion on SPECT and hypometabolism on PET remained. Neuroimaging showed that ataxia and tremor in this case were the result of cerebellitis. The patient has no neurological deficit except for mild truncal ataxia. This patient is a rare example of RSV encephalitis. (orig.) With 4 figs., 16 refs.

  4. Experimental neurocysticercosis: absence of IL-4 induces lower encephalitis

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    Hidelberto Matos Silva

    Full Text Available ABSTRACT Neurocysticercosis (NCC is the most severe clinical manifestation of cysticercosis. One of the factors responsible for its symptomatology is the host inflammatory response. Therefore the influence of interleukin 4 (IL-4 on the induction of encephalitis in experimental NCC was evaluated. Methods BALB/c (WT and BALB/c (IL-4-KO mice were inoculated intracranially with Taenia crassiceps cysticerci and euthanized at 7, 30, 60 and 90 days later, the encephala removed and histopathologically analyzed. Results The absence of IL-4 induced greater parasitism. In the initial phase of the infection, IL-4-KO showed a lower intensity in the inflammatory infiltration of polimorphonuclear cells in the host-parasite interface and intra-parenquimatous edema. The IL-4-KO animals, in the late phase of the infection, showed lower intensity of ventriculomegaly, encephalitis, and meningitis, and greater survival of the parasites in comparison with the WT animals. Conclusion The absence of IL-4 induced lower inflammatory infiltration, ventriculomegaly and perivasculitis in experimental NCC.

  5. Diagnosis of Swine Encephalitis%猪乙型脑炎病的诊断

    Institute of Scientific and Technical Information of China (English)

    史秀春; 刁富花

    2012-01-01

    猪乙型脑炎又称流行性乙型脑炎、日本脑炎,是由流行性乙型脑炎病毒引起的一种急性、人畜共患的自然疫源性传染病.作者对某养殖户的病死猪进行临床症状观察,病理剖检及实验室诊断,通过病毒分离及鉴定,确诊病死猪为乙型脑炎病毒感染.鉴于养殖户中该病的存在及对养猪业的危害,建议加强对猪乙型脑炎病的诊断及监控.%Swine encephalitis also known as epidemic encephalitis, Japanese encephalitis,caused by an epidemic of acute viral encephalitis,the natural foci of zoonotic diseases. The paper introduced a pig farm. The clinical symptoms and pathological lesions and laboratory diagnosis were observed, and swine encephalitis was detected by virus isolation culture and RT-PCR, the final diagnosis result was swine encephalitis. Because the existence of the disease and the harm fulness to the pig industry, the diagnosis and monitoring of swine encephalitis should be strengthening.

  6. RIG-I mediates innate immune response in mouse neurons following Japanese encephalitis virus infection.

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    Arshed Nazmi

    Full Text Available BACKGROUND: Neuroinflammation associated with Japanese encephalitis (JE is mainly due to the activation of glial cells with subsequent release of proinflammatory mediators from them. The recognition of viral RNA, in part, by the pattern recognition receptor retinoic acid-inducible gene I (RIG-I has been indicated to have a role in such processes. Even though neurons are also known to express this receptor, its role after JE virus (JEV infections is yet to be elucidated. METHODOLOGY/PRINCIPAL FINDINGS: Upon infecting murine neuroblastoma cells and primary cortical neurons with JEV the expression profile of key proinflammatory cyto/chemokines were analyzed by qRT-PCR and bead array, both before and after ablation of RIG-I. Immunoblotting was performed to evaluate the levels of key molecules downstream to RIG-I leading to production of proinflammatory mediators. Changes in the intracellular viral antigen expression were confirmed by intracellular staining and immunoblotting. JEV infection induced neuronal expression of IL-6, IL-12p70, MCP-1, IP-10 and TNF-α in a time-dependent manner, which showed significant reduction upon RIG-I ablation. Molecules downstream to RIG-I showed significant changes upon JEV-infection, that were modulated following RIG-I ablation. Ablation of RIG-I in neurons also increased their susceptibility to JEV. CONCLUSIONS/SIGNIFICANCE: In this study we propose that neurons are one of the potential sources of proinflammatory cyto/chemokines in JEV-infected brain that are produced via RIG-I dependent pathways. Ablation of RIG-I in neurons leads to increased viral load and reduced release of the cyto/chemokines.

  7. Treatment of VGKC complex antibody-associated limbic encephalitis: a systematic review.

    Science.gov (United States)

    Radja, Guirindhra Koumar; Cavanna, Andrea Eugenio

    2013-01-01

    Limbic encephalitis is an autoimmune neuropsychiatric condition characterized by subacute cognitive symptoms, seizures, and affective changes. Although limbic encephalitis is usually caused by an immune reaction secondary to neoplasms, different types of potentially treatable non-paraneoplastic limbic encephalitis (nPLE) have recently been described. In particular, published studies have reported variable responses to immunosuppressive therapy in Voltage-Gated Potassium Channel (VGKC) complex antibody-associated nPLE. This systematic literature review found that the most significant improvements were reported by patients presenting with affective symptoms and consistent neuroradiological changes. In these patients, improved clinical outcomes correlated with the largest decreases in antibody titers.

  8. A case report on paraneoplastic encephalitis associated with astrocytoma - An unknown entity

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    Yogeshwari S Deshmukh

    2016-01-01

    Full Text Available Paraneoplastic encephalitis is a multifocal inflammatory disorder of the central nervous system (CNS that is associated with remote neoplasias. The most common malignancy associated with it is bronchial carcinoma, typically small cell carcinoma of lung. It has never been described in association with intracranial neoplasm. We present and discuss the clinical, radiological, and histopathological findings of paraneoplastic encephalitis with intracranial space-occupying lesions (SOLs in a 55-year-old man. He was thoroughly investigated and biopsy revealed presence of astrocytoma with changes of paraneoplastic encephalitis.

  9. Association between high expression macrophage migration inhibitory factor (MIF) alleles and West Nile virus encephalitis.

    Science.gov (United States)

    Das, Rituparna; Loughran, Kerry; Murchison, Charles; Qian, Feng; Leng, Lin; Song, Yan; Montgomery, Ruth R; Loeb, Mark; Bucala, Richard

    2016-02-01

    Infection with mosquito-borne West Nile virus (WNV) is usually asymptomatic but can lead to severe WNV encephalitis. The innate cytokine, macrophage migration inhibitory factor (MIF), is elevated in patients with WNV encephalitis and promotes viral neuroinvasion and mortality in animal models. In a case-control study, we examined functional polymorphisms in the MIF locus in a cohort of 454 North American patients with neuroinvasive WNV disease and found patients homozygous for high-expression MIF alleles to be >20-fold (p=0.008) more likely to have WNV encephalitis. These data indicate that MIF is an important determinant of severity of WNV neuropathogenesis and may be a therapeutic target.

  10. N-Methyl-D-aspartate receptor antibody could be a cause of catatonic symptoms in psychiatric patients: case reports and methods for detection

    Science.gov (United States)

    Tsutsui, Ko; Kanbayashi, Takashi; Takaki, Manabu; Omori, Yuki; Imai, Yumiko; Nishino, Seiji; Tanaka, Keiko; Shimizu, Tetsuo

    2017-01-01

    The symptoms of catatonia have been reported to be similar to the initial symptoms of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Subsequently, this autoimmune limbic encephalitis has been noticed by many psychiatrists. For a differential diagnosis of catatonic state, it is important to detect anti-NMDAR encephalitis. This encephalitis is expected to be in remission by early detection and treatment. We should be more cautious about catatonic symptoms of schizophrenia. When a patient is suspected of having encephalitis, we should screen for anti-NMDAR antibodies in cerebrospinal fluid samples using a cell-based assay. We describe the methods of NMDAR antibody detection and the etiology of this encephalitis with case reports. Two representative cases with catatonia and non-catatonia (brief psychotic disorder) were reported. Schizophrenia is a general, heterogeneous, and complicated disorder, and its pathophysiology is unknown. There is an established evidence of NMDAR hypofunction, which is the functional disconnection of the central component; this is one of the most recognized models for schizophrenia. Furthermore, it is said that autoimmune mechanisms have been involved, at least in subgroups of schizophrenia patients. Further study of anti-NMDAR antibody and its related encephalitis would give essential clues for the research of schizophrenia, catatonia, and atypical psychosis. PMID:28223808

  11. HIV positive patient with HSV-2 encephalitis: case report.

    Science.gov (United States)

    Pagliano, Pasquale; Ascione, Tiziana; Carleo, Maria Aurora; Boccia, Giovanni; De Caro, Francesco; Tortora, Fabio

    2016-09-01

    Incidence of brain infections in Human Immunodeficiency Virus (HIV) positive patients is reduced after the availability of current high active antiretroviral therapy (HAART). Herpes Simplex Virus type 2 (HSV-2) is an infrequent cause of encephalitis in HIV patients despite it is frequently involved in sexual transmitted infections. Here, we report a case of HSV-2 encephalitis occurring in a patient without full suppression of HIV replication within the brain. A 38 year-old HIV infected man was admitted to our department because of recurrent generalized seizure and fever during the previous 24 hours. Eight months before our observation the patient was switched from a protease inhibitor based regimen to a rilpivirine-based regimen without any evidence of HIV-RNA replication in the plasma. When the patient was admitted in our hospital, he was febrile and moderately confused, no deficit of cranial nerves was reported, motility was conserved, but he was unable to walk. Laboratory examinations performed at admission demonstrated an increase of cerebrospinal fluid (CSF) protein and cells with lymphocyte prevalence, and normal CSF glucose. HSV-2-DNA and HIV-RNA were present within CSF at admission. Nuclear Magnetic Resonance imaging of the brain revealed lesions of the medial part of both temporal lobes including hippocampus without any sign of bleeding. A 21-day course of acyclovir therapy was administered with consistent improvement of clinical findings and disappearance of HSV-2-DNA within CSF. After the episode, HAART was switched to a regimen with high CSF penetrability containing abacavir, lamivudine, darunavir and ritonavir. Twelve months after HSV-2 encephalitis neurologic evaluation was normal, but symptoms of depression were reported, HIV-RNA remained undetectable both in the plasma and CSF, and CD4+ lymphocytes were above 500/μL. No opportunistic infection was reported. Patients switched to regimen well tolerated such those containing rilpivirine, that have

  12. A clinical case of pseudotumorous chronic parainfectious limbic encephalitis

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    N. A. Shnaider

    2014-01-01

    Full Text Available Parainfectous limbic encephalitis (PILE associated with viruses of the Herpesviridae family is one of the forms of chronic herpes encephalitis characterized by limbic system dysfunction and a prolonged course with frequent exacerbations. There are two types of the course of the disease: latent autoimmune limbic encephalitis (LE progressing to mesial temporal sclerosis and pseudotumorous granulomatous LE. The latter (inflammatory pseudotumor or granuloma is characterized by the formation of a polymorphic inflammatory infiltrate with the elements of fibrosis, necrosis, and a granulomatous reaction and by myofibroblast cells. This is a slowly growing benign pseudotumor that contains much more plasma cells than inflammatory ones. The diagnosis of pseudotumorous LE is difficult and requires the participation of a neurologist, an immunologist, an oncologist, and a neurosurgeon. Perfusion computed tomography, magnetic resonance imaging, and magnetic resonance spectroscopy give proof to the adequacy of the term inflammatory pseudotumor because it is histologically difficult to characterize the lesion as a tumor or inflammation. When a chronic lesion in the central nervous system is lately diagnosed, the prognosis of the disease may be poor and complicated by the development of resistant symptomatic focal epilepsy and emotional, volitional, and cognitive impairments. It was differentially diagnosed from brain tumors (astrocytic, oligodendroglial, and mixed gliomas, ependymal, neuronal, neuroglial, and embryonal tumors, meningiomas, cholesteatomas, dermoid cysts, teratomas, and cysts, other reactive and inflammatory processes (leukemic infiltrations, systemic lupus erythematosus, multiple sclerosis, encephalomyelitis, hypoparathyroidism, Addison's disease, vitamin A intoxication, and the long-term use of glucocorticoids and contraceptives. The authors describe a clinical case of the pseudotumorous course of chronic PILE in a 28-year-old woman

  13. Multiple Paths to Encephalization and Technical Civilizations: Constraints Inferred from the Biosphere's Evolution

    Science.gov (United States)

    Schwartzman, D. W.; Middendorf, G.

    2010-04-01

    Two possible evolutionary paths should be considered for the emergence of intelligent life with the potential for technical civilization, encephalization in warm blooded animals, and swarm intelligence in superorganisms (e.g., termite colonies).

  14. Some patients with advanced malignancies also have reversible catatonia or limbic encephalitis

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    Joseph Martin Alisky

    2015-12-01

    Full Text Available Two potentially treatable disorders, paraneoplastic catatonia and paraneoplastic limbic encephalitis, may be hidden within the presentation of end stage cancer patients, because catatonia and limbic encephalitis usually feature severely altered mental status, confusion, anorexia, and minimal responsiveness that are also common with people dying of cancer. If catatonia and limbic encephalitis are correctly diagnosed and treated, there should be definite and dramatic improvement that would translate into better quality of life and perhaps even resumption of cancer therapy. This editorial reviews basic features of catatonia and limbic encephalitis, and then presents a strategy to systematically screen for these in end stage cancer patients who are about to enter hospice. A protocol is outlined that could be adapted for clinical practice or for designing clinical studies.

  15. Elevated cerebrospinal fluid endothelin 1 associated with neurogenic pulmonary edema in children with enterovirus 71 encephalitis

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    Yi-Fang Tu

    2015-05-01

    Conclusion: ET-1 in the central nervous system may play a role in the development of NPE in children with EV71 infection and could be used as a biomarker or therapeutic target for NPE in EV71 encephalitis.

  16. Purification of the envelope glycoproteins of western equine encephalitis virus by glass wool column chromatography.

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    Yamamoto, K.; Simizu, B

    1980-01-01

    Glass wool column chromatography was used for separation of the two glycoproteins of western equine encephalitis virus. Cross-contamination of each protein separated was confirmed to be negligible by sodium dodecyl sulfate-polyacrylamide gel electrophoresis.

  17. Post-marketing surveillance of live-attenuated Japanese encephalitis vaccine safety in China.

    Science.gov (United States)

    Wang, Yali; Dong, Duo; Cheng, Gang; Zuo, Shuyan; Liu, Dawei; Du, Xiaoxi

    2014-10-07

    Japanese encephalitis (JE) is the most severe form of viral encephalitis in Asia and no specific treatment is available. Vaccination provides an effective intervention to prevent JE. In this paper, surveillance data for adverse events following immunization (AEFI) related to SA-14-14-2 live-attenuated Japanese encephalitis vaccine (Chengdu Institute of Biological Products) was presented. This information has been routinely generated by the Chinese national surveillance system for the period 2009-2012. There were 6024 AEFI cases (estimated reported rate 96.55 per million doses). Most common symptoms of adverse events were fever, redness, induration and skin rash. There were 70 serious AEFI cases (1.12 per million doses), including 9 cases of meningoencephalitis and 4 cases of death. The post-marketing surveillance data add the evidence that the Chengdu institute live attenutated vaccine has a reasonable safety profile. The relationship between encephalitis and SA-14-14-2 vaccination should be further studied.

  18. Encephalitis caused by pathogens transmitted through organ transplants, United States, 2002-2013.

    Science.gov (United States)

    Basavaraju, Sridhar V; Kuehnert, Matthew J; Zaki, Sherif R; Sejvar, James J

    2014-09-01

    The cause of encephalitis among solid organ transplant recipients may be multifactorial; the disease can result from infectious or noninfectious etiologies. During 2002-2013, the US Centers for Disease Control and Prevention investigated several encephalitis clusters among transplant recipients. Cases were caused by infections from transplant-transmitted pathogens: West Nile virus, rabies virus, lymphocytic choriomeningitis virus, and Balamuthia mandrillaris amebae. In many of the clusters, identification of the cause was complicated by delayed diagnosis due to the rarity of the disease, geographic distance separating transplant recipients, and lack of prompt recognition and reporting systems. Establishment of surveillance systems to detect illness among organ recipients, including communication among transplant center physicians, organ procurement organizations, and public health authorities, may enable the rapid discovery and investigation of infectious encephalitis clusters. These transplant-transmitted pathogen clusters highlight the need for greater awareness among clinicians, pathologists, and public health workers, of emerging infectious agents causing encephalitis among organ recipients.

  19. Imaging Appearance of Human Immunodeficiency Virus Encephalitis on the Diffusion Weighted Images: A Case Report

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    Lim, Hun Cheol; Yu, In Kyu [Dept. of Radiology, Daejeon Eulji University Hospital, Daejeon (Korea, Republic of); Oh, Keon Se [Dept. of Neurology, Daejeon Eulji University Hospital, Daejeon (Korea, Republic of)

    2011-09-15

    Imaging finding of human immunodeficiency virus (HIV) encephalitis contain bilateral, symmetric, patchy, or diffuse increased T2WI signal intensities in the basal ganglia, cerebellum, brainstem, and centrum semiovale. In particular, the centrum semiovale is most commonly involved. Most of the HIV encephalitis cases are accompanied by brain atrophy. No previous study has reported symmetric increased signal intensity at the bilateral centrum semiovale without brain atrophy on diffusion weighted images in HIV encephalitis patients. Here, we report a case of this. We suggest that radiologists should consider the possibility of HIV encephalitis if there are symmetric increases in signal intensity at the bilateral centrum semiovale on diffusion weighted images of patients with a history of HIV infection.

  20. Isolation of saint louis encephalitis virus from a horse with neurological disease in Brazil.

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    Roberta Rosa

    2013-11-01

    Full Text Available St. Louis encephalitis virus (SLEV is a causative agent of encephalitis in humans in the Western hemisphere. SLEV is a positive-sense RNA virus that belongs to the Flavivirus genus, which includes West Nile encephalitis virus, Japanese encephalitis virus, Dengue virus and other medically important viruses. Recently, we isolated a SLEV strain from the brain of a horse with neurological signs in the countryside of Minas Gerais, Brazil. The SLEV isolation was confirmed by reverse-transcription RT-PCR and sequencing of the E protein gene. Virus identity was also confirmed by indirect immunofluorescence using commercial antibodies against SLEV. To characterize this newly isolated strain in vivo, serial passages in newborn mice were performed and led to hemorrhagic manifestations associated with recruitment of inflammatory cells into the central nervous system of newborns. In summary this is the first isolation of SLEV from a horse with neurological signs in Brazil.

  1. Encephalitis in a child with H1N1 infection: First case report from India

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    Rajesh Kulkarni

    2010-01-01

    Full Text Available Neurological complications have been described with seasonal influenza infection. We report encephalitis manifesting as seizures in a child with confirmed H1N1 infection. Treatment with oseltamivir was started. Child was discharged without any neurological sequelae.

  2. Limbic encephalitis as the presenting symptom of oesophageal adenocarcinoma: another cancer to search?

    Science.gov (United States)

    Menezes, Renata Barbosa; de Lucena, Adson Freitas; Maia, Fernanda Martins; Marinho, Antônia Rosivalda Teixeira

    2013-04-16

    Limbic encephalitis is a syndrome characterised by irritability, depression, sleeping disturbance, convulsion, hallucination and short-period memory loss that is commonly associated with a malignancy even if there is no evidence of it by the time of presentation. Most reported cases of limbic encephalitis as a paraneoplastic syndrome are associated with small-cell lung cancer and lymphoma. This article is a case report of a patient with limbic encephalitis associated with an oesophageal adenocarcinoma. The patient is a middle-aged man who presented apathy and unstable mood. After months, developed diplopia, reduced visual acuity and involuntary movements. Later, gait disability, disorientation, memory loss and aggressive behaviour were detected, associated with seizures. After investigation, limbic encephalitis was diagnosed and, as the patient developed dysphagia, oesophageal adenocarcinoma was detected. Oesophageal carcinoma usually does not have neurological symptoms associated.

  3. Rabies viral encephalitis with proable 25 year incubation period!

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    S K Shankar

    2012-01-01

    Full Text Available We report a case of rabies viral encephalitis in a 48-year-old male with an unusually long incubation period, historically suspected to be more than 20 years. The case was referred for histological diagnosis following alleged medical negligence to the forensic department. The histology and immunocytochemical demonstration of rabies viral antigen established the diagnosis unequivocally. The case manifested initially with hydrophobia and aggressive behavior, although he suddenly went to the bathroom and drank a small amount of water. History of dog bite 25 years back was elicited retrospectively following clinical suspicion. There was no subsequent history to suggest nonbite exposure to a rabid dog to consider recent event causing the disease, although this cannot be totally excluded.

  4. Herpes Simplex Encephalitis of the Parietal Lobe: A Rare Presentation

    Science.gov (United States)

    Tkachenko, Lara; Moisi, Marc; Rostad, Steven; Umeh, Randle; Zwillman, Michael E; Tubbs, R. Shane; Page, Jeni; Newell, David W.; Delashaw, Johnny B

    2016-01-01

    A 69-year-old female with a history of breast cancer and hypertension presented with a rare case of herpes simplex encephalitis (HSE) isolated to her left parietal lobe. The patient’s first biopsy was negative for herpes simplex virus (HSV) I/II antigens, but less than two weeks later, the patient tested positive on repeat biopsy. This initial failure to detect the virus and the similarities between HSE and symptoms of intracranial hemorrhage (ICH) suggests repeat testing for HSV in the presence of ICH. Due to the frequency of patients with extra temporal HSE, a diagnosis of HSE should be more readily considered, particularly when a patient may not be improving and a concrete diagnosis has not been solidified. PMID:27774355

  5. Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis

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    Robert Gross

    2016-01-01

    Full Text Available Mild cognitive impairment (MCI can be the initial manifestation of autoimmune limbic encephalitis (ALE, a disorder that at times presents a diagnostic challenge. In addition to memory impairment, clinical features that might suggest this disorder include personality changes, agitation, insomnia, alterations of consciousness, and seizures. Once recognized, ALE typically responds to treatment with immune therapies, but long-term cognitive deficits may remain. We report two cases of patients with MCI who were ultimately diagnosed with ALE with antibodies against the voltage gated potassium channel complex. Months after apparent resolution of their encephalitides, both underwent neuropsychological testing, which demonstrated persistent cognitive deficits, primarily in the domains of memory and executive function, for cases 1 and 2, respectively. A brief review of the literature is included.

  6. Testosterone correlates with Venezuelan equine encephalitis virus infection in macaques

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    Koterski James

    2006-03-01

    Full Text Available Abstract Here we briefly report testosterone and cytokine responses to Venezuelan equine encephalitis virus (VEEV in macaques which were used as part of a larger study conducted by the Department of Defense to better characterize pathological responses to aerosolized VEEV in non-human primates. Serial samples were collected and analyzed for testosterone and cytokines prior to and during infection in 8 captive male macaques. Infected animals exhibited a febrile response with few significant changes in cytokine levels. Baseline testosterone levels were positively associated with viremia following exposure and were significantly higher than levels obtained during infection. Such findings suggest that disease-induced androgen suppression is a reasonable area for future study. Decreased androgen levels during physiological perturbations may function, in part, to prevent immunosuppression by high testosterone levels and to prevent the use of energetic resources for metabolically-expensive anabolic functions.

  7. Advancements in pathogenesis studies of Rasmussen’s encephalitis

    Institute of Scientific and Technical Information of China (English)

    Sichang Chen; Shuai Chen; Yuguang Guan; Xueling Qi; Guoming Luan

    2016-01-01

    Rasmussen’s encephalitis ( RE ) , which was first described by Rasmussen in 1958 , is a rare, dispersed, and progressive neurological syndrome that is characterized by focal epilepsy, unilateral inflam-mation of the cerebral cortex, progressive hemiplegia and cognitive deterioration. The etiology of this syn-drome remains under investigation, and it is hypothe-sized and widely accepted that RE is a T-cell-mediated autoimmune response. However, the antigenic epitopes and mechanisms are still unknown. The patho-logical characteristics of RE are cortical inflammation, neuronal loss, and gliosis that are confined to one cere-bral hemisphere. Hemispherectomy remains the only cure for the seizures and cognitive deterioration associ-ated with the disease, but this procedure results in inev-itable functional loss in the brain. Compared with sur-gery, immunomodulatory treatments are expected to cause less neurological deficits, but with limited clinical effect.

  8. Disability from Japanese encephalitis in Cambodia and Viet Nam.

    Science.gov (United States)

    Hills, Susan L; Van Cuong, Nguyen; Touch, Sok; Mai, Hoang Hong; Soeung, Sann Chan; Lien, Tran Thi Huong; Samnang, Chham; Sovann, Ly; Van Diu, Pham; Lac, Luc Duy; Heng, Seng; Huong, Vu Minh; Grundy, John J; Huch, Chea; Lewthwaite, Penny; Solomon, Tom; Jacobson, Julie A

    2011-08-01

    A cohort of Japanese encephalitis (JE) survivors in Cambodia and Viet Nam were assessed at least 4 months after hospital discharge in order to understand the extent of disability after JE. We used a simple assessment tool which focuses on the impact on daily life. In total, 64 disability assessments were conducted: 38 in Cambodia and 26 in Viet Nam. In Cambodia, 4 (11%) children had severe sequelae, suggesting the children would likely be dependent, 15 (39%) had moderate sequelae and 17 (45%) had mild sequelae. In Viet Nam, two (8%) persons had severe sequelae, five (19%) had moderate sequelae and eight (31%) had mild sequelae. In many JE-endemic areas there are no multi-disciplinary teams with sophisticated equipment to assess patients after JE disease. This assessment tool can assist with patient management and generate data to support the need for programmes to prevent disease and improve outcomes for survivors.

  9. Cysticercal encephalitis presenting with a "Starry Sky" appearance on neuroimaging

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    Tushar B Patil

    2015-01-01

    Full Text Available A lady in her early forties was brought to our hospital in an unconscious state with history of generalized tonic-clonic seizures for last 6 h. She had multiple episodes of seizures in last 4 months, but did not receive any treatment. Relatives also gave a history of fever, headache, and vomiting for last 1 week. Her seizures were controlled with intravenous lorazepam and valproate. Clinical examination revealed a delirious patient with bilateral papilledema, brisk deep tendon reflexes, and extensor plantars. She had aspirated and had bilateral coarse crepitations on chest auscultation. Computed tomography (CT of brain showed multiple small hyperdense calcific lesions extending throughout both the cerebral hemispheres leading to a "starry sky" appearance, suggestive of cysticercal encephalitis. The patient succumbed to progressive aspiration pneumonitis on the 6 th day after hospitalization.

  10. New-onset refractory status epilepticus mimicking herpes virus encephalitis.

    Science.gov (United States)

    Puoti, Gianfranco; Elefante, Andrea; Saracino, Dario; Capasso, Antonella; Cotrufo, Roberto; Anello, Clara Belluomo

    2013-01-01

    New-onset refractory status epilepticus (NORSE) is a recently defined clinical entity that describes patients who present with status epilepticus of unclear etiology that is highly refractory to therapy. Magnetic resonance imaging (MRI) of NORSE usually discloses no specific abnormalities except for an occasional mild T2/FLAIR hyperintense signal of the mesial temporal lobe. Here, we report a peculiar case of NORSE in which brain MRI showed massive alteration of both temporal lobes, with features strongly supporting the diagnosis of herpes virus encephalitis, but lacking any laboratory evidence of viral infection in the blood and cerebrospinal fluid. It showed also striking signal alterations in the thalamus, which got worse in the course of the disease. This report emphasizes the possibility that seizure activity alone plays a critical role in both determining the disease and whether it will be self-sustaining.

  11. New-Onset Refractory Status Epilepticus Mimicking Herpes Virus Encephalitis

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    Gianfranco Puoti

    2013-09-01

    Full Text Available New-onset refractory status epilepticus (NORSE is a recently defined clinical entity that describes patients who present with status epilepticus of unclear etiology that is highly refractory to therapy. Magnetic resonance imaging (MRI of NORSE usually discloses no specific abnormalities except for an occasional mild T2/FLAIR hyperintense signal of the mesial temporal lobe. Here, we report a peculiar case of NORSE in which brain MRI showed massive alteration of both temporal lobes, with features strongly supporting the diagnosis of herpes virus encephalitis, but lacking any laboratory evidence of viral infection in the blood and cerebrospinal fluid. It showed also striking signal alterations in the thalamus, which got worse in the course of the disease. This report emphasizes the possibility that seizure activity alone plays a critical role in both determining the disease and whether it will be self-sustaining.

  12. Japanese encephalitis: Challenges and intervention opportunities in Nepal

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    Shristi Ghimire

    2015-01-01

    Full Text Available Japanese encephalitis (JE is a mosquito borne zoonotic disease caused by JE virus (JEV. JE has been endemic in Terai region, the lowland plains of Nepal bordering India, since 1978. However, in recent years cases of JE has been continuously reported from high altitude zones of hills and mountains. Irrigated rice farming system, expanded pig husbandry practices, inadequate vaccine coverage, low level of public awareness and climate change favoring mosquito breeding in higher altitudes might be the probable risk factors for emergence and re-emergence of JE in Nepal. Repeated outbreak in endemic areas and geographical expansion to newer areas have created huge challenge for JE prevention and control. At present, JE is one of the major public health concern of Nepal. Expanding vaccine coverage, improving agricultural practices, generating public awareness, supporting for use of mosquito avoiding practices and regional collaboration at border against JE can be helpful in getting better control over it in future.

  13. Tick-borne encephalitis virus infection in humans

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    Hrnjaković-Cvjetković Ivana

    2016-01-01

    Full Text Available Introduction. Tick-borne meningoencephalitis virus is a flavivirus that causes the most important vector-borne central nervous system infection in many countries of Europe and Asia. There are three subtypes of tick-borne encephalitis virus: European, Siberian and the Far-Eastern subtype. Transmission. In endemic areas, the virus remains in transmissive cycles between Ixodes ticks and small rodents. Clinical picture. In most cases (70−98% infection goes asymptomatically. In about one-third of meningitis cases, meningoencephalitis or meningomyelitis is developed. Postencephalytic syndrome may be the complication of the infection, presenting with neurological symptoms. Diagnosis. Etiologic diagnosis of tick-borne meningoencephalitis is only made on basis of laboratory analyses. Reverse transcription-polymerase chain reaction is used for determining the presence of virus in the blood and cerebrospinal fluid. Antibodies in blood and cerebrospinal fluid can be detected by serological tests. Prevention. The most efficient way to control this potentially severe disease with possible serious long-term consequences is vaccination. It should be recommended to persons who live or travel to endemic areas. Conclusion. In Serbia, tick-borne encephalitis virus infection belongs to the list of reportable diseases; however, there are no reported cases because the diagnostics is not performed routinely. We believe that the significance of this zoonosis must be examined in our country and some of its parts because of preliminary positive serological findings found out in Vojvodina as well as because of reported cases in neighboring countries such as Hungary and Croatia and its worldwide distribution.

  14. Varicella-zoster virus encephalitis in an AIDS patient

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    P.V. Toledo

    2004-06-01

    Full Text Available A 37-year-old man with a three-year history of Acquired Immunodeficiency Syndrome was admitted with impaired consciousness, seizures and fever. He was on highly active antiretroviral therapy and on neurotoxoplasmosis secondary prophylaxis. Laboratory exams from two months before showed a CD4 cell count of 37/µL and a viral load of 230,000 copies/mL. Three months before admission he developed herpetic skin rash in the right trunk and acyclovir was added to his treatment regimen. On physical exam he was drowsy and had motor and sensory aphasia. The patient had elevated protein levels and normal pressure in the cerebrospinal fluid (CSF. Contrast enhanced computed tomography scan of the brain showed a hypodense lesion in the left parietal lobe, with poorly defined margins and no contrast enhancement. The magnetic resonance scan (MRI showed multiple hyperintensities in T2-weighted image in white and grey matters and hypointense products of hemorrhage in both hemispheres and in the cerebellum. He was empirically treated with intravenous acyclovir and prednisone. Viral DNA of Varicella-zoster virus (VZV was detected in the CSF by means of polymerase chain reaction (PCR analysis. Acyclovir was continued for 10 days and the patient became well, with improvement of aphasia.We present a case of VZV encephalitis, confirmed by nested PCR, in a patient with suggestive MRI findings, who succeeded with treatment. VZV encephalitis is a rare opportunistic infection, occurring in 0.1 to 4% of AIDS patients with neurological disease; it is related to severe immunodeficiency and has a high mortality.

  15. Herpes simlex encephalitis. A neuropsychological and neuroradiological study

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    Koga, Masaya [Kurume Univ., Fukuoka (Japan). School of Medicine

    1994-10-01

    Herpes simplex virus (HSV) is the most common causative pathogen of nonepidemic encephalitis in Japan. The mortality rate is approximately 30% and surviving patients often have severe sequelae such as amnestic syndrome. Between 1979 and 1992, 13 patients with herpes simplex encephalitis (HSE) were admitted to our Department. Diagnosis was based on the neurological symptoms and signs, the findings on EEG, CT and MRI, and the results of serological examination including ELISA for HSV type 1. Neuropsychological tests were performed over a period of approximatly 2 years in seven of 13 patients. The results of the tests disclosed memory impairments as follows. Namely, immediate recall was well preserved, but disturbance of recent memory and retrograde amnesia over 20 years were observed. The neuropsychological features more closely resembled those of amnestic syndrome than those of Korsakoff syndrome. The amnesia in HSE patients was characterized in the finding that it was prone to recover more gradually as compared with that following head trauma or surgical brain resection. CT scan was performed in 13 patients during the acute and convalescent stages. The low density areas were found in the temporal lobes in 60% of the patients. MRI were obtained during the convalescent stage in ten of 13 patients. In seven of the ten patients, abnormal signals were distributed uni- or bilaterally to the temporal lobe, insular and limbic system such as the hippocampus and amygdala. In the remaining three patients, the abnormal signals were localized in both hippocampi and amygdalae in one patient and only in the brain stem in another. In the last case, the MRI was normal. The findings that signal intensity on MRI showing the lesion in the temporal lobe spread continuously to the opposite limbic area or occipital lobe strongly suggested the spreading of HSV through neural fiber connections in HSE. (author).

  16. Phylogeography of Japanese encephalitis virus: genotype is associated with climate.

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    Amy J Schuh

    Full Text Available The circulation of vector-borne zoonotic viruses is largely determined by the overlap in the geographical distributions of virus-competent vectors and reservoir hosts. What is less clear are the factors influencing the distribution of virus-specific lineages. Japanese encephalitis virus (JEV is the most important etiologic agent of epidemic encephalitis worldwide, and is primarily maintained between vertebrate reservoir hosts (avian and swine and culicine mosquitoes. There are five genotypes of JEV: GI-V. In recent years, GI has displaced GIII as the dominant JEV genotype and GV has re-emerged after almost 60 years of undetected virus circulation. JEV is found throughout most of Asia, extending from maritime Siberia in the north to Australia in the south, and as far as Pakistan to the west and Saipan to the east. Transmission of JEV in temperate zones is epidemic with the majority of cases occurring in summer months, while transmission in tropical zones is endemic and occurs year-round at lower rates. To test the hypothesis that viruses circulating in these two geographical zones are genetically distinct, we applied Bayesian phylogeographic, categorical data analysis and phylogeny-trait association test techniques to the largest JEV dataset compiled to date, representing the envelope (E gene of 487 isolates collected from 12 countries over 75 years. We demonstrated that GIII and the recently emerged GI-b are temperate genotypes likely maintained year-round in northern latitudes, while GI-a and GII are tropical genotypes likely maintained primarily through mosquito-avian and mosquito-swine transmission cycles. This study represents a new paradigm directly linking viral molecular evolution and climate.

  17. Neuropathogenesis of Japanese encephalitis in a primate model.

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    Khin Saw Aye Myint

    2014-08-01

    Full Text Available Japanese encephalitis (JE is a major cause of mortality and morbidity for which there is no treatment. In addition to direct viral cytopathology, the inflammatory response is postulated to contribute to the pathogenesis. Our goal was to determine the contribution of bystander effects and inflammatory mediators to neuronal cell death.Material from a macaque model was used to characterize the inflammatory response and cytopathic effects of JE virus (JEV. Intranasal JEV infection induced a non-suppurative encephalitis, dominated by perivascular, infiltrates of mostly T cells, alongside endothelial cell activation, vascular damage and blood brain barrier (BBB leakage; in the adjacent parenchyma there was macrophage infiltration, astrocyte and microglia activation. JEV antigen was mostly in neurons, but there was no correlation between intensity of viral infection and degree of inflammatory response. Apoptotic cell death occurred in both infected and non-infected neurons. Interferon-α, which is a microglial activator, was also expressed by both. Tumour Necrosis Factor-α, inducible nitric oxide synthase and nitrotyrosine were expressed by microglial cells, astrocytes and macrophages. The same cells expressed matrix metalloproteinase (MMP-2 whilst MMP-9 was expressed by neurons.The results are consistent with JEV inducing neuronal apoptotic death and release of cytokines that initiate microglial activation and release of pro-inflammatory and apoptotic mediators with subsequent apoptotic death of both infected and uninfected neurons. Activation of astrocytes, microglial and endothelial cells likely contributes to inflammatory cell recruitment and BBB breakdown. It appears that neuronal apoptotic death and activation of microglial cells and astrocytes play a crucial role in the pathogenesis of JE.

  18. Human T cell responses to Japanese encephalitis virus in health and disease

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    Turtle, L.; Bali, T; Buxton, G; Chib, S.; Chan, S; Soni, M.; Hussain, M; H. Isenman; Fadnis, P; Venkataswamy, MM; Satishkumar, V; Lewthwaite, P; Kurioka, A; Krishna, S.; Shankar, MV

    2016-01-01

    Japanese encephalitis (JE) virus (JEV) is an important cause of encephalitis in children of South and Southeast Asia. However, the majority of individuals exposed to JEV only develop mild symptoms associated with long-lasting adaptive immunity. The related flavivirus dengue virus (DENV) cocirculates in many JEV-endemic areas, and clinical data suggest cross-protection between DENV and JEV. To address the role of T cell responses in protection against JEV, we conducted the first full-breadth a...

  19. The evolution of orbit orientation and encephalization in the Carnivora (Mammalia).

    Science.gov (United States)

    Finarelli, John A; Goswami, Anjali

    2009-05-01

    Evolutionary change in encephalization within and across mammalian clades is well-studied, yet relatively few comparative analyses attempt to quantify the impact of evolutionary change in relative brain size on cranial morphology. Because of the proximity of the braincase to the orbits, and the inter-relationships among ecology, sensory systems and neuroanatomy, a relationship has been hypothesized between orbit orientation and encephalization for mammals. Here, we tested this hypothesis in 68 fossil and living species of the mammalian order Carnivora, comparing orbit orientation angles (convergence and frontation) to skull length and encephalization. No significant correlations were observed between skull length and orbit orientation when all taxa were analysed. Significant correlations were observed between encephalization and orbit orientation; however, these were restricted to the families Felidae and Canidae. Encephalization is positively correlated with frontation in both families and negatively correlated with convergence in canids. These results indicate that no universal relationship exists between encephalization and orbit orientation for Carnivora. Braincase expansion impacts orbit orientation in specific carnivoran clades, the nature of which is idiosyncratic to the clade itself.

  20. [Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma].

    Science.gov (United States)

    Launay, M; Bozzolo, E; Venissac, N; Delmont, E; Fredenrich, A; Thomas, P

    2008-01-01

    We report the case of a 49-year-old man who was admitted for progressive behaviorial disorders with frontal elements. There was no sensorial nor motor deficiency. Clinical examination revealed android obesity, cutaneous and mucous paleness, pubic and axillary depilation and gynecomastia. Encephalic MRI found a lesion of the left amygdalian region with high T2 intensity and low T1 intensity associated with gadolinium-enhancement. Cerebrospinal fluid analysis showed a lymphocytic meningitis. Panhypopituitarism was found on the endocrine investigations. Anti-RI antibodies were positive, leading to the diagnosis of paraneoplastic limbic encephalitis. The CT-scan showed a node of the lower part of the thymic area. Surgical resection revealed an ectopic mediastinal seminoma. The evolution consisted of paraneoplastic fever and crossed-syndrome with right hemiparesia and left common oculomotor nerve paralysis. Treatment was completed by two cycles of carboplatin, corticosteroids and substitutive opotherapy. Paraneoplastic fever disappeared, but behavioral disorders and palsy remain unchanged. The patient died two years later in a bedridden state. This case of paraneoplastic limbic encephalitis associated with positive anti-RI antibodies and mediastinal seminoma is exceptional and has not to our knowledge been described in the literature. Cancers usually associated with anti-RI antibody are breast and lung cancer. Paraneoplastic limbic encephalitis is not the classical clinical presentation, which usually is brainstem encephalitis. Hypothalamic involvement, uncommon in paraneoplastic limbic encephalitis is mainly associated with positive antineuronal anti-Ma2 antibodies. Finally, the gadolinium enhancement on encephalic MRI is unusual in paraneoplastic limbic encephalitis.

  1. Temporal and Pontine Involvement in a Case of Herpes Simplex Encephalitis, Presenting as Kluver Bucy Syndrome - A Case Report

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    Suresh Thirunavukarasu

    2011-01-01

    Full Text Available Bilateral temporal and frontal lobe involvement is a common characteristic of herpes simplex encephalitis (HSE. Clinical sequelae of herpes simplex encephalitis may manifest sometimes as Kluver Bucy syndrome (KBS. In herpes simplex encephalitis, apart from frontal lobe, extra temporal involvement is rare and uncommon. We report a case of HSE manifesting clinically as KBS with a rare radiological finding of temporal and extratemporal involvement of pons.

  2. N-Methyl-D-aspartate receptor antibody could be a cause of catatonic symptoms in psychiatric patients: case reports and methods for detection

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    Tsutsui K

    2017-02-01

    Full Text Available Ko Tsutsui,1 Takashi Kanbayashi,1,2 Manabu Takaki,3 Yuki Omori,1 Yumiko Imai,4 Seiji Nishino,5 Keiko Tanaka,6 Tetsuo Shimizu1,2 1Department of Neuropsychiatry, Akita University Graduate School of Medicine, Akita, 2International Institute for Integrative Sleep Medicine (WPI-IIIS, University of Tsukuba, Tsukuba, 3Department of Neuropsychiatry, Okayama University, Okayama, 4Biological Informatics and Experimental Therapeutics, Akita University Graduate School of Medicine, Akita, Japan; 5Sleep and Circadian Neurobiology Laboratory, Stanford University School of Medicine, Palo Alto, California, USA; 6Brain Research Institute, Niigata University, Niigata, Japan Abstract: The symptoms of catatonia have been reported to be similar to the initial symptoms of anti-N-methyl-D-aspartate receptor (NMDAR encephalitis. Subsequently, this autoimmune limbic encephalitis has been noticed by many psychiatrists. For a differential diagnosis of catatonic state, it is important to detect anti-NMDAR encephalitis. This encephalitis is expected to be in remission by early detection and treatment. We should be more cautious about catatonic symptoms of schizophrenia. When a patient is being doubted with encephalitis, we should screen for anti-NMDAR antibodies in cerebrospinal fluid samples using a cell-based assay. We describe the methods of NMDAR antibody detection and the etiology of this encephalitis with case reports. Two representative cases with catatonia and non-catatonia (brief psychotic disorder were reported. Schizophrenia is a general, heterogeneous, and complicated disorder, and its pathophysiology is unknown. There is an established evidence of NMDAR hypofunction, which is the functional disconnection of the central component; this is one of the most recognized models for schizophrenia. Furthermore, it is said that autoimmune mechanisms have been involved, at least in subgroups of schizophrenia patients. Further study of anti-NMDAR antibody and its related

  3. Cluster analysis for identifying sub-groups and selecting potential discriminatory variables in human encephalitis

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    Crowcroft Natasha S

    2010-12-01

    Full Text Available Abstract Background Encephalitis is an acute clinical syndrome of the central nervous system (CNS, often associated with fatal outcome or permanent damage, including cognitive and behavioural impairment, affective disorders and epileptic seizures. Infection of the central nervous system is considered to be a major cause of encephalitis and more than 100 different pathogens have been recognized as causative agents. However, a large proportion of cases have unknown disease etiology. Methods We perform hierarchical cluster analysis on a multicenter England encephalitis data set with the aim of identifying sub-groups in human encephalitis. We use the simple matching similarity measure which is appropriate for binary data sets and performed variable selection using cluster heatmaps. We also use heatmaps to visually assess underlying patterns in the data, identify the main clinical and laboratory features and identify potential risk factors associated with encephalitis. Results Our results identified fever, personality and behavioural change, headache and lethargy as the main characteristics of encephalitis. Diagnostic variables such as brain scan and measurements from cerebrospinal fluids are also identified as main indicators of encephalitis. Our analysis revealed six major clusters in the England encephalitis data set. However, marked within-cluster heterogeneity is observed in some of the big clusters indicating possible sub-groups. Overall, the results show that patients are clustered according to symptom and diagnostic variables rather than causal agents. Exposure variables such as recent infection, sick person contact and animal contact have been identified as potential risk factors. Conclusions It is in general assumed and is a common practice to group encephalitis cases according to disease etiology. However, our results indicate that patients are clustered with respect to mainly symptom and diagnostic variables rather than causal agents

  4. Encephalitis hospitalization rates and inpatient mortality in the United States, 2000-2010.

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    Benjamin P George

    Full Text Available Encephalitis rates by etiology and acute-phase outcomes for encephalitis in the 21st century are largely unknown. We sought to evaluate cause-specific rates of encephalitis hospitalizations and predictors of inpatient mortality in the United States.Using the Nationwide Inpatient Sample (NIS from 2000 to 2010, a retrospective observational study of 238,567 patients (mean [SD] age, 44.8 [24.0] years hospitalized within non-federal, acute care hospitals in the U.S. with a diagnosis of encephalitis was conducted. Hospitalization rates were calculated using population-level estimates of disease from the NIS and population estimates from the United States Census Bureau. Adjusted odds of mortality were calculated for patients included in the study.In the U.S. from 2000-2010, there were 7.3±0.2 encephalitis hospitalizations per 100,000 population (95% CI: 7.1-7.6. Encephalitis hospitalization rates were highest among females (7.6±0.2 per 100,000 and those 65 years of age with rates of 13.5±0.9 and 14.1±0.4 per 100,000, respectively. Etiology was unknown for approximately 50% of cases. Among patients with identified etiology, viral causes were most common (48.2%, followed by Other Specified causes (32.5%, which included predominantly autoimmune conditions. The most common infectious agents were herpes simplex virus, toxoplasma, and West Nile virus. Comorbid HIV infection was present in 7.7% of hospitalizations. Average length of stay was 11.2 days with mortality of 5.6%. In regression analysis, patients with comorbid HIV/AIDS or cancer had increased odds of mortality (odds ratio [OR]  = 1.70; 95% CI: 1.30-2.22 and OR = 2.26; 95% CI: 1.88-2.71, respectively. Enteroviral, postinfectious, toxic, and Other Specified causes were associated with lower odds vs. herpes simplex encephalitis.While encephalitis and encephalitis-related mortality impose a considerable burden in the U.S. in the 21st Century, the reported demographics of hospitalized

  5. Glucagon-Like Peptide-1 Analog, Liraglutide, Delays Onset of Experimental Autoimmune Encephalitis in Lewis Rats.

    Science.gov (United States)

    DellaValle, Brian; Brix, Gitte S; Brock, Birgitte; Gejl, Michael; Landau, Anne M; Møller, Arne; Rungby, Jørgen; Larsen, Agnete

    2016-01-01

    Introduction: Recent findings indicate that metabolic disturbances are involved in multiple sclerosis (MS) pathology and influence the susceptibility to treatment, directing attention toward anti-diabetic drugs such as metformin and pioglitazone. Liraglutide, a drug of the glucagon-like peptide-1 (GLP-1) family, is also anti-diabetic and weight-reducing and is, moreover, directly neuroprotective and anti-inflammatory in a broad spectrum of experimental models of brain disease. In this study we investigate the potential for this FDA-approved drug, liraglutide, as a treatment for MS by utilizing the experimental model, experimental autoimmune encephalitis (EAE). Methods: EAE was induced in 30 female Lewis rats that subsequently received twice-daily liraglutide (200 μg/kg s.c.) or saline. Healthy controls were included (saline, n = 6, liraglutide, n = 7). Clinical score and weight were assessed daily by blinded observers. Animals were killed at peak disease severity (day 11) or if exceeding humane endpoint (clinical score ≥4). Protein levels of manganese superoxide dismutase (MnSOD), amyloid precursor protein (APP), and glial fibrillary acidic protein (GFAP) were determined. Results: Liraglutide treatment delayed disease onset (group clinical score significantly >0) by 2 days and markedly reduced disease severity (median clinical score 2 vs. 5; p = 0.0003). Fourteen of 15 (93%) of vehicle-treated rats reached the humane endpoint (clinical score ≥4) by day 11 compared to 5 of 15 (33%) of liraglutide-treated rats (p = 0.0004). Liraglutide substantially increased the mitochondrial antioxidant MnSOD (p drug treatment (p = 0.09). Conclusion: We demonstrate, for the first time, that liraglutide treatment delays onset of EAE in Lewis rats and is associated with improved protective capacity against oxidative stress. These data suggest GLP-1 receptor agonists should be investigated further as a potential therapy for MS.

  6. Respiratory syncytial virus-related encephalitis: magnetic resonance imaging findings with diffusion-weighted study

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    Park, Arim; Suh, Sang-il; Seol, Hae-Young [Korea University College of Medicine, Department of Radiology, Korea University Guro Hospital, Seoul (Korea, Republic of); Son, Gyu-Ri; Lee, Nam-Joon [Korea University College of Medicine, Department of Radiology, Korea University Anam Hospital, Seoul (Korea, Republic of); Lee, Young Hen; Seo, Hyung Suk [Korea University College of Medicine, Department of Radiology, Korea University Ansan Hospital, Gyeonggi-do (Korea, Republic of); Eun, Baik-Lin [Korea University College of Medicine, Department of Pediatrics, Korea University Guro Hospital, Seoul (Korea, Republic of)

    2014-02-15

    Respiratory syncytial virus (RSV) is a common pathogen causing acute respiratory infection in children. Herein, we describe the incidence and clinical and magnetic resonance imaging (MRI) findings of RSV-related encephalitis, a major neurological complication of RSV infection. We retrospectively reviewed the medical records and imaging findings of the patients over the past 7 years who are admitted to our medical center and are tested positive for RSV-RNA by reverse transcriptase PCR. In total, 3,856 patients were diagnosed with RSV bronchiolitis, and 28 of them underwent brain MRI for the evaluation of neurologic symptoms; 8 of these 28 patients had positive imaging findings. Five of these 8 patients were excluded because of non-RSV-related pathologies, such as subdural hemorrhage, brain volume loss due to status epilepticus, periventricular leukomalacia, preexisting ventriculomegaly, and hypoxic brain injury. The incidence of RSV-related encephalitis was as follows: 3/3,856 (0.08 %) of the patients are positive for RSV RNA, 3/28 (10.7 %) of the patient underwent brain MRI for neurological symptom, and 3/8 (37.5 %) of patients revealed abnormal MR findings. The imaging findings were suggestive of patterns of rhombenmesencephalitis, encephalitis with acute disseminated encephalomyelitis, and limbic encephalitis. They demonstrated no diffusion abnormality on diffusion-weighted image and symptom improvement on the follow-up study. Encephalitis with RSV bronchiolitis occurs rarely. However, on brain MRI performed upon suspicion of neurologic involvement, RSV encephalitis is not infrequently observed among the abnormal MR findings and may mimic other viral and limbic encephalitis. Physicians should be aware of this entity to ensure proper diagnosis and neurologic care of RSV-positive patients. (orig.)

  7. Provenance and geographic spread of St. Louis encephalitis virus.

    Science.gov (United States)

    Kopp, Anne; Gillespie, Thomas R; Hobelsberger, Daniel; Estrada, Alejandro; Harper, James M; Miller, Richard A; Eckerle, Isabella; Müller, Marcel A; Podsiadlowski, Lars; Leendertz, Fabian H; Drosten, Christian; Junglen, Sandra

    2013-06-11

    St. Louis encephalitis virus (SLEV) is the prototypic mosquito-borne flavivirus in the Americas. Birds are its primary vertebrate hosts, but amplification in certain mammals has also been suggested. The place and time of SLEV emergence remain unknown. In an ecological investigation in a tropical rainforest in Palenque National Park, Mexico, we discovered an ancestral variant of SLEV in Culex nigripalpus mosquitoes. Those SLEV-Palenque strains form a highly distinct phylogenetic clade within the SLEV species. Cell culture studies of SLEV-Palenque versus epidemic SLEV (MSI-7) revealed no growth differences in insect cells but a clear inability of SLEV-Palenque to replicate in cells from birds, cotton rats, and free-tailed bats permissive for MSI-7 replication. Only cells from nonhuman primates and neotropical fruit bats were moderately permissive. Phylogeographic reconstruction identified the common ancestor of all epidemic SLEV strains to have existed in an area between southern Mexico and Panama ca. 330 years ago. Expansion of the epidemic lineage occurred in two waves, the first representing emergence near the area of origin and the second involving almost parallel appearances of the virus in the lower Mississippi and Amazon delta regions. Early diversification events overlapped human habitat invasion during the post-Columbian era. Several documented SLEV outbreaks, such as the 1964 Houston epidemic or the 1990 Tampa epidemic, were predated by the arrival of novel strains between 1 and 4 years before the outbreaks. Collectively, our data provide insight into the putative origins of SLEV, suggesting that virus emergence was driven by human invasion of primary rainforests. IMPORTANCE St. Louis encephalitis virus (SLEV) is the prototypic mosquito-transmitted flavivirus of the Americas. Unlike the West Nile virus, which we know was recently introduced into North America from the Old World, the provenience of SLEV is obscure. In an ecological investigation in a primary

  8. Dual Function of Ccr5 during Langat Virus Encephalitis: Reduction in Neutrophil-Mediated Central Nervous System Inflammation and Increase in T Cell-Mediated Viral Clearance.

    Science.gov (United States)

    Michlmayr, Daniela; Bardina, Susana V; Rodriguez, Carlos A; Pletnev, Alexander G; Lim, Jean K

    2016-06-01

    Tick-borne encephalitis virus (TBEV) is a vector-transmitted flavivirus that causes potentially fatal neurologic infection. There are thousands of cases reported annually, and despite the availability of an effective vaccine, the incidence of TBEV is increasing worldwide. Importantly, up to 30% of affected individuals develop long-term neurologic sequelae. We investigated the role of chemokine receptor Ccr5 in a mouse model of TBEV infection using the naturally attenuated tick-borne flavivirus Langat virus (LGTV). Ccr5-deficient mice presented with an increase in viral replication within the CNS and decreased survival during LGTV encephalitis compared with wild-type controls. This enhanced susceptibility was due to the temporal lag in lymphocyte migration into the CNS. Adoptive transfer of wild-type T cells, but not Ccr5-deficient T cells, significantly improved survival outcome in LGTV-infected Ccr5-deficient mice. Concomitantly, a significant increase in neutrophil migration into the CNS in LGTV-infected Ccr5(-/-) mice was documented at the late stage of infection. Ab-mediated depletion of neutrophils in Ccr5(-/-) mice resulted in a significant improvement in mortality, a decrease in viral load, and a decrease in overall tissue damage in the CNS compared with isotype control-treated mice. Ccr5 is crucial in directing T cells toward the LGTV-infected brain, as well as in suppressing neutrophil-mediated inflammation within the CNS.

  9. Leucine-Rich Glioma Inactivated-1 and Voltage-Gated Potassium Channel Autoimmune Encephalitis Associated with Ischemic Stroke: A Case Report.

    Science.gov (United States)

    McGinley, Marisa; Morales-Vidal, Sarkis; Ruland, Sean

    2016-01-01

    Autoimmune encephalitis is associated with a wide variety of antibodies and clinical presentations. Voltage-gated potassium channel (VGKC) antibodies are a cause of autoimmune non-paraneoplastic encephalitis characterized by memory impairment, psychiatric symptoms, and seizures. We present a case of VGKC encephalitis likely preceding an ischemic stroke. Reports of autoimmune encephalitis associated with ischemic stroke are rare. Several hypotheses linking these two disease processes are proposed.

  10. Argonaute 2 Suppresses Japanese Encephalitis Virus Infection in Aedes aegypti.

    Science.gov (United States)

    Sasaki, Toshinori; Kuwata, Ryusei; Hoshino, Keita; Isawa, Haruhiko; Sawabe, Kyoko; Kobayashi, Mutsuo

    2017-01-24

    There are three main innate immune mechanisms against viruses in mosquitoes. Infection with the flavivirus dengue virus is controlled by RNA interference (RNAi) and the JAK-STAT and Toll signaling pathways. This study showed that another flavivirus, Japanese encephalitis virus (JEV), did not invade the salivary glands of Aedes aegypti and that this may be a result of the innate immune resistance to the virus. Argonaute 2 (Ago2) plays a critical role in the RNAi pathway. To understand the mechanism of JEV resistance, we focused on Ago2 as a possible target of JEV. Here, we show that the expression of MyD88 (a mediator of Toll signaling) and Ago2 mRNAs was induced by JEV in the salivary glands of Ae. aegypti mosquitoes and that Ago2, JAK, and domeless (DOME) mRNAs were induced by JEV in the bodies of Ae. aegypti mosquitoes. Double-stranded (ds) Ago2 RNA enhanced JEV infection, and the virus was detected in salivary glands by immunofluorescence assay. In contrast, MyD88 dsRNA had no effect on JEV infection. These data suggest that Ago2 plays a crucial role in mediating the innate immune response of Ae. aegypti to JEV in a manner similar to that employed by dengue virus.

  11. Endemic eastern equine encephalitis in the Amazon region of Peru.

    Science.gov (United States)

    Aguilar, Patricia V; Robich, Rebecca M; Turell, Michael J; O'Guinn, Monica L; Klein, Terry A; Huaman, Alfredo; Guevara, Carolina; Rios, Zonia; Tesh, Robert B; Watts, Douglas M; Olson, James; Weaver, Scott C

    2007-02-01

    Eastern equine encephalitis virus (EEEV) causes severe neurologic disease in North America, but only two fatal human cases have been documented in South America. To test the hypothesis that alphavirus heterologous antibodies cross-protect, animals were vaccinated against other alphaviruses and challenged up to 3 months later with EEEV. Short-lived cross-protection was detected, even in the absence of cross-neutralizing antibodies. To assess exposure to EEEV in Peru, sera from acutely ill and healthy persons were tested for EEEV and other alphavirus antibodies, as well as for virus isolation. No EEEV was isolated from patients living in an EEEV-enzootic area, and only 2% of individuals with febrile illness had EEEV-reactive IgM. Only 3% of healthy persons from the enzootic region had EEEV-neutralizing antibodies. Our results suggest that humans are exposed but do not develop apparent infection with EEEV because of poor infectivity and/or avirulence of South American strains.

  12. Impaired autonomic responses to emotional stimuli in autoimmune limbic encephalitis

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    Olga eSchröder

    2015-11-01

    Full Text Available Limbic encephalitis (LE is an autoimmune-mediated disorder that affects structures of the limbic system, in particular the amygdala. The amygdala constitutes a brain area substantial for processing of emotional, especially fear-related signals. The amygdala is also involved in neuroendocrine and autonomic functions, including skin conductance responses (SCRs to emotionally arousing stimuli. This study investigates behavioral and autonomic responses to discrete emotion-evoking and neutral film clips in a patient suffering from LE associated with contactin-associated protein-2 (CASPR2-antibodies as compared to a healthy control group. Results show a lack of SCRs in the patient while watching the film clips, with significant differences compared to healthy controls in the case of fear-inducing videos. There was no comparable impairment in behavioral data (emotion report, valence and arousal ratings. The results point to a defective modulation of sympathetic responses during emotional stimulation in patients with LE, probably due to impaired functioning of the amygdala.

  13. A case of Bickerstaff's brainstem encephalitis in childhood

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    Ji Youn Kim

    2010-04-01

    Full Text Available Bickerstaff's brainstem encephalitis (BBE is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ in the serum or cerebrospinal fluid (CSF are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI, routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

  14. HHV-6 encephalitis in pediatric pazient: case report

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    Agata Calvario

    2009-12-01

    Full Text Available We report the case of a seventeen-month-old child, with a monocular amaurosis, hospitalized for inconsolable crying followed by a deep sleepiness.At neurological examination, and in the absence of clearly focal neurological signs, the child seemed drowsy and could wake only by moderately intense stimuli.A modest metabolic acidosis and an occasional delay of brain electrical activity at EEG, especially on posterior regions of the right hemisphere, were reported. Cranial CT scan, encephalic NMR and ECG were negative. Standard analysis and isofocusing of cerebro-spinal fluid (CSF were normal, while the virological analysis by Real Time PCR, performed on CSF and whole blood, revealed the presence of HHV-6 DNA. Guthrie Card, tested in triplicate, was positive for HHV-6 and negative for CMV. An antiviral, antibiotic and glucorticoid therapy was started. Following clinical improvement, the little patient was dismissed with a diagnosis of HHV-6 encephalitis. Neurological damages linked to HHV-6 are documented in pediatric patients. But while the infection is suspected of possible vertical viral transmission, HHV-6 remains a little known and misdiagnosed virus.

  15. Purpura fulminans associated with acute West Nile virus encephalitis.

    Science.gov (United States)

    Shah, Sheevam; Fite, Laura Paul; Lane, Natalie; Parekh, Palak

    2016-02-01

    Purpura fulminans is a progressive thrombotic disorder that presents with widespread purpura due to deficiency or dysfunction of protein C or protein S. Lesions present as well-demarcated erythematous macules that progress to irregular areas of hemorrhagic necrosis.West Nile virus is a member of the Flaviviridae family transmitted to humans through the bite of various mosquito species. It manifests as West Nile fever in 25% of those infected and less commonly as neuroinvasive disease. An African American man in his fortiespresented with altered mental status and was noted to have evidence of disseminated intravascular coagulation according to his lab data. He then developed dusky skin discoloration and systemic flaccid bullae with desquamation. Biopsy was consistent with purpura fulminans and the patient eventually developed symmetric peripheral gangrene, requiring amputations of all four extremities. Infectious work up revealed positive testing for IgM and IgG antibodies in serum and cerebrospinal fluid leading to the diagnosis of acute West Nile Virus encephalitis. We present this case to describe the rarely reported association of purpura fulminans with West Nile Virus infection.

  16. Vectors expressing chimeric Japanese encephalitis dengue 2 viruses.

    Science.gov (United States)

    Wei, Y; Wang, S; Wang, X

    2014-01-01

    Vectors based on self-replicating RNAs (replicons) of flaviviruses are becoming powerful tool for expression of heterologous genes in mammalian cells and development of novel antiviral and anticancer vaccines. We constructed two vectors expressing chimeric viruses consisting of attenuated SA14-14-2 strain of Japanese encephalitis virus (JEV) in which the PrM/M-E genes were replaced fully or partially with those of dengue 2 virus (DENV-2). These vectors, named pJED2 and pJED2-1770 were transfected to BHK-21 cells and produced chimeric viruses JED2V and JED2-1770V, respectively. The chimeric viruses could be passaged in C6/36 but not BHK-21 cells. The chimeric viruses produced in C6/36 cells CPE 4-5 days after infection and RT-PCR, sequencing, immunofluorescence assay (IFA) and Western blot analysis confirmed the chimeric nature of produced viruses. The immunogenicity of chimeric viruses in mice was proved by detecting DENV-2 E protein-specific serum IgG antibodies with neutralization titer of 10. Successful preparation of infectious clones of chimeric JEV-DENV-2 viruses showed that JEV-based expression vectors are fully functional.

  17. Experimental St. Louis encephalitis virus infection of sloths and cormorants.

    Science.gov (United States)

    Seymour, C; Kramer, L D; Peralta, P H

    1983-07-01

    Experimental infection of 11 Bradypus variegatus and Choloepus hoffmanni sloths with St. Louis encephalitis (SLE) virus produced detectable viremias of seven to 27 (median 13) days duration and maximum titers of 2.7 to 6.5 (median 5.1) log10 median suckling mouse intracranial lethal doses (SMicLD50) per ml. Experimental SLE viremia onset was delayed and maximum titer depressed in two sloths concurrently infected with naturally acquired viruses. SLE viremias in four experimentally inoculated cormorants Phalacrocorax olivaceus were shorter, and of equal or lower titer, than in sloths. Colonized Culex pipiens quinquefasciatus mosquitoes were infected by feeding on sloths circulating at least 4.8 log10 SMicLD50 of SLE virus per ml, and subsequently transmitted the infection to mice and chicks. An uninoculated baby Bradypus became infected by contact transmission from its mother. The antibody response of sloths to SLE virus was slow, being undetectable until several weeks post-inoculation. However, both sloth species developed high and long-lasting neutralizing and hemagglutination-inhibition antibody titers. The complement-fixation antibody response in Bradypus was lower and slower to develop than in Choloepus. Sloths with naturally acquired SLE virus antibody did not become detectably viremic after experimental inoculation. Neither sloths nor cormorants become overly ill from SLE virus infection.

  18. The clinical usefulness of Tc-99m ECD brain SPECT in acute measles encephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Seok Tae; Sohn, Myung Hee [School of Medicine, Chonbuk National Univ., Chonju (Korea, Republic of)

    2003-08-01

    Since the prognosis of measles encephalitis is poor, early diagnosis and proper management are very important to improve clinical outcomes. We compared Tc-99m ECD brain SPECT (SPECT) with MR imaging (MRI) for the detection of acute measles encephalitis. Eleven patients (M : F=4 : 7, age range 18 months-14 yrs) with acute measles encephalitis were enrolled in this studies. All of them underwent both MRI and SPECT. The results of SPECT were scored from 0 (normal) to 3 (most severe defect) according to perfusion state. We compared two image modalities for the detection of brain abnormality in acute measles encephalitis. Seven of 11 patients (63.6%) revealed high signal intensity in the white matter on T2WI of MRI, on the other hand all patients (100%) showed hypoperfusion on SPECT. Severe perfusion deficits above score 2 were located with decreasing frequencies in the frontal lobe (81.8%), temporal lobe (72.7%), occipital lobe (27.3%), basal ganglia (27.3%), and parietal lobe (9.1%). We conclude that SPECT is more useful than MRI for the detection of brain involvement in patients with acute measles encephalitis.

  19. Analysis of the surveillance situation for viral encephalitis and meningitis in Europe.

    Science.gov (United States)

    Donoso Mantke, O; Vaheri, A; Ambrose, H; Koopmans, M; de Ory, F; Zeller, H; Beyrer, K; Windorfer, A; Niedrig, M

    2008-01-17

    Infective processes in the brain, spinal cord and meninges are considered to be the main causes of encephalitis, myelitis and meningitis. However, most cases remain unexplained. The incidence of different viral aetiologies (zoonotic and non-zoonotic) is especially poorly estimated, due to the lack of a standard case definition and of agreed diagnostic algorithms, including harmonised diagnostic methods and sample collection. It is important to clarify the incidence of viral encephalitis/meningitis and to optimise the diagnosis of infectious neurological illness, particularly to ensure early recognition of outbreaks or emerging infectious such a West Nile encephalitis. The European Network for Diagnostics of 'Imported' Viral Diseases (ENIVD) has analysed the present surveillance situation for viral encephalitis/meningitis in Europe. Here we give an overview of the existing epidemiological sources of information in European Union (EU) Member States, mapping the laboratory capacity and identifying key requirements for a possible future surveillance study at European level. The data presented will help design a harmonised/standardised Europe-wide surveillance study investigating patients with encephalitis and/or meningitis in order to obtain more information on the role of infections in these rarely analysed syndromes, both from a clinical and an epidemiological perspective.

  20. Shuanghuanglian injection downregulates nuclear factor-kappa B expression in mice with viral encephalitis

    Institute of Scientific and Technical Information of China (English)

    Naibing Gu; Ye Tian; Zhengli Di; Caiping Han; Hui Lei; Gejuan Zhang

    2012-01-01

    A mouse model of viral encephalitis was induced by intracranial injection of a Coxsackie virus B3 suspension.Quantitative real-time reverse transcription-PCR and western blot assay were applied to detect mRNA and protein expression of intelectin-2 and nuclear factor-kappa B in the viral encephalitis and control groups.Nuclear factor-kappa B and intelectin-2 mRNA and protein expression were significantly increased in mice with viral encephalitis.After intraperitoneal injection of Shuanghuanglian at a dose of 1.5 mg/kg for 5 successive days,intelectin-2 and nuclear factor-kappa B protein and mRNA expression were significantly decreased.To elucidate the relationship between intelectin-2 and nuclear factor-kappa B,mice with viral encephalitis were administered an intracerebral injection of 107 pfu recombinant lentivirus expressing intelectin shRNA.Both protein and mRNA levels of intelectin and nuclear factor-kappa B in brain tissue of mice were significantly decreased.Experimental findings suggest that Shuanghuanglian injection may downregulate nuclear factor-kappa B production via suppression of intelectin production,thus inhibiting inflammation associated with viral encephalitis.

  1. Estimating the burden of Japanese encephalitis virus and other encephalitides in countries of the mekong region.

    Directory of Open Access Journals (Sweden)

    Arnaud Tarantola

    Full Text Available Diverse aetiologies of viral and bacterial encephalitis are widely recognized as significant yet neglected public health issues in the Mekong region. A robust analysis of the corresponding health burden is lacking. We retrieved 75 articles on encephalitis in the region published in English or in French from 1965 through 2011. Review of available data demonstrated that they are sparse and often derived from hospital-based studies with significant recruitment bias. Almost half (35 of 75 of articles were on Japanese encephalitis virus (JEV alone or associated with dengue. In the Western Pacific region the WHO reported 30,000-50,000 annual JEV cases (15,000 deaths between 1966 and 1996 and 4,633 cases (200 deaths in 2008, a decline likely related to the introduction of JEV vaccination in China, Vietnam, or Thailand since the 1980s. Data on dengue, scrub typhus and rabies encephalitis, among other aetiologies, are also reviewed and discussed. Countries of the Mekong region are undergoing profound demographic, economic and ecological change. As the epidemiological aspects of Japanese encephalitis (JE are transformed by vaccination in some countries, highly integrated expert collaborative research and objective data are needed to identify and prioritize the human health, animal health and economic burden due to JE and other pathogens associated with encephalitides.

  2. Characteristic abnormalities in cerebrospinal fluid biochemistry in children with cerebral malaria compared to viral encephalitis

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    Atmakuri RM

    2006-06-01

    Full Text Available Abstract Background In developing countries where Plasmodium falciparum malaria is endemic, viral encephalitis and cerebral malaria are found in the same population, and parasitemia with Plasmodium falciparum is common in asymptomatic children. The objective of this study was to investigate the cerebrospinal fluid (CSF biochemistry in children with cerebral malaria compared to those with presumed viral encephalitis. Methods We studied the following CSF parameters: cell count, glucose, protein, lactic dehydrogenase (LDH and adenosine deaminase (ADA levels, in children with cerebral malaria, with presumed viral encephalitis, and in control subjects who had a lumbar puncture after a febrile convulsion with postictal coma. Results We recruited 12 children with cerebral malaria, 14 children with presumed viral encephalitis and 20 controls prospectively, over 2 years in the Government General Hospital in Kakinada, India. Patients with cerebral malaria had significantly lower CSF glucose, and higher protein, LDH, CSF/blood LDH ratio and CSF ADA levels but a lower CSF/serum ADA ratio compared to controls (p Conclusion CSF/serum ADA ratio and CSF glucose levels were the best discriminators of cerebral malaria from presumed viral encephalitis in our study. Further studies are needed to explore their usefulness in epidemiological studies.

  3. Análisis de la densidad de receptores tipo NMDA R1 en el núcleo espinal de trigémino humano Analysis of the density of NMDA R1 receptors in the spinal nucleus of human trigeminal nucleus

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    I. C. Suazo

    2008-09-01

    Full Text Available El sistema trigeminal es encargado de la sensibilidad dolorosa de la mayor parte de los territorios orales y maxilofaciales, en esta función participan los receptores glutamatérgicos tipo NMDA que se encuentran en el núcleo espinal de trigémino. El núcleo espinal de trigémino se encuentra subdividido en tres subnúcleos en sentido rostrocaudal, denominados subnúcleo oral, interpolar y caudal. Clásicamente el subnúcleo caudal se ha considerado el sitio de relevo de la información dolorosa trigeminal. Objetivos. El objetivo del presente estudio fue analizar la distribución del receptor glutamatérgico NMDA en el núcleo espinal de trigémino en humanos. Material y Métodos. En este estudio se utilizaron 10 troncos encefálicos humanos obtenidos de cadáveres con una data postmortem de 8,7 horas en promedio (devesta 2,75, los cuales fueron sometidos a secciones transversales seriadas, obteniéndose 120 controles anatómicos teñidos con tinción de Mulligan y 120 placas sometidas a inmunohistoquímica con anticuerpos monoclonales anti-NMDA R1 (SigmaR en dilución 1:500 en 0,3% de Triton X-100 a ph 7,3 0,1 M. Resultados. Los resultados comprobaron la existencia de receptores glutamatérgicos tipo NMDA R1 en el núcleo espinal de trigémino humano, con un leve predominio en el subnúcleo caudal, sin encontrar una diferencia estadísticamente significativa. Debido a la importante presencia de estos receptores en los subnúcleos oral e interpolar. Conclusiones. Los resultados sugieren que todos los niveles del núcleo espinal de trigémino participarían en la transmisión dolorosa originada en los territorios orales y maxilofaciales.The trigeminal system is in charge of the painful sensibility of most the oral and maxilofaciales territory, in this function take part the NMDA type receptors that are in the spinal nucleus of trigeminal nerve. The spinal nucleus of trigéminal is subdivided in three subnucleuses in rostrocaudal orientation

  4. The Combination of Marketed Antagonists of α1b-Adrenergic and 5-HT2A Receptors Inhibits Behavioral Sensitization and Preference to Alcohol in Mice: A Promising Approach for the Treatment of Alcohol Dependence.

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    Fabrice Trovero

    Full Text Available Alcohol-dependence is a chronic disease with a dramatic and expensive social impact. Previous studies have indicated that the blockade of two monoaminergic receptors, α1b-adrenergic and 5-HT2A, could inhibit the development of behavioral sensitization to drugs of abuse, a hallmark of drug-seeking and drug-taking behaviors in rodents. Here, in order to develop a potential therapeutic treatment of alcohol dependence in humans, we have blocked these two monoaminergic receptors by a combination of antagonists already approved by Health Agencies. We show that the association of ifenprodil (1 mg/kg and cyproheptadine (1 mg/kg (α1-adrenergic and 5-HT2 receptor antagonists marketed as Vadilex ® and Periactine ® in France, respectively blocks behavioral sensitization to amphetamine in C57Bl6 mice and to alcohol in DBA2 mice. Moreover, this combination of antagonists inhibits alcohol intake in mice habituated to alcohol (10% v/v and reverses their alcohol preference. Finally, in order to verify that the effect of ifenprodil was not due to its anti-NMDA receptors property, we have shown that a combination of prazosin (0.5 mg/kg, an α1b-adrenergic antagonist, Mini-Press ® in France and cyproheptadine (1 mg/kg could also reverse alcohol preference. Altogether these findings strongly suggest that combined prazosin and cyproheptadine could be efficient as a therapy to treat alcoholism in humans. Finally, because α1b-adrenergic and 5-HT2A receptors blockade also inhibits behavioral sensitization to psychostimulants, opioids and tobacco, it cannot be excluded that this combination will exhibit some efficacy in the treatment of addiction to other abused drugs.

  5. [F-18]FHPG positron emission tomography for detection of herpes simplex virus (HSV) in experimental HSV encephalitis

    NARCIS (Netherlands)

    Buursma, AR; de Vries, EFJ; Garssen, J; Kegler, D; van Waarde, A; Schirm, J; Hospers, GAP; Mulder, NH; Vaalburg, W; Klein, HC

    2005-01-01

    Herpes simplex virus type 1 (HSV-1) is one of the most common causes of sporadic encephalitis. The initial clinical course of HSV encephalitis (HSE) is highly variable, and the infection may be rapidly fatal. For effective treatment with antiviral medication, an early diagnosis of HSE is crucial. Su

  6. 日本脑炎流行病学和疫苗研制%Japanese Encephalitis Epidemiology and Vaccine Production

    Institute of Scientific and Technical Information of China (English)

    Akira Oya

    2002-01-01

    @@ Japanese encephalitis (JE) caused by Japanese encephalitis virus (JEV) is widely spread in the east and southeast part of Asia and number of human cases are estimated approximately 20000 annually in the world. The epidemic area is expanding recently as a newly emerging disease in the Oceanic countries.

  7. A systematic review of the literature to identify and quantify host and vector competence and abundance of Japanese Encephalitis Virus

    Science.gov (United States)

    Japanese Encephalitis virus (JEV) is a mosquito-borne arbovirus that causes endemic and epidemic encephalitis in Eastern and Southeastern Asia. Swine and wading birds serve as reservoirs for the virus, which can be transmitted to humans via mosquitos. Currently, there is no specific treatment availa...

  8. Viral Etiology of Encephalitis in Children in Southern Vietnam: Results of a One-Year Prospective Descriptive Study

    NARCIS (Netherlands)

    Tan, L.V.; Qui, P.T.; Ha, D.Q.; Hue, N.B.; Bao, L.Q.; Cam, B.V.; Khanh, T.H.; Hien, T.T.; Chau, N.V.V.; Tram, T.T.; Hien, V.M.; Nga, T.V.T.; Schultsz, C.; Farrar, J.; van Doorn, H.R.; de Jong, M.D.

    2010-01-01

    Background: Acute encephalitis is an important and severe disease in children in Vietnam. However, little is known about the etiology while such knowledge is essential for optimal prevention and treatment. To identify viral causes of encephalitis, in 2004 we conducted a one-year descriptive study at

  9. Family outbreak of alimentary tick-borne encephalitis in Slovakia associated with a natural focus of infection.

    Science.gov (United States)

    Kohl, I; Kozuch, O; Elecková, E; Labuda, M; Zaludko, J

    1996-08-01

    A family outbreak of tick-borne encephalitis involving 7 people, all of them hospitalized, was observed in the district of Povazská Bystrica (central Slovakia). The disease was associated with the drinking of unboiled goat milk and tick-borne encephalitis virus was recovered from Ixodes ricinus ticks collected from places where goats were grazing.

  10. Quantification of vector and host competence and abundance for Japanese Encephalitis Virus: a systematic review of the literature.

    Science.gov (United States)

    Japanese encephalitis (JE) is a vector-borne disease caused by the Japanese encephalitis virus (JEV) that affects humans in Eastern and Southeastern Asia. Although it could be prevented by a vaccine, JE has no treatment and the inadvertent introduction of the virus into JEV-free countries, such as t...

  11. Japanese encephalitis virus infection decrease endogenous IL-10 production: correlation with microglial activation and neuronal death.

    Science.gov (United States)

    Swarup, Vivek; Ghosh, Joydeep; Duseja, Rachna; Ghosh, Soumya; Basu, Anirban

    2007-06-13

    The anti-inflammatory cytokine interleukin (IL)-10 is synthesized in the central nervous system (CNS) and acts to limit clinical symptoms of stroke, multiple sclerosis, Alzheimer's disease, meningitis, and the behavioral changes that occur during bacterial infections. Expression of IL-10 is critical during the course of most major diseases in the CNS and promotes survival of neurons and all glial cells in the brain by blocking the effects of proinflammatory cytokines and by promoting expression of cell survival signals. In order to assess functional importance of this cytokine in viral encephalitis we have exploited an experimental model of Japanese encephalitis (JE). We report for the first time that in Japanese encephalitis, there is a progressive decline in level of IL-10. The extent of progressive decrease in IL-10 level following viral infection is inversely proportional to the increase in the level of proinflammatory cytokines as well as negative consequences that follows viral infection.

  12. The clinical diagnosis and treatment about 22 cases of limbic encephalitis were retrospectively analyzed.

    Science.gov (United States)

    Zang, Weiping; Zhang, Zhijun; Feng, Laihui; Zhang, Ailing

    2016-03-01

    To summarize and analyze the clinical characteristics and treatment of limbic encephalitis, in order to provide the basis for clinical work. We retrospectively analyzed the clinical characteristics, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) and self immune antibody results of 22 patients with limbic encephalitis in Zheng zhou people's Hospital from March 2013 to May 2014. 22 cases of patients with psychiatric disturbance, such as hallucinations being typical clinical manifestations: Memory decline in 18 cases: Seizures in 13 patients: Altered level of consciousness in 10 cases; Movement disorders in 7 cases and 9 cases with febrile.14 cases have relieved after treating with antiviral and immunosuppressive therapy, 5 cases left memory decline, 2 patients left overwhelmingly excited, 1 cases of seizures. The clinical symptoms of patients with limbic encephalitis are complicated changeable and unspecific. so earlier diagnosis and treatment are very important for the prognosis of patients.

  13. Herpetic brainstem encephalitis: report of a post-mortem case studied electron microscopically and immunohisiochemically

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    José Eymard Homem Pitella

    1987-03-01

    Full Text Available A post-mortem examined case of herpetic brainstem encephalitis is presented. Clinically, the patient had cephalea followed by ataxia, drowsiness and multiple palsies of some cranial nerves, developing into death in eight days. The pathologic examination of the brain showed necrotizing encephalitis in multiple foci limited to the brainstem, more distinctly in the pons and medula oblongata. The technique of immunoperoxidase revealed rare glial cells with intranuclear immunoreactivity for herpes antigen. Rare viral particles with the morphological characteristics of the herpesvirus were identified in the nuclei of neurons in 10% formol fixed material. This is the second reported case of herpetic brainstem encephalitis confirmed by post-mortem examination. The pathway used by the virus to reach the central nervous system and its posterior dissemination to the oral cavity, the orbitofrontal region and the temporal lobes as well as to the brainstem, after a period of latency and reactivation, are discussed.

  14. Infectious causes of encephalitis and meningoencephalitis in Thailand, 2003-2005.

    Science.gov (United States)

    Olsen, Sonja J; Campbell, Angela P; Supawat, Krongkaew; Liamsuwan, Sahas; Chotpitayasunondh, Tawee; Laptikulthum, Somsak; Viriyavejakul, Akravudh; Tantirittisak, Tasanee; Tunlayadechanont, Supoch; Visudtibhan, Anannit; Vasiknanonte, Punnee; Janjindamai, Supachai; Boonluksiri, Pairoj; Rajborirug, Kiatsak; Watanaveeradej, Veerachai; Khetsuriani, Nino; Dowell, Scott F

    2015-02-01

    Acute encephalitis is a severe neurologic syndrome. Determining etiology from among ≈100 possible agents is difficult. To identify infectious etiologies of encephalitis in Thailand, we conducted surveillance in 7 hospitals during July 2003-August 2005 and selected patients with acute onset of brain dysfunction with fever or hypothermia and with abnormalities seen on neuroimages or electroencephalograms or with cerebrospinal fluid pleocytosis. Blood and cerebrospinal fluid were tested for >30 pathogens. Among 149 case-patients, median age was 12 (range 0-83) years, 84 (56%) were male, and 15 (10%) died. Etiology was confirmed or probable for 54 (36%) and possible or unknown for 95 (64%). Among confirmed or probable etiologies, the leading pathogens were Japanese encephalitis virus, enteroviruses, and Orientia tsutsugamushi. No samples were positive for chikungunya, Nipah, or West Nile viruses; Bartonella henselae; or malaria parasites. Although a broad range of infectious agents was identified, the etiology of most cases remains unknown.

  15. The Prognostic Role of Magnetic Resonance Imaging and Single-Photon Emission Computed Tomography in Viral Encephalitis

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    Misra, U.K.; Kalita, J.; Srivastav, A.; Pradhan, P.K. (Depts. of Neurology and Nuclear Medicine, Sanjay Gandhi Post Graduate Inst. of Medical Sciences, Lucknow (India))

    2008-09-15

    Background: There is a paucity of studies evaluating the prognostic role of magnetic resonance imaging (MRI) and single-photon emission computed tomography (SPECT) changes in viral encephalitis. Purpose: To study MRI and SPECT changes in patients with viral encephalitis, and to correlate these changes with clinical findings and outcome. Material and Methods: During 1997-2006, 31 encephalitis patients (aged 2-60 years; nine females, 22 males) underwent both MRI and SPECT studies. Their demographic and clinical data and 6-month outcome were recorded. For the diagnosis of encephalitis, polymerase chain reaction (PCR) and IgM enzyme-linked immunosorbent assay (ELISA) were carried out. Cranial MRI was done on a 1.5 T scanner, and 99mTc ethylene cysteine dimer (ECD) SPECT using a gamma camera. Outcome was defined at 6 months as complete, partial, or poor recovery. Results: 19 patients had Japanese encephalitis (JE), one had herpes simplex encephalitis (HSE), and 11 had nonspecific encephalitis. Movement disorders were present in 21, parkinsonian features in 19, and dystonia in 16 patients. MRI was abnormal in 20 patients, and revealed thalamic involvement in 17, basal ganglia in eight, brainstem in 11, and cortical in two. SPECT revealed hypoperfusion in 22 patients, which was cortical in 11, thalamic in 10, basal ganglia in six, and midbrain in one. Cortical involvement was more frequently found by SPECT and brainstem involvement by MRI. Outcome of encephalitis did not differ in the different groups of encephalitis and MRI changes. Conclusion: MRI and SPECT show a spectrum of findings in encephalitis, but these do not correlate with 6-month outcome

  16. Chikungunya virus infection amongst the acute encephalitis syndrome cases in West Bengal, India

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    D Taraphdar

    2015-01-01

    Full Text Available Chikungunya virus (CHIKV infection from the acute encephalitis syndrome cases is an uncommon form and has been observed in the year 2010-11 from West Bengal, India. The case-1 and case-2 had the acute encephalitis syndrome; case-3 was of acute disseminated encephalomyelitis whereas the case-4 had the symptoms of meningo-encephalopathy with bulbar involvement. We are reporting four cases with neurological complications involving central nervous system (CNS due to CHIKV infection from this state for the first time. The virus has spread almost every districts of this state rapidly. At this stage, these cases are public health threat.

  17. Status Epilepticus Due to Severe HHV-6 Encephalitis in an Allogeneic Stem Cell Transplant Recipient

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    Poorvi Chordia

    2013-12-01

    Full Text Available Reactivation of human herpes virus-6 (HHV-6 after stem cell transplantation occurs frequently. It is associated with clinical manifestations varying from nonspecific symptoms such as fevers or rash, to severe life threatening complications including post-transplantation limbic encephalitis. We report a case of severe HHV-6 encephalitis with viremia in an allogeneic peripheral stem cell transplant recipient who presented with status epilepticus unresponsive to antiepileptic therapy.  With intravenous ganciclovir and supportive care, the patient’s condition improved. Awareness of HHV-6 infection in stem cell transplant recipients may help with early diagnosis and improved outcome.

  18. [Nonstructural protein 1 of tick-borne encephalitis virus activates the expression of immunoproteasome subunits].

    Science.gov (United States)

    Kuzmenko, Y V; Starodubova, E S; Karganova, G G; Timofeev, A V; Karpov, V L

    2016-01-01

    The interaction of viral proteins with host cell components plays an important role in antiviral immune response. One of the key steps of antiviral defense is the formation of immunoproteasomes. The effect of nonstructural protein 1 (NS1) of tick-borne encephalitis virus on the immunoproteasome formation was studied. It was shown that cell expression of NS1 does not reduce the efficacy of the immunoproteasome generation in response to interferon-γ stimulation and even increases the content of the immunoproteasome subunits without the interferon-γ treatment. Thus, NS1 of tick-borne encephalitis virus activates, rather than blocks the mechanisms of immune defense in the cell.

  19. Numerous cerebral hemorrhages in a patient with influenza-associated encephalitis: A case report

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    Lee, Ji Ye; Seong, Su Ok; Park, Noh Hyuck; Park, Chan Sup [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2016-02-15

    Influenza-associated encephalitis (IAE) is a complication of a common disease that is rare even during an epidemic. Awareness of magnetic resonance imaging features of IAE is important in treatment planning and prognosis estimation. Several reports have described necrotizing encephalopathy in children with influenza. However, few reports have described multifocal hemorrhages in both cerebral hemispheres in adults with concomitant infection with influenza A and B. Here, we describe a case of influenza A- and B-associated encephalitis accompanied by numerous cerebral hemorrhages.

  20. Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma.

    Science.gov (United States)

    Morales La Madrid, Andres; Rubin, Charles M; Kohrman, Michael; Pytel, Peter; Cohn, Susan L

    2012-03-01

    Opsoclonus-myoclonus syndrome (OMS) is seen in 2-3% of children with neuroblastoma and is believed to be caused by an autoimmune process elicited by the tumor. Although long-term neurologic sequelae are common in children with OMS, limbic encephalitis has not previously been reported. We report a child who developed limbic encephalitis associated with anti-Hu antibodies, 6 years after her initial diagnosis of neuroblastoma and OMS. This case demonstrates that patients with neuroblastoma and OMS are at risk for developing new paraneoplastic symptoms years after their original diagnosis and emphasizes the need for careful long-term follow-up.

  1. Natural and nosocomial infection in a patient with West Nile encephalitis and extrapyramidal movement disorders.

    Science.gov (United States)

    Solomon, Tom; Fisher, Ann F; Beasley, David W C; Mandava, Pitchaiah; Granwehr, Bruno P; Langsjoen, Hans; Travassos Da Rosa, Amelia P; Barrett, Alan D T; Tesh, Robert B

    2003-06-01

    Since its first recognition in North America in 1999, West Nile virus (WNV) has spread rapidly across the continent, but in many communities, rapid diagnostic tests for detection of WNV infection are not fully available. We describe a patient with extrapyramidal movement disorders and changes in the basal ganglia noted on magnetic resonance images that are characteristic of other flavivirus encephalitides and may help in the recognition of patients with West Nile encephalitis. Detailed molecular analysis suggested that, although our patient received a blood transfusion infected with WNV, the virus that caused his initial infection and encephalitis was probably acquired naturally from a mosquito.

  2. Affinity (tropism) of caprine arthritis encephalitis virus for brain cells.

    Science.gov (United States)

    Adebayo, I A; Olaleye, O D; Awoniyi, T A M

    2010-12-01

    One of the constraints in unraveling the mysteries blurring the advancement of research in the quest to totally put HIV problems under control is getting the appropriate animal model that would truly simulate human cases. This problem is more apparent in studies involving the central nervous system. Consequently, a viable animal model to generate information for the production of drugs and vaccines for the prevention and or control of lentiviral induced dementia in affected host animals is pertinent and vital. In this study, explant cultures prepared from the brain of new-born goat-kid were infected with CaprineArthritis Encephalitis (CAE) virus- a retrovirus affecting goats. The specific brain cell types infected by the (CAE) virus were determined using reverse-transcription polymerase chain reaction (RT-PCR) and transmission electron microscopy (TEM techniques). TEM showed that in 85 - 90% cases, microglia were the cells specifically infected by the virus. Amplification of the genomic sequence of the envelope and the gag genes by RT-PCR confirmed the presence of CAEV proviral DNA in the brain cells of affected animals. No productive infection of the astrocytes was observed. The results of this study showed a lot of similarities in the tropism of CAE virus infection of goat brain cells to that of HIV infection in humans thus suggesting the potential usefulness of the caprine model for the study of HIV neuropathology. The goat model system as a non-primate model therefore could be more adaptable as a simple animal model than primate models with their complexity of anthropological, environmental and safety problems.

  3. Von Willebrand Factor Gene Variants Associate with Herpes simplex Encephalitis.

    Science.gov (United States)

    Abdelmagid, Nada; Bereczky-Veress, Biborka; Atanur, Santosh; Musilová, Alena; Zídek, Václav; Saba, Laura; Warnecke, Andreas; Khademi, Mohsen; Studahl, Marie; Aurelius, Elisabeth; Hjalmarsson, Anders; Garcia-Diaz, Ana; Denis, Cécile V; Bergström, Tomas; Sköldenberg, Birgit; Kockum, Ingrid; Aitman, Timothy; Hübner, Norbert; Olsson, Tomas; Pravenec, Michal; Diez, Margarita

    2016-01-01

    Herpes simplex encephalitis (HSE) is a rare complication of Herpes simplex virus type-1 infection. It results in severe parenchymal damage in the brain. Although viral latency in neurons is very common in the population, it remains unclear why certain individuals develop HSE. Here we explore potential host genetic variants predisposing to HSE. In order to investigate this we used a rat HSE model comparing the HSE susceptible SHR (Spontaneously Hypertensive Rats) with the asymptomatic infection of BN (Brown Norway). Notably, both strains have HSV-1 spread to the CNS at four days after infection. A genome wide linkage analysis of 29 infected HXB/BXH RILs (recombinant inbred lines-generated from the prior two strains), displayed variable susceptibility to HSE enabling the definition of a significant QTL (quantitative trait locus) named Hse6 towards the end of chromosome 4 (160.89-174Mb) containing the Vwf (von Willebrand factor) gene. This was the only gene in the QTL with both cis-regulation in the brain and included several non-synonymous SNPs (single nucleotide polymorphism). Intriguingly, in human chromosome 12 several SNPs within the intronic region between exon 43 and 44 of the VWF gene were associated with human HSE pathogenesis. In particular, rs917859 is nominally associated with an odds ratio of 1.5 (95% CI 1.11-2.02; p-value = 0.008) after genotyping in 115 HSE cases and 428 controls. Although there are possibly several genetic and environmental factors involved in development of HSE, our study identifies variants of the VWF gene as candidates for susceptibility in experimental and human HSE.

  4. Von Willebrand Factor Gene Variants Associate with Herpes simplex Encephalitis.

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    Nada Abdelmagid

    Full Text Available Herpes simplex encephalitis (HSE is a rare complication of Herpes simplex virus type-1 infection. It results in severe parenchymal damage in the brain. Although viral latency in neurons is very common in the population, it remains unclear why certain individuals develop HSE. Here we explore potential host genetic variants predisposing to HSE. In order to investigate this we used a rat HSE model comparing the HSE susceptible SHR (Spontaneously Hypertensive Rats with the asymptomatic infection of BN (Brown Norway. Notably, both strains have HSV-1 spread to the CNS at four days after infection. A genome wide linkage analysis of 29 infected HXB/BXH RILs (recombinant inbred lines-generated from the prior two strains, displayed variable susceptibility to HSE enabling the definition of a significant QTL (quantitative trait locus named Hse6 towards the end of chromosome 4 (160.89-174Mb containing the Vwf (von Willebrand factor gene. This was the only gene in the QTL with both cis-regulation in the brain and included several non-synonymous SNPs (single nucleotide polymorphism. Intriguingly, in human chromosome 12 several SNPs within the intronic region between exon 43 and 44 of the VWF gene were associated with human HSE pathogenesis. In particular, rs917859 is nominally associated with an odds ratio of 1.5 (95% CI 1.11-2.02; p-value = 0.008 after genotyping in 115 HSE cases and 428 controls. Although there are possibly several genetic and environmental factors involved in development of HSE, our study identifies variants of the VWF gene as candidates for susceptibility in experimental and human HSE.

  5. The role of IKKβ in Venezuelan equine encephalitis virus infection.

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    Moushimi Amaya

    Full Text Available Venezuelan equine encephalitis virus (VEEV belongs to the genus Alphavirus, family Togaviridae. VEEV infection is characterized by extensive inflammation and studies from other laboratories implicated an involvement of the NF-κB cascade in the in vivo pathology. Initial studies indicated that at early time points of VEEV infection, the NF-κB complex was activated in cells infected with the TC-83 strain of VEEV. One upstream kinase that contributes to the phosphorylation of p65 is the IKKβ component of the IKK complex. Our previous studies with Rift valley fever virus, which exhibited early activation of the NF-κB cascade in infected cells, had indicated that the IKKβ component underwent macromolecular reorganization to form a novel low molecular weight form unique to infected cells. This prompted us to investigate if the IKK complex undergoes a comparable macromolecular reorganization in VEEV infection. Size-fractionated VEEV infected cell extracts indicated a macromolecular reorganization of IKKβ in VEEV infected cells that resulted in formation of lower molecular weight complexes. Well-documented inhibitors of IKKβ function, BAY-11-7082, BAY-11-7085 and IKK2 compound IV, were employed to determine whether IKKβ function was required for the production of infectious progeny virus. A decrease in infectious viral particles and viral RNA copies was observed with inhibitor treatment in the attenuated and virulent strains of VEEV infection. In order to further validate the requirement of IKKβ for VEEV replication, we over-expressed IKKβ in cells and observed an increase in viral titers. In contrast, studies carried out using IKKβ(-/- cells demonstrated a decrease in VEEV replication. In vivo studies demonstrated that inhibitor treatment of TC-83 infected mice increased their survival. Finally, proteomics studies have revealed that IKKβ may interact with the viral protein nsP3. In conclusion, our studies have revealed that the host IKK

  6. Spatial epidemiology of eastern equine encephalitis in Florida

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    Vander Kelen Patrick T

    2012-11-01

    Full Text Available Abstract Background Eastern Equine Encephalitis virus (EEEV is an alphavirus with high pathogenicity in both humans and horses. Florida continues to have the highest occurrence of human cases in the USA, with four fatalities recorded in 2010. Unlike other states, Florida supports year-round EEEV transmission. This research uses GIS to examine spatial patterns of documented horse cases during 2005–2010 in order to understand the relationships between habitat and transmission intensity of EEEV in Florida. Methods Cumulative incidence rates of EEE in horses were calculated for each county. Two cluster analyses were performed using density-based spatial clustering of applications with noise (DBSCAN. The first analysis was based on regional clustering while the second focused on local clustering. Ecological associations of EEEV were examined using compositional analysis and Euclidean distance analysis to determine if the proportion or proximity of certain habitats played a role in transmission. Results The DBSCAN algorithm identified five distinct regional spatial clusters that contained 360 of the 438 horse cases. The local clustering resulted in 18 separate clusters containing 105 of the 438 cases. Both the compositional analysis and Euclidean distance analysis indicated that the top five habitats positively associated with horse cases were rural residential areas, crop and pastureland, upland hardwood forests, vegetated non-forested wetlands, and tree plantations. Conclusions This study demonstrates that in Florida tree plantations are a focus for epizootic transmission of EEEV. It appears both the abundance and proximity of tree plantations are factors associated with increased risk of EEE in horses and therefore humans. This association helps to explain why there is are spatially distinct differences in the amount of EEE horse cases across Florida.

  7. Peripheral facial palsy in patients with tick-borne encephalitis.

    Science.gov (United States)

    Lotric-Furlan, S; Strle, F

    2012-10-01

    Although tick-borne encephalitis (TBE) has been recognized in Europe for more than 70 years and has been the topic of numerous reports, information on the involvement of facial nerves in the course of the disease is limited. Our study conducted at a single medical centre revealed that facial nerve involvement in the course of TBE in Central Europe is (i) infrequent--it was found in only 11 of 1218 (0.9%) consecutive adult patients diagnosed with TBE; (ii) manifests with unilateral or rarely bilateral peripheral facial palsy (PFP) (nine and two patients, respectively); (iii) appears late in the course of acute illness--in our patients 10-20 days after the onset of the meningoencephalitic phase of TBE, and often after defervescence (in 8/11 patients; 6-13 days after normalization of body temperature); (iv) develops more often in patients with more severe illness, i.e. more frequently in those with encephalitic than in those with meningitic clinical presentation, and more commonly in patients with monophasic than biphasic illness; and (v) has a favourable outcome--our patients had a clinically complete recovery from PFP within 7-90 (median 30) days after its onset. Moreover, the finding of Borrelia infection in 3/11 (27.3%) patients (diagnosis of confirmed Lyme neuroborreliosis was established in 1/11 patients and two patients fulfilled criteria for possible Lyme neuroborreliosis) suggests that in countries where TBE and Lyme borreliosis are endemic, concomitant infection with Borrelia burgdorferi sensu lato should be considered and searched for in patients who develop PFP in the course of TBE.

  8. Nucleoside Inhibitors of Tick-Borne Encephalitis Virus

    Science.gov (United States)

    Eyer, Luděk; Valdés, James J.; Gil, Victor A.; Nencka, Radim; Hřebabecký, Hubert; Šála, Michal; Salát, Jiří; Černý, Jiří; Palus, Martin; De Clercq, Erik

    2015-01-01

    Tick-borne encephalitis virus (TBEV) is a leading cause of human neuroinfections in Europe and Northeast Asia. There are no antiviral therapies for treating TBEV infection. A series of nucleoside analogues was tested for the ability to inhibit the replication of TBEV in porcine kidney cells and human neuroblastoma cells. The interactions of three nucleoside analogues with viral polymerase were simulated using advanced computational methods. The nucleoside analogues 7-deaza-2′-C-methyladenosine (7-deaza-2′-CMA), 2′-C-methyladenosine (2′-CMA), and 2′-C-methylcytidine (2′-CMC) inhibited TBEV replication. These compounds showed dose-dependent inhibition of TBEV-induced cytopathic effects, TBEV replication (50% effective concentrations [EC50]of 5.1 ± 0.4 μM for 7-deaza-2′-CMA, 7.1 ± 1.2 μM for 2′-CMA, and 14.2 ± 1.9 μM for 2′-CMC) and viral antigen production. Notably, 2′-CMC was relatively cytotoxic to porcine kidney cells (50% cytotoxic concentration [CC50] of ∼50 μM). The anti-TBEV effect of 2′-CMA in cell culture diminished gradually after day 3 posttreatment. 7-Deaza-2′-CMA showed no detectable cellular toxicity (CC50 > 50 μM), and the antiviral effect in culture was stable for >6 days posttreatment. Computational molecular analyses revealed that compared to the other two compounds, 7-deaza-2′-CMA formed a large cluster near the active site of the TBEV polymerase. High antiviral activity and low cytotoxicity suggest that 7-deaza-2′-CMA is a promising candidate for further investigation as a potential therapeutic agent in treating TBEV infection. PMID:26124166

  9. Spatiotemporal patterns of Japanese encephalitis in China, 2002-2010.

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    Li-Ya Wang

    Full Text Available OBJECTIVE: The aim of the study is to examine the spatiotemporal pattern of Japanese Encephalitis (JE in mainland China during 2002-2010. Specific objectives of the study were to quantify the temporal variation in incidence of JE cases, to determine if clustering of JE cases exists, to detect high risk spatiotemporal clusters of JE cases and to provide evidence-based preventive suggestions to relevant stakeholders. METHODS: Monthly JE cases at the county level in mainland China during 2002-2010 were obtained from the China Information System for Diseases Control and Prevention (CISDCP. For the purpose of the analysis, JE case counts for nine years were aggregated into four temporal periods (2002; 2003-2005; 2006; and 2007-2010. Local Indicators of Spatial Association and spatial scan statistics were performed to detect and evaluate local high risk space-time clusters. RESULTS: JE incidence showed a decreasing trend from 2002 to 2005 but peaked in 2006, then fluctuated over the study period. Spatial cluster analysis detected high value clusters, mainly located in Southwestern China. Similarly, we identified a primary spatiotemporal cluster of JE in Southwestern China between July and August, with the geographical range of JE transmission increasing over the past years. CONCLUSION: JE in China is geographically clustered and its spatial extent dynamically changed during the last nine years in mainland China. This indicates that risk factors for JE infection are likely to be spatially heterogeneous. The results may assist national and local health authorities in the development/refinement of a better preventive strategy and increase the effectiveness of public health interventions against JE transmission.

  10. Combined alphavirus replicon particle vaccine induces durable and cross-protective immune responses against equine encephalitis viruses.

    Science.gov (United States)

    Reed, Douglas S; Glass, Pamela J; Bakken, Russell R; Barth, James F; Lind, Cathleen M; da Silva, Luis; Hart, Mary Kate; Rayner, Jonathan; Alterson, Kim; Custer, Max; Dudek, Jeanne; Owens, Gary; Kamrud, Kurt I; Parker, Michael D; Smith, Jonathan

    2014-10-01

    Alphavirus replicons were evaluated as potential vaccine candidates for Venezuelan equine encephalitis virus (VEEV), western equine encephalitis virus (WEEV), or eastern equine encephalitis virus (EEEV) when given individually or in combination (V/W/E) to mice or cynomolgus macaques. Individual replicon vaccines or the combination V/W/E replicon vaccine elicited strong neutralizing antibodies in mice to their respective alphavirus. Protection from either subcutaneous or aerosol challenge with VEEV, WEEV, or EEEV was demonstrated out to 12 months after vaccination in mice. Individual replicon vaccines or the combination V/W/E replicon vaccine elicited strong neutralizing antibodies in macaques and demonstrated good protection against aerosol challenge with an epizootic VEEV-IAB virus, Trinidad donkey. Similarly, the EEEV replicon and V/W/E combination vaccine elicited neutralizing antibodies against EEEV and protected against aerosol exposure to a North American variety of EEEV. Both the WEEV replicon and combination V/W/E vaccination, however, elicited poor neutralizing antibodies to WEEV in macaques, and the protection conferred was not as strong. These results demonstrate that a combination V/W/E vaccine is possible for protection against aerosol challenge and that cross-interference between the vaccines is minimal. Importance: Three related viruses belonging to the genus Alphavirus cause severe encephalitis in humans: Venezuelan equine encephalitis virus (VEEV), western equine encephalitis virus (WEEV), and eastern equine encephalitis virus (EEEV). Normally transmitted by mosquitoes, these viruses can cause disease when inhaled, so there is concern that these viruses could be used as biological weapons. Prior reports have suggested that vaccines for these three viruses might interfere with one another. We have developed a combined vaccine for Venezuelan equine encephalitis, western equine encephalitis, and eastern equine encephalitis expressing the surface

  11. Acute infection with venezuelan equine encephalitis virus replicon particles catalyzes a systemic antiviral state and protects from lethal virus challenge.

    Science.gov (United States)

    Konopka, Jennifer L; Thompson, Joseph M; Whitmore, Alan C; Webb, Drue L; Johnston, Robert E

    2009-12-01

    The host innate immune response provides a critical first line of defense against invading pathogens, inducing an antiviral state to impede the spread of infection. While numerous studies have documented antiviral responses within actively infected tissues, few have described the earliest innate response induced systemically by infection. Here, utilizing Venezuelan equine encephalitis virus (VEE) replicon particles (VRP) to limit infection to the initially infected cells in vivo, a rapid activation of the antiviral response was demonstrated not only within the murine draining lymph node, where replication was confined, but also within distal tissues. In the liver and brain, expression of interferon-stimulated genes was detected by 1 to 3 h following VRP footpad inoculation, reaching peak expression of >100-fold over that in mock-infected animals. Moreover, mice receiving a VRP footpad inoculation 6, 12, or 24 h prior to an otherwise lethal VEE footpad challenge were completely protected from death, including a drastic reduction in challenge virus titers. VRP pretreatment also provided protection from intranasal VEE challenge and extended the average survival time following intracranial challenge. Signaling through the interferon receptor was necessary for antiviral gene induction and protection from VEE challenge. However, VRP pretreatment failed to protect mice from a heterologous, lethal challenge with vesicular stomatitis virus, yet conferred protection following challenge with influenza virus. Collectively, these results document a rapid modulation of the host innate response within hours of infection, capable of rapidly alerting the entire animal to pathogen invasion and leading to protection from viral disease.

  12. Outcomes of West Nile encephalitis patients after 1 year of West Nile encephalitis outbreak in Kerala, India: A follow-up study.

    Science.gov (United States)

    Balakrishnan, Anukumar; Thekkekara, Romy Jose; Tandale, Babasheb V

    2016-11-01

    We reported an acute encephalitis syndrome outbreak in Alappuzha district in Kerala, India during the year 2011. The etiology was confirmed to be West Nile virus lineage 1. Many encephalitis patients from this outbreak exhibited neurological sequelae post recovery. This study was aimed to assess the clinical outcomes of West Nile encephalitis confirmed case-patients after 1 year of acute illness. Forty West Nile virus confirmed encephalitis patients were selected from the 2011 outbreak was included in this study. Out of 40 cases, only 30 survived after 12 months. Among these 30 recovered case-patients, 27 (90%) consented for clinical follow-up and 23 (73.67%) of them consented for assessment of cognitive impairment and deposition of blood sample for antibody testing. The most common symptom observed in these patients was fatigue (25.93%). Other symptoms included dizziness (7.4%), decreased sense of hearing (7.4%) and decreased sense of smell (7.4%). Reduced power in limbs was found in 33.33% of the cases. Most of the patients (23.1%) were dependent on others for normal daily living activities. The patients also had probable risk of poor cognition (29.41%) and dementia (57.14%). None of the patients were positive for WNV specific IgM at 12 months post onset of disease. The study concluded that the long-term sequelae were noticed in WNV positive patients. Prevention effort should be focused on the elderly (≥60 years old) people who have a higher risk of severe sequelae. The state health authorities should create awareness among people in order to prevent the transmission of disease. J. Med. Virol. 88:1856-1861, 2016. © 2016 Wiley Periodicals, Inc.

  13. Glucagon-Like Peptide-1 Analog, Liraglutide, Delays Onset of Experimental Autoimmune Encephalitis in Lewis Rats

    Science.gov (United States)

    DellaValle, Brian; Brix, Gitte S.; Brock, Birgitte; Gejl, Michael; Landau, Anne M.; Møller, Arne; Rungby, Jørgen; Larsen, Agnete

    2016-01-01

    Introduction: Recent findings indicate that metabolic disturbances are involved in multiple sclerosis (MS) pathology and influence the susceptibility to treatment, directing attention toward anti-diabetic drugs such as metformin and pioglitazone. Liraglutide, a drug of the glucagon-like peptide-1 (GLP-1) family, is also anti-diabetic and weight-reducing and is, moreover, directly neuroprotective and anti-inflammatory in a broad spectrum of experimental models of brain disease. In this study we investigate the potential for this FDA-approved drug, liraglutide, as a treatment for MS by utilizing the experimental model, experimental autoimmune encephalitis (EAE). Methods: EAE was induced in 30 female Lewis rats that subsequently received twice-daily liraglutide (200 μg/kg s.c.) or saline. Healthy controls were included (saline, n = 6, liraglutide, n = 7). Clinical score and weight were assessed daily by blinded observers. Animals were killed at peak disease severity (day 11) or if exceeding humane endpoint (clinical score ≥4). Protein levels of manganese superoxide dismutase (MnSOD), amyloid precursor protein (APP), and glial fibrillary acidic protein (GFAP) were determined. Results: Liraglutide treatment delayed disease onset (group clinical score significantly >0) by 2 days and markedly reduced disease severity (median clinical score 2 vs. 5; p = 0.0003). Fourteen of 15 (93%) of vehicle-treated rats reached the humane endpoint (clinical score ≥4) by day 11 compared to 5 of 15 (33%) of liraglutide-treated rats (p = 0.0004). Liraglutide substantially increased the mitochondrial antioxidant MnSOD (p < 0.01) and reduced the neurodegenerative marker APP (p = 0.036) in the brain. GFAP levels were not significantly changed with drug treatment (p = 0.09). Conclusion: We demonstrate, for the first time, that liraglutide treatment delays onset of EAE in Lewis rats and is associated with improved protective capacity against oxidative stress. These data suggest GLP-1

  14. Mycotic encephalitis, sinus osteomyelitis, and guttural pouch mycosis in a 3-year-old Arabian colt.

    Science.gov (United States)

    Hunter, Barbara; Nation, Patrick N

    2011-12-01

    Mycotic encephalitis caused severe ataxia and other neurologic deficits in a horse. The finding of a single, large focus of cerebral malacia, with histopathologic evidence of fungal elements, suggested infection was a result of direct transfer from the frontal sinuses, rather than hematogenous spread from the guttural pouch.

  15. Human parvovirus 4 as potential cause of encephalitis in children, India.

    Science.gov (United States)

    Benjamin, Laura A; Lewthwaite, Penny; Vasanthapuram, Ravi; Zhao, Guoyan; Sharp, Colin; Simmonds, Peter; Wang, David; Solomon, Tom

    2011-08-01

    To investigate whether uncharacterized infectious agents were associated with neurologic disease, we analyzed cerebrospinal fluid specimens from 12 children with acute central nervous system infection. A high-throughput pyrosequencing screen detected human parvovirus 4 DNA in cerebrospinal fluid of 2 children with encephalitis of unknown etiology.

  16. Review of tick-borne encephalitis and vaccines : clinical and economical aspects

    NARCIS (Netherlands)

    Smit, Renata; Postma, Maarten J

    2015-01-01

    Tick-borne encephalitis (TBE) disease is an increasing burden not only locally but also globally. In most endemic countries, vaccination coverage is too low to reduce the TBE burden significantly; however, vaccination is the most effective protection against TBE, with various vaccines currently avai

  17. Dengue encephalitis with predominant cerebellar involvement: Report of eight cases with MR and CT imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Hegde, Vinay; Bhat, Maya; Prasad, Chandrajit; Gupta, A.K.; Saini, Jitender [National Institute of Mental Health and Neurosciences, Department of Neuroimaging and Interventional Radiology, Bangalore, Karnataka (India); Aziz, Zarina [Sri Sathya Sai Institute of Medical Science, Department of Radiology, Bangalore (India); Kumar, Sharath [Apollo Hospital, Department of Neuroradiology, Bangalore (India); Netravathi, M. [National Institute of Mental Health and Neurosciences, Department of Neurology, Bangalore (India)

    2014-11-01

    CNS dengue infection is a rare condition and the pattern of brain involvement has not been well described. We report the MR imaging (MRI) features in eight cases of dengue encephalitis. We retrospectively searched cases of dengue encephalitis in which imaging was performed. Eight cases (three men, five women; age range: 8-42 years) diagnosed with dengue encephalitis were included in the study. MR studies were performed on 3-T and 1.5-T MR clinical systems. Two neuroradiologists retrospectively reviewed the MR images and analysed the type of lesions, as well as their distribution and imaging features. All eight cases exhibited MRI abnormalities and the cerebellum was involved in all cases. In addition, MRI signal changes were also noted in the brainstem, thalamus, basal ganglia, internal capsule, insula, mesial temporal lobe, and cortical and cerebral white matter. Areas of susceptibility, diffusion restriction, and patchy post-contrast enhancement were the salient imaging features in our cohort of cases. A pattern of symmetrical cerebellar involvement and presence of microbleeds/haemorrhage may serve as a useful imaging marker and may help in the diagnosis of dengue encephalitis. (orig.)

  18. Successful treatment with ganciclovir of presumed Epstein-Barr meningo-encephalitis following bone marrow transplant

    NARCIS (Netherlands)

    Dellemijn, P L; Brandenburg, A; Niesters, H G; van den Bent, M J; Rothbarth, P H; Vlasveld, L T

    1995-01-01

    Epstein-Barr virus-specific polymerase chain reaction was used to diagnose EBV-meningo-encephalitis in a bone marrow transplant recipient. The patient made complete recovery with ganciclovir treatment. Pitfalls in diagnosis with EBV-PCR and the potential therapeutic efficacy of ganciclovir in EBV in

  19. Japanese encephalitis in a Danish short-term traveler to Cambodia

    DEFF Research Database (Denmark)

    Werlinrud, Anne M; Christiansen, Claus B; Koch, Anders

    2011-01-01

    We present a recent case of Japanese encephalitis in a Danish male traveler to Cambodia, who we believe is the second Danish case within the last 15 years. Although both this and a number of other travel-related cases occurred in short-term travelers, change in vaccination recommendations...

  20. Susceptibility of a North American Culex quinquefasciatus to Japanese encephalitis virus

    Science.gov (United States)

    Japanese encephalitis virus (JEV) is a flavivirus that is transmitted by Culex (Cx.) tritaeniorhynchus in tropical and subtropical regions of Asia. The endemic transmission cycle involves domestic pigs and avian species that serve as amplification hosts; humans are incidental hosts that cannot devel...

  1. Comparative evaluation of the FilmArray meningitis/encephalitis molecular panel in a pediatric population.

    Science.gov (United States)

    Graf, Erin H; Farquharson, Maria Victoria; Cárdenas, Ana María

    2017-01-01

    We compared an FDA cleared molecular meningitis/encephalitis panel to lab developed viral PCRs and bacterial culture. Of the 67 viral PCR or bacterial culture-positive samples, 92.5% were positive for the same target by the panel. Of the 66 negative samples tested, no targets were detected by the panel, for an agreement of 96.2%.

  2. Fatal Granulomatous Amebic Encephalitis Due to Balamuthia mandrillaris in New Mexico: A Case Report.

    Science.gov (United States)

    Pindyck, Talia N; Dvorscak, Lauren E; Hart, Blaine L; Palestine, Michael D; Gallant, Joel E; Allen, Sarah E; SantaCruz, Karen S

    2014-09-01

    Balamuthia mandrillaris is a free-living amoeba that can cause granulomatous amebic encephalitis (GAE). We report a case in an individual with a history of alcohol abuse, cocaine use, and ditch water exposure. This is the first reported case of GAE due to B mandrillaris in New Mexico.

  3. THE GROWTH OF JAPANESE AND WEST NILE ENCEPHALITIS VIRUSES IN TISSUE CULTURES,

    Science.gov (United States)

    extinguishing it with a homologous immune serum. In the HeLa cells a specific cytopathogenic effect was registered after six cultural passages of the strain R...1. The cytopathogenic effect was retained in subinoculation. The West Nile encephalitis virus induced a cytopathogenic effect in the HeLa cells from

  4. Paraneoplastic limbic encephalitis presenting as a neurological emergency: a case report

    Directory of Open Access Journals (Sweden)

    Mehta Brijesh P

    2010-03-01

    Full Text Available Abstract Introduction Paraneoplastic limbic encephalitis remains a challenging clinical diagnosis with poor outcome if it is not recognized and treated early in the course of the disease. Case Presentation A 65-year-old Caucasian woman presented with generalized tonic-clonic seizures and increasing confusion shortly after a lung biopsy that led to the diagnosis of small-cell lung cancer. She had a complicated hospital course, and had recurrent respiratory distress due to aspiration pneumonia, and fluctuating mental status and seizures that were refractory to anti-epileptic drug treatment. Routine laboratory testing, magnetic resonance imaging of the brain, electroencephalogram, lumbar puncture, serum and cerebrospinal fluid tests for paraneoplastic antibodies, and chest computed tomography were performed on our patient. The diagnosis was paraneoplastic limbic encephalitis in the setting of small-cell lung cancer with positive N-type voltage-gated calcium channel antibody titer. Anti-epileptic drugs for seizures, chemotherapy for small-cell lung cancer, and intravenous immunoglobulin and steroids for paraneoplastic limbic encephalitis led to a resolution of her seizures and improved her mental status. Conclusion Early recognition of paraneoplastic limbic encephalitis and prompt intervention with immune therapies at the onset of presentation will probably translate into more favorable neurological outcomes.

  5. Prevalence of intrathecal acyclovir resistant virus in herpes simplex encephalitis patients

    NARCIS (Netherlands)

    J.G. Mitterreiter (Johanna G.); M.J. Titulaer (Maarten); G.P. van Nierop (Gijsbert); J.J.A. van Kampen (Jeroen); G.I. Aron; A.D.M.E. Osterhaus (Albert); G.M.G.M. Verjans (George); W.J.D. Ouwendijk (Werner )

    2016-01-01

    textabstractHerpes simplex encephalitis (HSE) is a life-threatening complication of herpes simplex virus (HSV) infection. Acyclovir (ACV) is the antiviral treatment of choice, but may lead to emergence of ACV-resistant (ACVR) HSV due to mutations in the viral UL23 gene encoding for the ACV-targeted

  6. Acute Measles Encephalitis in an Immigrant Syrian Child: Case Report and Review of the Literature.

    Science.gov (United States)

    Al-Qayoudhi, Abdullah; Al-Kindi, Hanan; Meki, Nabil; Al-Maani, Amal

    2016-03-01

    The introduction of measles vaccination programs and broad coverage worldwide has meant this infection a rare encounter for pediatricians. In Oman, with almost 100% measles vaccination coverage for children, this infection disappeared from the list of fever and rash differential diagnoses. Encephalitis is a well-known complication of measles infection and sometimes can be the only manifestation especially in adults. We report a seven-year-old Syrian immigrant who was admitted to the Royal Hospital, Muscat, with acute encephalitis secondary to wild measles infection. Although she had a classical presentation of measle infection, the diagnosis was missed in the private and regional hospital she attended before getting referred to Royal Hospital. She was later identified to be exposed to an outbreak of the infection in an unvaccinated population. Magnetic resonance imaging showed high signal intensity of both basal ganglia suggestive of measles encephalitis. The diagnosis was confirmed by detection of measles virus from her urine and blood, and a throat swab. The isolated measles virus was D8 serotype, which was prevalent in Syria around the same time. The child was treated with steroids and vitamin A. She achieved full recovery despite her severe presentation. A high degree of suspicion for measles infection should be maintained in unvaccinated children with a compatible presentation of the infection or its complications. There might be a role for steroid use in cases of acute measles encephalitis.

  7. Acute Measles Encephalitis in an Immigrant Syrian Child: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Abdullah Al-Qayoudhi

    2016-03-01

    Full Text Available The introduction of measles vaccination programs and broad coverage worldwide has meant this infection a rare encounter for pediatricians. In Oman, with almost 100% measles vaccination coverage for children, this infection disappeared from the list of fever and rash differential diagnoses. Encephalitis is a well-known complication of measles infection and sometimes can be the only manifestation especially in adults. We report a seven-year-old Syrian immigrant who was admitted to the Royal Hospital, Muscat, with acute encephalitis secondary to wild measles infection. Although she had a classical presentation of measle infection, the diagnosis was missed in the private and regional hospital she attended before getting referred to Royal Hospital. She was later identified to be exposed to an outbreak of the infection in an unvaccinated population. Magnetic resonance imaging showed high signal intensity of both basal ganglia suggestive of measles encephalitis. The diagnosis was confirmed by detection of measles virus from her urine and blood, and a throat swab. The isolated measles virus was D8 serotype, which was prevalent in Syria around the same time. The child was treated with steroids and vitamin A. She achieved full recovery despite her severe presentation. A high degree of suspicion for measles infection should be maintained in unvaccinated children with a compatible presentation of the infection or its complications. There might be a role for steroid use in cases of acute measles encephalitis.

  8. Encephalitis Caused by Pathogens Transmitted through Organ Transplants, United States, 2002–2013

    Centers for Disease Control (CDC) Podcasts

    2014-10-21

    Dr. Mike Miller reads an abridged version of the article, Encephalitis Caused by Pathogens Transmitted through Organ Transplants, United States, 2002–2013.  Created: 10/21/2014 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 10/23/2014.

  9. 抗NMDA受体脑炎三例误诊原因分析并文献复习%Misdiagnosis Analysis of Anti-NMDAR Encephalitis

    Institute of Scientific and Technical Information of China (English)

    李莹; 夏振西; 刘楠; 丁继朝; 黄勇华

    2015-01-01

    目的:加强对抗N-甲基-D-天冬氨酸( N-methyl-D-asparate, NMDA)受体脑炎临床特点的认识,减少临床误诊。方法对我科收治的3例抗NMDA受体脑炎误诊病例资料进行回顾性分析并复习相关文献。结果本组2例以癫痫发作就诊,1例以精神行为异常就诊。2例在疾病初期误诊为病毒性脑炎,1例误诊为脑胶质瘤。入院后因脑脊液病毒学检测阴性或血肿瘤标志物正常,综合分析病情考虑自身免疫性脑炎可能,行脑脊液抗NMDA受体抗体检测阳性,诊断为抗NMDA受体脑炎。给予糖皮质激素和(或)免疫球蛋白治疗,症状控制,生活可自理,但遗留记忆力稍减退和(或)轻度反应迟钝。结论接诊以癫痫或精神行为异常起病,伴有运动异常或自主神经症状者,应考虑抗NMDA受体脑炎可能,及时行血或脑脊液抗NMDA受体抗体检测明确诊断,以免误漏诊。%Objective To improve awareness of N-methyl-D-aspartate receptor encephalitis and prevent misdiagnosis. Methods The misdiagnosis cause was retrospective analyzed for 3 cases in our department and related literature was re-viewed. Results In 3 cases, 2 cases showed seizure and 1 case showed behavioral abnormalities at onset. 2 cases were misdi-agnosed as viral encephalitis and 1 case was misdiagnosed as brain glioma on admission. The virus testing in cerebrospinal flu-id ( CSF) and the serum tumor maker were normal. Autoimmune encephalitis was considered based on different clinical symp-toms. Lumber puncture was performed and anti-NMDAR antibody was positive in CSF. Anti-NMDAR encephalitis was con-firmed. The patients were treated with glucocorticoids and/or immune globulin and recovered with mild memory loss and/or dull reaction. Conclusion Anti-NMDAR encephalitis should be considered if the patients show seizure or psychological symp-toms with movement disorders and autonomic dysfunction. To test anti-NMDAR antibody in CSF and/or in serum is necessary in

  10. Complexity of the Microglial Activation Pathways that Drive Innate Host Responses During Lethal Alphavirus Encephalitis in Mice

    Directory of Open Access Journals (Sweden)

    Nilufer Esen

    2012-04-01

    Full Text Available Microglia express multiple TLRs (Toll-like receptors and provide important host defence against viruses that invade the CNS (central nervous system. Although prior studies show these cells become activated during experimental alphavirus encephalitis in mice to generate cytokines and chemokines that influence virus replication, tissue inflammation and neuronal survival, the specific PRRs (pattern recognition receptors and signalling intermediates controlling microglial activation in this setting remain unknown. To investigate these questions directly in vivo, mice ablated of specific TLR signalling molecules were challenged with NSV (neuroadapted Sindbis virus and CNS viral titres, inflammatory responses and clinical outcomes followed over time. To approach this problem specifically in microglia, the effects of NSV on primary cells derived from the brains of wild-type and mutant animals were characterized in vitro. From the standpoint of the virus, microglial activation required viral uncoating and an intact viral genome; inactivated virus particles did not elicit measurable microglial responses. At the level of the target cell, NSV triggered multiple PRRs in microglia to produce a broad range of inflammatory mediators via non-overlapping signalling pathways. In vivo, disease survival was surprisingly independent of TLR-driven responses, but still required production of type-I IFN (interferon to control CNS virus replication. Interestingly, the ER (endoplasmic reticulum protein UNC93b1 facilitated host survival independent of its known effects on endosomal TLR signalling. Taken together, these data show that alphaviruses activate microglia via multiple PRRs, highlighting the complexity of the signalling networks by which CNS host responses are elicited by these infections.

  11. Clinical Significance of Human Metapneumovirus in Refractory Status Epilepticus and Encephalitis: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aysel Vehapoglu

    2015-01-01

    Full Text Available Encephalitis is a complex neurological disease that is associated with significant morbidity and mortality, and the etiology of the disease is often not identified. Human metapneumovirus (hMPV is a common cause of upper and lower respiratory tract infections in children. Few reports are available showing possible involvement of hMPV in development of neurologic complications. Here, we describe an infant, the youngest case in literature, with refractory status epilepticus and severe encephalitis in whom hMPV was detected in respiratory samples and review diagnostic workup of patient with encephalitis.

  12. Herpes simplex type 2 encephalitis and methotrexate medication: a fortuitous or causative association in a patient with spondyloarthritis?

    Science.gov (United States)

    Lupo, Julien; Dos Santos, Ophélie; Germi, Raphaele; Baccard-Longère, Monique; Stahl, Jean-Paul; Epaulard, Olivier; Morand, Patrice

    2016-11-23

    It is unclear whether immunosuppression is a risk factor for herpes encephalitis. Herein, we describe a rare case of herpes simplex virus (HSV) type 2 encephalitis in a patient treated with low-dose methotrexate for HLA-B27-associated spondyloarthritis. The patient was successfully treated with acyclovir but presented sequelae of encephalitis. Here we discuss the possible role of low-dose MTX therapy as a risk factor of neurological herpes reactivation and severe disease. The host-related and viral risk factors are also addressed.

  13. A Special Electroencephalography Pattern Might Help in the Diagnosis of Antibody-positive Encephalitis

    Institute of Scientific and Technical Information of China (English)

    Li-Ping Mei; Li-Ping Li; Jing Ye; Yu-Ping Wang; Jun Zhao; Tong Zhang

    2015-01-01

    Background:Patterns observed with electroencephalography (EEG) for patients who have encephalitis are usually known as generalized nonspecific cerebral abnormalities.The aim of this study was to investigate the presence of a special EEG pattern for patients with encephalitis and to explore features related to this special and uncommon pattern.Methods:EEG monitoring was performed for every patient aged > 15 years with encephalitis who was hospitalized between December 2011 and March 2014.Clinical characteristics and EEG recordings were collected and evaluated.Results:Fifty-two patients with encephalitis were enrolled in our study with a 2-h median EEG recording time,and extreme beta brushes (EBBs) occurred in 17 patients (32.7%).Its presence was not significant regarding gender,age,psychiatric medication use,EEG rhythmic disorganization (P > 0.05).Nevertheless,among the patients with EBBs,nine patients (52.9%) had epileptic seizures that had a significant detection rate (P < 0.05); moreover,the cerebrospinal fluid (CSF) or serum of 15 patients (88.2%) with EBBs was positive for antibodies (P < 0.05).Four patients (23.5%) who had EBB had corresponding regional distributions on neuroimaging scans.The EBBs completely correlated with the regional distributions of spike discharges for four patients.Conclusion:EBB is a special EEG pattern for patients with encephalitis,especially those with epileptic seizures or who have antibody-positive CSF/serum,and should be considered in clinical practice.

  14. Transcranial direct current stimulation improves seizure control in patients with Rasmussen encephalitis.

    Science.gov (United States)

    Tekturk, Pinar; Erdogan, Ezgi Tuna; Kurt, Adnan; Kocagoncu, Ece; Kucuk, Zeynep; Kinay, Demet; Yapici, Zuhal; Aksu, Serkan; Baykan, Betul; Karamursel, Sacit

    2016-03-01

    Rasmussen encephalitis is associated with severe seizures that are unresponsive to antiepileptic drugs, as well as immunosuppressants. Transcranial direct current stimulation (t-DCS) is a non-invasive and safe method tried mostly for focal epilepsies with different aetiologies. To date, there is only one published study with two case reports describing the effect of t-DCS in Rasmussen encephalitis. Our aim was to investigate the effect of t-DCS on seizures in Rasmussen encephalitis and to clarify its safety. Five patients (mean age: 19; three females), diagnosed with Rasmussen encephalitis were included in this study. Patients received first cathodal, then anodal (2 mA for 30 minutes on three consecutive days for non-sham stimulations), and finally sham stimulation with two-month intervals, respectively. Three patients received classic (DC) cathodal t-DCS whereas two patients received cathodal stimulation with amplitude modulation at 12 Hz. Afterwards, all patients received anodal stimulation with amplitude modulation at 12 Hz. In the last part of the trial, sham stimulation (a 60-second stimulation with gradually decreasing amplitude to zero in the last 15 seconds) was applied to three patients. Maximum current density was 571 mA/m2 using 70 mm x 50 mm wet sponge electrodes with 2-mA maximum, current controlled stimulator, and maximum charge density was 1028 C/m2 for a 30-minute stimulation period. After cathodal stimulation, all but one patient had a greater than 50% decrease in seizure frequency. Two patients who received modulated cathodal t-DCS had better results. The longest positive effect lasted for one month. A second trial with modulated anodal stimulation and a third with sham stimulation were not effective. No adverse effect was reported with all types of stimulations. Both classic and modulated cathodal t-DCS may be suitable alternative methods for improving seizure outcome in Rasmussen encephalitis patients.

  15. Rapidly-progressive catatonia responsive to zolpidem in a patient with ovarian teratoma-associated paraneoplastic encephalitis.

    Science.gov (United States)

    Amorim, Edilberto; McDade, Eric M

    2016-08-01

    Psychiatric symptoms and catatonia are key components of the clinical presentation of paraneoplastic encephalitis; additionally symptoms can be long-lasting and often difficult to treat. We report a 73-year-old patient with rapidly progressive catatonia not responsive to immunotherapy, tumor resection, electroconvulsive therapy, or benzodiazepines who had significant improvement after zolpidem administration. This report suggests that zolpidem is an option in the treatment of patients with refractory catatonia and paraneoplastic encephalitis.

  16. Influence of Malnutrition on Adverse Outcome in Children with Confirmed or Probable Viral Encephalitis: A Prospective Observational Study

    Directory of Open Access Journals (Sweden)

    Priyanka Singh

    2015-01-01

    Full Text Available A prospective observational study was conducted in a tertiary care teaching hospital from August 2008 to August 2009 to explore the independent predictors of adverse outcome in the patients with confirmed/probable viral encephalitis. The primary outcome variable was the incidence of adverse outcomes defined as death or severe neurological deficit such as loss of speech, motor deficits, behavioural problems, blindness, and cognitive impairment. Patients with confirmed or probable viral encephalitis were classified into two groups based on their Z-score of weight-for-age as per WHO growth charts. Group I. Patients with confirmed or probable viral encephalitis with weight-for-age (W/A Z-scores below −2SD were classified as undernourished. Group II. Patients with confirmed or probable viral encephalitis were classified as having normal nutritional status (weight-for-age Z-score >−2SD. A total of 114 patients were classified as confirmed or probable viral encephalitis based on detailed investigations. On multivariate logistic regression, undernutrition (adjusted OR: 5.05; 95% CI: 1.92 to 13.44 and requirement of ventilation (adjusted OR: 6.75; 95% CI: 3.63 to 77.34 were independent predictors of adverse outcomes in these patients. Thus, the results from our study highlight that the association between undernutrition and adverse outcome could be extended to the patients with confirmed/probable viral encephalitis.

  17. A historical analysis of the relationship between encephalitis lethargica and postencephalitic parkinsonism: a complex rather than a direct relationship.

    Science.gov (United States)

    Vilensky, Joel A; Gilman, Sid; McCall, Sherman

    2010-07-15

    Postencephalitic parkinsonism has been considered unique among disorders with parkinsonian features because it is believed to have a unitary etiology associated with the virus that presumably caused encephalitis lethargica. Careful analysis of the historical record, however, suggests that this relationship is more complex than commonly perceived. In most cases, the diagnosis of acute encephalitis lethargica was made post hoc, and virtually any catarrh-like illness was considered to have represented encephalitis lethargica, often after an oral history-taking that was undoubtedly subject to patient recall and physician bias. Also, postencephalitic parkinsonism and oculogyric crises were not recognized as sequelae to encephalitis lethargica until well after other sequelae such as movement disorders and mental disturbances had been identified (see previous paper). We suggest here that the relationship between encephalitis lethargica and postencephalitic parkinsonism is not simplistic, i.e., encephalitis lethargica was not solely responsible for the etiology of postencephalitic parkinsonism, thus aligning the latter with most other parkinsonian disorders that are now believed to have multiple causes.

  18. Scavenger receptor b2 as a receptor for hand, foot, and mouth disease and severe neurological diseases.

    Science.gov (United States)

    Yamayoshi, Seiya; Fujii, Ken; Koike, Satoshi

    2012-01-01

    Enterovirus 71 (EV71) is one of the major causative agents of hand, foot, and mouth disease (HFMD). Infection with EV71 is occasionally associated with severe neurological diseases such as acute encephalitis, acute flaccid paralysis, and cardiopulmonary failure. Because cellular receptors for viruses play an important role in cell, tissue, and species tropism, it is important to identify and characterize the receptor molecule. Recently, cellular receptors and host factors that stimulate EV71 infection have been identified. Several lines of evidence suggest that scavenger receptor class B, member 2 (SCARB2) plays critical roles in efficient EV71 infection and the development of disease in humans. In this review, we will summarize the findings of recent studies on EV71 infection and on the roles of SCARB2.

  19. Blockage of indoleamine 2,3-dioxygenase regulates Japanese encephalitis via enhancement of type I/II IFN innate and adaptive T-cell responses

    OpenAIRE

    2016-01-01

    Background Japanese encephalitis (JE), a leading cause of viral encephalitis, is characterized by extensive neuroinflammation following infection with neurotropic JE virus (JEV). Indoleamine 2,3-dioxygenase (IDO) has been identified as an enzyme associated with immunoregulatory function. Although the regulatory role of IDO in viral replication has been postulated, the in vivo role of IDO activity has not been fully addressed in neurotropic virus-caused encephalitis. Methods Mice in which IDO ...

  20. Unusual amnesia in a patient with VGKC-Ab limbic encephalitis: a case study.

    Science.gov (United States)

    Kartsounis, Luke D; de Silva, Rajith

    2011-04-01

    We describe the case of a patient with confirmed voltage-gated potassium channel antibody-associated encephalitis (VGKC-Ab). MRI studies revealed bilateral hyper-intensity in the hippocampi, with their volumes preserved. At presentation, the patient's anterograde and retrograde memory skills were found to be impaired and he showed fluctuation in his ability to recall familiar information. Following treatment with immunotherapy, his condition improved considerably and, in a series of follow up assessments, he performed satisfactorily (i.e., within the average range or above) on formal tests of memory, as well as on a range of other cognitive tests, including tests of executive function. By contrast, in the context of contemporaneous unstructured interviews, he showed a strong tendency to confabulate. We argue that the reported case broadens the phenomenology of VGKC-Ab limbic encephalitis and raises important theoretical questions about the aetiology of this patient's most unusual memory disorder.

  1. Limbic encephalitis. A rare presentation of the small-cell lung carcinoma.

    Science.gov (United States)

    den Hollander, A M; van Hulst, A M; Meerwaldt, J D; Haasjes, J G

    1989-11-01

    Two patients with an acute organic brain syndrome and accompanying neurological symptoms are described. Extensive work up showed that both patients suffered from small-cell lung cancer. Cerebral metastases were absent. Following chemotherapy and radiotherapy to the primary tumor one of the two patients showed a complete remission of psychiatric symptoms for one year. A paraneoplastic origin of this syndrome, in the literature known as limbic encephalitis, is postulated. The exact cause of this syndrome is yet unknown. Recent research reveals data indicating an immunological pathogenesis. The major clinical importance of this (neuro)-psychiatric syndrome is that its appearance may serve as a warning sign for an occult malignancy; furthermore, effective treatment of the primary malignancy can reverse the encephalitis. Thus antitumor therapy can result in a prolonged survival and considerably improved quality of life.

  2. A tropical menace of co-infection of Japanese encephalitis and neurocysticercosis in two children

    Directory of Open Access Journals (Sweden)

    Sangeetha Yoganathan

    2016-01-01

    Full Text Available Japanese encephalitis (JE is a mosquito borne encephalitis caused by Flavivirus. Neurocysticercosis (NCC is a parasitic disease of the central nervous system caused by Taenia solium. In this report, we describe the clinical profile, imaging findings, and outcome of two children with JE and coexisting NCC. Eleven and thirteen-year-old boys from the same town of Jharkhand state were brought with history of fever, seizures, altered sensorium, and extrapyramidal symptoms. Dystonia, hypomimia, bradykinesia, and dyskinesia were observed. Meige syndrome observed in one of the children is a novel finding. Magnetic resonance imaging of the brain revealed findings suggestive of JE with cysticercal granulomas. There are few reports of coexistence of JE and NCC in children. Both children were treated with ribavirin, and follow-up imaging had shown significant resolution of signal changes. Both the children had shown marked clinical improvement. Ribavirin was found to beneficial in reducing the morbidity in our patients.

  3. A tropical menace of co-infection of Japanese encephalitis and neurocysticercosis in two children

    Science.gov (United States)

    Yoganathan, Sangeetha; Sudhakar, Sniya Valsa; Thomas, Maya Mary; Yadav, Vikas Kapildeo

    2016-01-01

    Japanese encephalitis (JE) is a mosquito borne encephalitis caused by Flavivirus. Neurocysticercosis (NCC) is a parasitic disease of the central nervous system caused by Taenia solium. In this report, we describe the clinical profile, imaging findings, and outcome of two children with JE and coexisting NCC. Eleven and thirteen-year-old boys from the same town of Jharkhand state were brought with history of fever, seizures, altered sensorium, and extrapyramidal symptoms. Dystonia, hypomimia, bradykinesia, and dyskinesia were observed. Meige syndrome observed in one of the children is a novel finding. Magnetic resonance imaging of the brain revealed findings suggestive of JE with cysticercal granulomas. There are few reports of coexistence of JE and NCC in children. Both children were treated with ribavirin, and follow-up imaging had shown significant resolution of signal changes. Both the children had shown marked clinical improvement. Ribavirin was found to beneficial in reducing the morbidity in our patients. PMID:27606026

  4. Venezuelan equine encephalitis virus activity in the Gulf Coast region of Mexico, 2003-2010.

    Directory of Open Access Journals (Sweden)

    A Paige Adams

    Full Text Available Venezuelan equine encephalitis virus (VEEV has been the causative agent for sporadic epidemics and equine epizootics throughout the Americas since the 1930s. In 1969, an outbreak of Venezuelan equine encephalitis (VEE spread rapidly from Guatemala and through the Gulf Coast region of Mexico, reaching Texas in 1971. Since this outbreak, there have been very few studies to determine the northward extent of endemic VEEV in this region. This study reports the findings of serologic surveillance in the Gulf Coast region of Mexico from 2003-2010. Phylogenetic analysis was also performed on viral isolates from this region to determine whether there have been substantial genetic changes in VEEV since the 1960s. Based on the findings of this study, the Gulf Coast lineage of subtype IE VEEV continues to actively circulate in this region of Mexico and appears to be responsible for infection of humans and animals throughout this region, including the northern State of Tamaulipas, which borders Texas.

  5. Protein amino acid composition: a genomic signature of encephalization in mammals.

    Directory of Open Access Journals (Sweden)

    Humberto Gutierrez

    Full Text Available Large brains relative to body size represent an evolutionarily costly adaptation as they are metabolically expensive and demand substantial amounts of time to reach structural and functional maturity thereby exacerbating offspring mortality while delaying reproductive age. In spite of its cost and adaptive impact, no genomic features linked to brain evolution have been found. By conducting a genome-wide analysis in all 37 fully sequenced mammalian genomes, we show that encephalization is significantly correlated with overall protein amino acid composition. This correlation is not a by-product of changes in nucleotide content, lifespan, body size, absolute brain size or genome size; is independent of phylogenetic effects; and is not restricted to brain expressed genes. This is the first report of a relationship between this fundamental and complex trait and changes in protein AA usage, possibly reflecting the high selective demands imposed by the process of encephalization across mammalian lineages.

  6. The effect of cyclin-dependent kinases inhibitor treatment on experimental herpes simplex encephalitis mice.

    Science.gov (United States)

    Zhou, Yu; Zeng, Yan-Ping; Zhou, Qin; Guan, Jing-Xia; Lu, Zu-Neng

    2016-08-01

    Herpes simplex encephalitis(HSE) is the most common and serious viral encephalitis in humans. There is a lack of effective medication to date for HSE. A better understanding of the mediators of tissue damage is essential for finding new targets for therapeutic intervention. In this project, we explored the effect of cyclin-dependent kinases inhibitor olomoucine treatment on experimental HSE mice. The following results were obtained: (1) olomoucine increased survival in HSE mice; (2) olomoucine inhibited microglial activation and reduced HSV-1-induced cytokines release; (3) olomoucine prevented neural cells apoptosis and attenuated brain tissue pathological changes following HSV-1 infection; (4) olomoucine reduced brain edema and improved neurological function in HSE. Overall, olomoucine can induce a blunted inflammatory response, maintain the blood vessel wall intact, improve neurological function and increase survival in HSE mice.

  7. Limbic encephalitis: Potential impact of adaptive autoimmune inflammation on neuronal circuits of the amygdala

    Directory of Open Access Journals (Sweden)

    Nico eMelzer

    2015-08-01

    Full Text Available Limbic encephalitis is characterized by adaptive autoimmune inflammation of the gray matter structures of the limbic system. It has recently been identified as major cause of temporal lobe epilepsy accompanied by progressive declarative – mainly episodic – memory disturbance as well as a variety of rather poorly defined emotional and behavioral changes. While autoimmune inflammation of the hippocampus is likely to be responsible for declarative memory disturbance, consequences of autoimmune inflammation of the amygdala are largely unknown. The amygdala is central for the generation of adequate homoeostatic behavioral responses to emotionally significant external stimuli following processing in a variety of parallel neuronal circuits. Here, we hypothesize, that adaptive cellular and humoral autoimmunity may target and modulate distinct inhibitory or excitatory neuronal networks within the amygdala and thereby strongly impact processing of emotional stimuli and corresponding behavioral responses. This may explain some of the rather poorly understood neuropsychiatric symptoms in limbic encephalitis.

  8. Comparative analysis of encephalization in mammals reveals relaxed constraints on anthropoid primate and cetacean brain scaling.

    Science.gov (United States)

    Boddy, A M; McGowen, M R; Sherwood, C C; Grossman, L I; Goodman, M; Wildman, D E

    2012-05-01

    There is a well-established allometric relationship between brain and body mass in mammals. Deviation of relatively increased brain size from this pattern appears to coincide with enhanced cognitive abilities. To examine whether there is a phylogenetic structure to such episodes of changes in encephalization across mammals, we used phylogenetic techniques to analyse brain mass, body mass and encephalization quotient (EQ) among 630 extant mammalian species. Among all mammals, anthropoid primates and odontocete cetaceans have significantly greater variance in EQ, suggesting that evolutionary constraints that result in a strict correlation between brain and body mass have independently become relaxed. Moreover, ancestral state reconstructions of absolute brain mass, body mass and EQ revealed patterns of increase and decrease in EQ within anthropoid primates and cetaceans. We propose both neutral drift and selective factors may have played a role in the evolution of brain-body allometry.

  9. Venezuelan Equine Encephalitis Virus Activity in the Gulf Coast Region of Mexico, 2003–2010

    Science.gov (United States)

    Adams, A. Paige; Navarro-Lopez, Roberto; Ramirez-Aguilar, Francisco J.; Lopez-Gonzalez, Irene; Leal, Grace; Flores-Mayorga, Jose M.; Travassos da Rosa, Amelia P. A.; Saxton-Shaw, Kali D.; Singh, Amber J.; Borland, Erin M.; Powers, Ann M.; Tesh, Robert B.; Weaver, Scott C.; Estrada-Franco, Jose G.

    2012-01-01

    Venezuelan equine encephalitis virus (VEEV) has been the causative agent for sporadic epidemics and equine epizootics throughout the Americas since the 1930s. In 1969, an outbreak of Venezuelan equine encephalitis (VEE) spread rapidly from Guatemala and through the Gulf Coast region of Mexico, reaching Texas in 1971. Since this outbreak, there have been very few studies to determine the northward extent of endemic VEEV in this region. This study reports the findings of serologic surveillance in the Gulf Coast region of Mexico from 2003–2010. Phylogenetic analysis was also performed on viral isolates from this region to determine whether there have been substantial genetic changes in VEEV since the 1960s. Based on the findings of this study, the Gulf Coast lineage of subtype IE VEEV continues to actively circulate in this region of Mexico and appears to be responsible for infection of humans and animals throughout this region, including the northern State of Tamaulipas, which borders Texas. PMID:23133685

  10. [A young patient of acute encephalitis complicated with acyclovir encephalopathy without renal dysfunction].

    Science.gov (United States)

    Tomori, Koji; Isozumi, Kazuo; Motohashi, Sachiko; Komatsumoto, Satoru; Fukuuchi, Yasuo

    2003-08-01

    A previously healthy 30-year-old woman was admitted to our hospital because of impaired consciousness after convulsion. A temporary diagnosis of herpes simplex encephalitis was made, and intravenous acyclovir (ACV) therapy (250 mg four times daily in normal saline over 2 hours) was started. Three days later, she became confused, and was having hallucinations, dysarthria and generalized painful seizures occurred without focal neurologic deficit. Whether the neuropsychiatric symptoms were related to herpes simplex encephalitis or acyclovir neurotoxity was initially unclear. The brain MRI and lumbar puncture findings were initially normal, but abnormal FLAIR lesions appeared later. ACV-associated encephalopathy was considered. ACV was discontinued, and she recovered from the neurological disorder within 24 hours. Although blood levels of acyclovir were not determined, it is unlikely that they were in a toxic range, in view of her normal renal function.

  11. Anterior opercular syndrome as a first presentation of herpes simplex encephalitis.

    Science.gov (United States)

    De Kleermaeker, Floriaan G C M; Bouwmans, Angela E P; Nicolai, Joost; Klinkenberg, Sylvia

    2014-04-01

    We report a 5-year-old girl who presented with fever, drooling, dysphagia, and anarthria. Moreover, voluntary facial movements were disturbed, but the emotional facial movements were completely normal. This clinical phenomenon is known as the anterior opercular syndrome. There was a positive polymerase chain reaction for herpes simplex in the cerebrospinal fluid. The diagnosis herpes simplex encephalitis was supported by both magnetic resonance images (MRI) as by electroencephalogram (EEG). Herpes simplex encephalitis is a rare, but severe, cause of the anterior opercular syndrome that demands treatment as soon as possible in order to prevent high morbidity or mortality. The phenomenon of autonomic-voluntary dissociation, associated with other clinical and radiologic findings related to an underlying neurologic disorder, alerts clinicians to the anterior opercular syndrome as a critical diagnostic observation with time-dependent therapeutic consequences.

  12. [Autochthonous acute viral and bacterial infections of the central nervous system (meningitis and encephalitis)].

    Science.gov (United States)

    Pérez-Ruiz, Mercedes; Vicente, Diego; Navarro-Marí, José María

    2008-07-01

    Rapid diagnosis of acute viral and bacterial infections of the central nervous system (meningitis and encephalitis) is highly important for the clinical management of the patient and helps to establish early therapy that may solve life-threatening situations, to avoid unnecessary empirical treatments, to reduce hospital stay, and to facilitate appropriate interventions in the context of public health. Molecular techniques, especially real-time polymerase chain reaction, have become the fastest and most sensitive diagnostic procedures for autochthonous viral meningitis and encephalitis, and their role is becoming increasingly important for the diagnosis and control of most frequent acute bacterial meningitides. Automatic and closed systems may encourage the widespread and systematic use of molecular techniques for the diagnosis of these neurological syndromes in most laboratories.

  13. Seroprevalence of St. Louis Encephalitis Virus and West Nile Virus (Flavivirus, Flaviviridae in Horses, Uruguay

    Directory of Open Access Journals (Sweden)

    Analía Burgueño

    2013-01-01

    Full Text Available St. Louis encephalitis virus (SLEV and West Nile virus (WNV belong to the Japanese encephalitis antigenic complex (Flavivirus genus, Flaviviridae family. They show antigenic close relationships and share many similarities in their ecology. Both are responsible for serious human diseases. The aim of this study was to investigate the presence of neutralizing antibodies to these viruses in horses from Uruguay. To do this, 425 horse sera were collected in 2007 and analyzed by plaque reduction neutralization tests. As a result, 205 sera (48.2% were found positive for SLEV, with titers ranging between 10 and 80. Two sera remained inconclusive, since they showed low titers to WNV and SLEV (10 and 20, not allowing us to demonstrate activity of WNV in our territory. This is the first report of circulation of SLEV in horses in Uruguay.

  14. Seroprevalence of St. Louis encephalitis virus and West Nile virus (Flavivirus, Flaviviridae) in horses, Uruguay.

    Science.gov (United States)

    Burgueño, Analía; Spinsanti, Lorena; Díaz, Luis Adrián; Rivarola, María Elisa; Arbiza, Juan; Contigiani, Marta; Delfraro, Adriana

    2013-01-01

    St. Louis encephalitis virus (SLEV) and West Nile virus (WNV) belong to the Japanese encephalitis antigenic complex (Flavivirus genus, Flaviviridae family). They show antigenic close relationships and share many similarities in their ecology. Both are responsible for serious human diseases. The aim of this study was to investigate the presence of neutralizing antibodies to these viruses in horses from Uruguay. To do this, 425 horse sera were collected in 2007 and analyzed by plaque reduction neutralization tests. As a result, 205 sera (48.2%) were found positive for SLEV, with titers ranging between 10 and 80. Two sera remained inconclusive, since they showed low titers to WNV and SLEV (10 and 20), not allowing us to demonstrate activity of WNV in our territory. This is the first report of circulation of SLEV in horses in Uruguay.

  15. Expression of N-methyl-D-aspartic acid 2A-B and 2B receptors in anterior thalamic nucleus and subiculum complex of rats

    Institute of Scientific and Technical Information of China (English)

    Yuanshan Fu; Xiaokai Ma; Xiaoling Yue; Bin Wang

    2008-01-01

    BACKGROUND: Glutamate acid ionotropic receptor N-methyl-D-aspartic acid (NMDA) takes part in long-term potentiation, thereby influencing the process of learning and memory.OBJECTIVE: To verify expression of NMDA 2A/B and 2B receptors in the anterior thalamic nucleus and subieulum complex of rats.DESIGN, TIME AND SETTING: A single-sample observation was performed at Department of Anatomy in Dalian Mcdical University (Dalian, Liaoning, China) from April to September in 2007.MATERIALS: Ten adult Wistar rats were used for this study, as well as rabbit anti-NMDA 2A/B and 2Bantibodies.METHODS: The rats were anesthetized and perfused, followed by brain resection and coronal sectioning of the brain tissue. A 1:3 series was selected for immunohistochemistry, using antibodies specific to NMDA 2A/B and 2B receptors. Photos were taken using the Nikon image analysis system.MAIN OUTCOME MEASURES: Expression and distribution of immunohistochemistry staining of NMDA 2A/B and 2B receptor subunits.RESULTS: There were a large number of NMDA 2A/B and 2B receptor-positive neurons distributed throughout the anterior dorsal thalamic nucleus. In the anterior ventral thalamic nucleus, distribution of positive neurons was rare, staining intensity was lighter, and cell bodies were smaller compared with the anterior dorsal thalamic nucleus. In the subiculum complex, staining intensity of NMDA 2A/B and 2B-positive neurons was weakest in the molecular layer and stronger in the pyramidal layer, in particular the region with large cell bodies adjacent to the molecular layer. In the multiform layer, more positive neurons of various sizes were detected.CONCLUSION: NMDA 2A/B and 2B receptor subunits were richly distributed in the anterior thalamic nucleus, with a small difference existing between the anterior dorsal nucleus and anterior ventral nucleus.These neurons were also differentially distributed within the three layers of the subiculum complex.

  16. [Anti-VGKC antibody-associated limbic encephalitis/Morvan syndrome].

    Science.gov (United States)

    Misawa, Tamako; Mizusawa, Hidehiro

    2010-04-01

    Anti-voltage-gated potassium channel antibodies (anti-VGKC-Ab) cause hyperexcitability of the peripheral nerve and central nervous system. Peripheral nerve hyperexcitability is the chief manifestation of Issacs syndrome and cramp-fasciculation syndrome. Morvan syndrome is characterized by neuromyotonia with autonomic and CNS involvement. Manifestations involving the CNS without peripheral involvement are characteristic of limbic encephalitis and epilepsy. The clinical features of anti-VGKC-Ab-associated limbic encephalitis are subacute onset of episodic memory impairment, disorientation and agitation. Hyponatremia is also noted in most patients. Cortico-steroid therapy, plasma exchange and intravenous immunoglobulin are effective in treating to not only the clinical symptoms but also hyponatremia. Unlike other anti-VGKC-Ab-associated neurological disorders, paraneoplastic cases are rare. Thus, anti-VGKC-Ab-associated limbic encephalopathy is considered to be an autoimmune, non-paraneoplastic, potentially treatable encephalitis. Morvan syndrome is characterized by widespread neurological symptoms involving the peripheral nervous system (neuromyotonia), autonomic system (hyperhidrosis, severe constipation, urinary incontinence, and cardiac arrhythmia) and the CNS (severe insomnia, hallucinations, impairment of short-term memory and epilepsy). Many patients have an underlying tumor, for example thymoma, lung cancer, testicular cancer and lymphoma; this indicates the paraneoplastic nature of the disease. Needle electro-myography reveals myokimic discharge. In nerve conduction study, stimulus-induced repetitive descharges are frequently demonstrated in involved muscles. Plasma exchange is an effective treatment approach, and tumor resection also improves symptoms. Both VGKC-Ab-associated limbic encephalitis and Morvan syndrome can be successfully treated. Therefore, when these diseases are suspected, it's important to measure the anti-VGKC-Ab level.

  17. A pathogenetic study of the early connective tissue lesions of viral caprine arthritis-encephalitis.

    OpenAIRE

    Adams, D. S.; Crawford, T B; Klevjer-Anderson, P

    1980-01-01

    Experiments were designed to correlate morphologic lesions with the presence of caprine arthritis-encephalitis virus (CAEV). Twenty-one cesarean-derived goat kids were infected with 10(6) to 10(7) TCID50 of virus, killed sequentially, and examined for viral antigens by immunofluorescence, viral infectivity by isolation and titration, and morphologic changes by light microscopy. Fluorescent viral antigens were detected from 1 to 10 days postinoculation (DPI) and only in synovial cells. Virus w...

  18. Activation of caprine arthritis-encephalitis virus expression during maturation of monocytes to macrophages.

    OpenAIRE

    Narayan, O; Kennedy-Stoskopf, S; Sheffer, D; Griffin, D E; Clements, J E

    1983-01-01

    Lentiviruses, which cause arthritis-encephalitis and maedi-visna in goats and sheep, respectively, cause persistent infections in these animals. The viruses replicate productively at low levels in macrophages in diseased organs such as the "maedi lung" and nonproductively in other cell types such as leukocytes in peripheral blood. Nonproductive infections become productive during in vitro cultivation of the cells. This study showed that monocytes were the only cells in the peripheral blood le...

  19. Paroxysmal dysarthria-ataxia in remitting-relapsing Bickerstaff's-like encephalitis.

    Science.gov (United States)

    Piffer, Silvio; Turri, Giulia; Acler, Michele; Richelli, Silvia; Cerini, Roberto; Fiaschi, Antonio; Monaco, Salvatore; Bonetti, Bruno

    2014-06-15

    Paroxysmal dysarthria-ataxia is a rare neurological condition due to ephaptic transmission, generally appearing in multiple sclerosis patients characterized by stereotyped attacks of slurred speech usually accompanied by ataxia, appearing many times a day. Here we describe a patient with an unusual remitting-relapsing form of Bickerstaff's-like brainstem encephalitis who manifested PDA after a relapse with the involvement of a peculiar region below the red nuclei and benefited from lamotrigine.

  20. Characterization and Pathogenesis of Aerosolized Eastern Equine Encephalitis in the Common Marmoset (Callithrix jacchus)

    Science.gov (United States)

    2016-08-10

    to assess intranasal EEEV infection 70 (Adams, 2008). The small size of the marmoset allows for easy handling, reasonable housing 71 space , and...P.D., Boolukos, P.J. 1969. Eastern equine encephalitis. Distribution of 599 central nervous system lesions in man and Rhesus monkey . J Comp Pathol...pp. 831-838. 654 Wyckoff, R.W. 1939. Encephalomyelitis in monkeys . Science. 89(2319), 542-543. 655 Wyckoff, R.W., Tesar, W.C. 1939. Equine

  1. Protein Amino Acid Composition: A Genomic Signature of Encephalization in Mammals

    OpenAIRE

    Humberto Gutierrez; Atahualpa Castillo; Jimena Monzon; Urrutia, Araxi O.

    2011-01-01

    Large brains relative to body size represent an evolutionarily costly adaptation as they are metabolically expensive and demand substantial amounts of time to reach structural and functional maturity thereby exacerbating offspring mortality while delaying reproductive age. In spite of its cost and adaptive impact, no genomic features linked to brain evolution have been found. By conducting a genome-wide analysis in all 37 fully sequenced mammalian genomes, we show that encephalization is sign...

  2. Diffusion-weighted imaging in the diagnosis of enterovirus 71 encephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Lian, Zhou-yang; Huang, Biao; Liang, Chang-hong (Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong (China)), Email: cjr.huangbiao@vip.163.com; He, Shaoru; Guo, Yuxiong (Department of Pediatrics, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong (China))

    2012-03-15

    Background. In the early phase of viral encephalitis, conventional MRI may appear normal. Diffusion-weighted imaging (DWI) is a sensitive tool for detecting early changes in cellular function in the central nervous system. Purpose. To investigate the usefulness of DWI in the diagnosis of enterovirus 71 (EV71) encephalitis, and to determine whether DWI is superior to conventional MR sequences. Material and Methods. MRI scans in 26 patients were retrospectively evaluated for distribution of lesions on T1-weighted images (T1WI), T2-weighted images (T2WI), fluid-attenuated inversion recovery (FLAIR), and DWI. Contrast-to-noise ratios (CNRs) were calculated for all regions on each sequence and differences in the four MRI sequences were assessed using CNRs. Apparent diffusion coefficient (ADC) values were measured for all regions to look for true restriction of diffusion. Results. Fifteen out of 26 cases showed positive findings on MR imaging. The brain stem was involved in 11 patients, cortex and subcortical white matter in four patients. DWI was more sensitive in detecting the abnormalities (89.7%) compared to T2WI (48.7%), FLAIR (41.0%), and T1WI (35.9%), and the positive ratio of DWI was significantly higher compared to other sequences. Furthermore, no significant difference was found between T2WI and FLAIR (P 0.649). The corresponding mean CNRs were 8.73 +- 2.57, 83.59 +- 29.28, 24.22 +- 6.22, and 132.27 +- 78.32 on T1WI, T2WI, FLAIR, and DWI, respectively. The absolute values of CNRs of lesions on DWI were significantly greater than those on other sequences. Conclusion. DWI appears to be more sensitive in detecting EV71 encephalitis than conventional MRI sequences. This capability may improve the accuracy in diagnosing EV71 encephalitis, especially at the early stage

  3. Generation of Constructs for DNA-Directed RNA Interference of Venezuelan Equine Encephalitis Virus Genes

    Science.gov (United States)

    2006-12-01

    20(Xi-237 Executive summary Introduction: Venezuelan equine encephalitis virus (VEE) is one of a number of different alphaviruses , which can cause...required for replication . It is hypothesized that targeting essential virus genes, either individually or simultaneously, will lead to knockdown or...silencing of the genes, and subsequent inhibition of virus replication . This paper describes the PCR-based approach used to generate DNA cassettes that

  4. Demographics of natural oral infection of mosquitos by Venezuelan equine encephalitis virus.

    Science.gov (United States)

    Gutiérrez, Serafín; Thébaud, Gaël; Smith, Darci R; Kenney, Joan L; Weaver, Scott C

    2015-04-01

    The within-host diversity of virus populations can be drastically limited during between-host transmission, with primary infection of hosts representing a major constraint to diversity maintenance. However, there is an extreme paucity of quantitative data on the demographic changes experienced by virus populations during primary infection. Here, the multiplicity of cellular infection (MOI) and population bottlenecks were quantified during primary mosquito infection by Venezuelan equine encephalitis virus, an arbovirus causing neurological disease in humans and equids.

  5. Travel-Related Tick-Borne Encephalitis, Israel, 2006–2014

    Science.gov (United States)

    Paran, Yael; Lustig, Yaniv; Stienlauf, Shmuel; Weinberger, Miriam; Schwartz, Eli

    2017-01-01

    During 2006–2014, four tick-borne encephalitis (TBE) cases occurred among Israeli travelers. We calculated TBE incidence at 321.0, 45.0, 13.2, and 7.5 cases/100,000 travelers/year of travel to Sweden, Switzerland, Austria, and Germany, respectively. TBE incidence among travelers to these destinations appears to justify TBE vaccination in accordance with World Health Organization recommendations. PMID:27779467

  6. Acute Measles Encephalitis in an Immigrant Syrian Child: Case Report and Review of the Literature

    OpenAIRE

    Abdullah Al-Qayoudhi; Hanan Al-Kindi; Nabil Meki; Amal Al-Maani

    2016-01-01

    The introduction of measles vaccination programs and broad coverage worldwide has meant this infection a rare encounter for pediatricians. In Oman, with almost 100% measles vaccination coverage for children, this infection disappeared from the list of fever and rash differential diagnoses. Encephalitis is a well-known complication of measles infection and sometimes can be the only manifestation especially in adults. We report a seven-year-old Syrian immigrant who was admitted to the Royal Hos...

  7. [The immune status of the population of the Crimea to the tick-borne encephalitis virus].

    Science.gov (United States)

    Markeshin, S Ia; Karavanov, A S; Kovin, V V; Zakharova, T F; Evstratov, Iu V; Bychkova, M V; Evstaf'ev, I L

    1991-10-01

    The immune status of the Crimean population with respect to tick-borne encephalitis (TBE) virus has been studied. The results of the study confirm the existence of natural foci of TBE in the Crimea. The most active and potentially dangerous foci are located in forests of the mountain area of the peninsula. The study has revealed that humans are mainly exposed to the risk of contacting TBE virus infection during their work and rest in the forest.

  8. [Characteristics of clinical features of tick-borne encephalitis in Crimea].

    Science.gov (United States)

    Borisova, M A; Markeshin, S Ia; Riazanova, N Ia; Degtiareva, A A; Zakharova, T F; Bychkova, M V

    1989-12-01

    The analysis of data collected from the clinical epidemiological and virological surveys in 1985-1988 on the incidence of arbovirus infection in population provided evidence for circulation of tick-borne encephalitis (TBE) virus in the Crimea. Etiological relevance of TBE virus in the onset of the diseases was revealed. Clinical picture of TBE in the Crimea is characterized by severe forms and diphasic fever with the relapse running more grave and protracted course.

  9. Computed Tomography Perfusion Usefulness in Early Imaging Diagnosis of Herpes Simplex Virus Encephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Marco de Lucas, E.; Mandly, Gonzalez A.; Gutierrez, A.; Sanchez, E.; Arnaiz, J.; Piedra, T.; Rodriguez, E.; Diez, C. [Hospital Univ. Marques de Valdecilla, Santander (Spain). Depts. of Radiology and Neurology

    2006-10-15

    An early diagnosis is crucial in herpes simplex virus encephalitis patients in order to institute acyclovir therapy and reduce mortality rates. Magnetic resonance imaging (MRI) is considered the gold standard for evaluation of these patients, but is frequently not available in the emergency setting. We report the first case of a computed tomography (CT) perfusion study that helped to establish a prompt diagnosis revealing abnormal increase of blood flow in the affected temporoparietal cortex at an early stage.

  10. Chandipura virus: a major cause of acute encephalitis in children in North Telangana, Andhra Pradesh, India.

    Science.gov (United States)

    Tandale, Babasaheb V; Tikute, Sanjaykumar S; Arankalle, Vidya A; Sathe, Padmakar S; Joshi, Manohar V; Ranadive, Satish N; Kanojia, Phoolchand C; Eshwarachary, D; Kumarswamy, M; Mishra, Akhilesh C

    2008-01-01

    A hospital-based surveillance was undertaken between May 2005 and April 2006 to elucidate the contribution of Chandipura virus (CHPV) to acute viral encephalitis cases in children, seroconversion in recovered cases and to compare the seroprevalences of anti-CHPV IgM and N antibodies in areas reporting cases with those without any case of acute viral encephalitis. During this period, 90 cases of acute encephalitis were hospitalized in the pediatric wards of Mahatma Gandhi Memorial (MGM) Hospital, Warangal. There were 49 deaths (Case Fatality Rate, i.e., CFR of 54.4%). Clinical samples and records were obtained from 52 suspected cases. The cases were below 15 years, majority in 0-4 years (35/52, 67.3%). Computerized tomography (CT) scans and cerebro-spinal fluid (CSF) picture favored viral etiology. No neurological sequelae were observed. CHPV etiology was detected in 25 cases (48.1%, n = 52; RNA in 20, IgM in 3 and N antibody seroconversion in 2). JEV etiology was detected in 5 cases (IgM in 4 cases and seroconversion in 1 case). Anti-CHPV IgM seroprevalence in contacts (26/167, 15.6%) was significantly higher (P < 0.05) than in non-contacts (11/430, 2.6%); which was also observed in children <15 years (19/90, 21.1% vs. 3/109, 2.7%). Anti-CHPV N antibody seroprevalence in <15 years contacts (66/90, 73.3%) and non-contacts (77/109, 70.6%) was significantly lower (P < 0.05) than in contacts (75/77, 97.4%) and non-contacts (302/321, 94.1%) more than 15 years respectively. CHPV appears to be the major cause of acute viral encephalitis in children in endemic areas during early monsoon months.

  11. Serosurveillance of Eastern Equine Encephalitis Virus in Amphibians and Reptiles from Alabama, USA

    OpenAIRE

    Graham, Sean P.; HASSAN, HASSAN K.; Chapman, Taryn; White, Gregory; Guyer, Craig; Unnasch, Thomas R.

    2012-01-01

    Eastern equine encephalitis virus (EEEV) is among the most medically important arboviruses in North America, and studies suggest a role for amphibians and reptiles in its transmission cycle. Serum samples collected from 351 amphibians and reptiles (27 species) from Alabama, USA, were tested for the presence of antibodies against EEEV. Frogs, turtles, and lizards showed little or no seropositivity, and snakes had high seropositivity rates. Most seropositive species were preferred or abundant h...

  12. Preventive strategies for frequent outbreaks of Japanese encephalitis in Northern India

    Indian Academy of Sciences (India)

    Vandana Saxena; Tapan N Dhole

    2008-11-01

    Japanese encephalitis (JE) remains the most important cause of acute viral encephalitis and continues to spread to hitherto unaffected regions like Indonesia, Pakistan and Australia. Approximately 60% of the world population inhabits JE endemic areas. Despite its restricted range mostly in the developing countries, a high annual incidence of 50,000 cases and about 10,000 deaths has been reported. Disease can be fatal in 25% cases. Magnitude of the problem is even more alarming since the survivors are left with serious long-term neuropsychiatric sequelae. Almost every two years, epidemics of JE occur in Indian subcontinent with a high mortality. JE virus infection results in different disease manifestations in host from mild subclinical febrile illness to clinical infections leading to encephalitis. No antiviral treatment is so far available for JE. The prevention of JE can be achieved by controlling the vector or by immunization regime. The vector control in the rural areas, which are the worst affected ones, is practically almost impossible. Three vaccines that have been implicated against JE include inactivated mouse brain derived, inactivated cell culture derived and cell culture derived live attenuated JE vaccine. But each has its own limitation. Currently, attempts to synthesize recombinant DNA vaccine are being made. New therapeutics are on the way of development like use of minocycline, short interfering RNA, arctigenin, rosmarinic acid, DNAzymes etc. However, the immune mechanisms that lead to JE are complex and need to be elucidated further for the development of therapeutics as well as safe and efficacious JE vaccines.

  13. [Aids-related toxoplasma-encephalitis presenting with acute psychotic episode].

    Science.gov (United States)

    Ilniczky, Sándor; Debreczeni, Róbert; Kovács, Tibor; Várkonyi, Viktória; Barsi, Péter; Szirmai, Imre

    2006-07-20

    The most frequent neurological manifestations of the Acquired Immunodeficiency Syndrome-(AIDS) are Cerebral Toxoplasmosis, Primary Central Nervous System Lymphoma (PCNSL), Progressive Multifocal Leukoencephalopathy (PML) and AIDS-encephalitis (AIDS-dementia complex, multinucleated giant cell encephalitis, HIV-encephalopathy). Neurological complications usually occur in the advanced stages of the disease, and they are uncommon in the beginning as presenting illness, but may result in life-threatening condition or in death. Rarely the disease presents as a neuropsychiatric illness in an undiagnosed AIDS patient, delaying a proper diagnosis. We present the case of a 34 years old patient treated for AIDS-related Toxoplasma-encephalitis in our department. His illness started as an acute psychosis followed by rapid mental and somatic decline, leading to death in three months. His HIV-seropositivity was not known at his admission, and the extraneural manifestations were slight. The diagnosis was established by serology, imaging methods and histopathological investigation. After presenting the medical history and results of autopsy studies of the patient we discuss the problems of the differential diagnosis, especially regarding the findings of the imaging methods.

  14. Acute psychosis followed by fever: Malignant neuroleptic syndrome or viral encephalitis?

    Directory of Open Access Journals (Sweden)

    Stojanović Zvezdana

    2014-01-01

    Full Text Available Introduction. Neuroleptic malignant syndrome is rare, but potentially fatal idiosyncratic reaction to antipsychotic medications. It is sometimes difficult to diagnose some clinical cases as neuroleptic malignant syndrome and differentiate it from the acute viral encephalitis. Case report. We reported a patient diagnosed with acute psychotic reaction which appeared for the first time. The treatment started with typical antipsychotic, which led to febrility. The clinical presentation of the patient was characterised by the signs and symptoms that might have indicated the neuroleptic malignant syndrome as well as central nervous system viral disease. In order to make a detailed diagnosis additional procedures were performed: electroencephalogram, magnetic resonance imaging of the head, lumbar puncture and a serological test of the cerebrospinal fluid. Considering that after the tests viral encephalitis was ruled out and the diagnosis of neuroleptic malignant syndrome made, antipsychotic therapy was immediately stopped. The patient was initially treated with symptomatic therapy and after that with atypical antipsychotic and electroconvulsive therapy, which led to complete recovery. Conclusion. We present the difficulties of early diagnosis at the first episode of acute psychotic disorder associated with acute febrile condition. Concerning the differential diagnosis it is necessary to consider both neuroleptic malignant syndrome and viral encephalitis, i.e. it is necessary to make the neuroradiological diagnosis and conduct cerebrospinal fluid analysis and blood test. In neuroleptic malignant syndrome treatment a combined use of electroconvulsive therapy and low doses of atypical antipsychotic are confirmed to be successful.

  15. Seroprevalence of Cysticercus Antibodies in Japanese Encephalitis Patients in Upper Assam, India: A Hospital Based Study

    Science.gov (United States)

    Mazumdar, Himangshu; Saikia, Lahari

    2016-01-01

    Introduction Co-infection of Japanese Encephalitis (JE) and Cysticercosis is attributed mainly to the common epidemiological features between the two diseases. Not much is known about the clinical implications of one infection over the other. Aim The study aimed at establishing whether JE-Cysticercosis co-infection is prevalent in the Upper Assam districts and to explore additional details about such co-infections both clinically and epidemiologically. Materials and Methods The present study was a retrospective cross-sectional hospital based study conducted between July 2013 and June 2014 and included 272 Acute Encephalitis Syndrome (AES) patients. Out of this, 137 JE positive and 135 non-JE Acute encephalitis patients were taken as cases and controls respectively. The diagnosis of JE and Cysticercosis was established by ELISA. Statistical Analysis EpiInfo ver. 7 was used for statistical analysis. Chi-square was used and p-value < 0.05 was considered to be statistically significant. Results The association of Cysticercosis with JE was found to be statistically significant (14.6%, p = 0.0019) in the cases with reference to the controls (3.7%). Moreover, the co-infections were found to be more common in case of adults (19.32%, p = 0.0360); with males having a greater odds (5.25, p = 0.0008) of harbouring the parasite as compared to females. Conclusion The study proves that the association of Cysticercosis and JE holds true in this region. PMID:27437215

  16. Evaluation of antiviral activity of essential oil of Trachyspermum Ammi against Japanese encephalitis virus

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    Soumen Roy

    2015-01-01

    Full Text Available Background: Japanese encephalitis is a leading form of viral encephalitis, prevalent mostly in South Eastern Asia caused by Japanese encephalitis virus (JEV. It is transmitted by the mosquitoes of the Culex sp. The disease affects children and results in 50% result in permanent neuropsychiatric disorder. There arises a need to develop a safe, affordable, and potent anti-viral agent against JEV. This study aimed to assess the antiviral activity of ajwain (Trachyspermum ammi: Umbellifereae essential oil against JEV. Materials and Methods: Ajwain oil was extracted by distillation method and in vitro cytotoxicity assay was performed in vero cell line by 3-(4, 5-dimethylthiazol-2-yl-2, 5-diphenyltetrazolium bromide (MTT assay method. JEV titer was determined by plaque assay and in vitro antiviral activity of ajwain oil was quantified by the plaque reduction neutralization test (PRNT. Results: Cytotoxic concentration of the oil was found to be 1 mg/ml by MTT assay. The titer of the virus pool was found to be 50× 10 7 PFU/ml. we observed 80% and 40% virus inhibition in 0.5mg/ml of ajwain oil by PRNT method in preexposure treatment and postexposure treatment (antiviral activity, respectively. Conclusion: Our data indicate ajwain oil has potential in vitro antiviral activity against JEV. Further, the active biomolecule will be purified and evaluated for anti-JEV activity and also to scale up for in vivo trial to evaluate the efficacy of ajwain oil in future.

  17. Is there a need for a travel vaccination against tick-borne encephalitis?

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    Kunze, Ursula

    2008-11-01

    Tick - borne encephalitis is a vector-borne disease primarily transmitted to humans by the bite of infected ticks. Clinical presentations range from mild meningitis to severe encephalitis with or without myelitis and may result in long-term neurological equelae or death. The disease can successfully be prevented through active immunization. BE is endemic in regions of at least 26 European countries. The risk of contracting TBE in the most affected countries has increased in the past 20 to 30 years, between 1974 and 2003, the number of reported clinical TBE cases increased by an appalling 400%, the only exception having been Austria. Because of increasing international travel streams of tourists from non-endemic countries to TBE risk areas, the infection is now becoming a more important issue of travel medicine.The risk depends on both the travelling season and the degree of unprotected outdoor exposure to forest areas (e.g. bicycling, camping, hiking, etc.). Tourists are by no means aware of the actual risk potential, especially when travelling to a knowingly 'safe' Europe. Vaccination against tick-borne encephalitis may be required for some tourists, depending on travel destination and behaviour. Implementing general guidelines for the use of TBE travel vaccination should be put on the agenda for travel medicine.

  18. Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba.

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    Martínez, A J; Sotelo-Avila, C; Alcalá, H; Willaert, E

    1980-01-01

    Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and low-grade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/microliters, 705 WBC/microliters with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were trombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.

  19. Monoclonal antibody against Saint Louis encephalitis prM viral protein.

    Science.gov (United States)

    Pupo-Antúnez, M; Vázquez, S; Sosa, A L; Caballero, Y; Vásquez, Y; Morier, L; Álvarez, M; Guzmán, M G

    2015-06-15

    Saint Louis encephalitis virus belongs to Flavivirus genus; Flaviviridae family jointly with other medically important flaviviruses including dengue virus and West Nile virus. The biological properties and functions of prM flavivirus protein are under investigation due to its importance in the generation of infectious virion and host interactions. Monoclonal antibodies have become powerful tools in this approach. Also the use of monoclonal antibodies has been successfully applied for antigenic analysis, clinical diagnosis and treatments. Here, using an immunofluorescence assay we describe a monoclonal antibody (mAb 3D2) that uniquely recognizes native prM Saint Louis encephalitis virus protein expressed in either C6/36-HT or Vero cells. In conclusion, mAb3D2 has significant potential for use in (a) the diagnosis of infections caused by this virus and (b) therapeutic use to treat patients infected by this virus and fundamental research to understand the role of the prM in the Saint Louis encephalitis virus infectious process.

  20. Transient asymptomatic white matter lesions following Epstein-Barr virus encephalitis

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    Yoo Young Jang

    2011-09-01

    Full Text Available We present the case of a patient with Epstein-Barr virus (EBV encephalitis who developed abnormal white matter lesions during the chronic phases of the infection. A 2-year-old-boy was admitted for a 2 day history of decreased activity with ataxic gait. The results of the physical examination were unremarkable except for generalized lethargy and enlarged tonsils with exudates. Brain magnetic resonance imaging (MRI at admission showed multiple high signal intensities in both basal ganglia and thalami. The result of EBV polymerase chain reaction (PCR of the cerebral spinal fluid was positive, and a serological test showed acute EBV infection. The patient was diagnosed with EBV encephalitis and recovered fully without any residual neurologic complications. Subsequently, follow-up MRI at 5 weeks revealed extensive periventricular white matter lesions. Since the patient remained clinically stable and asymptomatic during the follow-up period, no additional studies were performed and no additional treatments were provided. At the 1-year follow-up, cranial MRI showed complete disappearance of the abnormal high signal intensities previously seen in the white matter. The patient continued to remain healthy with no focal neurologic deficits on examination. This is the first case of asymptomatic self-limited white matter lesions seen in serial MRI studies in a Korean boy with EBV encephalitis.

  1. Molecular identification of Saint Louis encephalitis virus genotype IV in Colombia.

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    Hoyos-López, Richard; Soto, Sandra Uribe; Rúa-Uribe, Guillermo; Gallego-Gómez, Juan Carlos

    2015-09-01

    Saint Louis encephalitis virus (SLEV) is a member of the Japanese-encephalitis virus serocomplex of the genus Flavivirus. SLEV is broadly distributed in the Americas and the Caribbean Islands, where it is usually transmitted by mosquitoes of the genus Culex and primarily to birds and mammalian-hosts. Humans are occasionally infected by the virus and are dead-end hosts. SLEV causes encephalitis in temperate regions, while in tropical regions of the Americas, several human cases and a wide biological diversity of SLEV-strains have been reported. The phylogenetic analysis of the envelope (E) protein genes indicated eight-genotypes of SLEV with geographic overlap. The present paper describes the genotyping of two SLEV viruses detected in mosquito-pools collected in northern Colombia (department of Cordoba). We used reverse transcription-polymerase chain reaction to amplify a fragment of the E-gene to confirm the virus identity and complete E-gene sequencing for phylogenetic analysis and genotyping of the two-SLEV viruses found circulating in Córdoba. This is the first report of SLEV genotype IV in Colombia (Córdoba) in mosquitoes from a region of human inhabitation, implicating the risk of human disease due to SLEV infection. Physicians should consider SLEV as a possible aetiology for undiagnosed febrile and neurologic syndromes among their patients who report exposure to mosquito-bites.

  2. Isolation and characterization of tick-borne encephalitis virus from Ixodes persulcatus in Mongolia in 2012.

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    Muto, Memi; Bazartseren, Boldbaatar; Tsevel, Bazartseren; Dashzevge, Erdenechimeg; Yoshii, Kentaro; Kariwa, Hiroaki

    2015-07-01

    Tick-borne encephalitis virus (TBEV) is a zoonotic virus belonging to the genus Flavivirus, in the family Flaviviridae. The virus, which is endemic in Europe and northern parts of Asia, causes severe encephalitis. Tick-borne encephalitis (TBE) has been reported in Mongolia since the 1980s, but details about the biological characteristics of the endemic virus are lacking. In this study, 680 ticks (Ixodes persulcatus) were collected in Selenge aimag, northern Mongolia, in 2012. Nine Mongolian TBEV strains were isolated from tick homogenates. A sequence analysis of the envelope protein gene revealed that all isolates belonged to the Siberian subtype of TBEV. Two strains showed similar growth properties in cultured cells, but their virulence in mice differed. Whole genome sequencing revealed only thirteen amino acid differences between these Mongolian TBEV strains. Our results suggest that these naturally occurring amino acid mutations affected the pathogenicity of Mongolian TBEV. Our results may be an important platform for monitoring TBEV to evaluate the epidemiological risk in TBE endemic areas of Mongolia.

  3. Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up

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    Guimarães Catarina Abraão

    2002-01-01

    Full Text Available Rasmussen's encephalitis is characterized by refractory epilepsy, neurological deterioration and progressive atrophy of one cerebral hemisphere. The objective of this study is to describe the importance of neuropsychological evaluation in the treatment decision and follow-up of patients with Rasmussen´s encephalitis. Neuropsychological assessment was performed in two steps. Firstly, the clinical history was obtained and the Vineland adaptative behavior scale (VABS was applied. After this first step, the patients with social maturity level equal or higher than the inferior limit underwent a battery of neuropsychological assessment. We evaluated three patients before any specific treatment was started, and six months after the intervention (surgery or plasmapheresis. Patient 1 underwent left hemispherectomy and had global improvement on second neuropsychological assessment. This suggests that the decision of performing surgery was adequate. Patients 2 and 3 underwent plasmapheresis. They did not present cognitive decline between both evaluations which suggest that our decision of postponing surgery was adequate as well. We conclude that neuropsychological assessment is important when evaluating patients with Rasmussen´s encephalitis. That is especially true for patients in whom disease progression is slow, and surgery timing has to be carefully planned.

  4. Suramin Inhibits the In Vitro Expression of Encephalitis B Virus Proteins NS3 and E

    Institute of Scientific and Technical Information of China (English)

    徐可树; 任宏宇; 朱剑文; 杨昀; 廖芳

    2003-01-01

    In this study, the mechanism by which Suramin inhibits the replication of epidemic encephalitis B virus was explored to provide a theoretical basis for its further application in clinical practice. After viral infection of HepG2 and IMR-32 cells, different concentrations of Suramin were added to the culture media, and then the cultural supernatants and infected cells were collected 48 h later. For the evaluation of the curative effect, cytopathic effect (CPE), virus titers, the expression of viral protein and viral RNA were determined by Western blot, RT-PCR and in vitro RNA synthesis, respectively. At the concentration of 50 μg/ml of Suramin, HepG2 and IMR-32 infected with epidemic encephalitis B virus decreased by 51.8 % and 0.03 % respectively, as compared with controls. It was suggested that expression of encephalitis B virus proteins NS3 and E was notably reduced by Suramin. This is especially true of E protein. At RNA level, however, no difference in RNA virus was found between Suramin-treated virus and non-treated cells. Our results suggest that Suramin can inhibit viral replication by blocking the production of viral proteins.

  5. Acute Transverse Myelitis (Ascending Myelitis as the Initial Manifestation of Japanese Encephalitis: A Rare Presentation

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    Varshney Ankur Nandan

    2013-01-01

    Full Text Available Japanese encephalitis, an inflammatory brain disease prevalent in Southeast Asia, usually presented with fever, headache, convulsions, brain stem signs with pyramidal and extrapyramidal features, and altered sensorium. Acute transverse myelitis, as the initial manifestation of Japanese encephalitis, is an unusual manifestation and is seldom reported. We hereby report a case of 13-year-old adolescent boy who presented to us with fever and acute onset paraparesis with urinary retention initially, progressing to quadriparesis and then followed by headache and altered sensorium. Brain MRI revealed bilateral basal ganglia that were grossly swollen with vasogenic edema tracking along internal capsule and midbrain. Adjacent ventrolateral thalamus and internal capsule also showed mild abnormal intensities. Spinal screening showed abnormal cord intensities in entire cord with gross edema in cervical and conus regions. He had elevated IgM titres against JE virus in cerebrospinal fluid. The patient was treated conservatively along with intravenous methyl prednisolone for 5 days. He regained near normal power at 3 months in followup, but hesitancy, dysarthria, and slowness of movement still persisted. To conclude, a young boy presenting with ATM in an endemic region of JE, then a possibility of Japanese encephalitis, should be sought by clinicians as early use of immunomodulator shows survival benefit.

  6. Molecular identification of Saint Louis encephalitis virus genotype IV in Colombia

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    Richard Hoyos-López

    2015-09-01

    Full Text Available Saint Louis encephalitis virus (SLEV is a member of the Japanese-encephalitis virus serocomplex of the genus Flavivirus. SLEV is broadly distributed in the Americas and the Caribbean Islands, where it is usually transmitted by mosquitoes of the genus Culex and primarily to birds and mammalian-hosts. Humans are occasionally infected by the virus and are dead-end hosts. SLEV causes encephalitis in temperate regions, while in tropical regions of the Americas, several human cases and a wide biological diversity of SLEV-strains have been reported. The phylogenetic analysis of the envelope (E protein genes indicated eight-genotypes of SLEV with geographic overlap. The present paper describes the genotyping of two SLEV viruses detected in mosquito-pools collected in northern Colombia (department of Cordoba. We used reverse transcription-polymerase chain reaction to amplify a fragment of theE-gene to confirm the virus identity and completeE-gene sequencing for phylogenetic analysis and genotyping of the two-SLEV viruses found circulating in Córdoba. This is the first report of SLEV genotype IV in Colombia (Córdoba in mosquitoes from a region of human inhabitation, implicating the risk of human disease due to SLEV infection. Physicians should consider SLEV as a possible aetiology for undiagnosed febrile and neurologic syndromes among their patients who report exposure to mosquito-bites.

  7. Molecular detection and genotyping of Japanese Encephalitis Virus in mosquitoes during a 2010 outbreak in the Republic of Korea

    Science.gov (United States)

    Seo, Hyun-Ji; Kim, Heung Chul; Klein, Terry A.; Ramey, Andrew M.; Lee, Ji-Hyee; Kyung, Soon-Goo; Park, Jee-Yong; Cho, In-Soo; Yeh, Jung-Yong

    2013-01-01

    Japanese encephalitis virus (JEV), a mosquito-borne zoonotic pathogen, is one of the major causes of viral encephalitis. To reduce the impact of Japanese encephalitis among children in the Republic of Korea (ROK), the government established a mandatory vaccination program in 1967. Through the efforts of this program only 0-7 (mean 2.1) cases of Japanese encephalitis were reported annually in the ROK during the period of 1984-2009. However, in 2010 there was an outbreak of 26 confirmed cases of Japanese encephalitis, including 7 deaths. This represented a >12-fold increase in the number of confirmed cases of Japanese encephalitis in the ROK as compared to the mean number reported over the last 26 years and a 3.7-fold increase over the highest annual number of cases during this same period (7 cases). Surveillance of adult mosquitoes was conducted during the 2010 outbreak of Japanese encephalitis in the ROK. A total of 6,328 culicine mosquitoes belonging to 12 species from 5 genera were collected at 6 survey sites from June through October 2010 and assayed by reverse-transcription polymerase chain reaction (RT-PCR) for the presence of JEV. A total of 34/371 pooled samples tested positive for JEV (29/121 Culex tritaeniorhynchus, 4/64 Cx. pipiens, and 1/26 Cx. bitaeniorhynchus) as confirmed by sequencing of the pre-membrane and envelope protein coding genes. The maximum likelihood estimates of JEV positive individuals per 1,000 culicine vectors for Cx. tritaeniorhynchus, Cx. pipiens, and Cx. bitaeniorhynchus were 11.8, 5.6, and 2.8, respectively. Sequences of the JEV pre-membrane and envelope protein coding genes amplified from the culicine mosquitoes by RT-PCR were compared with those of JEV genotypes I-V. Phylogenetic analyses support the detection of a single genotype (I) among samples collected from the ROK in 2010.

  8. Cellular receptors for human enterovirus species A

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    Yorihiro eNishimura

    2012-03-01

    Full Text Available Human enterovirus species A (HEV-A is one of the four species of HEV in the genus Enterovirus in the family Picornaviridae. Among HEV-A, coxsackievirus A16 (CVA16 and enterovirus 71 (EV71 are the major causative agents of hand, foot, and mouth disease (HFMD. Some other types of HEV-A are commonly associated with herpangina. Although HFMD and herpangina due to HEV-A are common febrile diseases among infants and children, EV71 can cause various neurological diseases, such as aseptic meningitis and fatal encephalitis.Recently, two human transmembrane proteins, P-selectin glycoprotein ligand-1 (PSGL-1 and scavenger receptor class B, member 2 (SCARB2, were identified as functional receptors for EV71 and CVA16. In in vitro infection experiments using the prototype HEV-A strains, PSGL-1 and SCARB2 could be responsible for the specific receptors for EV71 and CVA16. However, the involvement of both receptors in the in vitro and in vivo infections of clinical isolates of HEV-A has not been clarified yet. To elucidate a diverse array of the clinical outcome of HEV-A-associated diseases, the identification and characterization of HEV-A receptors may provide useful information in understanding the HEV-A pathogenesis at a molecular level.

  9. Validity of the coding for herpes simplex encephalitis in the Danish National Patient Registry

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    Jørgensen LK

    2016-05-01

    Full Text Available Laura Krogh Jørgensen,1 Lars Skov Dalgaard,1 Lars Jørgen Østergaard,1 Nanna Skaarup Andersen,2 Mette Nørgaard,3 Trine Hyrup Mogensen1 1Department of Infectious Diseases, Aarhus University Hospital, Aarhus, 2Department of Clinical Microbiology, Odense University Hospital, Odense, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Background: Large health care databases are a valuable source of infectious disease epidemiology if diagnoses are valid. The aim of this study was to investigate the accuracy of the recorded diagnosis coding of herpes simplex encephalitis (HSE in the Danish National Patient Registry (DNPR. Methods: The DNPR was used to identify all hospitalized patients, aged ≥15 years, with a first-time diagnosis of HSE according to the International Classification of Diseases, tenth revision (ICD-10, from 2004 to 2014. To validate the coding of HSE, we collected data from the Danish Microbiology Database, from departments of clinical microbiology, and from patient medical records. Cases were classified as confirmed, probable, or no evidence of HSE. We estimated the positive predictive value (PPV of the HSE diagnosis coding stratified by diagnosis type, study period, and department type. Furthermore, we estimated the proportion of HSE cases coded with nonspecific ICD-10 codes of viral encephalitis and also the sensitivity of the HSE diagnosis coding. Results: We were able to validate 398 (94.3% of the 422 HSE diagnoses identified via the DNPR. Hereof, 202 (50.8% were classified as confirmed cases and 29 (7.3% as probable cases providing an overall PPV of 58.0% (95% confidence interval [CI]: 53.0–62.9. For “Encephalitis due to herpes simplex virus” (ICD-10 code B00.4, the PPV was 56.6% (95% CI: 51.1–62.0. Similarly, the PPV for “Meningoencephalitis due to herpes simplex virus” (ICD-10 code B00.4A was 56.8% (95% CI: 39.5–72.9. “Herpes viral encephalitis” (ICD-10 code G05.1E had a PPV

  10. Viral etiology of encephalitis in children in southern Vietnam: results of a one-year prospective descriptive study.

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    Van Tan Le

    Full Text Available BACKGROUND: Acute encephalitis is an important and severe disease in children in Vietnam. However, little is known about the etiology while such knowledge is essential for optimal prevention and treatment. To identify viral causes of encephalitis, in 2004 we conducted a one-year descriptive study at Children's Hospital Number One, a referral hospital for children in southern Vietnam including Ho Chi Minh City. METHODOLOGY/PRINCIPAL FINDINGS: Children less than 16 years of age presenting with acute encephalitis of presumed viral etiology were enrolled. Diagnostic efforts included viral culture, serology and real time (RT-PCRs. A confirmed or probable viral causative agent was established in 41% of 194 enrolled patients. The most commonly diagnosed causative agent was Japanese encephalitis virus (n = 50, 26%, followed by enteroviruses (n = 18, 9.3%, dengue virus (n = 9, 4.6%, herpes simplex virus (n = 1, cytomegalovirus (n = 1 and influenza A virus (n = 1. Fifty-seven (29% children died acutely. Fatal outcome was independently associated with patient age and Glasgow Coma Scale (GCS on admission. CONCLUSIONS/SIGNIFICANCE: Acute encephalitis in children in southern Vietnam is associated with high mortality. Although the etiology remains unknown in a majority of the patients, the result from the present study may be useful for future design of treatment and prevention strategies of the disease. The recognition of GCS and age as predictive factors may be helpful for clinicians in managing the patient.

  11. Transient widespread cortical and splenial lesions in acute encephalitis/encephalopathy associated with primary Epstein–Barr virus infection

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    Shuo Zhang

    2016-01-01

    Full Text Available Infection with Epstein–Barr virus (EBV is very common and usually occurs in childhood or early adulthood. Encephalitis/encephalopathy is an uncommon but serious neurological complication of EBV. A case of EBV-associated encephalitis/encephalopathy with involvement of reversible widespread cortical and splenial lesions is presented herein. An 8-year-old Chinese girl who presented with fever and headache, followed by seizures and drowsiness, was admitted to the hospital. Magnetic resonance imaging revealed high signal intensities on diffusion-weighted imaging in widespread cortical and splenial lesions. The clinical and laboratory examination results together with the unusual radiology findings suggested acute encephalitis/encephalopathy due to primary EBV infection. After methylprednisolone pulse therapy together with ganciclovir, the patient made a full recovery without any brain lesions. The hallmark clinical–radiological features of this patient included severe encephalitis/encephalopathy at onset, the prompt and complete recovery, and rapidly reversible widespread involvement of the cortex and splenium. Patients with EBV encephalitis/encephalopathy who have multiple lesions, even with the widespread involvement of cortex and splenium of the corpus callosum, may have a favorable outcome with complete disappearance of all brain lesions.

  12. Cerebrospinal Fluid Biomarkers of Japanese Encephalitis [v2; ref status: indexed, http://f1000r.es/5uq

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    Nabonita Sengupta

    2015-09-01

    Full Text Available Japanese encephalitis (JE is the leading cause of viral encephalitis in Asia. Acute encephalitis syndrome (AES is a group of central nervous system (CNS disorders caused by a wide range of viruses, bacteria, fungi, chemicals and toxins. It is important to distinguish between various forms of infectious encephalitis with similar clinical manifestations in order to ensure specific and accurate diagnosis and development of subsequent therapeutic strategies. Cerebrospinal fluid (CSF is in direct contact with the CNS and hence it is considered to be an excellent source for identifying biomarkers for various neurological disorders. With the recent advancement in proteomic methodologies, the field of biomarker research has received a remarkable boost.  The present study identifies potential biomarkers for JE using a proteomics based approach. The CSF proteomes from ten patients each with JE and Non-JE acute encephalitis were analyzed by 2D gel electrophoresis followed by mass spectrometry. Vitamin D-binding protein (DBP, fibrinogen gamma chain, fibrinogen beta chain, complement C4-B, complement C3 and cytoplasmic actin were found to be significantly elevated in case of JE indicating severe disruption of the blood brain barrier and DBP can be suggested to be an important diagnostic marker.

  13. Genetic diversity of Venezuelan alphaviruses and circulation of a Venezuelan equine encephalitis virus subtype IAB strain during an interepizootic period.

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    Medina, Gladys; Garzaro, Domingo J; Barrios, Miguel; Auguste, Albert J; Weaver, Scott C; Pujol, Flor H

    2015-07-01

    Several species of alphaviruses have been previously described in the Americas, some of which are associated with encephalitis and others are associated with arthralgia. Venezuelan equine encephalitis virus (VEEV) and eastern equine encephalitis virus (EEEV) are endemic to Venezuela, with the former being responsible for major outbreaks of severe and often fatal disease in animals and humans. The aim of this study was to analyze the genetic diversity of Venezuelan alphaviruses isolated during two decades (1973-1999) of surveillance in northern Venezuela. Phylogenetic analysis indicated the circulation of a VEEV subtype IAB strain 8 years after the last reported outbreak. Thirteen strains within two subclades of South American lineage III of EEEV were also found in Venezuela. Considerable genetic variability was observed among Venezuelan Una virus strains, which were widely distributed among the clades. The first Venezuelan Mayaro sequence was also characterized.

  14. Asymptomatic aneurysm of the cavernous and supraclinoid internal carotid artery in a patient with Balamuthia mandrillaris encephalitis.

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    Orozco, Ludwig D; Khan, Majid A; Fratkin, Jonathan D; Hanigan, William C

    2011-08-01

    This is the first report to our knowledge of the successful treatment of an asymptomatic mycotic aneurysm associated with Balamuthia mandrillaris encephalitis. A 27-year-old male with end-stage renal disease presented with generalized seizures following renal transplantation. MRI demonstrated multiple brain masses and an aneurysm of the cavernous and supraclinoid carotid artery. Autopsy of the donor's brain revealed Balamuthia encephalitis. The patient was placed on an anti-amebic regimen, his condition improved, and 126 days after the kidney transplant, MRI brain showed resolution of the aneurysm and improvement of the enhancing lesions. Balamuthia mandrillaris has been shown to cause a granulomatous encephalitis, with prominent vasculitis. This is the first report to demonstrate the risk of aneurysm formation associated with this infection. Prolonged anti-amebic treatment resulted in resolution of the aneurysm without clinical evidence of subarachnoid hemorrhage.

  15. Genetic Diversity of Venezuelan Alphaviruses and Circulation of a Venezuelan Equine Encephalitis Virus Subtype IAB Strain During an Interepizootic Period

    Science.gov (United States)

    Medina, Gladys; Garzaro, Domingo J.; Barrios, Miguel; Auguste, Albert J.; Weaver, Scott C.; Pujol, Flor H.

    2015-01-01

    Several species of alphaviruses have been previously described in the Americas, some of which are associated with encephalitis and others are associated with arthralgia. Venezuelan equine encephalitis virus (VEEV) and eastern equine encephalitis virus (EEEV) are endemic to Venezuela, with the former being responsible for major outbreaks of severe and often fatal disease in animals and humans. The aim of this study was to analyze the genetic diversity of Venezuelan alphaviruses isolated during two decades (1973–1999) of surveillance in northern Venezuela. Phylogenetic analysis indicated the circulation of a VEEV subtype IAB strain 8 years after the last reported outbreak. Thirteen strains within two subclades of South American lineage III of EEEV were also found in Venezuela. Considerable genetic variability was observed among Venezuelan Una virus strains, which were widely distributed among the clades. The first Venezuelan Mayaro sequence was also characterized. PMID:25940191

  16. Identification and properties of proteases from an Acanthamoeba isolate capable of producing granulomatous encephalitis

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    Jarroll Edward L

    2006-05-01

    Full Text Available Abstract Background Granulomatous amoebic encephalitis due to Acanthamoeba is often a fatal human disease. However, the pathogenesis and pathophysiology of Acanthamoeba encephalitis remain unclear. In this study, the role of extracellular Acanthamoeba proteases in central nervous system pathogenesis and pathophysiology was examined. Results Using an encephalitis isolate belonging to T1 genotype, we observed two major proteases with approximate molecular weights of 150 KD and 130 KD on SDS-PAGE gels using gelatin as substrate. The 130 KD protease was inhibited with phenylmethylsulfonyl fluoride (PMSF suggesting that it is a serine protease, while the 150 KD protease was inhibited with 1, 10-phenanthroline suggesting that it is a metalloprotease. Both proteases exhibited maximal activity at neutral pH and over a range of temperatures, indicating their physiological relevance. These proteases degrade extracellular matrix (ECM, which provide structural and functional support to the brain tissue, as shown by the degradation of collagen I and III (major components of collagenous ECM, elastin (elastic fibrils of ECM, plasminogen (involved in proteolytic degradation of ECM, as well as casein and haemoglobin. The proteases were purified partially using ion-exchange chromatography and their effects were tested in an in vitro model of the blood-brain barrier using human brain microvascular endothelial cells (HBMEC. Neither the serine nor the metalloprotease exhibited HBMEC cytotoxicity. However, the serine protease exhibited HBMEC monolayer disruptions (trypsin-like suggesting a role in blood-brain barrier perturbations. Conclusion Overall, these data suggest that Acanthamoeba proteases digest ECM, which may play crucial role(s in invasion of the brain tissue by amoebae.

  17. Characteristic Cytokine and Chemokine Profiles in Encephalitis of Infectious, Immune-Mediated, and Unknown Aetiology.

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    Benedict D Michael

    Full Text Available Encephalitis is parenchymal brain inflammation due to infectious or immune-mediated processes. However, in 15-60% the cause remains unknown. This study aimed to determine if the cytokine/chemokine-mediated host response can distinguish infectious from immune-mediated cases, and whether this may give a clue to aetiology in those of unknown cause.We measured 38 mediators in serum and cerebrospinal fluid (CSF of patients from the Health Protection Agency Encephalitis Study. Of serum from 78 patients, 38 had infectious, 20 immune-mediated, and 20 unknown aetiology. Of CSF from 37 patients, 20 had infectious, nine immune-mediated and eight unknown aetiology.Heat-map analysis of CSF mediator interactions was different for infectious and immune-mediated cases, and that of the unknown aetiology group was similar to the infectious pattern. Higher myeloperoxidase (MPO concentrations were found in infectious than immune-mediated cases, in serum and CSF (p = 0.01 and p = 0.006. Serum MPO was also higher in unknown than immune-mediated cases (p = 0.03. Multivariate analysis selected serum MPO; classifying 31 (91% as infectious (p = 0.008 and 17 (85% as unknown (p = 0.009 as opposed to immune-mediated. CSF data also selected MPO classifying 11 (85% as infectious as opposed to immune-mediated (p = 0.036. CSF neutrophils were detected in eight (62% infective and one (14% immune-mediated cases (p = 0.004; CSF MPO correlated with neutrophils (p<0.0001.Mediator profiles of infectious aetiology differed from immune-mediated encephalitis; and those of unknown cause were similar to infectious cases, raising the hypothesis of a possible undiagnosed infectious cause. Particularly, neutrophils and MPO merit further investigation.

  18. CLEC5A regulates Japanese encephalitis virus-induced neuroinflammation and lethality.

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    Szu-Ting Chen

    Full Text Available CLEC5A/MDL-1, a member of the myeloid C-type lectin family expressed on macrophages and neutrophils, is critical for dengue virus (DV-induced hemorrhagic fever and shock syndrome in Stat1⁻/⁻ mice and ConA-treated wild type mice. However, whether CLEC5A is involved in the pathogenesis of viral encephalitis has not yet been investigated. To investigate the role of CLEC5A to regulate JEV-induced neuroinflammation, antagonistic anti-CLEC5A mAb and CLEC5A-deficient mice were generated. We find that Japanese encephalitis virus (JEV directly interacts with CLEC5A and induces DAP12 phosphorylation in macrophages. In addition, JEV activates macrophages to secrete proinflammatory cytokines and chemokines, which are dramatically reduced in JEV-infected Clec5a⁻/⁻ macrophages. Although blockade of CLEC5A cannot inhibit JEV infection of neurons and astrocytes, anti-CLEC5A mAb inhibits JEV-induced proinflammatory cytokine release from microglia and prevents bystander damage to neuronal cells. Moreover, JEV causes blood-brain barrier (BBB disintegrity and lethality in STAT1-deficient (Stat1⁻/⁻ mice, whereas peripheral administration of anti-CLEC5A mAb reduces infiltration of virus-harboring leukocytes into the central nervous system (CNS, restores BBB integrity, attenuates neuroinflammation, and protects mice from JEV-induced lethality. Moreover, all surviving mice develop protective humoral and cellular immunity against JEV infection. These observations demonstrate the critical role of CLEC5A in the pathogenesis of Japanese encephalitis, and identify CLEC5A as a target for the development of new treatments to reduce virus-induced brain damage.

  19. Abrogated inflammatory response promotes neurogenesis in a murine model of Japanese encephalitis.

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    Sulagna Das

    Full Text Available BACKGROUND: Japanese encephalitis virus (JEV induces neuroinflammation with typical features of viral encephalitis, including inflammatory cell infiltration, activation of microglia, and neuronal degeneration. The detrimental effects of inflammation on neurogenesis have been reported in various models of acute and chronic inflammation. We investigated whether JEV-induced inflammation has similar adverse effects on neurogenesis and whether those effects can be reversed using an anti-inflammatory compound minocycline. METHODOLOGY/PRINCIPAL FINDINGS: Here, using in vitro studies and mouse models, we observed that an acute inflammatory milieu is created in the subventricular neurogenic niche following Japanese encephalitis (JE and a resultant impairment in neurogenesis occurs, which can be reversed with minocycline treatment. Immunohistological studies showed that proliferating cells were replenished and the population of migrating neuroblasts was restored in the niche following minocycline treatment. In vitro, we checked for the efficacy of minocycline as an anti-inflammatory compound and cytokine bead array showed that production of cyto/chemokines decreased in JEV-activated BV2 cells. Furthermore, mouse neurospheres grown in the conditioned media from JEV-activated microglia exhibit arrest in both proliferation and differentiation of the spheres compared to conditioned media from control microglia. These effects were completely reversed when conditioned media from JEV-activated and minocycline treated microglia was used. CONCLUSION/SIGNIFICANCE: This study provides conclusive evidence that JEV-activated microglia and the resultant inflammatory molecules are anti-proliferative and anti-neurogenic for NSPCs growth and development, and therefore contribute to the viral neuropathogenesis. The role of minocycline in restoring neurogenesis may implicate enhanced neuronal repair and attenuation of the neuropsychiatric sequelae in JE survivors.

  20. European Aedes albopictus and Culex pipiens Are Competent Vectors for Japanese Encephalitis Virus

    Science.gov (United States)

    Desprès, Philippe

    2017-01-01

    Background Japanese encephalitis virus (JEV) is the causative agent of Japanese encephalitis, the leading cause of viral encephalitis in Asia. JEV transmission cycle involves mosquitoes and vertebrate hosts. The detection of JEV RNA in a pool of Culex pipiens caught in 2010 in Italy raised the concern of a putative emergence of the virus in Europe. We aimed to study the vector competence of European mosquito populations, such as Cx. pipiens and Aedes albopictus for JEV genotypes 3 and 5. Findings After oral feeding on an infectious blood meal, mosquitoes were dissected at various times post-virus exposure. We found that the peak for JEV infection and transmission was between 11 and 13 days post-virus exposure. We observed a faster dissemination of both JEV genotypes in Ae. albopictus mosquitoes, when compared with Cx. pipiens mosquitoes. We also dissected salivary glands and collected saliva from infected mosquitoes and showed that Ae. albopictus mosquitoes transmitted JEV earlier than Cx. pipiens. The virus collected from Ae. albopictus and Cx. pipiens saliva was competent at causing pathogenesis in a mouse model for JEV infection. Using this model, we found that mosquito saliva or salivary glands did not enhance the severity of the disease. Conclusions In this study, we demonstrated that European populations of Ae. albopictus and Cx. pipiens were efficient vectors for JEV transmission. Susceptible vertebrate species that develop high viremia are an obligatory part of the JEV transmission cycle. This study highlights the need to investigate the susceptibility of potential JEV reservoir hosts in Europe, notably amongst swine populations and local water birds. PMID:28085881