Characterization of a siberian virus isolated from a patient with progressive chronic tick-borne encephalitis.

Science.gov (United States)

Gritsun, T S; Frolova, T V; Zhankov, A I; Armesto, M; Turner, S L; Frolova, M P; Pogodina, V V; Lashkevich, V A; Gould, E A

2003-01-01

A strain of Tick-borne encephalitis virus designated Zausaev (Za) was isolated in Siberia from a patient who died of a progressive (2-year) form of tick-borne encephalitis 10 years after being bitten by a tick. The complete genomic sequence of this virus was determined, and an attempt was made to correlate the sequence with the biological characteristics of the virus. Phylogenetic analysis demonstrated that this virus belongs to the Siberian subtype of Tick-borne encephalitis virus. Comparison of Za virus with two related viruses, a Far Eastern isolate, Sofjin, and a Siberian isolate, Vasilchenko, revealed differences among the three viruses in pathogenicity for Syrian hamsters, cytopathogenicity for PS cells, plaque morphology, and the electrophoretic profiles of virus-specific nonstructural proteins. Comparative amino acid alignments revealed 10 individual amino acid substitutions in the Za virus polyprotein sequence that were different from those of other tick-borne flaviviruses. Notably, the dimeric form of the Za virus NS1 protein migrated in polyacrylamide gels as a heterogeneous group of molecules with a significantly higher electrophoretic mobility than those of the Sofjin and Vasilchenko viruses. Two amino acid substitutions, T(277)-->V and E(279)-->G, within the NS1 dimerization domain are probably responsible for the altered oligomerization of Za virus NS1. These studies suggest that the patient from whom Za virus was isolated died due to increased pathogenicity of the latent virus following spontaneous mutagenesis.

A case of Herpes encephalitis followed-up by CT

International Nuclear Information System (INIS)

Fukui, Yukiko; Nagai, Shinya; Nishibayashi, Yohei; Okamoto, Hirofumi; Goishi, Junji

1982-01-01

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. (Chiba, N.)

Imaging of limbic para-neoplastic encephalitis; Imagerie de l`encephalite limbique paraneoplastique

Energy Technology Data Exchange (ETDEWEB)

Rimmelin, A.; Sellat, F.; Morand, G.; Quoix, E.; Clouet, P.L.; Dietemann, J.L. [Centre Hospitalier Universitaire, 67 - Strasbourg (France)

1997-09-01

Para-neoplastic limbic encephalitis is a rare syndrome mostly associated with small cell lung cancer. We present the case of a 69-year-old man with selective amnesia suggesting limbic encephalitis. A neuroendocrine cell lung cancer was found, confirming the diagnostics of para-neoplastic limbic encephalitis. Contrast-enhanced cerebral CT was normal whether magnetic resonance imaging showed signal abnormalities of the medial part of temporal lobes and hippocampal regions. Because neurologic improvement may follow treatment of the primary tumor, early diagnosis is important. (authors). 10 refs.

Serial CT scannings in herpes simplex encephalitis

International Nuclear Information System (INIS)

Fukushima, Masashi; Sawada, Tohru; Kuriyama, Yoshihiro; Kinugawa, Hidekazu; Yamaguchi, Takenori

1981-01-01

Two patients with serologically confirmed herpes simplex encephalitis were studied by serial CT scannings. Case 1, a 60-year-old woman, was admitted to National Cardiovascular Center because of headache, fever, and attacks of Jacksonian seizure. Case 2, a 54-year-old man, was admitted because of fever, consciousness disturbance and right hemipare sis. Pleocytosis (mainly lymphocytes) and elevation of protein content in cerebrospinal fluid were observed in both cases. Both patients presented ''das apallische Syndrom'' one month after admission. The diagnosis of herpes simplex encephalitis was confirmed by typical clinical courses and by greater than fourfold rises in serum antibody titer for herpes simplex virus as well as that in cerebrospinal fluid in case 1. Characteristic CT findings observed in these two cases were summarized as follows: Within a week after the onset, no obvious abnormalities could be detected on CT scans (Case 1). Two weeks after the onset, a large low-density area appeared in the left temporal lobe and in the contralateral insular cortex with midline shift toward the right side (Case 2). One month later, an ill-defined linear and ring-like high-density area (Case 1), or a well-defined high-density area (Case 2), that was enhanced after contrast administration, was observed in the large low-density area in the temporal lobe. These findings were considered as characteristic for hemorrhagic encephalitis. These high-density areas disappeared two months later, however, widespread and intensified low-density areas still remained. In both cases, the basal ganglia and thalamus were completely spared on CT scans. From these observations, it can be concluded that serial CT scannings are quite useful for diagnosis of herpes simplex encephalitis. (author)

Herpes simplex virus encephalitis: neuroradiological diagnosis

International Nuclear Information System (INIS)

Struffert, T.; Reith, W.

2000-01-01

Herpes simplex virus encephalitis (HSE) is the most frequent viral encephalitis, as a rule with the starting point and centre within the temporal lobe. If untreated, HSE is usually fatal, thus diagnosis has to be established rapidly. Treatment with Acyclovir should begin as soon possible. As MRI is extremely sensitive in detecting the early inflammatory changes, it should be initially performed, especially as in the early stadium CT may be unspecific. We recommend the following examination protocol: coronar T1-weighted MR imaging before and after administration of gadopentetate dimeglumine, coronar FLAIR sequence and axial T2-weighted imaging. The diagnostic proof is to show the evidence of viral DNA by polymerase chain reaction (PCR) in cerebrospinal liquor. (orig.) [de

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

OpenAIRE

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 ...

Rasmussen's encephalitis presenting as focal cortical dysplasia

Science.gov (United States)

O'Rourke, D.J.; Bergin, A.; Rotenberg, A.; Peters, J.; Gorman, M.; Poduri, A.; Cryan, J.; Lidov, H.; Madsen, J.; Harini, C.

2014-01-01

Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic. PMID:25667877

Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Rasmussen-like Syndrome: An Association?

Science.gov (United States)

Gurcharran, Kevin; Karkare, Shefali

2017-01-01

N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia. We describe a two-year-old girl with progressive right weakness and epilepsia partialis continua, concerning for early Rasmussen's encephalitis, who tested positive for anti-NMDA receptor antibodies. She experienced complete clinical recovery after immunotherapy. Anti-NMDA receptor antibodies were absent at three weeks and again at one year after the first treatment of intravenous immunoglobulin. There are few reports of Rasmussen-like encephalitis in individuals with anti-NMDA receptor antibody positivity. Thus the clinical significance of this association is yet to be determined. In addition, several other antibodies have been documented in individuals with Rasmussen encephalitis. The lack of a consistently reported antibody in Rasmussen encephalitis patients and the temporary nature of the anti-NMDA receptor antibody in our patient raise the following question: Is the presence of anti-NMDA receptor antibodies the cause of the symptoms or secondary to the pathogenic process? Copyright © 2016 Elsevier Inc. All rights reserved.

Serial CT scannings in herpes simplex encephalitis

Energy Technology Data Exchange (ETDEWEB)

Fukushima, M.; Sawada, T.; Kuriyama, Y.; Kinugawa, H.; Yamaguchi, T. (National Cardivascular Center, Osaka (Japan))

1981-10-01

Two patients with serologically confirmed herpes simplex encephalitis were studied by serial CT scannings. Case 1, a 60-year-old woman, was admitted to National Cardiovascular Center because of headache, fever, and attacks of Jacksonian seizure. Case 2, a 54-year-old man, was admitted because of fever, consciousness disturbance and right hemiparesis. Pleocytosis (mainly lymphocytes) and elevation of protein content in cerebrospinal fluid were observed in both cases. Both patients presented ''das apallische Syndrom'' one month after admission. The diagnosis of herpes simplex encephalitis was confirmed by typical clinical courses and by greater than fourfold rises in serum antibody titer for herpes simplex virus as well as that in cerebrospinal fluid in case 1. Characteristic CT findings observed in these two cases were summarized as follows: Within a week after the onset, no obvious abnormalities could be detected on CT scans (Case 1). Two weeks after the onset, a large low-density area appeared in the left temporal lobe and in the contralateral insular cortex with midline shift toward the right side (Case 2). One month later, an ill-defined linear and ring-like high-density area (Case 1), or a well-defined high-density area (Case 2), that was enhanced after contrast administration, was observed in the large low-density area in the temporal lobe. These findings were considered as characteristic for hemorrhagic encephalitis. These high-density areas disappeared two months later, however, widespread and intensified low-density areas still remained. In both cases, the basal ganglia and thalamus were completely spared on CT scans. From these observations, it can be concluded that serial CT scannings are quite useful for diagnosis of herpes simplex encephalitis.

Association of Progressive Cerebellar Atrophy With Long-term Outcome in Patients With Anti-N-Methyl-d-Aspartate Receptor Encephalitis.

Science.gov (United States)

Iizuka, Takahiro; Kaneko, Juntaro; Tominaga, Naomi; Someko, Hidehiro; Nakamura, Masaaki; Ishima, Daisuke; Kitamura, Eiji; Masuda, Ray; Oguni, Eiichi; Yanagisawa, Toshiyuki; Kanazawa, Naomi; Dalmau, Josep; Nishiyama, Kazutoshi

2016-06-01

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disorder that occurs with IgG antibodies against the GluN1 subunit of NMDAR. Some patients develop reversible diffuse cerebral atrophy (DCA), but the long-term clinical significance of progressive brain and cerebellar atrophy is unknown. To report the long-term clinical implications of DCA and cerebellar atrophy in anti-NMDAR encephalitis. A retrospective observational study and long-term imaging investigation was conducted in the Department of Neurology at Kitasato University. Fifteen patients with anti-NMDAR encephalitis admitted to Kitasato University Hospital between January 1, 1999, and December 31, 2014, were included; data analysis was conducted between July 15, 2015, and January 18, 2016. Neurologic examination, immunotherapy, and magnetic resonance imaging (MRI) studies were performed. Long-term MRI changes in association with disease severity, serious complications (eg, pulmonary embolism, septic shock, and rhabdomyolysis), treatment, and outcome. The clinical outcome of 15 patients (median age, 21 years, [range, 14-46 years]; 10 [67%] female) was evaluated after a median follow-up of 68 months (range, 10-179 months). Thirteen patients (87%) received first-line immunotherapy (intravenous high-dose methylprednisolone, intravenous immunoglobulin, and plasma exchange alone or combined), and 4 individuals (27%) also received cyclophosphamide; 2 patients (13%) did not receive immunotherapy. In 5 patients (33%), ovarian teratoma was found and removed. Serious complications developed in 4 patients (27%). Follow-up MRI revealed DCA in 5 patients (33%) that, in 2 individuals (13%), was associated with progressive cerebellar atrophy. Long-term outcome was good in 13 patients (87%) and poor in the other 2 individuals (13%). Although cerebellar atrophy was associated with poor long-term outcome (2 of 2 vs 0 of 13 patients; P = .01), other features, such as DCA without cerebellar atrophy

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.

[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.

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.

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.

Rasmussen's encephalitis presenting as focal cortical dysplasia

Directory of Open Access Journals (Sweden)

D.J. O'Rourke

2014-01-01

Full Text Available Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD and discuss the literature on this topic.

Encephalitis (For Parents)

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... West Nile encephalitis, St. Louis encephalitis, and Western Equine encephalitis. Over the last several years in the ... lack of muscle control. Speech, physical, or occupational therapy may be needed in these cases. It's difficult ...

A rare case of autoimmune limbic encephalitis: an uncharted territory!

Science.gov (United States)

Ibrahim, Hatim; Al Jasser, Abdulelah N; Khan, Sonia A; Tlili, Kalthoum G

2017-10-01

Autoimmune encephalitis is rare. Several auto- antibodies are described in autoimmune encephalitis. We describe a case of autoimmune limbic encephalitis associated with positive voltage gated potassium channel (VGKC) antibodies and positive leucine-rich glioma inactivated protein 1 antibodies (LGI1). A 33-year-old Saudi housewife, she presented with 2 months history of cognitive deterioration and recurrent left facio-brachial dystonic seizures followed by generalized tonic clonic seizures. At times the seizures are preceded by rising epigastric aura and shortness of breath. The neurological examination was normal apart from upgoing left plantar reflex. She had borderline IQ of 76 with impaired verbal fluency and impaired visual and verbal memory. Magnetic resonance imaging of the brain showed right mesial temporal non-enhancing lesion. Cerebrospinal fluid examination was positive for LGI1 and VGKC. Optimal seizure control was achieved with immunotherapy.

Temporal and spatial alterations in mutant swarm size of St. Louis encephalitis virus in mosquito hosts.

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Ciota, Alexander T; Koch, Evan M; Willsey, Graham G; Davis, Lauren J; Jerzak, Greta V S; Ehrbar, Dylan J; Wilke, Claus O; Kramer, Laura D

2011-03-01

St. Louis encephalitis virus (SLEV; Flaviviridae; Flavivirus) is a member of the Japanese encephalitis serocomplex and a close relative of West Nile virus (WNV). Although SLEV remains endemic to the US, both levels of activity and geographical dispersal are relatively constrained when compared to the widespread distribution of WNV. In recent years, WNV appears to have displaced SLEV in California, yet both viruses currently coexist in Texas and several other states. It has become clear that viral swarm characterization is required if we are to fully evaluate the relationship between viral genomes, viral evolution, and epidemiology. Mutant swarm size and composition may be particularly important for arboviruses, which require replication not only in diverse tissues but also divergent hosts. In order to evaluate temporal, spatial, and host-specific patterns in the SLEV mutant swarm, we determined the size, composition, and phylogeny of the intrahost swarm within primary mosquito isolates from both Texas and California. Results indicate a general trend of decreasing intrahost diversity over time in both locations, with recent isolates being highly genetically homogeneous. Additionally, phylogenic analyses provide detailed information on the relatedness of minority variants both within and among strains and demonstrate how both geographic isolation and seasonal maintenance have shaped the viral swarm. Overall, these data generally provide insight into how time, space, and unique transmission cycles influence the SLEV mutant swarm and how understanding these processes can ultimately lead to a better understanding of arbovirus evolution and epidemiology. Copyright © 2010 Elsevier B.V. All rights reserved.

[Autoimmune encephalitis: possibilities in the laboratory investigation].

Science.gov (United States)

Böröcz, Katalin; Hayden, Zsófia; Mészáros, Viktória; Csizmadia, Zsuzsanna; Farkas, Kornélia; Kellermayer, Zoltán; Balogh, Péter; Nagy, Ferenc; Berki, Tímea

2018-01-01

The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABA B R, AMPAR) or synaptic proteins (LGI1, CASPR2). Our aim is to bring to awareness the increasing number of autoimmune encephalitis patients requiring neurologic, psychiatric and intensive care and to emphasize the significance of detecting various autoantibodies in diagnosing patients. In the past 6 years, our laboratory received 836 autoimmune encephalitis diagnostic test requests from a total of 717 patients. Serum and cerebrospinal fluid (CSF) samples were analysed with indirect immunofluorescence using a BIOCHIP consisting of cell lines transfected with 6 different receptor proteins. IgG autoantibodies against receptor proteins were present in 7.5% of patients. The frequency of positive samples was the following: NMDAR > LGI1 > GABA B R > CASPR2. Detecting autoantibodies facilitates the diagnosis of autoimmune encephalitis in an early stage. Patients diagnosed early can be effectively treated with plasmapheresis and immunosuppressive drugs. The efficiency of therapies can be monitored by autoantibody detection. Therefore, the diagnostic immune laboratory plays an important role in proper diagnosis and in the prevention of rapidly progressing symptoms. Orv Hetil. 2018; 159(3): 107-112.

Encephalitis Lethargica

Science.gov (United States)

... as a year after the illness. View Full Definition Treatment Treatment for encephalitis lethargica is symptomatic. Levodopa and other antiparkinson drugs often produce dramatic responses. × Treatment Treatment for encephalitis ...

Orchiectomy for suspected microscopic tumor in patients with anti-Ma2-associated encephalitis.

Science.gov (United States)

Mathew, R M; Vandenberghe, R; Garcia-Merino, A; Yamamoto, T; Landolfi, J C; Rosenfeld, M R; Rossi, J E; Thiessen, B; Dropcho, E J; Dalmau, J

2007-03-20

To report the presence of microscopic neoplasms of the testis in men with anti-Ma2-associated encephalitis (Ma2-encephalitis) and to discuss the clinical implications. Orchiectomy specimens were examined using immunohistochemistry with Ma2 and Oct4 antibodies. Among 25 patients with Ma2-encephalitis younger than 50 years, 19 had germ-cell tumors, and 6 had no evidence of cancer. These 6 patients underwent orchiectomy because they fulfilled five criteria: 1) demonstration of anti-Ma2 antibodies in association with MRI or clinical features compatible with Ma2-encephalitis, 2) life-threatening or progressive neurologic deficits, 3) age Ma2 was expressed by neoplastic cells in three of three patients examined. Even though most patients had severe neurologic deficits at the time of orchiectomy (median progression of symptoms, 10 months), 4 had partial improvement and prolonged stabilization (8 to 84 months, median 22.5 months) and two did not improve after the procedure. In young men with Ma2-encephalitis, 1) the disorder should be attributed to a germ-cell neoplasm of the testis unless another Ma2-expressing tumor is found, 2) negative tumor markers, ultrasound, body CT, or PET do not exclude an intratubular germ-cell neoplasm of the testis, and 3) if no tumor is found, the presence of the five indicated criteria should prompt consideration of orchiectomy.

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 ...

Psychiatric aspects of herpes simplex encephalitis, tick-borne encephalitis and herpes zoster encephalitis among immunocompetent patients.

Science.gov (United States)

Więdłocha, Magdalena; Marcinowicz, Piotr; Stańczykiewicz, Bartłomiej

2015-01-01

The psychopathological symptoms occurring in the course of diseases associated with infections are often initially isolated and non-characteristic, and may cause diagnostic difficulties. Moreover, such disorders tend to be less responsive to psychiatric management. Among possible causes such as trauma, neoplasm and vascular changes, inflammatory changes of the brain as a result of a viral infection should also be considered. There were 452 registered cases of viral encephalitis in Poland in 2010, and although not very prevalent they remain a severe and life-threatening condition. What is more, the frequently occurring neurological and psychiatric complications of viral encephalitis often result in permanent disabilities, causing a significant decrease in the quality of life. This article presents the three types of encephalitis that are most prevalent among immunocompetent patients in Poland, i.e. herpes simplex encephalitis (HSE), tick-borne encephalitis (TBE) and herpes zoster encephalitis (HZE). The psychopathology of the acute phase of the infection, the residual symptoms, features apparent in imaging studies and some neuropathological aspects are also presented. The paper also focuses on psychiatric aspects of the diagnostics and treatment of the described conditions. The clinical pictures of these infections are quite specific, although they cover a wide range of symptoms, and these characteristic features are described. The aim of this review is also to show the significance of thorough diagnostics and a multidisciplinary approach to patients with viral CNS infections.

Upbeat nystagmus in anti-Ma2 encephalitis.

Science.gov (United States)

Garcia-Reitboeck, Pablo; Thompson, Graham; Johns, Paul; Al Wahab, Yasir; Omer, Salah; Griffin, Colette

2014-02-01

Anti-Ma2 encephalitis is a paraneoplastic disorder characterised by brainstem and/or limbic involvement. Eye movement abnormalities can occur in this condition, often with confusion or somnolence. We describe a patient with progressive oscillopsia (with upbeat nystagmus) and unsteadiness, followed by acute pancreatitis. She did not respond to immunomodulatory treatment and subsequently died of complications related to pancreatitis and sepsis. There was no tumour identified at autopsy, but the anti-Ma2 antibodies in her serum and the discovery of a brainstem-predominant inflammatory infiltrate at autopsy strongly suggest a paraneoplastic disorder. Our case illustrates that upbeat nystagmus can be a predominant feature in anti-Ma2 encephalitis; clinicians should consider testing for anti-Ma2 antibodies in patients with upbeat nystagmus of unknown cause.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Children and Adolescents.

Science.gov (United States)

Scheer, Shelly; John, Rita Marie

2016-01-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease that is becoming increasingly recognized in the pediatric population. It may be the most common cause of treatable autoimmune encephalitis. The majority of cases of anti-NMDAR encephalitis are idiopathic in etiology, but a significant minority can be attributed to a paraneoplastic origin. Children with anti-NMDAR encephalitis initially present with a prodrome of neuropsychiatric symptoms, often with orofacial dyskinesias followed by progressively worsening seizures, agitation, and spasticity, which may result in severe neurologic deficits and even death. Definitive diagnosis requires detection of NMDAR antibodies in the cerebrospinal fluid. Optimal outcomes are associated with prompt removal of the tumor in paraneoplastic cases, as well as aggressive immunosuppressive therapy. Early detection is essential for increasing the chances for a good outcome. Close follow-up is required to screen for relapse and later onset tumor presentation. The nurse practitioner plays a major role in the research, screening, diagnosis, treatment, follow-up, and rehabilitation of a child or adolescent with anti-NMDAR encephalitis. Copyright © 2016 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.

Anti‐Ma2 associated paraneoplastic neurological syndrome presenting as encephalitis and progressive muscular atrophy

Science.gov (United States)

Waragai, M; Chiba, A; Uchibori, A; Fukushima, T; Anno, M; Tanaka, K

2006-01-01

A 36 year old man with a history of testicular germ cell tumour presented six months after bilateral orchidectomy with progressive amnesia, irritability, vertical gaze palsy, and generalised seizures. Eight months after initial onset of symptoms, he demonstrated a head drop with muscular atrophy of the upper limbs, shoulder girdle, and posterior neck. He reported no sensory disturbances and his sensory examination was normal. The overall clinical presentation was consistent with motor neurone disease. Cerebrospinal fluid analysis revealed mild pleocytosis and increased protein concentration. Serum and cerebrospinal fluid were positive for the anti‐Ma2 antibody by western blot analysis and immunostaining. Abnormal high signal in the grey matter was noted in the cervical spinal cord and brain by T2 weighted magnetic resonance imaging (MRI). The patient was treated with corticosteroids, intravenous immunoglobulin, and antiepileptic medication. The patient improved clinically and symptom progression ceased after initiation of treatment. There was complete resolution of the abnormal brain MRI lesions; however, the cervical spinal cord MRI lesion and muscular atrophy remained unchanged. It is suggested that the anti‐Ma2 antibody is involved not only in encephalitis, but may also play a role in the cervical spinal cord lesions resulting in a motor neurone disease‐like presentation. PMID:16361608

Anti-Ma2 associated paraneoplastic neurological syndrome presenting as encephalitis and progressive muscular atrophy.

Science.gov (United States)

Waragai, M; Chiba, A; Uchibori, A; Fukushima, T; Anno, M; Tanaka, K

2006-01-01

A 36 year old man with a history of testicular germ cell tumour presented six months after bilateral orchidectomy with progressive amnesia, irritability, vertical gaze palsy, and generalised seizures. Eight months after initial onset of symptoms, he demonstrated a head drop with muscular atrophy of the upper limbs, shoulder girdle, and posterior neck. He reported no sensory disturbances and his sensory examination was normal. The overall clinical presentation was consistent with motor neurone disease. Cerebrospinal fluid analysis revealed mild pleocytosis and increased protein concentration. Serum and cerebrospinal fluid were positive for the anti-Ma2 antibody by western blot analysis and immunostaining. Abnormal high signal in the grey matter was noted in the cervical spinal cord and brain by T2 weighted magnetic resonance imaging (MRI). The patient was treated with corticosteroids, intravenous immunoglobulin, and antiepileptic medication. The patient improved clinically and symptom progression ceased after initiation of treatment. There was complete resolution of the abnormal brain MRI lesions; however, the cervical spinal cord MRI lesion and muscular atrophy remained unchanged. It is suggested that the anti-Ma2 antibody is involved not only in encephalitis, but may also play a role in the cervical spinal cord lesions resulting in a motor neurone disease-like presentation.

A clinical case of pseudotumorous chronic parainfectious limbic encephalitis

Directory of Open Access Journals (Sweden)

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

HHV-6 symptoms in central nervous system. Encephalitis and encephalopathy

International Nuclear Information System (INIS)

Yoshinari, Satoshi; Hamano, Shinichiro

2007-01-01

Described is the present knowledge of central nervous symptoms, mainly encephalitis and encephalopathy, caused by the primary infection of human herpes virus-6 (HHV-6) in the pediatric field. Discovery of HHV-6 is in 1986, the virus, normally latent, has a high nervous affinity, and most infants are infected until the age of 3 years. Encephalitis and encephalopathy caused by the primary infection can be derived from direct viral invasion in nervous system or secondary like that through angitis. Most of early clinical symptoms are febrile convulsion. Imaging of the head by MRI particularly with diffusion weighted imaging and by cerebral blood flow SPECT with 123 I-infetamine (IMP) is important for classification of encephalitis and encephalopathy by HHV-6: Four types of them are defined according to the area of lesion observed in abnormal images, the basal nuclei-diencephalon-brainstem, frontal lobe-dominant one, cerebral hemisphere and diffusive one. Further reviewed are the diagnosis, treatment and prognosis together with other HHV-6 related problems like infection in neonate, temporal lobe epilepsy and drug-induced hypersensitivity syndrome. Current topics are related with activation of latent HHV-6. Despite numerous findings, many remain to be elucidated in acute encephalitis and encephalopathy which are most important in pediatrics. (R.T.)

The clinical usefulness of Tc-99m ECD brain SPECT in acute measles encephalitis

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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.

The clinical usefulness of Tc-99m ECD brain SPECT in acute measles encephalitis

International Nuclear Information System (INIS)

Lim, Seok Tae; Sohn, Myung Hee

2003-01-01

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

Anti-N-methyl-D-aspartate receptor encephalitis: a common cause of encephalitis in the intensive care unit.

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Chen, Xueping; Li, Jin-Mei; Liu, Fan; Wang, Qiong; Zhou, Dong; Lai, Xiaohui

2016-12-01

Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is the most common type of immune-mediated encephalitis. This study aimed to assess the incidence and mortality of anti-NMDAR encephalitis in intensive care unit (ICU) to evaluate the clinical manifestations, laboratory findings, managements and outcomes, and to compare these characteristics with patients with non-anti-NMDAR encephalitis admitted to ICU. Patients admitted to the neurological ICU with suspected encephalitis were included between January 1, 2012 and July 31, 2015. Cerebrospinal fluid (CSF) of enrolled patients was screened for anti-NMDAR antibodies using a cell-based assay. 72 critically ill patients with encephalitis of uncertain etiology were investigated, and 16 patients were positive for anti-NMDAR antibodies in CSF. Compared to patients with non-anti-NMDAR encephalitis, patients with anti-NMDAR encephalitis were younger, more likely to present with the psychiatric symptoms, dyskinesia, and autonomic dysfunction, and had longer ICU stays. The abnormal movements were so difficult to control that complicated the management. The outcome was favorable in ten patients 1 year after the disease onset, and the mortality was as high as 25 % overall. The incidence of anti-NMDAR encephalitis is high among critically ill patients with encephalitis of uncertain etiology. Controlling dyskinesia proved to be a challenge. Persistent dysautonomias were additional difficult to manage confounders. Same points being highlighted in this study may aid clinicians in the management of patients with anti-NMDAR encephalitis in intensive care practice.

[Anti-Ma2-associated encephalitis and paraneoplastic limbic encephalitis].

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Yamamoto, Tomotaka; Tsuji, Shoji

2010-08-01

Anti-Ma2-associated encephalitis (or anti-Ma2 encephalitis) is a paraneoplastic neurological syndrome (PNS) characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. Anti-Ma2 antibodies detected in the serum or cerebrospinal fluid of patients are highly specific for this disease entity and belong to a group of well-characterized onconeuronal antibodies (or classical antibodies). The corresponding antigen, Ma2 is selectively expressed intracellularly in neurons and tumors as is the case with other onconeuronal antigens targeted by classical antibodies. However, in most cases the clinical pictures are different from those of classical PNS and this creates a potential risk of underdiagnosis. Although limbic dysfunction is the most common manifestation in patients with anti-Ma2 encephalitis which is one of the major causes of paraneoplastic limbic encephalitis (LE), it has been reported that less than 30% of the patients with anti-Ma2 LE exhibit clinical presentations typical of the classical description of LE. Of the remaining, many exhibit excessive daytime sleepiness, vertical ophthalmoparesis, or both associated with LE, because of frequent involvement of the diencephalon and/or upper brainstem. Anti-Ma2 LE can also be manifested as a pure psychiatric disturbance such as obsessive-compulsive disorder in a few cases. Some patients develop mesodiencephalic encephalitis with minor involvement of the limbic system, and some may manifest severe hypokinesis. About 40% of the patients with anti-Ma2 antibodies also have antibodies against different epitopes on Ma1, a homologue of Ma2. These patients may have predominant cerebellar and/or brainstem dysfunctions due to more extensive involvement of subtentorial structures. Anti-Ma2 encephalitis is outstanding among other PNS associated with classical antibodies in that the response rate to treatment is relatively high. While it can cause severe neurological deficits or death in a substantial

Sarcocystis neurona encephalitis in a dog.

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Cooley, A J; Barr, B; Rejmanek, D

2007-11-01

A 1.5-year-old male Feist dog was presented to a veterinarian for reluctance to stand on the hind legs. Treatment included dexamethasone and resulted in a favorable initial response, but posterior paresis returned and progressed to recumbency, hyperesthesia, and attempts to bite the owner. The dog was euthanized. The brain was negative for rabies by fluorescent antibody analysis. Multiple foci of encephalitis were found in the cerebrum and particularly in the cerebellum. Protozoa morphologically consistent with Sarcocystis sp. were identified at sites of intense inflammation and malacia. Additionally, multiple schizonts were identified in areas without inflammation. Immunohistochemistry using both polyclonal and monoclonal antibodies specific for Sarcocystis neurona was strongly positive. No reaction to polyclonal antisera for Toxoplasma gondii or Neospora caninum was found. Polymerase chain reaction confirmed that the protozoa were S. neurona. Additional aberrant hosts for S. neurona other than horses have been identified, but S. neurona encephalitis has not been documented previously in the dog.

Frontal infraslow activity marks the motor spasms of anti-LGI1 encephalitis.

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Wennberg, Richard; Steriade, Claude; Chen, Robert; Andrade, Danielle

2018-01-01

The clinical and electrographic features of seizures in anti-LGI1 encephalitis are distinct from those seen in other autoimmune encephalitides or non-encephalitic epilepsies. One electroclinical phenomenon specific to the condition consists of lateralized motor spasms, known as faciobrachial dystonic seizures (FBDS). An electrodecremental pattern overriding a "DC shift" has been described as the EEG correlate of these spasms. We sought to further characterize this pre-spasm infraslow activity (ISA). Continuous video-EEG recordings were acquired in four patients with anti-LGI1 encephalitis: each had frequent motor spasms/FBDS as well as frequent subclinical temporal lobe seizures (an independent indicator of anti-LGI1 encephalitis). In artifact-free recordings obtained using clinical amplifiers equipped with a low frequency analog filter of 0.07 Hz, ISA reliably preceded clinical onset of the motor spasms by ∼1.2 s and preceded the electrodecremental pattern by ∼700 ms. Pre-spasm ISA was invariably recorded contralateral to FBDS, with a voltage topographic maximum over the mid frontal region. The pre-movement ISA differed from the Bereitschaftspotential in timing and topography and was an order of magnitude higher in amplitude. Sporadic FBDS that occurred in association with temporal lobe seizures were preceded by identical ISA. The motor spasms of anti-LGI1 encephalitis are preceded by frontal ISA. A paucity of data at the microscale level precludes mechanistic explanations at the macroscale level, or even determination of the relative contributions of neurons and glia in the generation of the ISA. Although fundamental cellular mechanisms await elucidation, the pre-spasm ISA represents a singular and readily identifiable EEG response to this autoimmune brain disorder. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Outbreaks of Eastern equine encephalitis in northeastern Brazil.

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Silva, Maria L C R; Galiza, Glauco J N; Dantas, Antônio F M; Oliveira, Rafael N; Iamamoto, Keila; Achkar, Samira M; Riet-Correa, Franklin

2011-05-01

Outbreaks of eastern equine encephalitis observed from May 2008 to August 2009 in the Brazilian states of Pernambuco, Ceará, and Paraíba are reported. The disease occurred in 93 farms affecting 229 equids with a case fatality rate of 72.92%. Main clinical signs were circling, depression or hyperexcitability, ataxia, and progressive paralysis with a clinical manifestation period of 3-15 days. Main histologic lesions were a diffuse lymphocytic encephalomyelitis with neuronal death, satellitosis, neuronophagia, and hemorrhages being more severe in the cerebral gray matter of the telencephalon, diencephalon, and mesencephalon. Some animals also had areas of malacia in the telencephalon, thalamus, and basal nuclei. From 1 case, the virus was isolated by mice inoculation, and in other 13 cases was identified as Eastern equine encephalitis virus by semi-nested reverse transcription polymerase chain reaction. After DNA sequencing, all samples were identified as eastern equine encephalitis through the BLASTn analysis, but samples from the Ceará and Paraíba states corresponded to the same cluster, while the sample from the state of Pernambuco corresponded to a different cluster. © 2011 The Author(s)

A spatial and temporal analysis of Japanese encephalitis in mainland China, 1963-1975: a period without Japanese encephalitis vaccination.

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Li, Xiaolong; Gao, Xiaoyan; Ren, Zhoupeng; Cao, Yuxi; Wang, Jinfeng; Liang, Guodong

2014-01-01

More than a million Japanese encephalitis (JE) cases occurred in mainland China from the 1960s to 1970s without vaccine interventions. The aim of this study is to analyze the spatial and temporal pattern of JE cases reported in mainland China from 1965 to 1973 in the absence of JE vaccination, and to discuss the impacts of climatic and geographical factors on JE during that period. Thus, the data of reported JE cases at provincial level and monthly precipitation and monthly mean temperature from 1963 to 1975 in mainland China were collected. Local Indicators of Spatial Association analysis was performed to identify spatial clusters at the province level. During that period, The epidemic peaked in 1966 and 1971 and the JE incidence reached up to 20.58/100000 and 20.92/100000, respectively. The endemic regions can be divided into three classes including high, medium, and low prevalence regions. Through spatial cluster analysis, JE epidemic hot spots were identified; most were located in the Yangtze River Plain which lies in the southeast of China. In addition, JE incidence was shown to vary among eight geomorphic units in China. Also, the JE incidence in the Loess Plateau and the North China Plain was showed to increase with the rise of temperature. Likewise, JE incidence in the Loess Plateau and the Yangtze River Plain was observed a same trend with the increase of rainfall. In conclusion, the JE cases clustered geographically during the epidemic period. Besides, the JE incidence was markedly higher on the plains than plateaus. These results may provide an insight into the epidemiological characteristics of JE in the absence of vaccine interventions and assist health authorities, both in China and potentially in Europe and Americas, in JE prevention and control strategies.

Paraneoplastic brain stem encephalitis.

Science.gov (United States)

Blaes, Franz

2013-04-01

Paraneoplastic brain stem encephalitis can occur as an isolated clinical syndrome or, more often, may be part of a more widespread encephalitis. Different antineuronal autoantibodies, such as anti-Hu, anti-Ri, and anti-Ma2 can be associated with the syndrome, and the most frequent tumors are lung and testicular cancer. Anti-Hu-associated brain stem encephalitis does not normally respond to immunotherapy; the syndrome may stabilize under tumor treatment. Brain stem encephalitis with anti-Ma2 often improves after immunotherapy and/or tumor therapy, whereas only a minority of anti-Ri positive patients respond to immunosuppressants or tumor treatment. The Opsoclonus-myoclonus syndrome (OMS) in children, almost exclusively associated with neuroblastoma, shows a good response to steroids, ACTH, and rituximab, some patients do respond to intravenous immunoglobulins or cyclophosphamide. In adults, OMS is mainly associated with small cell lung cancer or gynecological tumors and only a small part of the patients show improvement after immunotherapy. Earlier diagnosis and treatment seem to be one major problem to improve the prognosis of both, paraneoplastic brain stem encephalitis, and OMS.

CT images of infantile viral encephalitis

International Nuclear Information System (INIS)

Sugimoto, Tateo; Okazaki, Hitoshi; Woo, Man

1985-01-01

Cranial CT scanning was undertaken in 40 patients with infantile viral encephalitis seen from 1977 to 1983. According to the pathogenic viruses, abnormal CT findings were detected most frequently in cases of herpes simplex encephalitis (HSE), followed by non-eruptive viral encephalitis, measles encephalitis, and rubella encephalitis in that order, which coincided well with neurological prognosis. Although CT findings lay within a normal range in cases of measles encephalitis, except a case in which cerebral ventricle was slightly dilated, the degree of consciousness disturbance was unfavorable and it persisted long. This revealed that there is no distinct correlation between the degree of consciousness disturbance and CT findings. Normal CT findings were detected in 13% of patients aged less than 5 years and 76.5% of patients aged 5 years or more. In many patients who had an attack of viral encephalitis at the age of 5 years or more, epileptic seizures occurred frequently, even though CT findings were normal. (Namekawa, K.)

Tick-Borne Encephalitis (TBE)

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... virus, Siberian tick-borne encephalitis virus, and Far eastern Tick-borne encephalitis virus (formerly known as Russian ... viruses are closely related to TBEV and Far-eastern TBE, and include Omsk hemorrhagic fever virus in ...

Regional impact of climate on Japanese encephalitis in areas located near the three gorges dam.

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Yuntao Bai

Full Text Available BACKGROUND: In this study, we aim to identify key climatic factors that are associated with the transmission of Japanese encephalitis virus in areas located near the Three Gorges Dam, between 1997 and 2008. METHODS: We identified three geographical regions of Chongqing, based on their distance from the Three Gorges Dam. Collectively, the three regions consisted of 12 districts from which study information was collected. Zero-Inflated Poisson Regression models were run to identify key climatic factors of the transmission of Japanese encephalitis virus for both the whole study area and for each individual region; linear regression models were conducted to examine the fluctuation of climatic variables over time during the construction of the Three Gorges Dam. RESULTS: Between 1997 and 2008, the incidence of Japanese encephalitis decreased throughout the entire city of Chongqing, with noticeable variations taking place in 2000, 2001 and 2006. The eastern region, which is closest to the Three Gorges Dam, suffered the highest incidence of Japanese encephalitis, while the western region experienced the lowest incidence. Linear regression models revealed that there were seasonal fluctuations of climatic variables during this period. Zero-Inflated Poisson Regression models indicated a significant positive association between temperature (with a lag of 1 and 3 months and Japanese encephalitis incidence, and a significant negative association between rainfall (with a lag of 0 and 4 months and Japanese encephalitis incidence. CONCLUSION: The spatial and temporal trends of Japanese encephalitis incidence that occurred in the City of Chongqing were associated with temperature and rainfall. Seasonal fluctuations of climatic variables during this period were also observed. Additional studies that focus on long-term data collection are needed to validate the findings of this study and to further explore the effects of the Three Gorges Dam on Japanese

[Post-operative progress of patients with Rasmussen's encephalitis who have undergone hemispherotomy surgery].

Science.gov (United States)

Garófalo-Gómez, Nicolás; Hamad, Ana P; Centeno, Ricardo S; Ferrari, Taíssa P; Carrete, Henrique; Caboclo, Luís O; Targas-Yacubian, Elza M

2013-02-16

Rasmussen's encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. The sample selected for study consisted of the cases of RE attended in the Hospital Sao Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. Hemispherotomy must be considered an efficient option for treatment in children with RE.

[Herpetic encephalitis: a clinical case].

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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.

Herpes Simplex Virus-1 Encephalitis in Adults: Pathophysiology, Diagnosis, and Management.

Science.gov (United States)

Bradshaw, Michael J; Venkatesan, Arun

2016-07-01

Herpetic infections have plagued humanity for thousands of years, but only recently have advances in antiviral medications and supportive treatments equipped physicians to combat the most severe manifestations of disease. Prompt recognition and treatment can be life-saving in the care of patients with herpes simplex-1 virus encephalitis, the most commonly identified cause of sporadic encephalitis worldwide. Clinicians should be able to recognize the clinical signs and symptoms of the infection and familiarize themselves with a rational diagnostic approach and therapeutic modalities, as early recognition and treatment are key to improving outcomes. Clinicians should also be vigilant for the development of acute complications, including cerebral edema and status epilepticus, as well as chronic complications, including the development of autoimmune encephalitis associated with antibodies to the N-methyl-D-aspartate receptor and other neuronal cell surface and synaptic epitopes. Herein, we review the pathophysiology, differential diagnosis, and clinical and radiological features of herpes simplex virus-1 encephalitis in adults, including a discussion of the most common complications and their treatment. While great progress has been made in the treatment of this life-threatening infection, a majority of patients will not return to their previous neurologic baseline, indicating the need for further research efforts aimed at improving the long-term sequelae.

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.

Imaging findings of neonatal herpes simplex virus type 2 encephalitis

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Vossough, Arastoo; Zimmerman, Robert A.; Bilaniuk, Larissa T.; Schwartz, Erin M. [University of Pennsylvania, Children' s Hospital of Philadelphia, Philadelphia, PA (United States)

2008-04-15

The CT, MR, and diffusion-weighted initial and follow-up imaging findings in neonatal herpes simplex virus type 2 (HSV-2) encephalitis were assessed. The clinical, laboratory and imaging findings in 12 patients (eight girls and four boys) with proven neonatal HSV-2 encephalitis with follow-up were retrospectively reviewed. Patterns of brain involvement and distribution of lesions were studied and the contribution of diffusion-weighted imaging to the imaging diagnosis of this disease was explored. A total of 24 CT and 22 MRI studies were performed with a mean follow-up time of 38 months. Neonatal HSV-2 encephalitis can be multifocal or limited to only the temporal lobes, brainstem, or cerebellum. The deep gray matter structures were involved in 57% of patients, and hemorrhage was seen in more than half of the patients. CT images were normal or showed mild abnormalities in the early stages of the disease. Conventional MR images may be normal in the early stages of the disease. Lesions were initially seen only by diffusion-weighted imaging in 20% of the patients and this modality showed a substantially more extensive disease distribution in an additional 50% of patients. In 40% of patients, watershed distribution ischemic changes were observed in addition to areas of presumed direct herpetic necrosis. Neonatal HSV-2 encephalitis has a variable imaging appearance. Diffusion-weighted MRI is an important adjunct in the imaging evaluation of this disease. Watershed distribution ischemia in areas remote from the primary herpetic lesions may be seen. (orig.)

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....

Herpes simlex encephalitis. A neuropsychological and neuroradiological study

International Nuclear Information System (INIS)

Koga, Masaya

1994-01-01

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)

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).

Efficacy of VIFERON® in the combination therapy of viral encephalitis in infants

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N. V. Skripchenko

2015-01-01

Full Text Available The paper provides the clinical and ettological characteristics of viral encephalitis in 103 children aged 1 month to 3 years and the results of a comparative study of the efficiency of recombinant interferon-a 2b (VIFERON® as rectal suppositories incorporated into the combination therapy of the disease. The VIFERON® regimen and duration in the study were determined by the pattern of encephalitis. In the acute course of the latter, VIFERON® was given twice daily for 14 days; in its protracted or chronical course, the drug was used twice daily for 14 days, then thrice daily for 1 and 3 months, respectively. The administration of VIFERON® in viral encephalitis was established to accelerate sanogenetic processes and to reduce the time of progression and preservation of major extracerebral and cerebral symptoms and the length of sanitization of cerebrospinal fluid (CSF and viremia. The use of VIFERON® resulted in better disease outcomes, prevented deaths, decreased the rate of an autonomic state from 8 to 1,8%, increased recovery rates without defects from 22 to 51%, and reduced the rate of cystic and gliotic changes in the brain by 2,1 times. Slower CSF sanitization and longer viremia were ascertained in children with chronic and protracted viral encephalitis, which necessitated the longer administration of VIFERON®. Incorporation of recombinant interferon-a 2b into the therapy of viral encephalitis in infants ensures higher serum concentrations of interferon-a and helps keep compensatory capacities for its production, leading to the promptest recovery. The findings suggest that the incorporation of VIFERON® as rectal suppositories into the therapy of viral encephalitis is effective and safe in infants.

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

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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. Copyright © 2016 Elsevier B.V. All rights reserved.

Herpes simplex encephalitis presenting as stroke-like symptoms with atypical MRI findings and lacking cerebrospinal fluid pleocytosis.

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Tsuboguchi, Shintaro; Wakasugi, Takahiro; Umeda, Yoshitaka; Umeda, Maiko; Oyake, Mutsuo; Fujita, Nobuya

2017-07-29

A 73-year-old woman presented with sudden onset of right hemiparesis and was diagnosed as having cerebral infarction on the basis of diffusion-weighted brain MRI, which demonstrated lesions in the left parietal cortex. On the 3rd day, the patient developed right upper limb myoclonus, aphasia, and disturbance of consciousness with high fever. On the 6th day, she was transferred to our hospital with suspected viral encephalitis, and treatment with acyclovir was started. By the 6th day, the lesions detected by MRI had expanded to the gyrus cinguli, insula and thalamus, but not to the temporal lobe. At that time, the CSF cell count was 8/μl, and this later increased to 17/μl by the 13th day. Although herpes simplex virus DNA was detected in the CSF on the 6th day, there was no evidence of CSF pleocytosis or temporal lobe abnormalities demonstrable by brain MRI throughout the whole follow-up period. This was very atypical case of herpes simplex encephalitis characterized by a stroke-like episode, atypical MRI findings, and absence of cerebrospinal fluid pleocytosis. It is important to be mindful that herpes simplex encephalitis (HSE) can have an atypical presentation, and that sufficient acyclovir treatment should be initiated until HSE can be ruled out.

[Wernicke-Korsakoff syndrome secondary to cytomegalovirus encephalitis: A case report].

Science.gov (United States)

Uribe, Luis Guillermo; Pérez, María Alejandra; Lara, Camilo Andrés; Rueda, Natalia; Hernández, Javier Augusto

2017-12-01

Cytomegalovirus (CMV) is one of the opportunistic microorganisms with the highest prevalence in immunocompromised patients. Reactivation has decreased after the introduction of highly active antiretroviral therapy (HAART). Encephalitis has been reported in the coinfection as one of the most frequent presentations.We present the case of a young adult patient with HIV infection and rapid neurological deterioration due to classic clinical symptoms and signs of the Wernicke-Korsakoff syndrome, with no risk factors for thiamine deficiency, with images by nuclear magnetic resonance typical of the syndrome, and identification of cytomegalovirus in cerebrospinal fluid. The specific treatment for CMV managed to control the symptoms with neurological sequelae in progression towards improvement.This is one of the few cases reported in the literature of Wernicke syndrome secondary to cytomegalovirus encephalitis.

Mycoplasma pneumoniae encephalitis

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Schmidt, H.; Korinthenberg, R.; Fahrendorf, G.

1987-07-01

Clinical, CT and, in one case, autopsy findings indicated a diagnosis of a severe necrotising encephalitis in two patients. Although usually herpes simplex virus is blamed for this form of encephalitis, it was possible to prove in these two patients that mycoplasma was the causative agent of the disease. It is concluded that this organism can produce a serious disease in the central nervous system similar to that caused by herpes simplex.

Mycoplasma pneumoniae encephalitis

International Nuclear Information System (INIS)

Schmidt, H.; Korinthenberg, R.; Fahrendorf, G.; Muenster Univ.

1987-01-01

Clinical, CT and, in one case, autopsy findings indicated a diagnosis of a severe necrotising encephalitis in two patients. Although usually herpes simplex virus is blamed for this form of encephalitis, it was possible to prove in these two patients that mycoplasma was the causative agent of the disease. It is concluded that this organism can produce a serious disease in the central nervous system similar to that caused by herpes simplex. (orig.) [de

Fluent Aphasia From Herpes Simplex Encephalitis

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Fariba Yadegari

2006-09-01

Full Text Available The present case report introduces a patient with fluent aphasia, anterograde amnesia and anosmia due to herpes simplex encephalitis after her first delivery. The left medial temporal lobe was one of the main areas involved. On aphasia testing she showed severe anomia on both confrontation and free recall, agraphia, alexia, repetition disorder and some auditory comprehension impairments. Therapy was focused on the following issues: phonological output lexicon , using graphemes as a source of reestablishing phonological representation; describing pictures to reinforce free recall and self-cuing word retrieval strategies; sequencing the events for language memory reinforcement, etc. Results showed improvement in confrontational naming, free recall, correct responses without priming, writing on dictation, spontaneous writing and reading comprehension.

Prostate cancer may trigger paraneoplastic limbic encephalitis

DEFF Research Database (Denmark)

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

2013-01-01

-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...

Dengue encephalitis with predominant cerebellar involvement: Report of eight cases with MR and CT imaging features

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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.)

Dengue encephalitis with predominant cerebellar involvement: Report of eight cases with MR and CT imaging features

International Nuclear Information System (INIS)

Hegde, Vinay; Bhat, Maya; Prasad, Chandrajit; Gupta, A.K.; Saini, Jitender; Aziz, Zarina; Kumar, Sharath; Netravathi, M.

2015-01-01

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.)

Paraneoplastic limbic encephalitis with associated hypothalamitis mimicking a hyperdense hypothalamic tumor: a case report

International Nuclear Information System (INIS)

Bataduwaarachchi, Vipula R.; Tissera, Nirmali

2016-01-01

Paraneoplastic limbic encephalitis is an uncommon association of common malignancies such as small cell lung carcinoma, testicular teratoma, and breast carcinoma. The nonspecific nature of the clinical presentation, lack of freely available diagnostic markers, and requirement for advanced imaging techniques pose a great challenge in the diagnosis of this disease in resource-poor settings. A 64-year-old previously healthy Sri Lankan man was admitted to the general medical unit with subacute memory impairment regarding recent events that had occurred during the previous 3 weeks. Initial noncontrast computed tomography of the brain revealed a hyperdensity in the hypothalamic region surrounded by hypodensities extending toward the bilateral temporal lobes; these findings were consistent with a possible hypothalamic tumor with perilesional edema. The patient later developed cranial diabetes insipidus, which was further suggestive of hypothalamic disease. Interestingly, gadolinium-enhanced magnetic resonance imaging of the brain showed no such lesions; instead, it showed prominent T2-weighted signals in the inner mesial region, characteristic of encephalitis. The possibility of tuberculosis and viral encephalitis was excluded based on cerebrospinal fluid analysis results. Limbic encephalitis with predominant hypothalamitis was suspected based on the radiological pattern. Subsequent screening for underlying malignancy revealed a mass lesion in the right hilum on chest radiographs. Histological examination of the lesion showed small cell lung cancer of the “oat cell” variety. We suggest that the initial appearance of a hyperdensity in the hypothalamus region on noncontrast computed tomography is probably due to hyperemia caused by hypothalamitis. If hypothalamitis is predominant in a patient with paraneoplastic limbic encephalitis, magnetic resonance imaging will help to differentiate it from a hypothalamic secondary deposit. Limbic encephalitis should be considered in

Autoimmune encephalitis and sleep disorders

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Yan HUANG

2017-10-01

Full Text Available Research shows that autoimmune encephalitis is associated with sleep disorders. Paraneoplastic neurological syndrome (PNS with Ma2 antibodies can cause sleep disorders, particularly narcolepsy and rapid eye movement sleep behavior disorder (RBD. Limbic encephalitis (LE and Morvan syndrome, associated with voltage - gated potassium channel (VGKC-complex antibodies, which include leucine-rich glioma-inactivated 1 (LGI1 antibody and contactin-associated protein 2 (Caspr2, can result in profound insomnia and other sleep disorders. Central neurogenic hypoventilation are found in patients with anti-N-methyl-D-aspartate (NMDA receptor encephalitis, whereas obstructive sleep apnea (OSA, stridor and parasomnia are prominent features of encephalopathy associated with IgLON5 antibodies. Sleep disorders are cardinal manifestations in patients with autoimmune encephalitis. Immunotherapy possiblely can improve clinical symptoms and prognosis in a positive way. DOI: 10.3969/j.issn.1672-6731.2017.10.004

Multiphasic presentation of Rasmussen's encephalitis

NARCIS (Netherlands)

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

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

Paraneoplastic limbic encephalitis in a teenage girl with an immature ovarian teratoma

International Nuclear Information System (INIS)

Stein-Wexler, Rebecca; Wootton-Gorges, Sandra L.; Brunberg, James A.; Greco, Claudia M.

2005-01-01

Paraneoplastic limbic encephalitis (PLE) is an unusual disorder that is characterized by the association of clinical limbic system abnormalities with neoplasia, usually malignancy. It has rarely been reported in children and then manifests during the teenage years. Diagnosis is often delayed, especially when the tumor has not been recognized. In adults, the diagnosis can be revealed by the presence of antineuronal antibodies. We describe an unusual case of behavioral disturbance leading rapidly to coma in a 14-year-old girl with CSF pleocytosis who was found 10 weeks later to have an immature ovarian teratoma. Although her symptoms eventually improved slightly after tumor excision, she died while in rehabilitation. PLE is an important diagnosis to consider in the teenage girl with symptoms of a progressive limbic disorder and CSF pleocytosis, and whose brain MR imaging demonstrates no abnormality or mild T2-weighted temporal lobe signal abnormality. When this constellation of findings presents in a teenage girl, the possibility of an underlying ovarian teratoma should be considered. (orig.)

Atrophy progression in semantic dementia with asymmetric temporal involvement: a tensor-based morphometry study.

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Brambati, S M; Rankin, K P; Narvid, J; Seeley, W W; Dean, D; Rosen, H J; Miller, B L; Ashburner, J; Gorno-Tempini, M L

2009-01-01

We performed a longitudinal anatomical study to map the progression of gray matter atrophy in anatomically defined predominantly left (LTLV) and right (RTLV) temporal lobe variants of semantic dementia (SD). T1-weighted MRI scans were obtained at presentation and one-year follow-up from 13 LTLV, 6 RTLV, and 25 control subjects. Tensor-based morphometry (TBM) in SPM2 was applied to derive a voxel-wise estimation of regional tissue loss over time from the deformation field required to warp the follow-up scan to the presentation scan in each subject. When compared to controls, both LTLV and RTLV showed significant progression of gray matter atrophy not only within the temporal lobe most affected at presentation, but also in the controlateral temporal regions (p<0.05 FWE corrected). In LTLV, significant progression of volume loss also involved the ventromedial frontal and the left anterior insular regions. These results identified the anatomic substrates of the previously reported clinical evolution of LTLV and RTLV into a unique 'merged' clinical syndrome characterized by semantic and behavioral deficits and bilateral temporal atrophy.

[Limbic encephalitis with antibodies against intracellular antigens].

Science.gov (United States)

Morita, Akihiko; Kamei, Satoshi

2010-04-01

Limbic encephalitis is a paraneoplastic syndrome that is often associated with small cell lung cancer (SCLC), breast cancer, testicular tumors, teratoma, Hodgkin's lymphoma and thymoma. The common clinical manifestations of limbic encephalitis are subacute onset, cognitive dysfunction, seizures and psychiatric symptoms. Paraneoplastic neurological disorders are considered to occur because of cytotoxic T cell responses and antibodies against target neuronal proteins that are usually expressed by an underlying tumor. The main intracellular antigens related to limbic encephalitis are Hu, Ma2, and less frequently CV2/CRMP5 and amphiphysin. The anti-Hu antibody, which is involved in cerebellar degeneration and extensive or multifocal encephalomyelitis such as limbic encephalitis is closely associated with a history of smoking and SCLC. The anti-Ma2 antibody is associated with encephalitis of the limbic system, hypothalamus and brain-stem. For this reason, some patients with limbic encephalitis have sleep disorders (including REM sleep abnormalities), severe hypokinesis and gaze palsy in addition to limbic dysfunction. In men aged less than 50 years, anti-Ma2 antibody encephalitis is almost always associated with testicular germ-cell tumors that are occasionally difficult to detect. In older men and women, the most common tumors are non-SCLC and breast cancer. Limbic encephalitis associated with cell-surface antigens (e.g., voltage-gated potassium channels, NMDA receptors) is mediated by antibodies and often improves after a reduction in the antibody titer and after tumor resection. Patients with antibodies against intracellular antigens, except for those with anti-Ma2 antibodies and testicular tumors, are less responsive. Early diagnosis and treatment with immunotherapy, tumor resection or both are important for improving or stabilizing the condition of limbic encephalitis.

Anti-N-methyl-D-aspartate receptor encephalitis: analysis of three cases

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Hui SU

2015-07-01

Full Text Available Objective To study clinical features, diagnosis, therapy response and prognosis of anti-N-methyl-D-aspartate receptor (anti-NMDAR encephalitis.  Methods Three cases with anti-NMDAR encephalitis were reported. The clinical features, laboratory examinations, imaging, EEG and therapy response of 3 cases were retrospectively analyzed, and also related literatures were reviewed.  Results Two patients were young male and one patient was old female. Main symptoms included psychiatric symptoms in 3 cases (mania in 2 male patients and stupor in the female patient, epilepsy in 2 cases and respiratory failure in one case. The results of MRI examination revealed normal, while EEG examination showed abnormal in all cases. No tumor was detected in any of these patients. Lumbar puncture revealed normal cerebrospinal fluid (CSF pressure (3 cases, elevated white blood cell (WBC, 3 cases and protein quantification (one case. All cases were confirmed to have the disease by detection of anti-NMDAR antibodies in serum and CSF. One male patient got better after receiving immunotherapy with methylprednisolone and intravenous immunoglobulin (IVIg, but psychiatric symptoms were left over. Another male patient had no response to the above treatment. But the female patient was improved without immunotherapy. All 3 cases were followed up for one year after being discharged. One male patient died by accident because of mental disorders. Another male patient showed no sign of relief. The female patient got mild personality and memory change.  Conclusions Anti-NMDAR encephalitis is a new type of autoimmune encephalitis. It is characterized by fever, memory deficits, seizures, disturbance of consciousness, and autonomic dysfunction in males and females of all ages. This type of encephalitis is often associated with teratoma, and has a good response to immunotherapy. There is a certain correlation between progression and prognosis. DOI: 10.3969/j.issn.1672-6731.2015.07.013

Progressive gray matter reduction of the superior temporal gyrus during transition to psychosis.

Science.gov (United States)

Takahashi, Tsutomu; Wood, Stephen J; Yung, Alison R; Soulsby, Bridget; McGorry, Patrick D; Suzuki, Michio; Kawasaki, Yasuhiro; Phillips, Lisa J; Velakoulis, Dennis; Pantelis, Christos

2009-04-01

Longitudinal magnetic resonance imaging studies have shown progressive gray matter reduction in the superior temporal gyrus during the earliest phases of schizophrenia. It is unknown whether these progressive processes predate the onset of psychosis. To examine gray matter reduction of the superior temporal gyrus over time in individuals at risk for psychosis and in patients with first-episode psychosis. Cross-sectional and longitudinal comparisons. Personal Assessment and Crisis Evaluation Clinic and Early Psychosis Preventions and Intervention Centre. Thirty-five ultrahigh-risk individuals (of whom 12 later developed psychosis [UHRP] and 23 did not [UHRNP]), 23 patients with first-episode psychosis (FEP), and 22 control subjects recruited from the community. Volumes of superior temporal subregions (planum polare, Heschl gyrus, planum temporale, and rostral and caudal regions) were measured at baseline and follow-up (mean, 1.8 years) and were compared across groups. In cross-sectional comparisons, only the FEP group had significantly smaller planum temporale and caudal superior temporal gyrus than other groups at baseline, whereas male UHRP subjects also had a smaller planum temporale than controls at follow-up. In longitudinal comparison, UHRP and FEP patients showed significant gray matter reduction (approximately 2%-6% per year) in the planum polare, planum temporale, and caudal region compared with controls and/or UHRNP subjects. The FEP patients also exhibited progressive gray matter loss in the left Heschl gyrus (3.0% per year) and rostral region (3.8% per year), which were correlated with the severity of delusions at follow-up. A progressive process in the superior temporal gyrus precedes the first expression of florid psychosis. These findings have important implications for underlying neurobiologic features of emerging psychotic disorders and emphasize the importance of early intervention during or before the first episode of psychosis.

Distinct white matter integrity in glutamic acid decarboxylase and voltage-gated potassium channel-complex antibody-associated limbic encephalitis.

Science.gov (United States)

Wagner, Jan; Schoene-Bake, Jan-Christoph; Witt, Juri-Alexander; Helmstaedter, Christoph; Malter, Michael P; Stoecker, Winfried; Probst, Christian; Weber, Bernd; Elger, Christian E

2016-03-01

Autoantibodies against glutamic acid decarboxylase (GAD) and the voltage-gated potassium channel (VGKC) complex are associated with distinct subtypes of limbic encephalitis regarding clinical presentation, response to therapy, and outcome. The aim of this study was to investigate white matter changes in these two limbic encephalitis subtypes by means of diffusion tensor imaging (DTI). Diffusion data were obtained in 14 patients with GAD antibodies and 16 patients with VGKC-complex antibodies and compared with age- and gender-matched control groups. Voxelwise statistical analysis was carried out using tract-based spatial statistics. The results were furthermore compared with those of 15 patients with unilateral histologically confirmed hippocampal sclerosis and correlated with verbal and figural memory performance. We found widespread changes of fractional anisotropy and all diffusivity parameters in GAD-associated limbic encephalitis, whereas no changes were found in VGKC-complex-associated limbic encephalitis. The changes observed in the GAD group were even more extensive when compared against those of the hippocampal sclerosis group, although the disease duration was markedly shorter in patients with GAD antibodies. Correlation analysis revealed areas with a trend toward a negative correlation of diffusivity parameters with figural memory performance located mainly in the right temporal lobe in the GAD group as well. The present study provides further evidence that, depending on the associated antibody, limbic encephalitis features clearly distinct imaging characteristics by showing widespread white matter changes in GAD-associated limbic encephalitis and preserved white matter integrity in VGKC-complex-associated limbic encephalitis. Furthermore, our results contribute to a better understanding of the specific pathophysiologic properties in these two subforms of limbic encephalitis by revealing that patients with GAD antibodies show widespread affections of

Dynamic CRM occupancy reflects a temporal map of developmental progression.

Science.gov (United States)

Wilczyński, Bartek; Furlong, Eileen E M

2010-06-22

Development is driven by tightly coordinated spatio-temporal patterns of gene expression, which are initiated through the action of transcription factors (TFs) binding to cis-regulatory modules (CRMs). Although many studies have investigated how spatial patterns arise, precise temporal control of gene expression is less well understood. Here, we show that dynamic changes in the timing of CRM occupancy is a prevalent feature common to all TFs examined in a developmental ChIP time course to date. CRMs exhibit complex binding patterns that cannot be explained by the sequence motifs or expression of the TFs themselves. The temporal changes in TF binding are highly correlated with dynamic patterns of target gene expression, which in turn reflect transitions in cellular function during different stages of development. Thus, it is not only the timing of a TF's expression, but also its temporal occupancy in refined time windows, which determines temporal gene expression. Systematic measurement of dynamic CRM occupancy may therefore serve as a powerful method to decode dynamic changes in gene expression driving developmental progression.

Japanese encephalitis in a French traveler to Nepal.

Science.gov (United States)

Lagarde, S; Lagier, J-C; Charrel, R; Quérat, G; Vanhomwegen, J; Desprès, P; Pelletier, J; Kaphan, E

2014-02-01

Japanese encephalitis is frequent in Asia, with a severe prognosis, but rare in travelers. Culex mosquitoes transmit Japanese encephalitis virus. Risk factors are destination, duration of stay, summer and fall seasons, outdoor activities, and type of accommodation. We report the case of a French traveler to Nepal with neutralization-based serological confirmed Japanese encephalitis. He presented classical clinical (viral syndrome before an encephalitis status with behavioral disorder, global hypotonia, mutism, movement disorders, seizure, and coma), radiological (lesions of thalami, cortico-spinal tracts, and brainstem) and biological features (lymphocytic meningitis). Nowadays, the presence of Japanese encephalitis virus in Nepal, including mountain areas, is established but Japanese encephalitis remains rare in travelers returning from this area and neurologist physicians need to become familiar with this. We recommend vaccination for travelers spending a long period of time in Nepal and having at-risk outdoor activities.

Diagnostic imaging in 13 cases of Rasmussen's encephalitis. Can early MRI suggest the diagnosis?

International Nuclear Information System (INIS)

Chiapparini, L.; Farina, L.; Ciceri, E.; Erbetta, A.; Savoiardo, M.; Granata, T.; Ragona, F.; Freri, E.; Fusco, L.; Gobbi, G.; Capovilla, G.; Tassi, L.; Viri, M.; Giordano, L.; Bernardina, B.D.; Spreafico, R.

2003-01-01

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ( 1 HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). (orig.)

Relevance of Neuroinflammation and Encephalitis in Autism

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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.

Meningitis and encephalitis in Poland in 2014

Science.gov (United States)

Paradowska-Stankiewicz, Iwona; Piotrowska, Anna

The aim of this study was to assess the epidemiology of meningitis and/or encephalitis in Poland in 2014. In the last three years in Poland, about 3000 cases of meningitis and/or encephalitis of viral or bacterial etiology were recorded annually. Assessment of the epidemiological situation of meningitis and/or encephalitis in Poland in 2014, was based on the results of the analysis of epidemiological reports sent to the NIZP-PZH by the Regional Sanitary-Epidemiological Stations published in the annual bulletin “Infectious diseases and poisonings in Poland in 2014” and “Preventive immunizations in Poland in 2014”. In 2014 in Poland 3488 cases of bacterial meningitis and/or encephalitis were recorded. Almost 61.3% of these were viral infections. In 2014, in comparison to 2013, a 1.1% increase in the number of cases of meningitis and/or encephalitis was observed and 91% with viral etiology.

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...

Voltage-Gated Potassium Channel Antibody Paraneoplastic Limbic Encephalitis Associated with Acute Myeloid Leukemia

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Marion Alcantara

2013-05-01

Full Text Available Among paraneoplastic syndromes (PNS associated with malignant hemopathies, there are few reports of PNS of the central nervous system and most of them are associated with lymphomas. Limbic encephalitis is a rare neurological syndrome classically diagnosed in the context of PNS. We report the case of a 81-year-old man who presented with a relapsed acute myeloid leukemia (AML with minimal maturation. He was admitted for confusion with unfavorable evolution as he presented a rapidly progressive dementia resulting in death. A brain magnetic resonance imaging, performed 2 months after the onset, was considered normal. An electroencephalogram showed non-specific bilateral slow waves. We received the results of the blood screening of neuronal autoantibodies after the patient's death and detected the presence of anti-voltage-gated potassium channel (VGKC antibodies at 102 pmol/l (normal at <30 pmol/l. Other etiologic studies, including the screening for another cause of rapidly progressive dementia, were negative. To our knowledge, this is the first case of anti-VGKC paraneoplastic limbic encephalitis related to AML.

PD-1 Checkpoint Inhibitor Associated Autoimmune Encephalitis

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Stephanie Schneider

2017-05-01

Full Text Available Objective: To report first-hand narrative experience of autoimmune encephalitis and to briefly review currently available evidence of autoimmune encephalitis in cancer patients treated with immune checkpoint inhibitors. Setting: A case study is presented on the management of a patient who developed autoimmune encephalitis during nivolumab monotherapy occurring after 28 weeks on anti-PD-1 monotherapy (nivolumab 3 mg/kg every 2 weeks for non-small cell lung cancer. Results: No substantial improvement was observed by antiepileptic treatment. After administration of 80 mg methylprednisolone, neurologic symptoms disappeared within 24 h and the patient fully recovered. Conclusions: Immune checkpoint inhibitor treatment can lead to autoimmune encephalitis. Clinical trial data indicate a frequency of autoimmune encephalitis of ≥0.1 to <1% with a higher probability during combined or sequential anti-CTLA-4/anti-PD-1 therapy than during anti-PD-1 or anti-PD-L1 monotherapy. Further collection of evidence and translational research is warranted.

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. Copyright © 2016 Elsevier B.V. All rights reserved.

Computer tomography in herpes simplex encephalitis

International Nuclear Information System (INIS)

Hacke, W.; Zeumer, H.

1981-01-01

The CT findings in six patients with confirmed herpes simplex encephalitis were analysed and compared with the literature. Thirty-four examinations were performed, 27 within the first 14 days of the illness. The early findings show three characteristic features: The CT may be entirely normal up to the fourth day. Consistent with clinical and E.E.G. findings there then develops a hypodense temporal lesion which, even at this stage, acts as an expanding lesion in half the patients. Between the sixth and eighth day there is frequently involvement of the contra-lateral temporal lobe, not as a continuation, but as a new lesion. At the same time, or after several days, there is involvement of the basal portions of the frontal lobes. In this late phase there may be necrosis in the basal ganglia, thalamus or other parts of the brain. The CT findings in this late phase are uniform and characteristic of the disease. For early diagnosis and treatment the early clinical, electrophysiological and neuro-radiological findings are important. A normal CT scan two days after the onset of clinical symptoms may be regarded as significant. (orig.) [de

Clinical significance of measurement of changes of serum NSE and plasma NPY levels after treatment in pediatric patients with viral encephalitis

International Nuclear Information System (INIS)

Jin Bo; Zheng Guo

2007-01-01

Objective: To explore the significance ef changes of serum NSE and plasma NPY levels after treatment in pediatric patients with viral encephalitis. Methods: Serum NSE and plasma NPY levels were measured with RIA in 32 pediatric patients with viral encephalitis both before and after treatment as well as in 30 controls. Results: Before treatment, in the patients, the serum NSE and plasma NPY levels were significantly higher than those in controls (P<0.01). After 1 month's treatment the levels dropped markedly but still remained significantly higher than those in controls (P<0.05). Conclusion: Serum NSE and plasma NPY levels changes were closely related to the progress of viral encephalitis. (authors)

OUTCOMES OF TICK-BORNE ENCEPHALITIS IN THE TOMSK REGION

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T. S. Pinegina

2013-01-01

Full Text Available The results of the study outcomes of tick-borne encephalitis in adults in the Tomsk Region. Patients conducted a comprehensive clinical and laboratory examination. Revealed the prevalence of autonomic disorders in individuals who have had at different periods of tick-borne encephalitis, which is regarded as the effects of tick-borne infection. Residual effects of tick-borne encephalitis occurs mainly in the form of light paresis after suffering a focal forms. Among the chronic (progredient forms of tick-borne encephalitis often formed hyperkinetic options. Most of the study revealed the presence of precipitating factors that could have an influence on the outcome. Fundamental diffe rences in all-clinical and immunological analyses at patients with various outcomes of tick-borne encephalitis it wasn't noted. KEY WORDS: tick-borne encephalitis, Tomsk Region, the outcomes.

Ablation of CD11c(hi) dendritic cells exacerbates Japanese encephalitis by regulating blood-brain barrier permeability and altering tight junction/adhesion molecules.

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

2016-10-01

Japanese encephalitis (JE), characterized by extensive neuroinflammation following infection with neurotropic JE virus (JEV), is becoming a leading cause of viral encephalitis due to rapid changes in climate and demography. The blood-brain barrier (BBB) plays an important role in restricting neuroinvasion of peripheral leukocytes and virus, thereby regulating the progression of viral encephalitis. In this study, we explored the role of CD11c(hi) dendritic cells (DCs) in regulating BBB integrity and JE progression using a conditional depletion model of CD11c(hi) DCs. Transient ablation of CD11c(hi) DCs resulted in markedly increased susceptibility to JE progression along with highly increased neuro-invasion of JEV. In addition, exacerbated JE progression in CD11c(hi) DC-ablated hosts was closely associated with increased expression of proinflammatory cytokines (IFN-β, IL-6, and TNF-α) and CC chemokines (CCL2, CCL3, CXCL2) in the brain. Moreover, our results revealed that the exacerbation of JE progression in CD11c(hi) DC-ablated hosts was correlated with enhanced BBB permeability and reduced expression of tight junction and adhesion molecules (claudin-5, ZO-1, occluding, JAMs). Ultimately, our data conclude that the ablation of CD11c(hi) DCs provided a subsidiary impact on BBB integrity and the expression of tight junction/adhesion molecules, thereby leading to exacerbated JE progression. These findings provide insight into the secondary role of CD11c(hi) DCs in JE progression through regulation of BBB integrity and the expression of tight junction/adhesion molecules. Copyright © 2016 Elsevier Ltd. All rights reserved.

Findings of 99mTc-HMPAO Regional Cerebral Blood Flow SPECT in a Case of Herpes Simplex Encephalitis

International Nuclear Information System (INIS)

Ahn, Myeong Im; Lee, Sung Yong; Kim, Jong Woo; Bahk, Yong Whee

1989-01-01

Herpes simplex encephalitis (HSE) is one of the fulminant necrotizing, often fatal sporadic form of the encephalitis caused by herpes simplex type I virus. Characteristically, there is early and almost constant involvement of one or both temporal lobes, although there are common additional areas of involvement. Appropriate early treatment following correct diagnosis by clinical findings, CSF study, EEG and several radiological studies including angiography, radionuclide studies, CT or MRI can reduce its mortality and severity of the sequelae. We report a case of HSE diagnosed by adjuvant study of 99m Tc-HMPAO regional cerebral blood flow SPECT, which showed a marked increase in bitemporal cerebral blood flow in a 24-year-old man.

Children and encephalitis lethargica: a historical review.

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Vilensky, Joel A; Foley, Paul; Gilman, Sid

2007-08-01

Between 1917 and the late 1920s, encephalitis lethargica was an epidemic and often lethal neurologic disease. In adults, it typically elicited severe somatic effects, and in particular, various forms of cranial nerve and motor dysfunction. In children, the psychiatric effects were often as severe as the physical consequences. Approximately one third of affected children underwent a rapid transformation from normal behavior to delinquency, often leading to institutionalization. Many neurologic and psychological theories were advanced to explain these severe behavioral changes, and the therapeutic approaches employed ranged from training in dedicated schools to frontal leucotomy. Whereas epidemiologic associations provide both positive and negative support for an etiologic relationship between encephalitis lethargica and the approximately contemporaneous "Spanish" influenza epidemic, previously unutilized data from children provide some of the strongest links between influenza and encephalitis lethargica. Encephalitis lethargica triggered behavioral changes in children that are not duplicated by any other neurologic condition, with the possible exception of traumatic brain injury. These unique behavioral abnormalities may provide the earliest clear indication of new encephalitis lethargica cases, whether alone or in concert with an influenza epidemic.

Epstein-Barr virus encephalitis and encephalomyelitis: MR findings

International Nuclear Information System (INIS)

Shian, W.J.; Chi, C.S.

1996-01-01

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

Epstein-Barr virus encephalitis and encephalomyelitis: MR findings

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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.

Effect of high-dose dexamethasone on the outcome of acute encephalitis due to Japanese encephalitis virus.

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Hoke, C H; Vaughn, D W; Nisalak, A; Intralawan, P; Poolsuppasit, S; Jongsawas, V; Titsyakorn, U; Johnson, R T

1992-04-01

Death due to Japanese encephalitis usually occurs in the first 5 days of hospitalization as a result of deepening coma with respiratory arrest. Death may result from edema-induced increases in intracranial pressure that might be reduced by the administration of steroids. Sixty-five patients presenting in Thailand to four hospitals with a diagnosis of acute Japanese encephalitis were randomized in a double-masked fashion and stratified by initial mental status into a placebo group (saline) or a treatment group (dexamethasone 0.6 mg/kg intravenously as a loading dose followed by 0.2 mg/kg every 6 h for 5 days). Fifty-five of the 65 had confirmed Japanese encephalitis as demonstrated by detection of virus or by Japanese encephalitis virus-specific IgM antibody. Important outcome measures included mortality (24%, treatment group; 27%, control group), days to alert mental status (3.9 vs. 6.2), and neurologic status 3 months after discharge (45% abnormal in each group). No statistically significant benefit of high-dose dexamethasone could be detected.

Adult-onset Rasmussen encephalitis associated with focal cortical dysplasia.

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Hohenbichler, Katharina; Lelotte, Julie; Lhommel, Renaud; Tahry, Riëm El; Vrielynck, Pascal; Santos, Susana Ferrao

2017-12-01

Rasmussen encephalitis is a rare, devastating condition, typically presenting in childhood. Cases of adult-onset Rasmussen have also been described, but the clinical picture is less defined, rendering final diagnosis difficult. We present a case of adult-onset Rasmussen encephalitis with dual pathology, associated with focal cortical dysplasia and encephalitis. We interpreted the Rasmussen encephalitis to be caused by severe and continuous epileptic activity due to focal cortical dysplasia. The best therapeutic approach for such cases remains unclear.

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome mimicking herpes simplex encephalitis on imaging studies.

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Gieraerts, Christopher; Demaerel, Philippe; Van Damme, Philip; Wilms, Guido

2013-01-01

We present a case in which mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome mimicked the clinical and radiological signs of herpes simplex encephalitis. In a patient with subacute encephalopathy, on computed tomography and magnetic resonance imaging, lesions were present in both temporal lobes extending to both insular regions with sparing of the lentiform nuclei and in both posterior straight and cingulate gyri. Final diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome was based on biochemical investigations on cerebrospinal fluid, electromyogram, muscle biopsy, and genetic analysis. On diffusion-weighted imaging, diffusion restriction was present in some parts of the lesions but not throughout the entire lesions. We suggest that this could be an important sign in the differential diagnosis with herpes simplex encephalitis.

EEG-confirmed epileptic activity in a cat with VGKC-complex/LGI1 antibody-associated limbic encephalitis.

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Pakozdy, Akos; Glantschnigg, Ursula; Leschnik, Michael; Hechinger, Harald; Moloney, Teresa; Lang, Bethan; Halasz, Peter; Vincent, Angela

2014-03-01

A 5-year-old, female client-owned cat presented with acute onset of focal epileptic seizures with orofacial twitching and behavioural changes. Magnetic resonance imaging showed bilateral temporal lobe hyperintensities and the EEG was consistent with ictal epileptic seizure activity. After antiepileptic and additional corticosteroid treatment, the cat recovered and by 10 months of follow-up was seizure-free without any problem. Retrospectively, antibodies to LGI1, a component of the voltage-gated potassium channel-complex, were identified. Feline focal seizures with orofacial involvement have been increasingly recognised in client-owned cats, and autoimmune limbic encephalitis was recently suggested as a possible aetiology. This is the first report of EEG, MRI and long-term follow-up of this condition in cats which is similar to human limbic encephalitis.

Diagnostic imaging in 13 cases of Rasmussen's encephalitis. Can early MRI suggest the diagnosis?

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Chiapparini, L.; Farina, L.; Ciceri, E.; Erbetta, A.; Savoiardo, M. [Dept. of Neuroradiology, Ist. Nazionale Neurologico ' ' C. Besta' ' , Milano (Italy); Granata, T.; Ragona, F.; Freri, E. [Dept. of Paediatric Neurology, Ist. Nazionale Neurologico ' ' C. Besta' ' , Milano (Italy); Fusco, L. [Dept. of Paediatric Neurology, Ospedale Pediatrico Bambino Gesu, Roma (Italy); Gobbi, G. [Dept. of Paediatric Neurology, Ospedale Maggiore Pizzardi, Bologna (Italy); Capovilla, G. [Dept. of Paediatric Neurology, Ospedale Poma, Mantova (Italy); Tassi, L. [Dept. of Epilepsy Surgery, Ospedale Niguarda Ca' Granda, Milano (Italy); Viri, M. [Epilepsy Centre, Ospedale Fatebenefratelli-Oftalmico, Milano (Italy); Giordano, L. [Dept. of Paediatric Neurology, Ospedale Civile, Brescia (Italy); Bernardina, B.D. [Dept. of Paediatric Neurology, Policlinico Borgo Roma, Verona (Italy); Spreafico, R. [Dept. of Neurophysiology, Ist. Nazionale Neurologico ' ' C. Besta' ' , Milano (Italy)

2003-03-01

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ({sup 1}HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). (orig.)

Influenza-associated Encephalitis/Encephalopathy Identified by the Australian Childhood Encephalitis Study 2013-2015.

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Britton, Philip N; Dale, Russell C; Blyth, Christopher C; Macartney, Kristine; Crawford, Nigel W; Marshall, Helen; Clark, Julia E; Elliott, Elizabeth J; Webster, Richard I; Cheng, Allen C; Booy, Robert; Jones, Cheryl A

2017-11-01

Influenza-associated encephalitis/encephalopathy (IAE) is an important cause of acute encephalitis syndrome in children. IAE includes a series of clinicoradiologic syndromes or acute encephalopathy syndromes that have been infrequently reported outside East Asia. We aimed to describe cases of IAE identified by the Australian Childhood Encephalitis study. Children ≤ 14 years of age with suspected encephalitis were prospectively identified in 5 hospitals in Australia. Demographic, clinical, laboratory, imaging, and outcome at discharge data were reviewed by an expert panel and cases were categorized by using predetermined case definitions. We extracted cases associated with laboratory identification of influenza virus for this analysis; among these cases, specific IAE syndromes were identified where clinical and radiologic features were consistent with descriptions in the published literature. We identified 13 cases of IAE during 3 southern hemisphere influenza seasons at 5 tertiary children's hospitals in Australia; 8 children with specific acute encephalopathy syndromes including: acute necrotizing encephalopathy, acute encephalopathy with biphasic seizures and late diffusion restriction, mild encephalopathy with reversible splenial lesion, and hemiconvulsion-hemiplegia syndrome. Use of influenza-specific antiviral therapy and prior influenza vaccination were infrequent. In contrast, death or significant neurologic morbidity occurred in 7 of the 13 children (54%). The conditions comprising IAE are heterogeneous with varied clinical features, magnetic resonance imaging changes, and outcomes. Overall, outcome of IAE is poor emphasizing the need for optimized prevention, early recognition, and empiric management.

[Post-herpes simplex encephalitis chorea: Viral replication or immunological mechanism?].

Science.gov (United States)

Benrhouma, H; Nasri, A; Kraoua, I; Klaa, H; Turki, I; Gouider-Khouja, N

2015-09-01

Herpes simplex encephalitis is a severe neurological condition, whose outcome is improved if treated early with acyclovir. Post-herpes simplex encephalitis with acute chorea has rarely been reported. We report on two observations of children presenting with post-herpes simplex encephalitis with acute chorea, related to two different pathophysiological mechanisms. The first one is an 11-month-old girl developing relapsing herpes simplex encephalitis with chorea due to resumption of viral replication. The second one is a 2-year-old boy with relapsing post-herpes simplex encephalitis acute chorea caused by an immunoinflammatory mechanism. We discuss the different neurological presentations of herpetic relapses, notably those presenting with movement disorders, as well as their clinical, paraclinical, physiopathological, and therapeutic aspects. Post-herpes simplex encephalitis with acute chorea may involve two mechanisms: resumption of viral replication or an immunoinflammatory mechanism. Treatment of post-herpes simplex encephalitis with acute chorea depends on the underlying mechanism, while prevention is based on antiviral treatment of herpes simplex encephalitis with acyclovir at the dose of 20mg/kg/8h for 21 days. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Dietary quality and encephalization in platyrrhine primates

Science.gov (United States)

Allen, Kari L.; Kay, Richard F.

2012-01-01

The high energetic costs of building and maintaining large brains are thought to constrain encephalization. The ‘expensive-tissue hypothesis’ (ETH) proposes that primates (especially humans) overcame this constraint through reduction of another metabolically expensive tissue, the gastrointestinal tract. Small guts characterize animals specializing on easily digestible diets. Thus, the hypothesis may be tested via the relationship between brain size and diet quality. Platyrrhine primates present an interesting test case, as they are more variably encephalized than other extant primate clades (excluding Hominoidea). We find a high degree of phylogenetic signal in the data for diet quality, endocranial volume and body size. Controlling for phylogenetic effects, we find no significant correlation between relative diet quality and relative endocranial volume. Thus, diet quality fails to account for differences in platyrrhine encephalization. One taxon, in particular, Brachyteles, violates predictions made by ETH in having a large brain and low-quality diet. Dietary reconstructions of stem platyrrhines further indicate that a relatively high-quality diet was probably in place prior to increases in encephalization. Therefore, it is unlikely that a shift in diet quality was a primary constraint release for encephalization in platyrrhines and, by extrapolation, humans. PMID:21831898

Dietary quality and encephalization in platyrrhine primates.

Science.gov (United States)

Allen, Kari L; Kay, Richard F

2012-02-22

The high energetic costs of building and maintaining large brains are thought to constrain encephalization. The 'expensive-tissue hypothesis' (ETH) proposes that primates (especially humans) overcame this constraint through reduction of another metabolically expensive tissue, the gastrointestinal tract. Small guts characterize animals specializing on easily digestible diets. Thus, the hypothesis may be tested via the relationship between brain size and diet quality. Platyrrhine primates present an interesting test case, as they are more variably encephalized than other extant primate clades (excluding Hominoidea). We find a high degree of phylogenetic signal in the data for diet quality, endocranial volume and body size. Controlling for phylogenetic effects, we find no significant correlation between relative diet quality and relative endocranial volume. Thus, diet quality fails to account for differences in platyrrhine encephalization. One taxon, in particular, Brachyteles, violates predictions made by ETH in having a large brain and low-quality diet. Dietary reconstructions of stem platyrrhines further indicate that a relatively high-quality diet was probably in place prior to increases in encephalization. Therefore, it is unlikely that a shift in diet quality was a primary constraint release for encephalization in platyrrhines and, by extrapolation, humans.

Moyamoya Disease Mimicking Encephalitis

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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.

Shell Vial culture Assay for the rapid diagnosis of Japanese encephalitis, West Nile and Dengue-2 viral encephalitis

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Badrinath S

2006-01-01

Full Text Available Abstract Background Encephalitis caused by flaviviruses, Japanese encephalitis virus (JEV and West Nile virus (WNV is responsible for significant morbidity and mortality in many endemic countries. Dengue-2 (Den-2 virus is a recent addition to the list of encephalitogenic viruses, after its Central Nervous System (CNS invasion capability has been established. There is a wide array of laboratory tools that have helped us not only in the diagnosis of these conditions but also in understanding their pathogenesis and pathology. However, there are no reports of Shell Vial Culture (SVC, a centrifuge enhanced tissue culture assay that has revolutionized viral culturing in terms of rapidity and sensitivity being optimized for these flaviviral encephalitic conditions. The present study is an attempt to standardize and evaluate the usefulness of SVC for the laboratory diagnosis of JE, WN and Den-2 encephalitis cases and to compare it with Indirect Immunofluorescence (IIF technique that detects cell associated virus antigen. Analysis of the various clinical parameters with respect to viral etiology has also been carried out. Results Pediatric patients constituted the major group involved in the study (92%. Etiological diagnosis of viral encephalitis could be established in twenty nine (58% patients. JE encephalitis was the commonest with 19 (39% cases being positive followed by, WN (9 cases-18% and Den-2 (one case. IIF test could detect antigens of JE, WN and Den-2 viruses in 16(32%, 7(14% and 1 case respectively. Shell vial culture assay picked up all cases that were positive by IIF test. In addition, SVC assay could detect 3 and 2 more cases of JE and WN encephalitis respectively, that were negative by the IIF test. Conclusion Shell vial culture is a rapid and efficient tool for the etiological diagnosis of JE, WN and Den-2 encephalitis cases. Early, prompt collection, transport and processing of the CSF samples, would make SVC a better method for the

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

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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.

Temporal acoustic measures distinguish primary progressive apraxia of speech from primary progressive aphasia.

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Duffy, Joseph R; Hanley, Holly; Utianski, Rene; Clark, Heather; Strand, Edythe; Josephs, Keith A; Whitwell, Jennifer L

2017-05-01

The purpose of this study was to determine if acoustic measures of duration and syllable rate during word and sentence repetition, and a measure of within-word lexical stress, distinguish speakers with primary progressive apraxia of speech (PPAOS) from nonapraxic speakers with the agrammatic or logopenic variants of primary progressive aphasia (PPA), and control speakers. Results revealed that the PPAOS group had longer durations and reduced rate of syllable production for most words and sentences, and the measure of lexical stress. Sensitivity and specificity indices for the PPAOS versus the other groups were highest for longer multisyllabic words and sentences. For the PPAOS group, correlations between acoustic measures and perceptual ratings of AOS were moderately high to high. Several temporal measures used in this study may aid differential diagnosis and help quantify features of PPAOS that are distinct from those associated with PPA in which AOS is not present. Copyright © 2017 Elsevier Inc. All rights reserved.

Putaminal involvement in Rasmussen encephalitis

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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.)

Putaminal involvement in Rasmussen encephalitis

International Nuclear Information System (INIS)

Rajesh, Bhagavatheeswaran; Ashalatha, Radhakrishnan; Kesavadas, Chandrasekharan; Thomas, Bejoy

2006-01-01

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.)

MRI findings of herpes simplex encephalitis

International Nuclear Information System (INIS)

Yoon, Hye Kyung; Chang, Kee Hyun; Han, Moon Hee; Kim, In One; Cha, Sang Hoon; Kim, Sang Joon

1992-01-01

We retrospectively analyzed the MR findings of 12 patients with herpes simplex encephalitis (HSE) (8 months - 64 years old). MR imaging was performed on either a 0.5T (6 patients) or 2.0T (6 patients) superconducting unit with spin echo pulse sequences. The most common and characteristic MR finding consisted of non-hemorrhagic lesions in the cortices of the temporal lobes(12), and insular(6), either bilateral(7) or unilateral(5). The frontal lobe and cingulate gyrus were involved in 4 and 2 patients respectively. Petechial hemorrhage was found in 3 patients. Non-hemorrhagic lesions were shown as high signal intensities on proton and T2WI, and iso- or low signal intensities on T1WI. In conclusion, MR imaging findings described above appear to be characteristic of HSE and were found to be extremely valuable in the diagnosis of HSE

Case report of anti-N-methyl-D-aspartate receptor encephalitis in a middle-aged woman with a long history of major depressive disorder.

Science.gov (United States)

Rong, Xia; Xiong, Zhenzhen; Cao, Bingrong; Chen, Juan; Li, Mingli; Li, Zhe

2017-08-31

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease involving antibodies against the NR1 subunits of NMDARs. The disease shows variable clinical presentation, and involves new-onset acute psychotic symptoms, making it difficult to differentiate from major depressive disorder with psychotic symptoms. Potential associations between this autoimmune disorder and onset or progression of major depressive disorder remains unclear. We present a rare case of a patient who had both major depressive disorder and anti-NMDAR encephalitis and in whom the encephalitis initially went undetected. The patient had been suffering from depressive disorder for more than 6 years without any treatment, when she was hospitalized for new-onset psychotic symptoms. She was initially diagnosed only with major depressive disorder with psychotic symptoms, but antipsychotics did not alleviate symptoms and the patient's psychiatric course began to fluctuate rapidly. Anti-NR1 IgG autoantibodies were detected in cerebrospinal fluid, and the combination of immunotherapy and antipsychotics proved more effective than antipsychotics alone. The patient was then also diagnosed with anti-NMDAR encephalitis. Our case suggests that clinicians should consider anti-NMDAR encephalitis when a patient with depressive disorder shows sudden fluctuations in psychiatric symptoms. It also highlights the need for research into possible relationships between anti-NMDAR encephalitis and major depressive disorder.

[Viral encephalitis virus, a new bioterrorist menace].

Science.gov (United States)

Rigaudeau, Sophie; Micol, Romain; Bricaire, François; Bossi, Philippe

2005-01-29

Often responsible for little known infections, today viral encephalitis viruses appear as a new bioterrorist menace, because of their easy production and their great pathogenic potential. Spraying is the best way to permit the rapid diffusion of certain encephalitis viruses. Diagnosis of viral encephalitis, predominating in tropical surroundings, is difficult. In the majority of cases, symptoms differ little from those of common flu. With supplementary examinations, the biological abnormalities are usually non-specific. There are no characteristic images on scans or MRI. Identification of the virus in the nasopharynx, blood or cerebrospinal fluid, in serology, PCR or RT-PCR permits confirmation of the virus. Treatment is essentially symptomatic and relies on appropriate reanimation measures. Ribavirin can be indicated in some cases such as the Rift Valley fever, but is formally contraindicated in West Nile encephalitis. The aim of terrorist groups who would use this type of weapon is more to provoke panic and disorganisation than to kill as many people as possible.

Japanese Encephalitis in Malaysia: An Overview and Timeline.

Science.gov (United States)

Kumar, Kiven; Arshad, Siti Suri; Selvarajah, Gayathri Thevi; Abu, Jalila; Toung, Ooi Peck; Abba, Yusuf; Yasmin, A R; Bande, Faruku; Sharma, Reuben; Ong, Bee Lee

2018-05-29

Japanese encephalitis (JE) is a vector-borne zoonotic disease caused by the Japanese encephalitis virus (JEV). It causes encephalitis in human and horses, and may lead to reproductive failure in sows. The first human encephalitis case in Malaya (now Malaysia) was reported during World War II in a British prison in 1942. Later, encephalitis was observed among race horses in Singapore. In 1951, the first JEV was isolated from the brain of an encephalitis patient. The true storyline of JE exposure among humans and animals has not been documented in Malaysia. In some places such as Sarawak, JEV has been isolated from mosquitoes before an outbreak in 1992. JE is an epidemic in Malaysia except Sarawak. There are four major outbreaks reported in Pulau Langkawi (1974), Penang (1988), Perak and Negeri Sembilan (1998-1999), and Sarawak (1992). JE is considered endemic only in Sarawak. Initially, both adults and children were victims of JE in Malaysia, however, according to the current reports; JE infection is only lethal to children in Malaysia. This paper describes a timeline of JE cases (background of each case) from first detection to current status, vaccination programs against JE, diagnostic methods used in hospitals and factors which may contribute to the transmission of JE among humans and animals in Malaysia. Copyright © 2018. Published by Elsevier B.V.

Atypical presentation of anti-N-methyl-D-aspartate receptor encephalitis: two case reports.

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Maggio, Maria Cristina; Mastrangelo, Greta; Skabar, Aldo; Ventura, Alessandro; Carrozzi, Marco; Santangelo, Giuseppe; Vanadia, Francesca; Corsello, Giovanni; Cimaz, Rolando

2017-08-16

Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis. The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described. We report the cases of two caucasian girls with an atypical presentation. The first patient was an 8-year-old girl with normal psychomotor development. Over a 4-month period she developed behavioral problems, speech impairment, and deterioration in academic skills. Within 8 months from the onset of symptoms, choreic movements gradually appeared. Hematological, neuroradiological, and neurophysiological examinations were negative; however, her symptoms worsened and treatment with prednisone was started. Although her choreic movements improved within 1 month, her neuropsychological and behavioral symptoms continued. Anti-N-methyl-D-aspartate receptor antibodies in cerebrospinal fluid and in blood were detected. Therapy with intravenously administered immunoglobulins was administered, without improvement of symptoms. After 2 months of steroid treatment, she suddenly started to pronounce some words with a progressive improvement in language and behavior. The second patient was a 14-year-old girl with classic anti-N-methyl-D-aspartate receptor encephalitis, treated successfully with intravenously administered immunoglobulins and methylprednisolone, followed by orally administered prednisone, who developed chronic arthritis of the hip. The arthritis was confirmed by magnetic resonance imaging and associated to antinuclear antigen antibody positivity. One year after the encephalitis presentation, an ovarian cystic mass was identified as a teratoma. The surgical resection

Anti-N-methyl-D-aspartate receptor encephalitis: three cases report and review of literature

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Guan-en ZHOU

2014-07-01

Full Text Available Objective To study the clinical and laboratory features and diagnosis of the patient with anti-N-methyl-D-aspartate receptor (NMDAR encephalitis.  Methods The data of clinical features, laboratory findings, and radiological manifestations of 3 patients with anti-NMDAR encephalitis were reviewed and analyzed. Results Of the 3 patients, 2 were male and one was female. The age was from 33 to 34 years (33.30 years on average. Main symptoms included headache in 2 cases, psychiatric symptoms and speech disorder in 3 cases, different levels of movement disorder in one case and hallucinations in one case. The results of MRI examination revealed gyri swelling, abnormal signal and demyelination of temporal lobe. The EEG showed focal or diffuse slow waves. All cases were confirmed to have the disease by detection of anti-NMDAR antibodies. Both the white blood cell count (3 cases and protein quantification (2 cases elevated. No tumor was detected in any of the patients. All patients were coued after receiving immunotherapy with methylprednisolone and human immunoglobulin.  Conclusions Anti-NMDAR encephalitis is a severe but treatable disorder. The syndrome is highly recognizable clinically and can be confirmed with the demonstration of anti-NMDAR antibodies. Timely diagnosis and treatment may yield a favorable prognosis. doi: 10.3969/j.issn.1672-6731.2014.07.005

Allergy in patients with anti-N-methyl-d-aspartate receptor encephalitis.

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Jiang, Xin-Yue; Zhang, Le; Jiang, Xian; Abdulaziz, Ammar Taha Abdullah; Wang, Yun-Hui; Li, Jin-Mei; Zhou, Dong

2018-02-01

Allergy is a potential outcome of dysregulated immune system. Previous studies have shown the association of allergy and autoimmune diseases, however, there is few study to investigate the relationship between allergy and anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Thus, we investigate the rate of allergy in patients with anti-NMDAR encephalitis and analyze the risk factors. The rate of allergy was investigated in patients with anti-NMDAR encephalitis and was compared with patients with virus encephalitis. The clinical cutaneous characters were described in details. All patients with anti-NMDAR encephalitis were divided into allergic and nonallergic group. Clinical factors were compared in the two groups, and logistic regression model was also used to analyze possible risk factors of allergy. Patients with anti-NMDAR encephalitis had a higher rate of allergy than those with viral encephalitis (22.1% vs 9.2%, odds ratio (OR)=3.23, confidence interval (CI)=1.40-7.42, P=0.006). In patients with anti-NMDAR encephalitis, allergic patients exhibited longer days in hospital (30days vs 22days, P=0.005) and higher occurrence of decreased consciousness (81.5% vs 58.9%, P=0.031), higher rate of complications (77.8% vs 57.9%, P=0.046) and abnormal electroencephalography (EEG) (100% vs 78.6%, P=0.021) than patients without allergy. Cerebrospinal fluid (CSF) antibody titers of allergic patients during the disease course were also higher than nonallergic patients (P=0.004). However, further logistic regression analysis did not reveal independent predictors of allergy. Patients with anti-NMDAR encephalitis show higher allergic rate than those with virus encephalitis. Patients with allergy show higher CSF antibody titers and greater illness severity. However, the final outcome of anti-NMDAR encephalitis was not influenced. Copyright © 2017 Elsevier Inc. All rights reserved.

Anti-NMDA receptor encephalitis. Clinical manifestations and pathophysiology

International Nuclear Information System (INIS)

Iizuka, Takahiro; Sakai, Fumihiko

2008-01-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a new category of treatment-responsive encephalitis associated with 'anti-NMDAR antibodies', which are antibodies to the NR1/NR2 heteromers of NMDAR. The antibodies are detected in the CSF/serum of young women with ovarian teratoma, who typically develop schizophrenia-like psychiatric symptoms, usually preceded by fever, headache, or viral infection-like illness. After reaching the peak of psychosis, most patients developed seizures followed by an unresponsive/catatonic state, decreased level of consciousness, central hypoventilation frequently requiring mechanical ventilation, orofacial-limb dyskinesias, and autonomic symptoms. Brain MRI is usually unremarkable but focal enhancement or medial temporal lobe abnormalities can be observed. The CSF reveals nonspecific changes. Electroencephalography (EEG) often reveals diffuse delta slowing without paroxysmal discharges, despite frequent bouts of seizures. This is a highly characteristic syndrome evolving in 5 stages, namely, the prodromal phase, psychotic phase, unresponsive phase, hyperkinetic phase, and gradual recovery phase. The hyperkinetic phase is the most prolonged and crucial. This disorder is usually severe and can be fatal, but it is potentially reversible. Once patients overcome the hyperkinetic phase, gradual improvement is expected with in months and full recovery can also be expected over 3 or more years. Ovarian teratoma-associated limbic encephalitis (OTLE) was first reported in 1997 when this syndrome was reported independently in 1 Japanese girl and 1 woman, both of whom improved following tumor resection. In 2005, Dalmau and his research group first demonstrated antibodies to novel neuronal cell membrane antigens in 4 women with OTLE in a non-permeabilized culture of hippocampal neurons. Two years later, they identified conformal extracellular epitopes present in the NR1/NR2B heteromers of NMDAR, which are expressed in the hippocampus

Serum uric acid and anti-N-methyl-d-aspartate receptor encephalitis.

Science.gov (United States)

Shu, Yaqing; Wang, Yuge; Lu, Tingting; Li, Rui; Sun, Xiaobo; Li, Jing; Chang, Yanyu; Hu, Xueqiang; Lu, Zhengqi; Qiu, Wei

2017-09-01

Uric acid (UA) levels are associated with autoimmune and neurodegenerative disorders, but their relationship with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is unknown. UA levels were evaluated in 58 patients with anti-NMDAR encephalitis, and 58 age- and sex-matched healthy controls (CTLs). Follow-up evaluations of 30 out of the 58 patients with anti-NMDAR encephalitis were conducted 3 months after admission. Modified Rankin scale (mRS) scores and clinical and cerebrospinal fluid parameters were evaluated in all anti-NMDAR encephalitis patients. Serum UA levels were significantly lower in patients with anti-NMDAR encephalitis than those in CTLs (p anti-NMDAR encephalitis are reduced during attacks compared with those in CTLs, are normalized after treatment, and are associated with disease severity. Copyright © 2017. Published by Elsevier Ltd.

Tick-borne encephalitis: Pathogenesis and clinical implications

Czech Academy of Sciences Publication Activity Database

Růžek, Daniel; Dobler, G.; Mantke, O. D.

2010-01-01

Roč. 8, č. 4 (2010), s. 223-232 ISSN 1477-8939 R&D Projects: GA ČR GPP302/10/P438; GA MŠk(CZ) LC06009 Institutional research plan: CEZ:AV0Z60220518 Keywords : Tick-borne encephalitis * Tick-borne encephalitis virus * Pathogenesis * Clinical data Subject RIV: EE - Microbiology, Virology

Visual pathways involvement in children with acute viral encephalitis

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Voitenkov Vladislav Borisovich

2013-10-01

Full Text Available AIM: To investigate extent and nature of visual pathways involvement in children with acute viral encephalitis. METHODS: Thirty patients(age 5-12 yearswith acute viral encephalitis underwent visual evoked potentials(VEPinvestigation within 12 days from the appearance of the first signs of disease. Latency and amplitude of P100 peak were compared with normative data and between patients with varicella and tick-borne encephalitis. RESULTS: There were no significant differences between children with these two forms of encephalitis. In the whole group in 40% of the cases signs of the visual cortex dysfunction(P100 amplitude loweringand mild slowing of the conductivity along the visual pathways(P100 latency lengtheningwere seen. In 3% of the cases retrobulbar optic neuritis was diagnosed. CONCLUSION:The results indicate that visual pathway have good endurance to the viral encephalitis anatomically, but functionally visual cortex is quite vulnerable towards general disturbances caused by this kind of illness.

St. Louis Encephalitis

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... and Treatment Diagnosis Links & References Fact Sheet Other diseases transmitted by mosquitoes Chikungunya virus Dengue Eastern Equine Encephalitis ... Emerging and Zoonotic Infectious Diseases (NCEZID) Division of Vector-Borne Diseases (DVBD) Email Recommend Tweet YouTube Instagram Listen Watch ...

St. Louis encephalitis virus possibly transmitted through blood transfusion-Arizona, 2015.

Science.gov (United States)

Venkat, Heather; Adams, Laura; Sunenshine, Rebecca; Krow-Lucal, Elisabeth; Levy, Craig; Kafenbaum, Tammy; Sylvester, Tammy; Smith, Kirk; Townsend, John; Dosmann, Melissa; Kamel, Hany; Patron, Roberto; Kuehnert, Matthew; Annambhotla, Pallavi; Basavaraju, Sridhar V; Rabe, Ingrid B

2017-12-01

St. Louis encephalitis virus is a mosquito-borne flavivirus that infrequently causes epidemic central nervous system infections. In the United States, blood donors are not screened for St. Louis encephalitis virus infection, and transmission through blood transfusion has not been reported. During September 2015, St. Louis encephalitis virus infection was confirmed in an Arizona kidney transplant recipient. An investigation was initiated to determine the infection source. The patient was interviewed, and medical records were reviewed. To determine the likelihood of mosquito-borne infection, mosquito surveillance data collected at patient and blood donor residences in timeframes consistent with their possible exposure periods were reviewed. To investigate other routes of exposure, organ and blood donor and recipient specimens were obtained and tested for evidence of St. Louis encephalitis virus infection. The patient presented with symptoms of central nervous system infection. Recent St. Louis encephalitis virus infection was serologically confirmed. The organ donor and three other organ recipients showed no laboratory or clinical evidence of St. Louis encephalitis virus infection. Among four donors of blood products received by the patient via transfusion, one donor had a serologically confirmed, recent St. Louis encephalitis virus infection. Exposure to an infected mosquito was unlikely based on the patient's minimal outdoor exposure. In addition, no St. Louis encephalitis virus-infected mosquito pools were identified around the patient's residence. This investigation provides evidence of the first reported possible case of St. Louis encephalitis virus transmission through blood product transfusion. Health care providers and public health professionals should maintain heightened awareness for St. Louis encephalitis virus transmission through blood transfusion in settings where outbreaks are identified. © 2017 AABB.

Serum cystatin C and anti-N-methyl-D-aspartate receptor encephalitis.

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Shu, Y; Chang, Y; Wu, H; Li, J; Cao, B; Sun, X; Wang, J; Peng, L; Hu, X; Yu, X; Qiu, W

2018-05-01

Cystatin C (CysC) is associated with many neurodegenerative disorders and autoimmune diseases, but its relationship with anti-N-Methyl-D-aspartate receptor (anti-NMDAR) encephalitis is unknown. Serum levels of CysC were determined in 66 patients with anti-NMDAR encephalitis and 115 healthy controls. Of the 66 patients, 30 had a follow-up evaluation at 3 months after admission. Association of CysC with anti-NMDAR encephalitis and its clinical parameters were evaluated in the patients. The serum levels of CysC were significantly lower in patients with anti-NMDAR encephalitis than in controls (0.70 ± 0.13 vs 0.83 ± 0.17 mg/mL, P anti-NMDAR encephalitis patients had significantly increased serum CysC levels (P anti-NMDAR encephalitis and its clinical parameters and that the changes in CysC levels correlate with therapeutic effect. Therefore, our findings provide new insights into the association between serum CysC and anti-NMDAR encephalitis. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Eastern Equine Encephalitis Virus

Energy Technology Data Exchange (ETDEWEB)

Borucki, M. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States)

2010-08-05

Eastern equine encephalitis virus (EEEV) is a mosquito-borne virus capable of causing large outbreaks of encephalitis in humans and horses. In North America, EEEV infection has a very high mortality rate in humans, and survivors often suffer severe neurological sequelae. Interestingly, EEEV infections from South American isolates are generally subclinical. Although EEEV is divided into two antigenic varieties and four lineages, only eleven isolates have been sequenced and eight of these are from the North American variety (Lineage I). Most sequenced strains were collected from mosquitoes and only one human isolate has been sequenced. EEEV isolates exist from a variety of hosts, vectors, years, and geographical locations and efforts should focus on sequencing strains that represent this diversity.

Anti-N-methyl-D-aspartate receptor(NMDAR) antibody encephalitis presents in atypical types and coexists with neuromyelitis optica spectrum disorder or neurosyphilis.

Science.gov (United States)

Qin, Kaiyu; Wu, Wenqing; Huang, Yuming; Xu, Dongmei; Zhang, Lei; Zheng, Bowen; Jiang, Meijuan; Kou, Cheng; Gao, Junhua; Li, Wurong; Zhang, Jinglin; Wang, Sumei; Luan, Yanfei; Yan, Chaoling; Xu, Dan; Zheng, Xinmei

2017-01-05

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases of anti-NMDAR encephalitis with incomplete forms, 3 cases of which were accompanied by neuromyelitis optica spectrum disorder or neurosyphilis respectively. A 33-year-old man presented with dysarthria, movement disorder and occasional seizures. He had 6 relapses in 28 years. When suffered from upper respiratory tract syndrome, he developed behavioral and consciousness impairment. Cranial MRI was normal. Viral PCR studies and oncologic work-up were negative. Anti-NMDAR antibody was detected in CSF and serum. A 21-year-old female manifested dizziness and diplopia ten months and six months before, respectively. Both responded to steroid therapy and improved completely. This time she presented with progressive left limb and facial anesthesia, walking and holding unsteadily. Spinal cord MRI follow-up showed abnormality of medulla oblongata and cervical cord(C1). Anti-AQP4 and anti-NMDAR were positive in CSF. Steroid-pulse therapy ameliorated her symptoms. A 37-year-old male experienced worsening vision. He was confirmed neurosyphilis since the CSF tests for syphilis were positive. Protein was elevated and the oligoclonal IgG bands(OB) and anti-NMDAR was positive in CSF. Anti-aquaporin 4(AQP4) antibodies and NMO-IgG were negative. Cranial MRI showed high FLAIR signal on frontal lobe and low T2 signal adjacent to the right cornu posterious ventriculi lateralis. Treatment for neurosyphlis was commenced with gradual improvement. A 39-year-old male, developed serious behavioral and psychiatric symptoms. Examination showed abnormal pupils and unsteady gait. He was confirmed neurosyphilis according to the CSF tests for syphilis. Anti-NMDAR was positive in CSF and serum

Anti-NMDAR encephalitis, a mimicker of acute infectious encephalitis and a review of the literature

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Darren Wong

2014-01-01

Full Text Available Anti-N-methyl-d-aspartate receptor encephalitis has become an increasingly recognized etiology of acute psychosis in young patients. The diverse constellation of symptoms allows for misdiagnosis as an infectious, psychological, or toxicological entity resulting in delays in treatment with increasing morbidity. We describe a case of anti-NMDAR encephalitis that was a particular challenge to diagnose. Practitioners should maintain a high index of suspicion for anti-NMDAR and related neuroautoimmune syndromes, especially in young patients that present with acute mental status decline or dyskinesia.

Recent progress in West Nile virus diagnosis and vaccination

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De Filette Marina

2012-03-01

Full Text Available Abstract West Nile virus (WNV is a positive-stranded RNA virus belonging to the Flaviviridae family, a large family with 3 main genera (flavivirus, hepacivirus and pestivirus. Among these viruses, there are several globally relevant human pathogens including the mosquito-borne dengue virus (DENV, yellow fever virus (YFV, Japanese encephalitis virus (JEV and West Nile virus (WNV, as well as tick-borne viruses such as tick-borne encephalitis virus (TBEV. Since the mid-1990s, outbreaks of WN fever and encephalitis have occurred throughout the world and WNV is now endemic in Africa, Asia, Australia, the Middle East, Europe and the Unites States. This review describes the molecular virology, epidemiology, pathogenesis, and highlights recent progress regarding diagnosis and vaccination against WNV infections.

Operculum syndrome: unusual feature of herpes simplex encephalitis

NARCIS (Netherlands)

van der Poel, J. C.; Haenggeli, C. A.; Overweg-Plandsoen, W. C.

1995-01-01

Herpes simplex encephalitis in adults and young patients carries a high mortality and morbidity. Its presentation may be nonspecific, sometimes hampering early diagnosis. Two young children are reported with herpes simplex encephalitis in whom the operculum syndrome was an outstanding feature. This

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

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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.

Hemorrhagic Transformation of Scrub Typhus Encephalitis: A Rare Entity.

Science.gov (United States)

Kim, H-C; Yoon, K-W; Yoo, D-S; Cho, C-S

2015-12-01

Central nervous system (CNS) involvement of scrub typhus infection is well known. Most CNS involvement of scrub typhus infection present as meningitis or encephalitis. We report on a patient suffering from hemorrhagic transformation of intracranial lesions caused by Orientia tsutsugamushi. A 53-year-old female farmer who was infected by scrub typhus was treated with doxycycline and recovered from the systemic illness. However, headache persisted. Brain radiologic studies revealed acute intracranial hemorrhage and enhancing lesion, which implied a CNS involvement. Hemorrhagic transformation of encephalitis by scrub typhus is very rare complication and to our best knowledge, this is the first report of hemorrhagic transformation of scrub typhus encephalitis. Clinician should consider the possibility of hemorrhagic transformation of encephalitis in cases of scrub typhus infection.

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.

Anti-N-methyl-D-aspartate receptor-mediated encephalitis in infants and toddlers: case report and review of the literature.

Science.gov (United States)

Goldberg, Ethan M; Titulaer, Maarten; de Blank, Peter M; Sievert, Angela; Ryan, Nicole

2014-02-01

Anti-N-methyl-D-aspartate receptor encephalitis is an increasingly well-recognized inflammatory encephalitis in children and adults. We report a previously healthy 21-month-old girl who presented with behavioral change, self-mutilatory behavior, and echolalia. Over the ensuing weeks, symptoms progressed to include unilateral upper extremity dystonia, gait impairment, dysphagia, and mutism. Magnetic resonance imaging of the brain showed a tiny area of signal abnormality in the subcortical white matter, but was otherwise normal. Continuous video electroencephalography showed slowing of the background rhythm, but was without epileptiform discharges. Lumbar puncture showed a mild pleocytosis of mixed cellularity; bacterial culture and testing for various viral encephalitides were negative. Serum and cerebrospinal fluid was positive for autoantibodies directed against the N-methyl-D-aspartate receptor, and she was diagnosed with anti-N-methyl-D-aspartate receptor encephalitis. The patient was successfully treated with a regimen of immunotherapy that included dexamethasone, intravenous immunoglobulin, and rituximab. One year after initial presentation, the patient remained symptom-free. We further review the clinical characteristics, results of diagnostic studies, treatment, and outcome of infants and toddlers diagnosed with anti-N-methyl-D-aspartate receptor encephalitis that have been previously reported in the literature. Anti-N-methyl-D-aspartate receptor encephalitis is relatively common among infants and toddlers and often presents with a pattern of defining characteristics in this age group, particularly the absence of associated tumor. Copyright © 2014 Elsevier Inc. All rights reserved.

Distemper virus encephalitis exerts detrimental effects on hippocampal neurogenesis.

Science.gov (United States)

von Rüden, E-L; Avemary, J; Zellinger, C; Algermissen, D; Bock, P; Beineke, A; Baumgärtner, W; Stein, V M; Tipold, A; Potschka, H

2012-08-01

Despite knowledge about the impact of brain inflammation on hippocampal neurogenesis, data on the influence of virus encephalitis on dentate granule cell neurogenesis are so far limited. Canine distemper is considered an interesting model of virus encephalitis, which can be associated with a chronic progressing disease course and can cause symptomatic seizures. To determine the impact of canine distemper virus (CDV) infection on hippocampal neurogenesis, we compared post-mortem tissue from dogs with infection with and without seizures, from epileptic dogs with non-viral aetiology and from dogs without central nervous system diseases. The majority of animals with infection and with epilepsy of non-viral aetiology exhibited neuronal progenitor numbers below the age average in controls. Virus infection with and without seizures significantly decreased the mean number of neuronal progenitor cells by 43% and 76% as compared to age-matched controls. Ki-67 labelling demonstrated that hippocampal cell proliferation was neither affected by infection nor by epilepsy of non-viral aetiology. Analysis of CDV infection in cells expressing caspase-3, doublecortin or Ki-67 indicated that infection of neuronal progenitor cells is extremely rare and suggests that infection might damage non-differentiated progenitor cells, hamper neuronal differentiation and promote glial differentiation. A high inter-individual variance in the number of lectin-reactive microglial cells was evident in dogs with distemper infection. Statistical analyses did not reveal a correlation between the number of lectin-reactive microglia cells and neuronal progenitor cells. Our data demonstrate that virus encephalitis with and without seizures can exert detrimental effects on hippocampal neurogenesis, which might contribute to long-term consequences of the disease. The lack of a significant impact of distemper virus on Ki-67-labelled cells indicates that the infection affected neuronal differentiation and

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

Science.gov (United States)

Ferrari, Taíssa P F; Hamad, Ana P A; Caboclo, Luís Otávio S F; Centeno, Ricardo S; Zaninotto, Ana Luiza; Scattolin, Monica; Carrete Junior, Henrique; Lancellotti, Carmem L P; Yacubian, Elza Márcia T

2011-09-01

A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].

Natural course of LGI1 encephalitis

DEFF Research Database (Denmark)

Szots, Monika; Marton, Annamaria; Kover, Ferenc

2014-01-01

. Follow-up brain MRI indicated early hippocampal sclerosis and global brain atrophy in one case characterized by more pronounced cognitive deficit. Memory and verbal fluency were affected most during the natural course of LGI1 encephalitis. LGI1 encephalitis had a monophasic course and spontaneously...... improved, suggesting that a relatively benign natural course may contribute to the favorable outcome observed after immunotherapy. Our data also indicate that LGI1 antibodies can be present in the sera without clinical disease activity....

Anti-N-Methyl-D-aspartate Receptor Encephalitis: A Severe, Potentially Reversible Autoimmune Encephalitis

Science.gov (United States)

Liu, Cai-yun; Zheng, Xiang-Yu; Ma, Chi

2017-01-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is potentially lethal, but it is also a treatable autoimmune disorder characterized by prominent psychiatric and neurologic symptoms. It is often accompanied with teratoma or other neoplasm, especially in female patients. Anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum are characteristic features of the disease, thereby suggesting a pathogenic role in the disease. Here, we summarize recent studies that have clearly documented that both clinical manifestations and the antibodies may contribute to early diagnosis and multidisciplinary care. The clinical course of the disorder is reversible and the relapse could occur in some patients. Anti-NMDAR encephalitis coexisting with demyelinating disorders makes the diagnosis more complex; thus, clinicians should be aware of the overlapping diseases. PMID:28698711

TIME AND PLACE DISTRIBUTION OFACUTE ENCEPHALITIS SYNDROME (AES JAPANESE ENCEPHALITIS (JE CASES IN GORAKHPUR

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G K Singh

2013-03-01

Full Text Available Introduction: 1000 children below the age of 15 years died from encephalitis in the states of UP, Bihar and Assam since 1978. JE vaccinations in 2010 and deep bore wells in 60 districts in India are the two preventive measures in use. Hypothesis generation through a time, place distribution study followed by a risk factor study would help target preventive and curative measures. A spatial temporal analysis of the 2012 encephalitis epidemic in the district of Gorakhpur, having the most cases, is reported. Material and Method Government of UP data on 714 cases of AES/JE occurring during 2012 in Gorakhpur district was analysed. Time and place distribution is described. Various hypotheses on mode of transmission besides other important features of the epidemic were generated. Data was used to create video maps of the 2012 AESJE epidemic using Epi-info 7. Onset of symptoms was used on the time axis and longitude-latitude data from residential details was used to describe the place distribution. Videos were interpreted to draw important inferences which may be used in planning a strategy to break the 2013 epidemic Result: Thirty (4.20% of 714 patients fitting case definitions were confirmed cases of Japanese encephalitis. 148 (20% died. 669 (93.69% were below 15 years of age. Male to female ratio was 1.45:1. On 9th Aug 2012 the usual 5 cases per day mark was crossed with 10 cases/day reported. On 22th August the peak of 19 cases/day was reached. On 11th September the epidemic started receding at rates slower than the rise showing multiple spurts. The medical college had 1.5 times the cases than anywhere else. On 10th Dec the daily incidence had returned to under 5 levels. District wise place distribution of the 2009, 2011 and 2012 cases shows Gorakhpur as having 714 i.e. twice the number of cases than anywhere else in 2012. Conclusion: The epidemic is seasonal and perhaps spreads man to man. Mosquito having a life time range of 5 miles cannot spread

Herpes Simplex Encephalitis Presenting with Normal CSF Analysis

International Nuclear Information System (INIS)

Ahmed, R.; Kiani, I. G.; Shah, F.; Rehman, R. N.; Haq, M. E.

2013-01-01

A 28 years old female presented with headache, fever, altered sensorium and right side weakness for one week. She was febrile and drowsy with right sided hemiplegia and papilledema. Tuberculous or bacterial meningitis, tuberculoma and abscess were at the top of the diagnosis list followed by Herpes simplex meningo-encephalitis (HSE). MRI showed abnormal signal intensity of left temporal lobe without significant post-contrast enhancement and midline shift. CSF examination was normal, gram stain and Ziehl-Neelsen stain showed no micro-organism, or acid fast bacilli. CSF for MTB PCR was negative. PCR DNA for Herpes simplex 1 on CSF was detected. Acyclovir was started and the patient was discharged after full recovery. A high index of suspicion is required for HSE diagnosis in Pakistan where other infections predominantly affect the brain and HSE may be overlooked as a potential diagnosis. (author)

CT findings in a case of Japanese encephalitis

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Toyomasu, Teruo; Nakashima, Kenichi; Matsumoto, Tomie; Shida, Kenshiro

1982-01-01

A 44-year-old man was admitted to a hospital on August 1980, with chief complaints of high fever and consciousness disturbance. Three months later he was referred to our hospital. Neurological examination revealed mental deterioration, amnesia, bilateral pyramidal signs, tremor, truncal ataxia and others. Serum CF titer to Japanese encephalitis virus was 1 : 16. He was diagnosed as having Japanese encephalitis from the clinical features and serological response. CT scans showed low density areas in bilateral thalami, the left ganglia, left internal capsule, left substantia nigra and others. It is noticeable that the CT findings were compatible with the pathological changes of Japanese encephalitis. (author)

Mycotic encephalitis: predilection for grey matter

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Knaap, M.S. van der; Valk, J.; Jansen, G.H.; Kapelle, L.J.; Nieuwenhuizen, O. van

1993-01-01

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.)

Mycotic encephalitis: predilection for grey matter

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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.)

MRI in tick-borne encephalitis

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Alkadhi, H.; Kollias, S.S.

2000-01-01

The tick-borne encephalitis (TBE) virus gives rise to epidemic encephalitis. Mild forms usually manifest as influenza-like episodes or are clinically silent. MRI is usually normal in TBE. We describe severe TBE in a patient who presented with fever and altered mental status after a tick bite and a specific antibody response to TBE. MRI revealed pronounced signal abnormalities in the basal ganglia and thalamus, without contrast enhancement. These findings coincide well with neuropathological studies of severe nerve cell degeneration with inflammatory cell infiltrates, neuronophagia and reactive astrocytosis in the deep grey matter. We review the literature and discuss the relevant differential diagnosis. (orig.)

[18F]-Fluoro-Deoxy-Glucose Positron Emission Tomography Scan Should Be Obtained Early in Cases of Autoimmune Encephalitis

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C. R. Newey

2016-01-01

Full Text Available Introduction. Autoimmune encephalitis (AE is a clinically challenging diagnosis with nonspecific neurological symptoms. Prompt diagnosis is important and often relies on neuroimaging. We present a case series of AE highlighting the importance of an early [18F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET scan. Methods. Retrospective review of seven consecutive cases of autoimmune encephalitis. Results. All patients had both magnetic resonance imaging (MRI and FDG-PET scans. Initial clinical presentations included altered mental status and/or new onset seizures. Six cases had serum voltage-gated potassium channel (VGKC antibody and one had serum N-methyl-D-aspartate (NMDA antibody. MRI of brain showed mesial temporal lobe hyperintensity in five cases of VGKC. The other two patients with VGKC or NMDA AE had restiform body hyperintensity on MRI brain or a normal MRI, respectively. Mesial temporal lobe hypermetabolism was noted in three cases on FDG-PET, despite initial unremarkable MRI. Malignancy workup was negative in all patients. Conclusion. A high index of suspicion for AE should be maintained in patients presenting with cognitive symptoms, seizures, and limbic changes on neuroimaging. In cases with normal initial brain MRI, FDG-PET can be positive. Additionally, extralimbic hyperintensity on MRI may also be observed.

[18F]-Fluoro-Deoxy-Glucose Positron Emission Tomography Scan Should Be Obtained Early in Cases of Autoimmune Encephalitis

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Sarwal, A.; Hantus, S.

2016-01-01

Introduction. Autoimmune encephalitis (AE) is a clinically challenging diagnosis with nonspecific neurological symptoms. Prompt diagnosis is important and often relies on neuroimaging. We present a case series of AE highlighting the importance of an early [18F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET) scan. Methods. Retrospective review of seven consecutive cases of autoimmune encephalitis. Results. All patients had both magnetic resonance imaging (MRI) and FDG-PET scans. Initial clinical presentations included altered mental status and/or new onset seizures. Six cases had serum voltage-gated potassium channel (VGKC) antibody and one had serum N-methyl-D-aspartate (NMDA) antibody. MRI of brain showed mesial temporal lobe hyperintensity in five cases of VGKC. The other two patients with VGKC or NMDA AE had restiform body hyperintensity on MRI brain or a normal MRI, respectively. Mesial temporal lobe hypermetabolism was noted in three cases on FDG-PET, despite initial unremarkable MRI. Malignancy workup was negative in all patients. Conclusion. A high index of suspicion for AE should be maintained in patients presenting with cognitive symptoms, seizures, and limbic changes on neuroimaging. In cases with normal initial brain MRI, FDG-PET can be positive. Additionally, extralimbic hyperintensity on MRI may also be observed. PMID:27559482

VGKC complex antibodies in pediatric severe acute encephalitis: a study and literature review.

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Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Wang, Huei-Shyong; Chiu, Cheng-Hsun; CHEESE Study Group

2013-08-01

Antibodies to surface proteins like voltage-gated potassium channel (VGKC) complexes are increasingly found in different neurologic diseases and encephalitis in adults and recently, in children. Detecting such antibodies can help identify forms of encephalitis that may respond to immuno-therapies. However, there are few reports on VGKC complex antibodies in pediatric severe acute encephalitis. This study retrospectively reviewed antibodies to VGKC, leucine-rich glioma-inactivated 1 (Lgi1), and contactin-associated protein-like 2 (Caspr2) in 46 children with severe acute encephalitis. Published cases of VGKC complex antibodies in pediatric encephalitis in the period of 2000-2012 were also reviewed. Elevated VGKC complex antibodies (>100pM) were detected in one of the 46 children with severe acute encephalitis. The 4-year and 6-month-old girl presented with seizure and disturbed consciousness. Viral PCR/culture and serologic evidence of influenza A infection was noted. She also had complications of epilepsy, impaired cognition, and altered behavior and psychology. Antibodies to Lgi1 and Caspr2 were not detected. Ten previously published reports revealed that VGKC complex antibodies can occur in children with limbic encephalitis and acute or sub-acute encephalitis. The incidence of VGKC complex antibodies in pediatric severe acute encephalitis is not high with only one (2.2%) of 46 children in this study. And, this is the first report on the association of VGKC complex antibodies and patients with influenza A-related severe acute encephalitis. The mechanism of VGKC complex antibodies in pediatric severe acute encephalitis warrants further study. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Management of Refractory Orofacial Dyskinesia Caused by Anti-N-methyl-d-aspartate Receptor Encephalitis Using Botulinum Toxin

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Feixia Zheng

2018-02-01

Full Text Available The use of botulinum neurotoxin serotype A (BoNT-A injections for the treatment of orofacial dyskinesia secondary to anti-N-methyl-d-aspartate receptor (NMDAR encephalitis is rarely reported. Here, we report a case of an urgent, successful management of severe orofacial dyskinesia in an 8-year-old girl with anti-NMDAR encephalitis using BoNT-A injection. The patient presented with de novo unilateral paroxysmal movement disorder progressing to generalized dystonia and repetitive orofacial dyskinesia. Diagnosis was confirmed by the presence of NMDAR antibodies in serum and cerebrospinal fluid. The orofacial dyskinesia worsened despite the aggressive use of first-line immunotherapy and second-line immunotherapy (rituximab, and resulted in a potentially fatal self-inflicted oral injury. We urgently attempted symptomatic management using BoNT-A injections in the masseter, and induced muscle paralysis using vecuronium. The patient’s severe orofacial dyskinesia was controlled. We observed the effects of the BoNT-A injections and a tapering off of the effects of vecuronium 10 days after the treatment. The movement disorder had improved significantly 4 weeks after the first administration of rituximab. The injection of BoNT-A into the masseter may be an effective treatment for medically refractory orofacial dyskinesia in pediatric patients with anti-NMDAR encephalitis. We propose that the use of BoNT-A injections should be considered early to avoid self-inflicted oral injury due to severe refractory orofacial dyskinesia in patients with anti-NMDAR encephalitis.

Biopsy histopathology in herpes simplex encephalitis and in encephalitis of undefined etiology.

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Booss, J.; Kim, J. H.

1984-01-01

The histopathology of herpes simplex encephalitis (HSE) has been described principally from postmortem studies which reveal end-stage disease. Biopsy material, which selects an earlier stage in disease development, has been used principally to isolate virus, identify viral particles, and locate viral antigens. Further, little attention has been paid to the histopathology of biopsies of encephalitis of undefined etiology. In the present study, sections from biopsies which yielded virus and those which were negative for virus were evaluated in a systematic and controlled manner. Biopsies yielding virus were characterized by meningeal inflammation, perivascular infiltrates, and glial nodules. Biopsies which did not yield virus and which failed to reveal another diagnosis were characterized by nonspecific gliosis. Thus the early histiopathology of HSE is characterized by early signs of inflammation in the absence of necrosis and generally differs from biopsies in which virus is not isolated. PMID:6098083

[Autoimmune Encephalitis Associated with Malignant Tumors].

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Inuzuka, Takashi

2016-09-01

Autoimmune encephalitis consists of limbic symptoms and signs associated with antibodies against neuronal cell-surface antigens or intracellular antigens. Some cases are known to be associated with anti-channel or anti-receptor-related molecule antibodies. Whether these cases are paraneoplastic depends on the kinds of antigens that the antibodies are produced against. Other cases due to well-characterized onco-neural antibodies are almost always paraneoplastic and are generally resistant to anti-tumor therapy and/or immunotherapy. An exception is anti-Ma2 antibody-positive encephalitis associated with a testicular tumor. Antibodies for intracellular antigens are considered not to be pathogenic. Rather, the T-cell response is thought to be responsible. These antibodies are useful markers for the diagnosis of paraneoplastic disorders and in the search for underlying cancer, as neurological symptoms often precede tumor diagnosis. There is a relationship among onco-neural antibodies, clinical features, tumor types, and response to immunotherapy. Here we describe the characteristics of autoimmune encephalitis cases with antibodies against different intracellular antigens, such as Hu, Ma2, CRMP5, or amphiphysin.

Diagnostic Pathways as Social and Participatory Practices: The Case of Herpes Simplex Encephalitis.

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Jessie Cooper

Full Text Available Herpes simplex virus (HSV encephalitis is a potentially devastating disease, with significant rates of mortality and co-morbidities. Although the prognosis for people with HSV encephalitis can be improved by prompt treatment with aciclovir, there are often delays involved in the diagnosis and treatment of the disease. In response, National Clinical Guidelines have been produced for the UK which make recommendations for improving the management of suspected viral encephalitis. However, little is currently known about the everyday experiences and processes involved in the diagnosis and care of HSV encephalitis. The reported study aimed to provide an account of the diagnosis and treatment of HSV encephalitis from the perspective of people who had been affected by the condition. Thirty narrative interviews were conducted with people who had been diagnosed with HSV encephalitis and their significant others. The narrative accounts reveal problems with gaining access to a diagnosis of encephalitis and shortfalls in care for the condition once in hospital. In response, individuals and their families work hard to obtain medical recognition for the problem and shape the processes of acute care. As a consequence, we argue that the diagnosis and management of HSV encephalitis needs to be considered as a participatory process, which is co-produced by health professionals, patients, and their families. The paper concludes by making recommendations for developing the current management guidelines by formalising the critical role of patients and their significant others in the identification, and treatment of, HSV encephalitis.

Diagnostic Pathways as Social and Participatory Practices: The Case of Herpes Simplex Encephalitis.

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Cooper, Jessie; Kierans, Ciara; Defres, Sylviane; Easton, Ava; Kneen, Rachel; Solomon, Tom

2016-01-01

Herpes simplex virus (HSV) encephalitis is a potentially devastating disease, with significant rates of mortality and co-morbidities. Although the prognosis for people with HSV encephalitis can be improved by prompt treatment with aciclovir, there are often delays involved in the diagnosis and treatment of the disease. In response, National Clinical Guidelines have been produced for the UK which make recommendations for improving the management of suspected viral encephalitis. However, little is currently known about the everyday experiences and processes involved in the diagnosis and care of HSV encephalitis. The reported study aimed to provide an account of the diagnosis and treatment of HSV encephalitis from the perspective of people who had been affected by the condition. Thirty narrative interviews were conducted with people who had been diagnosed with HSV encephalitis and their significant others. The narrative accounts reveal problems with gaining access to a diagnosis of encephalitis and shortfalls in care for the condition once in hospital. In response, individuals and their families work hard to obtain medical recognition for the problem and shape the processes of acute care. As a consequence, we argue that the diagnosis and management of HSV encephalitis needs to be considered as a participatory process, which is co-produced by health professionals, patients, and their families. The paper concludes by making recommendations for developing the current management guidelines by formalising the critical role of patients and their significant others in the identification, and treatment of, HSV encephalitis.

Herpes simplex encephalitis: MRI findings in two cases confirmed by polymerase chain reaction assay

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Lee, J.W.; Kim, I.O.; Kim, W.S.; Yeon, K.M. [Dept. of Radiology and the Institute of Radiation Medicine, Seoul National University Hospital, Seoul (Korea); Lee, H.-J.; Hwang, Y.S. [Dept. of Paediatrics, Seoul National University College of Medicine, Seoul (Korea)

2001-09-01

Herpes simplex virus (HSV) type I causes a fulminant necrotising meningoencephalitis distinguished from other encephalitides by its focal and often haemorrhagic nature. Specific antiviral therapy with acyclovir can significantly improve the prognosis. We present MRI findings of two cases of herpes simplex encephalitis (HSE) confirmed by PCR analysis, focusing on the serial changes after acyclovir therapy: gyral swelling, high signal intensity on T2-weighted images in the subfrontal region, temporal lobe and insula in the initial stage, then regional extension with enhancement and haemorrhage despite appropriate acyclovir therapy, and finally encephalomalacia and brain atrophy. (orig.)

Herpes simplex encephalitis: MRI findings in two cases confirmed by polymerase chain reaction assay

International Nuclear Information System (INIS)

Lee, J.W.; Kim, I.O.; Kim, W.S.; Yeon, K.M.; Lee, H.-J.; Hwang, Y.S.

2001-01-01

Herpes simplex virus (HSV) type I causes a fulminant necrotising meningoencephalitis distinguished from other encephalitides by its focal and often haemorrhagic nature. Specific antiviral therapy with acyclovir can significantly improve the prognosis. We present MRI findings of two cases of herpes simplex encephalitis (HSE) confirmed by PCR analysis, focusing on the serial changes after acyclovir therapy: gyral swelling, high signal intensity on T2-weighted images in the subfrontal region, temporal lobe and insula in the initial stage, then regional extension with enhancement and haemorrhage despite appropriate acyclovir therapy, and finally encephalomalacia and brain atrophy. (orig.)

Hypocretin-1 CSF levels in anti-Ma2 associated encephalitis.

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Overeem, S; Dalmau, J; Bataller, L; Nishino, S; Mignot, E; Verschuuren, J; Lammers, G J

2004-01-13

Idiopathic narcolepsy is associated with deficient hypocretin transmission. Narcoleptic symptoms have recently been described in paraneoplastic encephalitis with anti-Ma2 antibodies. The authors measured CSF hypocretin-1 levels in six patients with anti-Ma2 encephalitis, and screened for anti-Ma antibodies in patients with idiopathic narcolepsy. Anti-Ma autoantibodies were not detected in patients with idiopathic narcolepsy. Four patients with anti-Ma2 encephalitis had excessive daytime sleepiness; hypocretin-1 was not detectable in their cerebrospinal fluid, suggesting an immune-mediated hypocretin dysfunction.

Japanese encephalitis can trigger anti-N-methyl-D-aspartate receptor encephalitis.

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Ma, Jiannan; Zhang, Ting; Jiang, Li

2017-06-01

Japanese encephalitis (JE) is usually a monophasic disease; however, in rare cases, patients with JE may have an early relapse after a partial recovery, giving rise to a biphasic pattern for the disease. In this study, we report three pediatric cases in which post-JE relapse was characterized by movement disorder and/or behavioral problems, and was related to anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G (IgG). Serum and cerebrospinal fluid were examined for anti-NMDAR IgG in three patients who had confirmed JE and then developed relapsing symptoms which were similar to those of anti-NMDAR encephalitis. The main symptoms of the two young children were choreoathetosis, irritability, and sleep disorder; while for the teenager, agitation, mutism, rigidity, and sleep disorder were the main symptoms. Samples of cerebrospinal fluid from all patients were positive for anti-NMDAR IgG, and all patients gradually improved with immunotherapy. Testing for NMDAR antibodies is highly recommend in patients with JE, especially those with a relapsing syndrome involving movement disorder and/or behavioral problems, as these patients may benefit from immunotherapy.

The Spatio-temporal Distribution of Japanese Encephalitis Cases in Different Age Groups in Mainland China, 2004 - 2014.

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Xiaolong Li

2016-04-01

Full Text Available Japanese encephalitis (JE is very prevalent in China, but the incidence of JE among children has been greatly reduced by extensive promotion of vaccinations. The incidence of JE among adults, however, has increased in some parts of China.Data on JE in mainland China, in terms of incidence, gender, and age, were collected between 2004 and 2014. We conducted spatial and temporal analyses on data from different age groups. Generally, children aged 0-15 years still represent the major population of JE cases in China, despite the gradual decrease in incidence over years. However, the incidence of JE among adults in several provinces is notably higher than the national average, especially during the epidemic waves in 2006, 2009, and 2013. The JE cases in the 0-15-year-old group are distributed mainly in the area south of the Yangtze River, with peak incidence occurring from July to September. In the adult group, especially for those over 40 years old, the JE cases are concentrated mainly in the area north of the Yangtze River. JE incidence in the adult group in September and October is significantly greater compared to the other groups. Further analysis using Local Indicators of Spatial Association (LISA reveals that the distribution of adult JE cases in the six provinces north of the Yangtze River, between north 30-35° latitude and east 110-130° longitude, is a hotspot for adult JE cases.The rate of JE case increase for adults is much greater than for children and has become a public health issue. Therefore, studies on the necessity and feasibility of vaccinating adults who live in JE-endemic areas, but have never been vaccinated for JE, should become a new focus of JE prevention in the future.

Anti-N-methyl-D-aspartate receptor encephalitis associated with hepatic neuroendocrine carcinoma: A case report.

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Lim, Ee Wei; Yip, Chun Wai

2017-07-01

Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis can present with and without tumor. Tumor associations are less common in older patients. We report a 65-year-old gentleman who presented with one week history of cough, chills, rigor and altered behavior, followed by florid visual and auditory hallucinations. Mini mental status examination score was 16/30. Both cerebrospinal fluid and plasma anti-NMDA receptor antibodies were detected. A course of intravenous methylprednisolone was given with partial symptom improvement. A hepatic neuroendocrine carcinoma was detected and confirmed on biopsy. Unfortunately, he developed several medical complications: non-ST elevation myocardial infarction, infected foot gangrene and peripheral vascular disease, which made him unsuitable for both surgery and chemotherapy. He passed away 6months later due to the progression of the malignancy. This case illustrated that NMDAR encephalitis may be associated with an uncommon hepatic neuroendocrine carcinoma in an older person, which is responsive to early treatment. Copyright © 2017 Elsevier Ltd. All rights reserved.

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

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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. Copyright © 2016 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

MRI findings in a remitting-relapsing case of Bickerstaff encephalitis

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Mondejar, R.R.; Santos, J.M.G.; Villalba, E.F.

2002-01-01

A case of remitting-relapsing Bickerstaff encephalitis is reported. The article focuses on its imaging findings and their significance when a clinical differentiation between Bickerstaff encephalitis and Miller-Fisher syndrome is attempted. Signs and symptoms may occasionally overlap. However, because Miller-Fisher syndrome is related to the peripheral nervous system and Bickerstaff encephalitis is a central disease, the recognition of brain stem hypointense lesions on T1-weighted images, which are hyperintense on T2-weighted sequences, could be a reliable tool when the clinical diagnosis is unclear. (orig.)

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.

Rare case of acute dengue encephalitis with correlated MRI findings

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Mathew, Rishi Philip; Basti, Ram Shenoy; Hegde, Pavan; Devdas, Jaidev M.; Khan, Habeeb Ullah; Bukelo, Mario Joseph

2014-01-01

Dengue encephalitis is extremely rare, with most patients showing no significant abnormality on neuroimaging (CT/MRI). We report one of the very few documented cases of dengue encephalitis, with abnormal signal intensities on all major sequences on brain MRI.

Japanese encephalitis: a review of the Indian perspective

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Sarika Tiwari

Full Text Available Japanese encephalitis virus (JEV causes Japanese encephalitis, which is a leading form of viral encephalitis in Asia, with around 50,000 cases and 10,000 deaths per year in children below 15 years of age. The JEV has shown a tendency to extend to other geographic regions. Case fatality averages 30% and a high percentage of the survivors are left with permanent neuropsychiatric sequelae. Currently, there is no cure for JEV, and treatment is mainly supportive. Patients are not infectious, but should avoid further mosquito bites. A number of antiviral agents have been investigated; however, none of these have convincingly been shown to improve the outcome of JEV. In this review, the current knowledge of the epidemiology and the pathogenesis of this deadly disease have been summarized.

Japanese encephalitis: a review of the Indian perspective

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Sarika Tiwari

2012-12-01

Full Text Available Japanese encephalitis virus (JEV causes Japanese encephalitis, which is a leading form of viral encephalitis in Asia, with around 50,000 cases and 10,000 deaths per year in children below 15 years of age. The JEV has shown a tendency to extend to other geographic regions. Case fatality averages 30% and a high percentage of the survivors are left with permanent neuropsychiatric sequelae. Currently, there is no cure for JEV, and treatment is mainly supportive. Patients are not infectious, but should avoid further mosquito bites. A number of antiviral agents have been investigated; however, none of these have convincingly been shown to improve the outcome of JEV. In this review, the current knowledge of the epidemiology and the pathogenesis of this deadly disease have been summarized.

Unusual Clinical Presentation and Role of Decompressive Craniectomy in Herpes Simplex Encephalitis.

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Singhi, Pratibha; Saini, Arushi Gahlot; Sahu, Jitendra Kumar; Kumar, Nuthan; Vyas, Sameer; Vasishta, Rakesh Kumar; Aggarwal, Ashish

2015-08-01

Decompressive craniectomy in pediatric central nervous infections with refractory intracranial hypertension is less commonly practiced. We describe improved outcome of decompressive craniectomy in a 7-year-old boy with severe herpes simplex encephalitis and medically refractory intracranial hypertension, along with a brief review of the literature. Timely recognition of refractory intracranial hypertension and surgical decompression in children with herpes simplex encephalitis can be life-saving. Additionally, strokelike atypical presentations are being increasingly recognized in children with herpes simplex encephalitis and should not take one away from the underlying herpes simplex encephalitis. © The Author(s) 2014.

Anti-N-methyl-D-aspartate receptor encephalitis after Herpes simplex virus-associated encephalitis: an emerging disease with diagnosis and therapeutic challenges.

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Schein, Flora; Gagneux-Brunon, Amandine; Antoine, Jean-Christophe; Lavernhe, Sylvie; Pillet, Sylvie; Paul, Stéphane; Frésard, Anne; Boutet, Claire; Grange, Rémi; Cazorla, Céline; Lucht, Frédéric; Botelho-Nevers, Elisabeth

2017-08-01

Morbidity and mortality of Herpes simplex virus encephalitis (HSE) remain high. Relapses of neurological signs may occur after initial clinical improvement under acyclovir treatment. We report here a case of post-HSE anti-N-methyl-d-aspartate receptor-mediated encephalitis in an adult and perform a systematic search on PubMed to identify other cases in adults. We identified 11 previously published cases, to discuss diagnostic and therapeutic management. Symptoms in adults are often inappropriate behaviors, confusion and agitation. Diagnosis of anti-NMDA-R encephalitis after HSE is often delayed. Treatment consists in steroids, plasma exchange, and rituximab. Prognosis is often favorable. Anti-NMDA-R antibodies should be searched in cerebrospinal fluid of patients with unexpected evolution of HSE. This emerging entity reopens the hot debate about steroids in HSE.

A meta-analysis on progressive atrophy in intractable temporal lobe epilepsy

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Caciagli, Lorenzo; Bernasconi, Andrea; Wiebe, Samuel; Koepp, Matthias J.; Bernasconi, Neda

2017-01-01

Objective: It remains unclear whether drug-resistant temporal lobe epilepsy (TLE) is associated with cumulative brain damage, with no expert consensus and no quantitative syntheses of the available evidence. Methods: We conducted a systematic review and meta-analysis of MRI studies on progressive atrophy, searching PubMed and Ovid MEDLINE databases for cross-sectional and longitudinal quantitative MRI studies on drug-resistant TLE. Results: We screened 2,976 records and assessed eligibility of 248 full-text articles. Forty-two articles met the inclusion criteria for quantitative evaluation. We observed a predominance of cross-sectional studies, use of different clinical indices of progression, and high heterogeneity in age-control procedures. Meta-analysis of 18/1 cross-sectional/longitudinal studies on hippocampal atrophy (n = 979 patients) yielded a pooled effect size of r = −0.42 for ipsilateral atrophy related to epilepsy duration (95% confidence interval [CI] −0.51 to −0.32; p 80% of articles reported duration-related progression in extratemporal cortical and subcortical regions. Detailed analysis of study design features yielded low to moderate levels of evidence for progressive atrophy across studies, mainly due to dominance of cross-sectional over longitudinal investigations, use of diverse measures of seizure estimates, and absence of consistent age control procedures. Conclusions: While the neuroimaging literature is overall suggestive of progressive atrophy in drug-resistant TLE, published studies have employed rather weak designs to directly demonstrate it. Longitudinal multicohort studies are needed to unequivocally differentiate aging from disease progression. PMID:28687722

Limbic encephalitis: Clinical spectrum and long-term outcome from a developing country perspective

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Sujit Abajirao Jagtap

2014-01-01

Full Text Available Introduction: Limbic encephalitis (LE is characterized by rapidly progressive short-term memory loss, psychiatric symptoms and seizures. We describe the clinical spectrum, underlying etiology and long-term follow-up of patients with LE from India. Materials and Methods: This prospective study included patients during the period of January 2009 and December 2011 with the clinical features consistent with LE with one or more of the following: (1 Magnetic resonance imaging (MRI evidence of temporal lobe involvement; (2 cerebrospinal fluid inflammatory abnormalities, or (3 detection of antineuronal antibodies. Patients with metastasis, infection, metabolic and nutritional deficits, stroke, were excluded. Results: There were 16 patients (9 females, mean age of presentation was 36.6 years (range 15-69 years. The mean duration of symptoms before presentation was 11 months (range 5 days-2 years. The most common symptom at presentation was short-term memory impairment in 7 patients followed by seizures in 5 and behavioral changes in three. Nine patients had seizures, 11 had change in behavior, language involvement in eight, cerebellar features in 3 and autonomic dysfunction in two. Four patients had associated malignancy, 3 of four presented with neurological symptoms and on investigations found to be have malignancy. Antineuronal antibody testing was done in 6 of 12 non paraneoplastic and two paraneoplastic patients, one positive for N-methyl-D-aspartate and one for anti-Hu antibody. MRI brain showed typical fluid attenuated inversion recovery or T2 bilateral temporal lobe hyperintensities in 50% of patients. At a mean follow-up of 21 months (3-36 months, 10 patients improved, 4 patients remained same and two patients expired. Conclusion: Early recognition of LE is important based upon clinical, MRI data in the absence of antineuronal surface antibody screen in developing nations. Early institution of immunotherapy will help in improvement in outcome of

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

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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.

Relationship between imaging and pathological features and clinical factors in surgical cases of temporal lobe epilepsy

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Uesugi, Hideji; Matsuda, Hiroshi; Onuma, Teiichi [National Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo (Japan); Shimizu, Hiroyuki; Arai, Nobutaka; Nakayama, Hiroshi; Maehara, Taketoshi; Yanashita, Akira

1998-03-01

The relationships between imaging, pathology and presumed causes in surgical cases of temporal lobe epilepsy (TLE) was studied. The subject was 62 patients. MRI, PET and SPECT were performed. Hematoxylin and eosin was used for pathological judgement. On MRI, mesial temporal sclerosis (MTS) was detected in 48 of 52 patients (92%); 32 (62%) had high-signal intensity on T2-weighted images; 31 (60%) had atrophy {l_brace}23 (44%) had high-signal intensity on T2+atrophy{r_brace}; 5 (10%) had calcified lesions; and 2 (4%) had cystic lesions. On PET and SPECT, abnormal cerebral blood flow was noted in 33 of 36 (92%). On pathological findings (61 cases), Ammon`s horn sclerosis (AHS), tumors, gliosis in lateral temporal and meningeal inflammatory finding were detected in 42 (69%), 10 (23%) and 8 (13%) cases, respectively, whereas 2 showed no abnormalities. The 2 patients with normal pathology showed both high-signal intensity and atrophy on MRI. The presumed causes of TLE were encephalitis/meningitis and/or suspected of these diseases in 15 patients (24%), injuries at birth in 5 (8%), and none in 42 (68%). The presumed causes in the 43 patients with AHS were encephalitis/meningitis in 11, injuries at birth in 3, and none in 29. Of the 15 patients in whom encephalitis/meningitis was estimated as the causes of TLE, only 6 (40%) had pathological evidence of meningeal inflammatory finding. Of the 42 patients in whom cause could not be determined, 2 had pathological evidence of meningeal inflammatory finding. (K.H.)

The effect of vaccination coverage and climate on Japanese encephalitis in Sarawak, Malaysia.

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Daniel E Impoinvil

Full Text Available Japanese encephalitis (JE is the leading cause of viral encephalitis across Asia with approximately 70,000 cases a year and 10,000 to 15,000 deaths. Because JE incidence varies widely over time, partly due to inter-annual climate variability effects on mosquito vector abundance, it becomes more complex to assess the effects of a vaccination programme since more or less climatically favourable years could also contribute to a change in incidence post-vaccination. Therefore, the objective of this study was to quantify vaccination effect on confirmed Japanese encephalitis (JE cases in Sarawak, Malaysia after controlling for climate variability to better understand temporal dynamics of JE virus transmission and control.Monthly data on serologically confirmed JE cases were acquired from Sibu Hospital in Sarawak from 1997 to 2006. JE vaccine coverage (non-vaccine years vs. vaccine years and meteorological predictor variables, including temperature, rainfall and the Southern Oscillation index (SOI were tested for their association with JE cases using Poisson time series analysis and controlling for seasonality and long-term trend. Over the 10-years surveillance period, 133 confirmed JE cases were identified. There was an estimated 61% reduction in JE risk after the introduction of vaccination, when no account is taken of the effects of climate. This reduction is only approximately 45% when the effects of inter-annual variability in climate are controlled for in the model. The Poisson model indicated that rainfall (lag 1-month, minimum temperature (lag 6-months and SOI (lag 6-months were positively associated with JE cases.This study provides the first improved estimate of JE reduction through vaccination by taking account of climate inter-annual variability. Our analysis confirms that vaccination has substantially reduced JE risk in Sarawak but this benefit may be overestimated if climate effects are ignored.

The effect of vaccination coverage and climate on Japanese encephalitis in Sarawak, Malaysia.

Science.gov (United States)

Impoinvil, Daniel E; Ooi, Mong How; Diggle, Peter J; Caminade, Cyril; Cardosa, Mary Jane; Morse, Andrew P; Baylis, Matthew; Solomon, Tom

2013-01-01

Japanese encephalitis (JE) is the leading cause of viral encephalitis across Asia with approximately 70,000 cases a year and 10,000 to 15,000 deaths. Because JE incidence varies widely over time, partly due to inter-annual climate variability effects on mosquito vector abundance, it becomes more complex to assess the effects of a vaccination programme since more or less climatically favourable years could also contribute to a change in incidence post-vaccination. Therefore, the objective of this study was to quantify vaccination effect on confirmed Japanese encephalitis (JE) cases in Sarawak, Malaysia after controlling for climate variability to better understand temporal dynamics of JE virus transmission and control. Monthly data on serologically confirmed JE cases were acquired from Sibu Hospital in Sarawak from 1997 to 2006. JE vaccine coverage (non-vaccine years vs. vaccine years) and meteorological predictor variables, including temperature, rainfall and the Southern Oscillation index (SOI) were tested for their association with JE cases using Poisson time series analysis and controlling for seasonality and long-term trend. Over the 10-years surveillance period, 133 confirmed JE cases were identified. There was an estimated 61% reduction in JE risk after the introduction of vaccination, when no account is taken of the effects of climate. This reduction is only approximately 45% when the effects of inter-annual variability in climate are controlled for in the model. The Poisson model indicated that rainfall (lag 1-month), minimum temperature (lag 6-months) and SOI (lag 6-months) were positively associated with JE cases. This study provides the first improved estimate of JE reduction through vaccination by taking account of climate inter-annual variability. Our analysis confirms that vaccination has substantially reduced JE risk in Sarawak but this benefit may be overestimated if climate effects are ignored.

Histopathologic identification of Trypanosoma cruzi (Chagas' encephalitis in an AIDS patient

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Dimath Alyemni

2017-03-01

Full Text Available Trypanosoma cruzi (Chagas' encephalitis is an uncommon manifestation of T. cruzi infection, typically seen in immunocompromised patients. Encephalitis results from the reactivation of chronic infection predominately in individuals from endemic areas. Increased awareness of this complication is essential especially with increased migration of patients from endemic areas with concomitant HIV infection. Here we report a case of Chagas' encephalitis in an AIDS patient from Mexico in which there was no evidence of acute serologic, CSF, or blood infection by T. cruzi trypomastigotes.

Large cross-sectional study of presbycusis reveals rapid progressive decline in auditory temporal acuity.

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Ozmeral, Erol J; Eddins, Ann C; Frisina, D Robert; Eddins, David A

2016-07-01

The auditory system relies on extraordinarily precise timing cues for the accurate perception of speech, music, and object identification. Epidemiological research has documented the age-related progressive decline in hearing sensitivity that is known to be a major health concern for the elderly. Although smaller investigations indicate that auditory temporal processing also declines with age, such measures have not been included in larger studies. Temporal gap detection thresholds (TGDTs; an index of auditory temporal resolution) measured in 1071 listeners (aged 18-98 years) were shown to decline at a minimum rate of 1.05 ms (15%) per decade. Age was a significant predictor of TGDT when controlling for audibility (partial correlation) and when restricting analyses to persons with normal-hearing sensitivity (n = 434). The TGDTs were significantly better for males (3.5 ms; 51%) than females when averaged across the life span. These results highlight the need for indices of temporal processing in diagnostics, as treatment targets, and as factors in models of aging. Copyright © 2016 Elsevier Inc. All rights reserved.

Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review.

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Ravindra, Vijay M; Mazur, Marcus D; Mohila, Carrie A; Sweney, Matthew T; Hersh, Aimee; Bollo, Robert J

2015-11-01

Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of Rasmussen encephalitis. We report the case of a 12-year-old girl with a history of cognitive decline and right arm weakness. Magnetic resonance imaging demonstrated diffuse left hemispheric cortical and subcortical atrophy suggestive of Rasmussen encephalitis. The patient had no clinical history of seizures, and electroencephalography did not demonstrate epileptiform abnormalities. Craniotomy for open brain biopsy was performed, and histopathologic evaluation identified Rasmussen encephalitis with cortical dysplasia (dual pathology). To the best of our knowledge, this is the third case of Rasmussen encephalitis diagnosed by both imaging and histopathology that had no clinical or electroencephalographic evidence of seizures and is the only case of Rasmussen encephalitis with cortical dysplasia without epilepsy.

Clinical and cerebrospinal fluid findings contribute to the early differentiation between infectious and noninfectious encephalitis

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Miguel Wilken

2017-06-01

Full Text Available Early recognition and prompt specific treatment are crucial factors influencing the outcome of patients with acute encephalitis. The aim of this study was to determine the main causes of acute encephalitis in our population and to find predictors that may lead to specific diagnosis. Adult patients admitted to our hospital with suspected diagnosis of encephalitis in the period 2006-2013 were included. One hundred and five medical records were analyzed. Eighty-two patients with infectious encephalitis were identified (78% of total cases, 53 (65% men and 29 (35% women, mean age 47.8 years. The most common microorganisms identified were: HSV-1 (11%, VZV (10%, HSV-2 (5% and EBV (5%. Twenty-three patients (22% of the series had non-infectious encephalitis. Headache (p < 0.0001 and fever (p = 0.008 were more frequent in encephalitis of infectious origin. Protein levels and white blood cell counts in the cerebrospinal fluid were significantly higher in patients affected by infectious encephalitis than in those affected by noninfectious encephalitis (OR 95% CI 12.3 [2.9-51.7] and OR 95% CI 7.4 [2-27], respectively. Identifying specific causal agents of acute encephalitis remains a major challenge. Cerebrospinal fluid markers, as well as specific clinical findings, may however contribute to initial differentiation between infectious and noninfectious causes.

Computed tomographic brain scan findings in Q fever encephalitis

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Gomez Aranda, F.; Romero Acebal, M.; Maestre Moreno, J.; Pachon Diaz, J.; Lopez Cortes, L.; Navarro Rodriguez, A.

1984-07-01

Neurological involvement in Q Fever is unusual. We present a case of encephalitis due to Coxiella Burnetii with neuroradiologic findings on CT not described previously, consisting in areas of decreased absorption coefficient in the subcortical white matter of both hemispheres, predominantly in the right. Differential diagnosis must be established from viral encephalitis, of similar clinical presentation, which may show similar CT lesions to those in this case.

Temporal Progression of Lesions in Guinea Pigs Infected With Lassa Virus.

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Bell, T M; Shaia, C I; Bearss, J J; Mattix, M E; Koistinen, K A; Honnold, S P; Zeng, X; Blancett, C D; Donnelly, G C; Shamblin, J D; Wilkinson, E R; Cashman, K A

2017-05-01

Lassa virus (LASV) infection causes an acute, multisystemic viral hemorrhagic fever that annually infects an estimated 100 000 to 300 000 persons in West Africa. This pathogenesis study evaluated the temporal progression of disease in guinea pigs following aerosol and subcutaneous inoculation of the Josiah strain of LASV as well as the usefulness of Strain 13 guinea pigs as an animal model for Lassa fever. After experimental infection, guinea pigs ( Cavia porcellus; n = 67) were serially sampled to evaluate the temporal progression of infection, gross and histologic lesions, and serum chemistry and hematologic changes. Guinea pigs developed viremia on day 5 to 6 postexposure (PE), with clinical signs appearing by day 7 to 8 PE. Complete blood counts revealed lymphopenia and thrombocytopenia. Gross pathologic findings included skin lesions and congested lungs. Histologic lesions consisted of cortical lymphoid depletion by day 6 to 7 PE with lymphohistiocytic interstitial pneumonia at 7 to 8 days PE. Scattered hepatocellular degeneration and cell death were also noted in the liver and, to a lesser extent, in other tissues including the haired skin, lung, heart, adrenal gland, lymph nodes, thymus, and spleen. The first cell types to demonstrate staining for viral antigen were fibroblastic reticular cells and macrophages/dendritic cells in the lymph nodes on day 5 to 6 PE. This study demonstrates similarities between Lassa viral disease in human infections and experimental guinea pig infection. These shared pathologic characteristics support the utility of guinea pigs as an additional animal model for vaccine and therapeutic development under the Food and Drug Administration's Animal Rule.

CD8+ T-cells mediate immunopathology in tick-borne encephalitis

Czech Academy of Sciences Publication Activity Database

Růžek, Daniel; Salát, Jiří; Palus, M.; Gritsun, T. S.; Gould, E. A.; Dyková, Iva; Skallová, Anna; Jelínek, Jiří; Kopecký, Jan; Grubhoffer, Libor

2009-01-01

Roč. 384, č. 1 (2009), s. 1-6 ISSN 0042-6822 R&D Projects: GA MŠk(CZ) LC06009; GA ČR GA524/08/1509 Institutional research plan: CEZ:AV0Z60220518 Keywords : tick-borne encephalitis * immunopathology * encephalitis Subject RIV: EE - Microbiology, Virology Impact factor: 3.042, year: 2009

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.

Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis

International Nuclear Information System (INIS)

Sarria-Estrada, S.; Toledo, M.; Lorenzo-Bosquet, C.; Cuberas-Borrós, G.; Auger, C.; Siurana, S.; Rovira, À.

2014-01-01

Aim: To describe the characteristic magnetic resonance imaging (MRI) findings of paraneoplastic autoimmune encephalitis in patients with new-onset status epilepticus. Materials and methods: The neuroimaging and clinical data of five patients with paraneoplastic autoimmune encephalitis debuting as status epilepticus were retrospectively reviewed. All patients met the criteria for definite paraneoplastic syndrome and all underwent brain MRI during the status epilepticus episode or immediately after recovery. Results: All patients showed hyperintense lesions on T2-weighted imaging (WI) involving the limbic structures, specifically the hippocampus. Three of them showed additional extra-limbic areas of signal abnormalities. The areas of T2 hyperintensity were related to the electroclinical onset of the seizures. In three patients, various techniques were used to study cerebral perfusion, such as arterial spin labelling MRI, single photon-emission computed tomography (SPECT) and 2-[ 18 F]-fluoro-2-deoxy-D-glucose (FDG)-positron-emission tomography (PET). Arterial spin labelling showed hyperperfusion overlapping the inflammatory lesions, whereas PET and SPECT disclosed increased perfusion and increased metabolism. The subtraction SPECT co-registered to MRI (SISCOM) demonstrated hypermetabolism outside the areas of encephalitis. After clinical recovery, follow-up MRI revealed the development of atrophy in the initially affected hippocampus. Two patients who had recurrent paraneoplastic autoimmune encephalitis manifesting as status epilepticus showed new T2 lesions involving different structures. Conclusion: The presence of limbic and extra-limbic T2 signal abnormalities in new-onset status epilepticus should suggest the diagnosis of a paraneoplastic syndrome, especially when status epilepticus is refractory to treatment. The lesions are consistently seen as hyperintense on T2WI. - Highlights: • New onset status epilepticus can be caused by paraneoplastic encephalitis. �

Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis

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Sarria-Estrada, S., E-mail: ssarria@idi-cat.org [Magnetic Resonance Unit, Radiology Department, Vall d' Hebrón University Hospital, Barcelona (Spain); Toledo, M. [Epilepsy Unit, Neurology Department, Vall d' Hebrón University Hospital, Barcelona (Spain); Lorenzo-Bosquet, C.; Cuberas-Borrós, G. [Nuclear Medicine Department, Vall d' Hebrón University Hospital, Barcelona (Spain); Auger, C.; Siurana, S.; Rovira, À. [Magnetic Resonance Unit, Radiology Department, Vall d' Hebrón University Hospital, Barcelona (Spain)

2014-08-15

Aim: To describe the characteristic magnetic resonance imaging (MRI) findings of paraneoplastic autoimmune encephalitis in patients with new-onset status epilepticus. Materials and methods: The neuroimaging and clinical data of five patients with paraneoplastic autoimmune encephalitis debuting as status epilepticus were retrospectively reviewed. All patients met the criteria for definite paraneoplastic syndrome and all underwent brain MRI during the status epilepticus episode or immediately after recovery. Results: All patients showed hyperintense lesions on T2-weighted imaging (WI) involving the limbic structures, specifically the hippocampus. Three of them showed additional extra-limbic areas of signal abnormalities. The areas of T2 hyperintensity were related to the electroclinical onset of the seizures. In three patients, various techniques were used to study cerebral perfusion, such as arterial spin labelling MRI, single photon-emission computed tomography (SPECT) and 2-[{sup 18}F]-fluoro-2-deoxy-D-glucose (FDG)-positron-emission tomography (PET). Arterial spin labelling showed hyperperfusion overlapping the inflammatory lesions, whereas PET and SPECT disclosed increased perfusion and increased metabolism. The subtraction SPECT co-registered to MRI (SISCOM) demonstrated hypermetabolism outside the areas of encephalitis. After clinical recovery, follow-up MRI revealed the development of atrophy in the initially affected hippocampus. Two patients who had recurrent paraneoplastic autoimmune encephalitis manifesting as status epilepticus showed new T2 lesions involving different structures. Conclusion: The presence of limbic and extra-limbic T2 signal abnormalities in new-onset status epilepticus should suggest the diagnosis of a paraneoplastic syndrome, especially when status epilepticus is refractory to treatment. The lesions are consistently seen as hyperintense on T2WI. - Highlights: • New onset status epilepticus can be caused by paraneoplastic encephalitis

Computed tomography in young children with herpes simplex virus encephalitis

International Nuclear Information System (INIS)

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

1985-01-01

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. (orig.)

Venezuelan equine encephalitis virus infection causes modulation of inflammatory and immune response genes in mouse brain

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Puri Raj K

2008-06-01

Full Text Available Abstract Background Neurovirulent Venezuelan equine encephalitis virus (VEEV causes lethal encephalitis in equines and is transmitted to humans by mosquitoes. VEEV is highly infectious when transmitted by aerosol and has been developed as a bio-warfare agent, making it an important pathogen to study from a military and civilian standpoint. Molecular mechanisms of VEE pathogenesis are poorly understood. To study these, the gene expression profile of VEEV infected mouse brains was investigated. Changes in gene expression were correlated with histological changes in the brain. In addition, a molecular framework of changes in gene expression associated with progression of the disease was studied. Results Our results demonstrate that genes related to important immune pathways such as antigen presentation, inflammation, apoptosis and response to virus (Cxcl10, CxCl11, Ccl5, Ifr7, Ifi27 Oas1b, Fcerg1,Mif, Clusterin and MHC class II were upregulated as a result of virus infection. The number of over-expressed genes (>1.5-fold level increased as the disease progressed (from 197, 296, 400, to 1086 at 24, 48, 72 and 96 hours post infection, respectively. Conclusion Identification of differentially expressed genes in brain will help in the understanding of VEEV-induced pathogenesis and selection of biomarkers for diagnosis and targeted therapy of VEEV-induced neurodegeneration.

Case Report: Magnetic resonance imaging in rabies encephalitis

International Nuclear Information System (INIS)

Rao, Arekapudi Subramanyaswara; Varma, Dandu Ravi; Chalapathi Rao, Mamidi Venkata; Mohandas, Surat

2009-01-01

Rabies encephalitis is an invariably fatal disease characterized by typical clinical symptoms. Although the diagnosis of this condition can be made on the basis of the patient's history and the classical clinical presentation, neuroimaging may still play a role, especially for establishing an early diagnosis in cases with atypical presentations or when the history of animal bite is not forthcoming. We report the MRI findings in a case of furious rabies encephalitis and describe the utility of diffusion imaging in its diagnosis

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.

Hypothermia in VGKC antibody-associated limbic encephalitis.

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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.

Creutzfeldt-Jakob disease versus anti-LGI1 limbic encephalitis in a patient with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal electroencephalogram: a case report

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Sun L

2015-06-01

Full Text Available Li Sun, Jie Cao, Chang Liu, Yudan LvDepartment of Neurology, The First Hospital of JiLin University, ChangChun, People’s Republic of ChinaAbstract: Diagnosis of Creutzfeldt-Jakob disease (CJD is often challenging in elderly individuals, not only because of its variable clinical features but also because of nonspecific changes on the electroencephalogram (EEG in the early stages of the disease. Here we report on a patient who presented with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal EEG. We provide a detailed analysis and differential diagnosis between anti-leucine-rich glioma inactivated 1 (LGI1 limbic encephalitis versus CJD, in the hope of providing a new understanding of CJD. A 65-year-old Chinese man presented with slowly progressive cognitive decline with psychiatric symptoms. On admission, he presented with facial grimacing and brief left upper limb dystonic posturing lasting 1–2 seconds, with hyponatremia that was difficult to rectify. Neurological examination showed increased muscle tension in the left limb but without pathological reflexes. His early EEG showed focal periodic wave complexes. Diffusion-weighted magnetic resonance imaging showed a suspected “lace sign” in the occipital cortex. His cerebrospinal fluid was negative for LGI1 antibodies and positive for 14-3-3 brain protein. Therefore, we made a presumptive diagnosis of CJD. At the following visit, a second EEG showed paroxysmal sharp wave complexes, but the patient had a poor prognosis. Atypical facio-brachio-crural movement and nonspecific EEG changes may occasionally be found in patients with CJD or anti-LGI1 encephalitis. Clinicians should not be dissuaded from a diagnosis of CJD where the EEG does not show paroxysmal sharp wave complexes in the early stages but abnormal facio-brachio-crural movement is present.Keywords: abnormal facio-brachio-crural movement, hyponatremia, Creutzfeldt

Human Parechovirus and Neonatal Encephalitis

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J Gordon Millichap

2008-10-01

Full Text Available Clinical presentation, cranial ultrasound (cUS and MRi findings, and neurodevelopmental outcome of 10 neonates (70% term with human parechovirus (HPeV encephalitis are described by researchers at University Medical Center, Utrecht, The Netherlands; University of Toronto, Ontario, Canada; and Universitaire de Quebec, Canada.

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

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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

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

Characterization of tick-borne encephalitis virus from Latvia.

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Mavtchoutko, V; Vene, S; Haglund, M; Forsgren, M; Duks, A; Kalnina, V; Hörling, J; Lundkvist, A

2000-02-01

Viruses of the tick-borne encephalitis (TBE) antigenic complex, within the family Flaviviridae, cause a variety of diseases including uncomplicated febrile illness, encephalitis, meningo-encephalitis, hemorrhagic fever and chronic disease in humans, domesticated animals or wildlife species. TBE is a serious problem in Latvia with up to a 1,000 patients confirmed serologically annually 1994-1995. No previous data had been reported on the causative agent of TBE in Latvia. In the present study, a virus was isolated from serum of a patient with clinical symptoms of an acute TBE infection. Nucleotide sequence information obtained by direct reverse transcription-polymerase chain reaction (RT-PCR) and the serological characteristics of the isolated virus strain, designated TBE-Latvia-1-96, indicated a closer relationship to the Vasilchenko strain, isolated in Novosibirsk (Siberia, Russia), as compared to the western European or far eastern subtypes of TBE viruses. In a mouse neurovirulence assay, a significant difference in survival rates (days) was shown between Latvia-1-96 and the western European TBE virus subtype. Copyright 2000 Wiley-Liss, Inc.

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

Science.gov (United States)

Guo, Yao-Hong; Kuan, Ta-Shen; Hsieh, Pei-Chun; Lien, Wei-Chih; Chang, Chun-Kai; Lin, Yu-Ching

2014-01-01

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. PMID:25473290

Clinical profile and outcome of acute encephalitis syndrome (AES patients treated in College of Medical Sciences-Teaching Hospital

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Lekhjung J Thapa

2014-01-01

Full Text Available Objective: Acute encephalitis syndrome is a cause of significant morbidity and mortality in Nepal. Although Japanese encephalitis virus (JEV was thought to be a major cause for acute encephalitis syndrome, more non-Japanese encephalitis virus cases are reported. The outcome of patients with acute encephalitis syndrome is variable. Our study was designed to study the clinical profile and outcome of patients with acute encephalitis syndrome managed in tertiary care center in central Nepal. Methods: The record of patients admitted with diagnosis of acute encephalitis syndrome,from January 2010 to December 2010 in College of Medical Sciences-Teaching Hospital (CMS-TH was reviewed. They were classified clinically as meningitis, encephalitis and meningoencephalitis. The clinical details and reports of the patients were recorded and analyzed. Results: Total of 85 cases of meningitis and encephalitis were identified. Mean age was 19.18 years. Fifty-six (65.9% patients were males and 29 (34.1% were females. Sixty (70.58% patients had meningitis, 8 (9.41% had encephalitis, and 17 (20.0% had meningoencephalitis. JE serology was positive in 4 patients (4.7%. Seventy-two (84.7% patients made full recovery and were discharged from hospital. Thirteen (15.3% patients left against medical advice (LAMA. Conclusion: Acute encephalitis syndrome is still a major public health problem in Nepal. Few of these patients have Japanese Encephalitis. There is a trend towards improved outcome because of availability of improved health services. However, financial constraint remains a challenge in management of acute encephalitis syndrome. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-2, 31-37 DOI: http://dx.doi.org/10.3126/jcmsn.v9i2.9685

Postpartum Anti-N-methyl-D-aspartate Receptor Encephalitis: A Case Report and Literature Review.

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Doden, Tadashi; Sekijima, Yoshiki; Ikeda, Junji; Ozawa, Kazuki; Ohashi, Nobuhiko; Kodaira, Minori; Hineno, Akiyo; Tachibana, Naoko; Ikeda, Shu-Ichi

2017-01-01

We describe a 24-year-old woman with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis that developed 3 weeks after normal delivery. She was treated with methylprednisolone, intravenous immunoglobulin, and plasmapheresis, in addition to teratoma excision. However, her recovery was slow, and dysmnesia and mental juvenility persisted even two years after onset. To date, five patients with postpartum anti-NMDAR encephalitis have been reported. All of those patients showed psychotic symptoms and were suspected of having postpartum psychosis in the early period of the encephalitis. Changes in hormonal factors, modification of immune tolerance, or retrograde infection of the ovary may be contributing factors for postpartum anti-NMDAR encephalitis.

[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.

Repeated CT studies of a patient with herpes simplex encephalitis during his entire clinical course

International Nuclear Information System (INIS)

Shiraishi, Masahiro; Fukui, Keiji; Takeda, Sadanori; Sadamoto, Kazuhiko; Kimura, Hideki; Sakaki, Saburo.

1985-01-01

We encountered a patient with herpes simplex encephalitis whose cerebral lesions were studied by repeated CT scannings during his entire clinical course. The purpose of this paper is to report the earliest lesions of the brain as revealed by CT scans. A 63-year-old man was admitted to our clinic complaining of headache, nausea, fever, and disorientation. On admission, a physical examination showed a high fever, while a neurological examination revealed a stiff neck, a positive Kernig's sign, and disorientation. Laboratory examinations revealed a pleocytosis of the cerebrospinal fluid. Electroencephalograms showed the so-called ''periodic sharp-and-slow-waves complex''. The complement fixation titer for herpes simplex virus was x32 in the serum and x128 in the cerebrospinal fluid, suggesting the diagnosis of herpes simplex encephalitis. We treated him with adenine arabinoside and gamma-globulin, but the patient did not recover; rather, he died of pneumonia and gastrointestinal bleeding three months later. Plain CT scans taken on the 12th day after the onset revealed a low-density area with signs of a slight mass in the region from the right island of Reil to the right uncus. Contrast-enhanced CT scans revealed an irregular enhancement in the low-density area. CT scans taken on the 19th day after the onset showed an extensive low-density area with a streak-like enhancement in the right temporal lobe, which is in aggreement with the findings reported by others as characteristic CT findings for herpes simplex encephalitis. In order to make an early diagnosis of a patient, we should pay attention to a low-density area with an irregular contrast enhancement in the region from the island of Reil to the uncus on a CT scan. (author)

Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis.

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Favier, Marion; Joubert, Bastien; Picard, Géraldine; Rogemond, Véronique; Thomas, Laure; Rheims, Sylvain; Bailhache, Marion; Villega, Frédéric; Pédespan, Jean-Michel; Berzero, Giulia; Psimaras, Dimitri; Antoine, Jean-Christophe; Desestret, Virginie; Honnorat, Jérôme

2018-05-01

Autoimmune encephalitis with anti-N-methyl-d-aspartate receptor autoantibodies (NMDA-R-Abs) is a recently described disease affecting adult and pediatric patients. Symptoms of the disease are now perfectly described in the adult population but the clinical presentation is less known in young children. The aim of the present study was to describe the clinical presentation and the specificities of symptoms presented by young children with NMDA-R-Abs encephalitis to improve diagnosis of this disease, and to compare these to a series of previously published female adult patients. Fifty cases of children younger than twelve years of age diagnosed with NMDA-R-Abs encephalitis between January 1, 2007 and December 31, 2016 (27 females and 23 males) were retrospectively studied. The first neurological symptoms observed in young children with NMDA-R-Abs encephalitis were characterized by seizure (72%), especially focal seizure (42%), within a median of 15 days before other encephalitis symptoms; other patients mostly had behavioral disorders (26%). The seizures were frequently difficult to diagnose because of the transient unilateral dystonic or tonic posturing presentation or sudden unilateral pain in the absence of clonic movements. A post-ictal motor deficit was also frequently observed. This clinical presentation is different from that observed in adult females with NMDA-R-Abs encephalitis who initially present mainly psychiatric disorders (67%) or cognitive impairment (19%), and less frequently seizures (14%). The diagnosis of NMDA-R-Abs encephalitis should be systematically considered in young children of both sexes who present neurological symptoms suggesting recent seizures (focal or generalized) without obvious other etiology. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

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

International Nuclear Information System (INIS)

Park, Arim; Suh, Sang-il; Seol, Hae-Young; Son, Gyu-Ri; Lee, Nam-Joon; Lee, Young Hen; Seo, Hyung Suk; Eun, Baik-Lin

2014-01-01

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.)

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.)

CSF findings in patients with anti-N-methyl-D-aspartate receptor-encephalitis.

Science.gov (United States)

Wang, Rui; Guan, Hong-Zhi; Ren, Hai-Tao; Wang, Wei; Hong, Zhen; Zhou, Dong

2015-07-01

Anti-NMDAR-encephalitis is a recently described form of autoimmune encephalitis. Here, we characterize CSF changes in Chinese patients with anti-NMDAR encephalitis, and explore the relationship between CSF findings and disease outcome. The presence of NMDAR antibodies in serum or CSF samples was evaluated in patients diagnosed with encephalitis between October 1, 2010 and August 1, 2014 at the West China Hospital. All patients fulfilling our diagnostic criteria were included and CSF findings were analyzed. Patient outcome was assessed after 4, 8, 12, 16, 20, and 24 months using the modified Rankin scale (mRS). Out of 3000 people with encephalitis screened, 43 patients were anti-NMDAR antibody positive in CSF or serum and included in this study. 62.8% of the patients identified with positive CSFs had positive serum anti-NMDAR samples, while 100% patients with positive serum had positive CSF samples. In the CSF white cell counts were elevated in 58.1% of cases; protein was increased in 18.6%; QAlb>Qlim(Alb) of the blood-CSF barrier was found in 29.3%; intrathecal immunoglobulin synthesis was detected in 17.1%, and 39.5% patients exhibited increased CSF pressures. A longer follow-up period was associated with better outcomes. There was no relationship between changes in CSF findings and outcome. The sensitivity of NMDA receptor antibody testing is higher in CSF compared to serum. Other CSF abnormalities are present in some patients with Anti-NMDAR-encephalitis, however these changes do not appear to affect prognosis. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Diagnosis and genetic analysis of Japanese encephalitis virus infected in horses.

Science.gov (United States)

Lian, W C; Liau, M Y; Mao, C L

2002-10-01

Nervous disorders were found in two horses and verified as aseptic encephalitis by necropsy in the summer of 2000. To investigate agents that affected the horses, diagnostic procedures involving virus isolation, neutralization test and reverse transcription-polymerase chain reaction (RT-PCR) were performed. We intracranially inoculated litters of suckling mice with tissues suspected of containing aseptic encephalitis, including cerebrum, cerebellum, brain stem, thalamus, and cerebrospinal fluids; the mice were then observed for 14 days. Neutralizing antibodies against Japanese encephalitis (JE) viruses were present in the cerebrospinal fluid of the horses in titers of 10. Sequences of 500 nucleotides of the premembrane gene of JE virus, synthesized by RT-PCR, from both the cerebrum and cerebellum were determined. The phylogenetic analysis based on sequences of the premembrane gene revealed a relationship with the JE virus. The divergences at the nucleotide level of 1.2-5.7% and at the amino acid level of 0-4.3% were conserved with other JE strains. The results demonstrated that the pathogens causing equine encephalitis were JE viruses. The strains were closely related to Taiwanese isolates.

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.

First documented case of imported tick-borne encephalitis in Australia

Czech Academy of Sciences Publication Activity Database

Chaunhuri, A.; Růžek, Daniel

2013-01-01

Roč. 43, č. 1 (2013), s. 93-96 ISSN 0918-2918 R&D Projects: GA ČR GPP302/10/P438; GA ČR GAP502/11/2116 Institutional support: RVO:60077344 Keywords : tick-borne encephalitis virus * viral encephalitis * travel medicine * flavivirus Subject RIV: FN - Epidemiology, Contagious Diseases ; Clinical Immunology Impact factor: 0.967, year: 2013 http://onlinelibrary.wiley.com/doi/10.1111/imj.12017/pdf

{sup 11}C-Methionine positron emission tomography may monitor the activity of encephalitis

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Hirata, Kenji; Shiga, Tohru; Manabe, Osamu; Tamaki, Nagara [Department of Nuclear Medicine, Graduate School of Medicine, Hokkaido University, Sapporo (Japan)], E-mail: khirata@med.hokudai.ac.jp; Fujima, Noriyuki [Department of Radiology, Graduate School of Medicine, Hokkaido University, Sapporo (Japan); Usui, Reiko [Department of Nuclear Medicine, Graduate School of Medicine, Hokkaido University, Sapporo (Japan); Department of Psychiatry, Graduate School of Medicine, Hokkaido University, Sapporo (Japan); Kuge, Yuji [Central Institute of Isotope Science, Hokkaido University, Sapporo (Japan)

2012-12-15

Encephalitis is generally diagnosed by clinical symptoms, cerebrospinal fluid examination, and imaging studies including CT, magnetic resonance imaging (MRI), and perfusion single photon emission tomography (SPECT). However, the role of positron emission tomography (PET) in diagnosis of encephalitis remains unclear. A 49-year-old woman presenting with coma and elevated inflammatory reaction was diagnosed as having encephalitis according to slow activity on electroencephalogram, broad cortical lesion in MR fluid attenuated inversion recovery image, and increased blood flow demonstrated by SPECT. PET revealed increased accumulation of {sup 11}C-methionine (MET) in the affected brain tissues. After the symptom had improved 2 months later, the accumulation of MET as well as the abnormal findings of MR imaging and SPECT was normalized. This case indicated that MET PET may monitor the activity of encephalitis.

New Onset Insomnia in a Pediatric Patient: A Case of Anti-NMDA Receptor Encephalitis

OpenAIRE

Tamar N. Goldberg; Michael F. Cellucci

2017-01-01

Anti-NMDAR encephalitis is becoming more widely recognized as a cause of encephalopathy in both adults and children. Certain clinical features such as mood lability, movement disorders, speech dysfunction, seizures, and autonomic instability in a pediatric patient should prompt immediate concern and evaluation for autoimmune encephalitis among providers. We present the case of a pediatric patient with anti-NMDAR encephalitis in which the symptom prompting medical evaluation was insomnia. Inso...

Infection and injury of human astrocytes by tick-borne encephalitis virus

Czech Academy of Sciences Publication Activity Database

Palus, Martin; Bílý, Tomáš; Elsterová, Jana; Langhansová, Helena; Salát, J.; Vancová, Marie; Růžek, Daniel

2014-01-01

Roč. 95, Pt 11 (2014), s. 2411-2426 ISSN 0022-1317 R&D Projects: GA ČR GAP502/11/2116; GA ČR GAP302/12/2490; GA TA ČR TE01020118 Institutional support: RVO:60077344 Keywords : Tick-borne encephalitis * Tick-borne encephalitis virus * human Subject RIV: EE - Microbiology, Virology Impact factor: 3.183, year: 2014

Herpes Simplex Encephalitis during Treatment with Tumor Necrosis Factor-α Inhibitors

OpenAIRE

Bradford, Russell D.; Pettit, April C.; Wright, Patty W.; Mulligan, Mark J.; Moreland, Larry W.; McLain, David A.; Gnann, John W.; Bloch, Karen C.

2009-01-01

We report 3 cases of herpes simplex virus encephalitis in patients receiving tumor necrosis factor-alpha (TNF-α) inhibitors for rheumatologic disorders. Although TNF-α inhibitors have been reported to increase the risk of other infectious diseases, to our knowledge, an association between anti–TNF-α drugs and herpes simplex virus encephalitis has not been previously described.

Severe hypokinesis caused by paraneoplastic anti-Ma2 encephalitis associated with bilateral intratubular germ-cell neoplasm of the testes.

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Matsumoto, Lumine; Yamamoto, Tomotaka; Higashihara, Mana; Sugimoto, Izumi; Kowa, Hisatomo; Shibahara, Junji; Nakamura, Koichiro; Shimizu, Jun; Ugawa, Yoshikazu; Goto, Jun; Dalmau, Josep; Tsuji, Shoji

2007-04-15

We report a 40-year-old man with severe hypokinesis as paraneoplastic manifestation of a microscopic "carcinoma in situ" of the testis. The young age of the patient, along with progressive neurologic deterioration, detection of anti-Ma2 antibodies, and ultrasound findings of bilateral microcalcifications, led to bilateral orchiectomy, revealing the tumor in both testes. After orchiectomy, neurological symptoms stabilized, but the patient eventually died of systemic complications caused by his severe neurological deficits. Anti-Ma2 paraneoplastic encephalitis should be considered in patients with severe hypokinesis, and intensive investigation and aggressive approach to treatment is encouraged to prevent progression of the neurological deficits.

Limbic encephalitis and antibodies to Ma2: a paraneoplastic presentation of breast cancer.

Science.gov (United States)

Sutton, I; Winer, J; Rowlands, D; Dalmau, J

2000-08-01

A patient with atypical medullary breast cancer is described who presented with symptoms of limbic encephalitis. The patient's serum and CSF contained antibodies that reacted with the nervous system and the tumour. These antibodies recognised Ma2, a neuronal protein related to paraneoplastic limbic and brainstem encephalitis in men with testicular tumours. This report highlights the importance of testing for paraneoplastic antineuronal antibodies in cases of unexplained limbic encephalitis and suggests screening for breast cancer in women with antibodies predominantly directed to Ma2.

Serial EEG findings in anti-NMDA receptor encephalitis: correlation between clinical course and EEG.

Science.gov (United States)

Ueda, Jun; Kawamoto, Michi; Hikiami, Ryota; Ishii, Junko; Yoshimura, Hajime; Matsumoto, Riki; Kohara, Nobuo

2017-12-01

Anti-NMDA receptor encephalitis is a paraneoplastic encephalitis characterised by psychiatric features, involuntary movement, and autonomic instability. Various EEG findings in patients with anti-NMDA receptor encephalitis have been reported, however, the correlation between the EEG findings and clinical course of anti-NMDA receptor encephalitis remains unclear. We describe a patient with anti-NMDA receptor encephalitis with a focus on EEG findings, which included: status epilepticus, generalised rhythmic delta activity, excess beta activity, extreme delta brush, and paroxysmal alpha activity upon arousal from sleep, which we term"arousal alpha pattern". Initially, status epilepticus was observed on the EEG when the patient was comatose with conjugate deviation. The EEG then indicated excess beta activity, followed by the emergence of continuous slow activity, including generalised rhythmic delta activity and extreme delta brush, in the most severe phase. Slow activity gradually faded in parallel with clinical amelioration. Excess beta activity persisted, even after the patient became almost independent in daily activities, and finally disappeared with full recovery. In summary, our patient with anti-NMDA receptor encephalitis demonstrated slow activity on the EEG, including extreme delta brush during the most severe phase, which gradually faded in parallel with clinical amelioration, with excess beta activity persisting into the recovery phase.

Rasmussen's encephalitis | Dawodu | Nigerian Journal of Clinical ...

African Journals Online (AJOL)

complicated meningo.encephalitis and the third episode was associated with receptive aphasia, hemiparesis and intellectual impairment. Neuroimaging studies revealed cerebellar atrophy and infarction of territory of the middle cerebral artery.

Clinical analysis of anti-Ma2-associated encephalitis.

Science.gov (United States)

Dalmau, Josep; Graus, Francesc; Villarejo, Alberto; Posner, Jerome B; Blumenthal, Deborah; Thiessen, Brian; Saiz, Albert; Meneses, Patricio; Rosenfeld, Myrna R

2004-08-01

Increasing experience indicates that anti-Ma2-associated encephalitis differs from classical paraneoplastic limbic or brainstem encephalitis, and therefore may be unrecognized. To facilitate its diagnosis we report a comprehensive clinical analysis of 38 patients with anti-Ma2 encephalitis. Thirty-four (89%) patients presented with isolated or combined limbic, diencephalic or brainstem dysfunction, and four with other syndromes. Considering the clinical and MRI follow-up, 95% of the patients developed limbic, diencephalic or brainstem encephalopathy. Only 26% had classical limbic encephalitis. Excessive daytime sleepiness affected 32% of the patients, sometimes with narcolepsy-cataplexy and low CSF hypocretin. Additional hormonal or MRI abnormalities indicated diencephalic-hypothalamic involvement in 34% of the patients. Eye movement abnormalities were prominent in 92% of the patients with brainstem dysfunction, but those with additional limbic or diencephalic deficits were most affected; 60% of these patients had vertical gaze paresis that sometimes evolved to total external ophthalmoplegia. Three patients developed atypical parkinsonism, and two a severe hypokinetic syndrome with a tendency to eye closure and dramatic reduction of verbal output. Neurological symptoms preceded the tumour diagnosis in 62% of the patients. Brain MRI abnormalities were present in 74% of all patients and 89% of those with limbic or diencephalic dysfunction. Among the 34 patients with cancer, 53% had testicular germ-cell tumours. Two patients without evidence of cancer had testicular microcalcification and one cryptorchidism, risk factors for testicular germ-cell tumours. After neurological syndrome development, 17 of 33 patients received oncological treatment (nine also immunotherapy), 10 immunotherapy alone, and six no treatment. Overall, 33% of the patients had neurological improvement, three with complete recovery; 21% had long-term stabilization, and 46% deteriorated. Features

High sensitivity and specificity in proposed clinical diagnostic criteria for anti-N-methyl-D-aspartate receptor encephalitis.

Science.gov (United States)

Ho, Alvin C C; Mohammad, Shekeeb S; Pillai, Sekhar C; Tantsis, Esther; Jones, Hannah; Ho, Reena; Lim, Ming; Hacohen, Yael; Vincent, Angela; Dale, Russell C

2017-12-01

To determine the validity of the proposed clinical diagnostic criteria for anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in paediatric patients. The diagnostic criteria for anti-NMDAR encephalitis proposed by Graus et al. (2016) use clinical features and conventional investigations to facilitate early immunotherapy before antibody status is available. The criteria are satisfied if patients develop four out of six symptom groups within 3 months, together with at least one abnormal investigation (electroencephalography/cerebrospinal fluid) and reasonable exclusion of other disorders. We evaluated the validity of the criteria using a retrospective cohort of paediatric patients with encephalitis. Twenty-nine patients with anti-NMDAR encephalitis and 74 comparison children with encephalitis were included. As expected, the percentage of patients with anti-NMDAR encephalitis who fulfilled the clinical criteria increased over time. During the hospital inpatient admission, most patients (26/29, 90%) with anti-NMDAR encephalitis fulfilled the criteria, significantly more than the comparison group (3/74, 4%) (panti-NMDAR encephalitis was 2 weeks from first symptom onset (range 1-6). The sensitivity of the criteria was 90% (95% confidence interval 73-98) and the specificity was 96% (95% confidence interval 89-99). The proposed diagnostic criteria for anti-NMDAR encephalitis have good sensitivity and specificity. Incomplete criteria do not exclude the diagnosis. The proposed clinical diagnostic criteria for anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis by Graus et al. (2016) have high sensitivity and specificity in paediatric patients. The median time of fulfilling the criteria in patients with anti-NMDAR was 2 weeks from first symptom onset. © 2017 Mac Keith Press.

Paraneoplastic syndromes and autoimmune encephalitis

Science.gov (United States)

Rosenfeld, Myrna R.; Titulaer, Maarten J.

2012-01-01

Summary We review novel findings in paraneoplastic syndromes including the Lambert-Eaton myasthenic syndrome, and then focus on the novel disorders associated with antibodies against cell surface antigens, discussing the importance and caveats of antibody testing, and providing an algorithm for interpretation of results. In anti-NMDAR encephalitis 2 novel findings include the recognition of a characteristic EEG pattern (“extreme delta brush”) in 30% of patients and the demonstration of a fronto-temporo-occipital gradient of glucose metabolism that correlates with disease activity. In limbic encephalitis, antibodies to GABA(B) receptor are the most frequently detected in patients with small-cell lung cancer who are anti-Hu negative, and antibodies to mGluR5 distinctively associate with Hodgkin lymphoma (Ophelia syndrome). We also address the syndromes associated with “VGKC-complex antibodies,” a problematic term that groups well-characterized immune-mediated disorders (LGI1, Caspr2) with others that lack syndrome specificity, are less responsive to treatment, and for which the target antigens are unknown. PMID:23634368

Hemorrhagic herpes encephalitis: A difficult diagnosis in computed tomography

International Nuclear Information System (INIS)

Neumann, N.U.; Albert, H.H. von

1982-01-01

Herpes simplex encephalitis (HSE) is the most common sporadically appearing encephalitis in Central Europe. Differential diagnosis to brain tumors or spontaneous intercerebral hemorrhage is difficult. There are CT scan findings which are characteristic of HSE but there are no pathognomonic patterns. These characteristic findings are helpful in differential diagnosis to neoplastic or vascular processes. Thus, other diagnostic procedures (i.e. brain biopsy) to confirm diagnosis of HSE and effective therapy may be carried out in time. The difficulties in differential diagnosis are shown by the presented case. (orig.) [de

New Onset Insomnia in a Pediatric Patient: A Case of Anti-NMDA Receptor Encephalitis

Directory of Open Access Journals (Sweden)

Tamar N. Goldberg

2017-01-01

Full Text Available Anti-NMDAR encephalitis is becoming more widely recognized as a cause of encephalopathy in both adults and children. Certain clinical features such as mood lability, movement disorders, speech dysfunction, seizures, and autonomic instability in a pediatric patient should prompt immediate concern and evaluation for autoimmune encephalitis among providers. We present the case of a pediatric patient with anti-NMDAR encephalitis in which the symptom prompting medical evaluation was insomnia. Insomnia has not previously been emphasized in the literature as a presenting feature of this disease in children and has a broad differential. Recognition of the symptoms of anti-NMDAR encephalitis and its variable presentation are key to early diagnosis and prompt initiation of treatment which may help to improve outcomes.

Tick-borne encephalitis: What travelers should know when visiting an endemic country

Czech Academy of Sciences Publication Activity Database

Chrdle, A.; Chmelík, V.; Růžek, Daniel

2016-01-01

Roč. 12, č. 10 (2016), s. 2694-2699 ISSN 2164-5515 R&D Projects: GA MZd(CZ) NV16-34238A Institutional support: RVO:60077344 Keywords : endemic country * flavivirus * tick-borne encephalitis * tick-borne encephalitis virus * travel medicine * vaccination Subject RIV: EE - Microbiology, Virology Impact factor: 2.157, year: 2016

Entomologic studies after a St. Louis encephalitis epidemic in Grand Junction, Colorado.

Science.gov (United States)

Tasi, T F; Smith, G C; Ndukwu, M; Jakob, W L; Happ, C M; Kirk, L J; Francy, D B; Lampert, K J

1988-08-01

In 1986, after a St. Louis encephalitis epidemic in Grand Junction, Colorado, in 1985, vector mosquitoes in the city were surveyed to correlate their bionomics and infection rates with the occurrence of human disease. No human cases were reported, but mosquito surveillance disclosed St. Louis encephalitis virus in Culex tarsalis and Culex pipiens pipiens. Mosquitoes were collected with gravid traps designed to attract Cx. p. pipiens and with Centers for Disease Control light traps. Culex p. pipiens was the predominant vector mosquito collected and was captured chiefly in gravid traps. The Culex tarsalis population emerged and expanded approximately one month earlier than did the Cx. p. pipiens population. Consequently, Cx. p. pipiens was the predominant vector species after August. Infection rates throughout the surveillance period (June to September) were severalfold higher in Cx. tarsalis than in Cx. p. pipiens; however, in late summer, diminished numbers of Cx. tarsalis and a persistent population of Cx. p. pipiens resulted in relatively larger numbers of infected Cx. p. pipiens. Thus, the participation of Cx. p. pipiens as a St. Louis encephalitis vector would have been underestimated in previous studies employing light traps alone. These studies provide further evidence that Cx. p. pipiens-associated urban St. Louis encephalitis and rural Cx. tarsalis-associated St. Louis encephalitis cycles may coexist in the West.

Targeted blockade in lethal West Nile virus encephalitis indicates a crucial role for very late antigen (VLA-4-dependent recruitment of nitric oxide-producing macrophages

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Getts Daniel R

2012-10-01

Full Text Available Abstract Infiltration of Ly6Chi monocytes from the blood is a hallmark of viral encephalitis. In mice with lethal encephalitis caused by West Nile virus (WNV, an emerging neurotropic flavivirus, inhibition of Ly6Chi monocyte trafficking into the brain by anti-very late antigen (VLA-4 integrin antibody blockade at the time of first weight loss and leukocyte influx resulted in long-term survival of up to 60% of infected mice, with subsequent sterilizing immunity. This treatment had no effect on viral titers but appeared to be due to inhibition of Ly6Chi macrophage immigration. Although macrophages isolated from the infected brain induced WNV-specific CD4+ T-cell proliferation, T cells did not directly contribute to pathology, but are likely to be important in viral control, as antibody-mediated T-cell depletion could not reproduce the therapeutic benefit of anti-VLA-4. Instead, 70% of infiltrating inflammatory monocyte-derived macrophages were found to be making nitric oxide (NO. Furthermore, aminoguanidine-mediated inhibition of induced NO synthase activity in infiltrating macrophages significantly prolonged survival, indicating involvement of NO in the immunopathology. These data show for the first time the therapeutic effects of temporally targeting pathogenic NO-producing macrophages during neurotropic viral encephalitis.

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports.

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Bashiri, Fahad A; Al-Rasheed, Abdulrahman A; Hassan, Saeed M; Hamad, Muddathir H A; El Khashab, Heba Y; Kentab, Amal Y; AlBadr, Fahad B; Salih, Mustafa A

2017-08-01

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.

Loss of autonoetic consciousness of recent autobiographical episodes and accelerated long-term forgetting in a patient with previously unrecognized glutamic acid decarboxylase antibody related limbic encephalitis

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Juri-Alexander eWitt

2015-06-01

Full Text Available We describe a 35-year old male patient presenting with depressed mood and emotional instability who complained about severe anterograde and retrograde memory deficits characterized by accelerated long-term forgetting and loss of autonoetic consciousness regarding autobiographical memories of the last three years. Months before he had experienced two breakdowns of unknown etiology giving rise to the differential diagnosis of epileptic seizures after various practitioners and clinics had suggested different etiologies such as a psychosomatic condition, burnout, depression or dissociative amnesia. Neuropsychological assessment indicated selectively impaired figural memory performance. Extended diagnostics confirmed accelerated forgetting of previously learned and retrievable verbal material. Structural imaging showed bilateral swelling and signal alterations of temporomesial structures (left > right. Video-EEG monitoring revealed a left temporal epileptic focus and subclincal seizure, but no overt seizures. Antibody tests in serum and liquor were positive for glutamic acid decarboxylase antibodies. These findings led to the diagnosis of glutamic acid decarboxylase antibody related limbic encephalitis. Monthly steroid pulses over six months led to recovery of subjective memory and to intermediate improvement but subsequent worsening of objective memory performance. During the course of treatment the patient reported de novo paroxysmal non-responsive states. Thus, antiepileptic treatment was started and the patient finally became seizure free. At the last visit vocational reintegration was successfully in progress.In conclusion, amygdala swelling, retrograde biographic memory impairment, accelerated long-term forgetting and emotional instability may serve as indicators of limbic encephalitis, even in the absence of overt epileptic seizures. The monitoring of such patients calls for a standardized and concerted multilevel diagnostic approach with

Progressive contralateral hippocampal atrophy following surgery for medically refractory temporal lobe epilepsy.

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Elliott, Cameron A; Gross, Donald W; Wheatley, B Matt; Beaulieu, Christian; Sankar, Tejas

2016-09-01

Determine the extent and time course of volumetric changes in the contralateral hippocampus following surgery for medically refractory temporal lobe epilepsy (TLE). Serial T1-weighted MRI brain scans were obtained in 26 TLE patients pre- and post-temporal lobe epilepsy surgery as well as in 12 control subjects of similar age. Patients underwent either anterior temporal lobectomy (ATL) or selective amygdalohippocampectomy (SAH). Blinded, manual hippocampal volumetry (head, body, and tail) was performed in two groups: 1) two scan group [ATL (n=6); SAH (n=10)], imaged pre-surgery and on average at 5.4 years post-surgery; and 2) longitudinal group [ATL (n=8); SAH (n=2)] imaged pre-surgery and on post-operative day 1, 2, 3, 6, 60, 120 and a delayed time point (average 2.4 years). In the two scan group, there was atrophy by 12% of the unresected contralateral hippocampus (p<0.001), with atrophy being most pronounced (27%) in the hippocampal body (p<0.001) with no significant differences seen for the hippocampal head or tail. In the longitudinal group, significant atrophy was also observed for the whole hippocampus and the body with atrophy seen as early as post-operative day #1 which progressed significantly over the first post-operative week (1.3%/day and 3.0%./day, respectively) before stabilizing over the long-term to a 13% reduction in total volume. There was no significant difference in atrophy compared by surgical approach (ATL vs. SAH; p=0.94) or side (p=0.31); however, atrophy was significantly more pronounced in patients with ongoing post-operative seizures (hippocampal body, p=0.019; whole hippocampus, p=0.048). There were no detectable post-operative neuropsychological deficits attributable to contralateral hippocampal atrophy. Significant contralateral hippocampal atrophy occurs following TLE surgery, which begins immediately and progresses over the first post-operative week. The observation that seizure free patients had significantly less atrophy of the

Acute encephalitis associated with measles: MRI features

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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.)

Mycoplasma pneumoniae-associated encephalitis in childhood--nervous system disorder during or after a respiratory tract infection.

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Meyer Sauteur, P M; Streuli, J C; Iff, T; Goetschel, P

2011-07-01

Mycoplasma pneumoniae is an important aetiological agent of encephalitis in children, with encephalitis being the most frequent paediatric extrapulmonary manifestation of M. pneumoniae infections. Evidence of M. pneumoniae involvement in childhood encephalitis is difficult to obtain, because M. pneumoniae is seldom detected in the cerebrospinal fluid and the clinical picture shows gradual onset. Therefore, we present a small case-study as a paradigm of M. pneumoniae-associated encephalitis in childhood and illustrate the importance of this entity based on a review of previously published cases. We describe neurological signs and symptoms of 2 patients with M. pneumoniae-associated encephalitis in childhood. Respiratory symptoms with fever occurred in both children. They were seropositive for M. pneumoniae, but did not have the organism detected by PCR from cerebrospinal fluid. No long-term neurologic sequelae occurred. M. pneumoniae has to be considered as a responsible pathogen of encephalitis in children, even if respiratory symptoms do not occur. Due to the seldom detection of M. pneumoniae in cerebrospinal fluid, evidence of m. pneumoniae involvement in childhood encephalitis is difficult to obtain.Faced with a neurological disease with no organism detected in CNS in the majority of cases assumes that M. pneumoniae-associated encephalitis is most likely a paradigm for an autoimmune disease with uniform pathogenesis mediated by an immunologic response to an antecedent antigenic stimulus from M. pneumoniae. It is important to relate this organism to this relatively common and potentially devastating clinical syndrome. © Georg Thieme Verlag KG Stuttgart · New York.

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

International Nuclear Information System (INIS)

Lim, Hun Cheol; Yu, In Kyu; Oh, Keon Se

2011-01-01

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.

Electroconvulsive Therapy in Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Case Report and Review of the Literature.

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Coffey, M Justin; Cooper, Joseph J

2016-12-01

There is a growing scientific literature describing the neuropsychiatric symptoms of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, including the use of electroconvulsive therapy (ECT) to treat those symptoms. We sought to consolidate this literature into a review that highlights its relevance to ECT practitioners. We performed a PubMed search using the terms electroconvulsive therapy and encephalitis, autoimmune encephalitis, or anti-NMDA receptor encephalitis. We reviewed all relevant studies in detail, cross-referenced all bibliographies, and collected key clinical information related to the practice of ECT. We identified 6 studies offering patient-level descriptions of the use of ECT in patients with anti-NMDA receptor encephalitis. In all cases ECT was used to target symptoms of catatonia. Electroconvulsive therapy was delivered safely and effectively irrespective of the timing of diagnosis, tumor removal, or immunotherapy. There are no controlled data on the use of ECT in anti-NMDA receptor encephalitis. Further investigation is needed to determine whether ECT has a disease-modifying effect on this form of autoimmune encephalitis.

Nipah virus encephalitis: A cause for concern for Indian neurologists?

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Halder Amit

2006-01-01

Full Text Available The first and only recorded outbreak of Nipah virus (NV encephalitis in India occurred in the winter of 2001, although the causative organism could only be identified 5 years down the line in 2006. The first ever-recorded outbreak of NV encephalitis occurred in the Malaysian peninsula in 1998-99; though between 2001 and 2005, at least four outbreaks occurred in our neighboring country of Bangladesh. The threat of further outbreaks of this dangerous disease looms large on the Indian subcontinent, given the natural reservoir of the definitive host, namely, fruit-eating bats of the genus Pteropus. This review would briefly highlight the epidemiology, clinical aspects and diagnosis of NV encephalitis to enlighten the neurological community of the country for early detection and implementation of preventive measures in the event of further outbreaks, especially those which are generally passed of as ′mystery diseases′ in the lay press and even by governmental agencies.

Voltage-gated potassium channel-associated limbic encephalitis in the West of Scotland: case reports and literature review.

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Reid, J M; Foley, P; Willison, H J

2009-11-01

The syndrome of limbic encephalitis (LE) associated with antibodies against voltage-gated potassium channels (VGKC-LE) has recently been described. The number of published cases is however small. We therefore aimed to review all cases seen at our centre and compare with published cases. Retrospective cases of VGKC-LE were identified using a questionnaire to Neurologists at the Southern General hospital, Glasgow, and by reviewing patients with a positive VGKC antibody test (2002-2007). Case-note review of identified cases and a literature review of all published cases of VGKC-LE were performed. Seven cases were identified (four female, age range 51-81). Patients presented sub-acutely with seizures and anterograde memory loss. Five patients had medial temporal lobe change on cranial imaging. No paraneoplastic cases were identified. 5/7 patients made some improvement with immunotherapy. In 2006, 3/18 (17%) patients with a coded discharge of encephalitis were diagnosed with VGKC-LE. The literature review revealed 40 patients with VGKC-LE. Age, gender or VGKC level did not predict likelihood for a significant recovery. Patients treated VGKC-LE is being increasingly diagnosed and is best identified early and treated with immunotherapy to offer the greatest chance of recovery. This series and literature review expands the current published evidence in VGKC-LE.

Pre- and long-term postoperative courses of hippocampus-associated memory impairment in epilepsy patients with antibody-associated limbic encephalitis and selective amygdalohippocampectomy.

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Hansen, Niels; Ernst, Leon; Rüber, Theodor; Widman, Guido; Becker, Albert J; Elger, Christian E; Helmstaedter, Christoph

2018-02-01

Limbic encephalitis (LE) is defined by mesiotemporal lobe structure abnormalities, seizures, memory, and psychiatric disturbances. This study aimed to identify the long-term clinical and neuropsychological outcome of selective amygdalohippocampectomy (sAH) in drug-resistant patients with temporal lobe epilepsy due to known or later diagnosed subacute LE not responding to immunotherapy associated with neuronal autoantibodies. In seven patients with temporal lobe epilepsy due to antibody positive LE (glutamic acid decarboxylase (GAD65): n=5; voltage-gated potassium channel complex (VGKC), N-methyl d-aspartate receptor (NMDAR): n=1; Ma-2/Ta: n=1) sAH (6 left, 1 right) was performed. Those patients underwent repeated electroencephalography (EEG) recordings, magnetic resonance imaging (MRI) volumetry of the amygdala and hippocampus, and neuropsychological examinations and were followed up for 6-7years on average. Verbal memory and figural memory were affected in 57% of patients at baseline and 71% at the last follow-up. At the last follow-up, 14% of the patients had declined in verbal memory and figural memory. We observed improved memory in 43% of patients regarding figural memory, but not in a single patient regarding verbal memory. Repeated evaluations across the individual courses reveal cognitive and MRI dynamics that appear to be unrelated to surgery and drug treatment. Three of the seven patients with LE with different antibodies (NMDAR: n=1, Ma-2/Ta: n=1 and GAD65: n=1) achieved persistent seizure freedom along with no accelerated memory decline after surgery. Two of the five GAD65-antibody patients positive with LE showed progressive memory decline and a long-term tendency to contralateral hippocampus atrophy. While memory demonstrated some decline in the long run, what is most important is that a progressive decline in memory is seldom found after sAH in patients with LE. Moreover, the dynamics in performance and MRI before and after surgery reveal disease

Paraneoplastic limbic encephalitis presenting as a neurological emergency: a case report

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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.

Transient isolated lesion of the splenium associated with clinically mild influenza encephalitis

International Nuclear Information System (INIS)

Ganapathy, Srinivas; Ey, Elizabeth H.; Wolfson, Barbara J.; Khan, Nadir

2008-01-01

Transient isolated lesions of the splenium with restricted diffusion are rare in the pediatric population. We report two such cases with influenza-associated encephalitis/encephalopathy (IAEE). These reversible isolated central splenial lesions are not specific for IAEE, but the notable feature associated with this specific presentation is a comparatively milder form of encephalitis that resolves clinically and radiologically within a short time. (orig.)

Electroconvulsive therapy in a pediatric patient with malignant catatonia and paraneoplastic limbic encephalitis.

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Lee, Andrew; Glick, David B; Dinwiddie, Stephen H

2006-12-01

Paraneoplastic limbic encephalitis is a rare disorder that can cause memory loss, confusion, personality change, cognitive dysfunction, and psychosis. We present a case of an 11-year-old girl who was successfully treated with electroconvulsive therapy for a catatonic state associated with paraneoplastic limbic encephalitis caused by an ovarian teratoma.

Autoimmune encephalitis associated with voltage-gated potassium channels-complex and leucine-rich glioma-inactivated 1 antibodies - a national cohort study.

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Celicanin, M; Blaabjerg, M; Maersk-Moller, C; Beniczky, S; Marner, L; Thomsen, C; Bach, F W; Kondziella, D; Andersen, H; Somnier, F; Illes, Z; Pinborg, L H

2017-08-01

The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2 antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016. We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment. All magnetic resonance imaging, electroencephalography and 18 F-fluorodeoxyglucose positron emission tomography scans were re-evaluated by experts in the field. A total of 28/192 patients tested positive for VGKC-complex antibodies by radioimmunoassay and indirect immunofluorescence; 17 had antibodies to LGI1 and 6/7 of the available cerebrospinal fluids from these patients were seropositive. These 17 patients all had a clinical phenotype appropriate to LGI1 antibodies. The remaining 11 were LGI1 negative (n = 4) or not tested (n = 7). Of these, two had a phenotype consistent with limbic encephalitis. The remaining phenotypes were Guillain-Barré syndrome, Creutzfeldt-Jakob disease, neuromyotonia and anti-N-methyl-D-aspartate receptor encephalitis. Magnetic resonance imaging abnormalities were demonstrated in 69% of the LGI1-positive patients. Two patients with normal magnetic resonance imaging demonstrated temporal lobe hypermetabolism using 18 F-fluorodeoxyglucose positron emission tomography. Abnormal electroencephalography recordings were found in 86% of the patients. Upon follow-up (median 3.2 years), the median modified Rankin Scale score of anti-LGI1-positive patients was 2 and only two patients reported seizures in the past year. Patients diagnosed with anti-LGI1 autoimmune encephalitis increased significantly from 2009 to 2014, probably due to increased awareness. In contrast to seropositive anti-VGKC-complex patients, all anti-LGI1

Herpes simplex virus-induced anti-N-methyl-d-aspartate receptor encephalitis: a systematic literature review with analysis of 43 cases.

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Nosadini, Margherita; Mohammad, Shekeeb S; Corazza, Francesco; Ruga, Ezia Maria; Kothur, Kavitha; Perilongo, Giorgio; Frigo, Anna Chiara; Toldo, Irene; Dale, Russell C; Sartori, Stefano

2017-08-01

To conduct a systematic literature review on patients with biphasic disease with herpes simplex virus (HSV) encephalitis followed by anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. We conducted a case report and systematic literature review (up to 10 December 2016), focused on differences between herpes simplex encephalitis (HSE) and anti-NMDAR encephalitis phases, age-related characteristics of HSV-induced anti-NMDAR encephalitis, and therapy. For statistical analyses, McNemar's test, Fisher's test, and Wilcoxon rank sum test were used (two-tailed significance level set at 5%). Forty-three patients with biphasic disease were identified (31 children). Latency between HSE and anti-NMDAR encephalitis was significantly shorter in children than adults (median 24 vs 40.5d; p=0.006). Compared with HSE, anti-NMDAR encephalitis was characterized by significantly higher frequency of movement disorder (2.5% vs 75% respectively; panti-NMDAR encephalitis children had significantly more movement disorders (86.7% children vs 40% adults; p=0.006), fewer psychiatric symptoms (41.9% children vs 90.0% adults; p=0.025), and a slightly higher median modified Rankin Scale score (5 in children vs 4 in adults; p=0.015). During anti-NMDAR encephalitis, 84.6 per cent of patients received aciclovir (for ≤7d in 22.7%; long-term antivirals in 18.0% only), and 92.7 per cent immune therapy, but none had recurrence of HSE clinically or using cerebrospinal fluid HSV polymerase chain reaction (median follow-up 7mo). Our review suggests that movement disorder may help differentiate clinically an episode of HSV-induced anti-NMDAR encephalitis from HSE relapse. Compared with adults, children have shorter latency between HSE and anti-NMDAR encephalitis and, during anti-NMDAR encephalitis, more movement disorder, fewer psychiatric symptoms, and slightly more severe disease. According to our results, immune therapy given for HSV-induced anti-NMDAR encephalitis does not predispose patients to

Japanese encephalitis virus: from genome to infectome

Czech Academy of Sciences Publication Activity Database

Unni, S. K.; Růžek, Daniel; Chhatbar, C.; Mishra, R.; Johri, M. K.; Singh, S. K.

2011-01-01

Roč. 13, č. 4 (2011), 312-321 ISSN 1286-4579 Institutional research plan: CEZ:AV0Z60220518 Keywords : Japanese encephalitis * Neurotropic infection * Neuropathogenesis * Mosquito borne infections * Arboviral infections Subject RIV: EE - Microbiology, Virology Impact factor: 3.101, year: 2011

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.

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

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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. PMID:27242653

MR and CT imaging patterns in post-varicella encephalitis

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Darling, C.F. [Div. of Neuroimaging, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States); Larsen, M.B. [Div. of Neurology, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States); Byrd, S.E. [Div. of Neuroimaging, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States); Radkowski, M.A. [Div. of Neuroimaging, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States); Palka, P.S. [Div. of Neuroimaging, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States); Allen, E.D. [Div. of Neuroimaging, Children`s Memorial Center, Northwestern Univ. Medical School, Chicago, IL (United States)

1995-06-01

The aim of the investigation was to determine the patterns of cerebral involvement on computed tomography (CT) and magnetic resonance (MR) imaging in post-varicella encephalitis. Four children between the ages of 2 and 11 years presented over a 5-year period with a diagnosis of post-varicella encephalitis. Their imaging studies and clinical data were reviewed retrospectively. The medical histories of all four children were noncontributory except for recent bouts of chickenpox 1 week to 3 months prior to hospitalization. Three children presented with parkinsonian manifestations. Bilateral, symmetric hypodense, nonenhancing basal ganglia lesions were found on CT. These areas showed nonenhancing low signal intensity on T1-weighted images and high signal intensity on T2-weighted images on MR. One child presented with diffuse, multiple gray and white matter lesions of similar imaging characteristics; some lesions, however, did enhance. This child had no gait disturbances. Post-varicella encephalitis can produce two patterns of dramatic CT and MR findings. With an appropriate history and clinical findings, varicella as a cause of bilateral basal ganglia or diffuse cerebral lesions can be differentiated from other possible etiologies which include trauma, anoxia, metabolic disorders and demyelinating diseases. (orig.)

A case of urinary retention in the early stages of herpes simplex virus type-1 encephalitis.

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Fukuoka, Takuya; Nakazato, Yoshihiko; Miyake, Akifumi; Tamura, Naotoshi; Araki, Nobuo; Yamamoto, Toshimasa

2017-06-01

A 70-year-old man developed urinary retention in the early stages of herpes simplex virus (HSV) type-1 encephalitis. A nerve conduction study suggested latent myeloradiculitis. This is the first report of human herpes simplex virus-1 encephalitis followed by urinary retention at early stage from the onset like the Elsberg syndrome. Although relatively few similar cases have been reported, we consider that urinary retention is common in HSV-1 encephalitis, in which disturbances of consciousness usually require bladder catheterization from the onset. We further emphasize that urinary retention may occasionally occur in early stages of HSV-1 encephalitis, with a significant possibility of recovery. Copyright © 2017. Published by Elsevier B.V.

Cognitive outcomes following anti-N-methyl-D-aspartate receptor encephalitis: A systematic review.

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McKeon, Gemma L; Robinson, Gail A; Ryan, Alexander E; Blum, Stefan; Gillis, David; Finke, Carsten; Scott, James G

2018-04-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated neurological disorder that (among other severe neuropsychiatric symptoms) affects cognition. This study aimed to summarize current knowledge regarding the rates, nature, and predictors of neuropsychological dysfunction in patients recovering from anti-NMDAR encephalitis. A systematic review of studies describing neuropsychological outcomes following anti-NMDAR encephalitis was conducted. Electronic databases Medline, PsycINFO, EMBASE, and CINAHL were searched from inception to September 2016. Results were summarized using descriptive statistics and a series of chi-square analyses. Of 4030 identified studies, 44 were included. These reported neuropsychological outcomes for 109 treated patients (83.5% female, M age  = 22.5 years, range = 2-67) recovering from anti-NMDAR encephalitis. High rates of neuropsychological dysfunction were identified, with diverse impairments of variable severity documented in >75% of patients at assessments conducted during acute, subacute, and longer term recovery periods. Despite this, cognitive outcomes were ultimately considered favorable in most cases (74.3%). This estimate does not account for the potential impact of relapses. The frequency of impairments in overall intellectual functioning, language, attention, working memory, and visuospatial functions were significantly higher within the acute recovery period than in later phases of convalescence. However, rates of impaired processing speed, episodic memory, and aspects of executive functioning were consistent across time points. Adverse neuropsychological outcomes occurred at significantly higher frequency in patients where immunotherapy was delayed, χ 2 (1, N = 66) = 10.84, p anti-NMDAR encephalitis, although improvement in cognitive outcomes can be expected as patients recover. Some cognitive deficits may be less likely than others to resolve. Close neuropsychological monitoring is warranted

Anti-N-methyl-D-aspartate receptor encephalitis associated with acute Toxoplasma gondii infection: A case report.

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Cai, Xiaotang; Zhou, Hui; Xie, Yongmei; Yu, Dan; Wang, Zhiling; Ren, Haitao

2018-02-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been recognized as the most frequent autoimmune encephalitis in children. Several infectious agents have been implicated in anti-NMDA encephalitis. A previously healthy immunocompetent 9-year-old girl first presented with seizures, headaches and vomiting. Cerebrospinal fluid and brain magnetic resonance imaging were normal. After one week onset, the patient gradually developed unexplained personality and behavior changes, accompanied by fever and seizures again. Repeated CSF analysis revealed a slightly lymphocytic predominant pleocytosis and positive anti-NMDAR antibody. A variety of pathogenic examinations were negative, except for positive toxoplasma IgM and IgG. The patient was diagnoses for anti-NMDA encephalitis associated with acute acquired toxoplasma gondii infection. The patient received 10 days azithromycin for treatment of acquired toxoplasma infection. The parents refuse immunotherapy because substantial recovery from clinical symptoms. The patient was substantially recovered with residual mild agitation after therapy for acquired toxoplasma gondii infection. Two months later, the patient was completely devoid of symptoms, and the levels of serum IgM and IgG of toxoplasma gondii were decreased. Acquired toxoplasma gondii infection may trigger anti-NMDAR encephalitis in children, which has not been reported previously. Clinicians should assess the possibility of toxoplasma gondii infection when evaluating a patient with anti-NMDA encephalitis.

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

Early maternal death due to acute encephalitis

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M Vidanapathirana

2014-03-01

Full Text Available Maternal death in an unmarried woman poses a medico-legal challenge. A 24-year-old unmarried schoolteacher, residing at a boarding place, had been admitted to hospital in a state of cardiac arrest. At the autopsy, mild to moderate congestion of subarachnoid vessels and oedema of the brain was noted. An un-interfered foetus of 15 weeks with an intact sac and placental tissues were seen. Genital tract injuries were not present. Histopathological examination showed diffuse perivascular cuffing by mononuclear cells suggestive of viral encephalitis, considering the circumstances of death and the social stigma of pregnancy in this unmarried teacher, the possibility of attempted suicide by ingestion of a poison was considered. Abrus precatorius (olinda seeds commonly found in the area is known to produce acute encephalitis as well as haemorrhagic gastroenteritis and pulmonary congestion was also considered as a possible cause for this unusual presentation

Brain biopsy for diagnosis of chlamydia encephalitis

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I. A. Voznyuk

2017-01-01

Full Text Available This is the case of encephalitis associated with chlamydia infection of central nervous system. The diagnostic protocol of the patient included: a careful observation of somatic and neurological status, laboratory tests of blood and cerebrospinal fluid, neurovisualization. The results of the diagnostic protocol suggest that laboratory tests blood and cerebrospinal fluid possess low sensitivity and specificity. The MRI study has revealed the localization and inflammatory character of the changes in brain tissue; it has also helped to choose the most favorable area for the stereotaxic biopsy. The obtained tissue was evaluated by means of light (immunohistochemistry and electronic microscopy. The active chlamydia infection was estimated. The subsequent antibacterial etiotropic therapy resulted in the regression of the neurologic symptoms and remission.The intravitalpathomorphology study of the brain could be recommended for the management of the severe encephalitis of the unknown origin. 

New-Onset Headache in Patients With Autoimmune Encephalitis Is Associated With anti-NMDA-Receptor Antibodies.

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Schankin, Christoph J; Kästele, Fabian; Gerdes, Lisa Ann; Winkler, Tobias; Csanadi, Endy; Högen, Tobias; Pellkofer, Hannah; Paulus, Walter; Kümpfel, Tania; Straube, Andreas

2016-06-01

We tested the hypotheses (i) that autoimmune encephalitis is associated with new-onset headache, and (ii) that the occurrence of headache is associated with the presence of anti-N-methyl-D-aspartate (NMDA)-receptor antibodies. Autoimmune encephalitis presents with cognitive dysfunction as well as neuro-psychiatric symptoms. Its pathophysiology might involve antibody-mediated dysfunction of the glutamatergic system as indicated by the presence of anti-NMDA-receptor antibodies in some patients. In this cross-sectional study, patients with autoimmune encephalitis were assessed with a standardized interview for previous headache and headache associated with autoimmune encephalitis. Headache was classified according to the International Classification of Headache Disorders, second edition. Clinical and paraclinical findings were correlated with the occurrence of headache. Of 40 patients with autoimmune encephalitis, 19 did not have a history of headache. Of those, nine suffered from encephalitis-associated headache. Seven of these nine had anti-NMDA-receptor antibodies in contrast to only two among the remaining 10 patients without new-onset headache (P = .023, odds ratio: 14, 95% confidence interval: 1.5; 127). In most patients headache occurred in attacks on more than 15 days/month, was severe, and of short duration (less than 4 hours). International Headache Society criteria for migraine were met in three patients. New-onset headache is a relevant symptom in patients with autoimmune encephalitis who have no history of previous headache, especially in the subgroup with anti-NMDA-receptor antibodies. This indicates a thorough investigation for secondary headaches including anti-NMDA-R antibodies for patients with new-onset headache and neuropsychiatric findings. Glutamatergic dysfunction might be important for the generation of head pain but may only occasionally be sufficient to trigger migraine-like attacks in nonmigraineurs. © 2016 American Headache Society.

[Encephalitis due to the Epstein-Barr virus: a description of a clinical case and review of the literature].

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Barón, Johanna; Herrero-Velázquez, Sonia; Ruiz-Piñero, Marina; Pedraza, M Isabel; Rojo-Rello, Silvia; Guerrero-Peral, Ángel Luis

2013-11-16

INTRODUCTION. Infection by the Epstein-Barr virus (EBV) -either as a primary infection, a reactivation or an active chronic infection- can give rise to several clinical forms of involvement of the central nervous system. We report a case of encephalitis due to EBV produced by viral reactivation in an immunocompetent patient which initially mimicked, from the clinical and electroencephalographic point of view, encephalitis due to type 1 herpes simplex virus (HSV-1). CASE REPORT. A 51-year-old male who had reported the presence of dorsal herpes zoster some days earlier. The patient visited the emergency department after suffering a holocranial oppressive headache and febricula for seven days; 24 hours before admission to hospital, he was suffering from drowsiness and language disorder. The neurological examination revealed stiffness in the back of the neck and dysphasia. An analysis of the cerebrospinal fluid revealed pleocytosis (422 cells/mm(3)) with 98% of mononuclear cells and normal protein and glucose concentration levels in cerebrospinal fluid. Magnetic resonance imaging of the brain and electroencephalogram readings were normal with periodic lateralised epileptiform discharges in the left temporal region. Intravenous acyclovir treatment was initiated, but renal failure meant it had to be changed to oral valaciclovir with clinical resolution and improvement of the liquoral parameters. Polymerase chain reaction in the cerebrospinal fluid was positive for EBV and negative for the other neurotropic viruses. In blood, the serology test for EBV with IgG was positive, while IgM and heterophile antibody tests were negative. CONCLUSIONS. EBV infection can give rise to acute disseminated encephalomyelitis or affect several locations in the central nervous system, especially the cerebellum. Clinical pictures mimicking HSV-1 are less frequent. When encephalitis is related to viral reactivation, precipitating factors can be detected, as in our case.

Anti-ma2 paraneoplastic encephalitis in association with recurrent cervical cancer.

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Ney, Douglas E; Messersmith, Wells; Behbakht, Kian

2014-07-01

Paraneoplastic neurological syndromes are rare, and although they are frequently associated with gynecological malignancies, cervical cancer is a rare cause. The symptoms of anti-Ma2 encephalitis are diverse and often present prior to the diagnosis of malignancy. We report a case of a 37-year-old woman with a history of cervical cancer presenting with unexplained weight gain and vertical supranuclear gaze palsy. Magnetic resonance imaging of the brain revealed lesions within the bilateral hypothalami and midbrain. Anti-Ma2 antibodies were eventually found in the serum, prompting a search for malignancy. Recurrent metastatic cervical cancer was found in the retroperitoneal lymph nodes. This is the first report of cervical cancer in association with anti-Ma2 encephalitis, and highlights the need for a high degree of suspicion in patients with a cancer history presenting with neurological symptoms. The symptoms associated with anti-Ma2 encephalitis are diverse and typically precede the diagnosis of cancer in patients, and should trigger a search for an underlying malignancy.

Anti-N-methyl-D-aspartate receptor encephalitis with an imaging-invisible ovarian teratoma: a case report.

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Abdul-Rahman, Zainab M; Panegyres, Peter K; Roeck, Margareta; Hawkins, David; Bharath, Jude; Grolman, Paul; Neppe, Cliffe; Palmer, David

2016-10-24

Anti-N-methyl-D-aspartate receptor encephalitis is a recently discovered disease entity of paraneoplastic limbic encephalitis. It largely affects young women and is often associated with an ovarian teratoma. It is a serious yet treatable condition if diagnosed early. Its remedy involves immunotherapy and surgical removal of the teratoma of the ovaries. This case of anti-N-methyl-D-aspartate receptor encephalitis involves an early surgical intervention with bilateral oophorectomy, despite negative imaging evidence of a teratoma. A 25-year-old white woman with anti-N-methyl-D-aspartate receptor encephalitis presented with behavioral changes and seizures that were confirmed to be secondary to anti-N-methyl-D-aspartate receptor encephalitis. She required an admission to our intensive care unit for ventilator support and received a number of immunological therapies. Multiple imaging investigations showed no evidence of an ovarian teratoma; she had a bilateral oophorectomy 29 days after admission. Ovarian histology confirmed the presence of a teratoma with neuronal cells. A few days after the operation she began to show signs of improvement and, apart from mild short-term memory loss, she returned to normal function. Our patient is an example of teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis, in which the teratoma was identified only microscopically. Her case highlights that even with negative imaging evidence of a teratoma, ovarian pathology should still be considered and explored.

Pitfalls in diagnosing limbic encephalitis - a case report.

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Kerling, F; Blümcke, I; Stefan, H

2008-11-01

The syndrome of limbic encephalitis (LE) is characterized by subacute onset of temporal lobe epilepsy, loss of short-term memory, cognitive confusion and psychiatric symptoms. We report a patient with pharmacoresistant epilepsy who underwent presurgical video-electroencephalogram (EEG)-monitoring with normal psychiatric and neuropsychological findings. Magnetic resonance imaging (MRI) revealed a hyperintense lesion within the right amygdala but no contrast enhancement. Analysis of cerebrospinal fluid (CSF) showed pleocytosis and positive oligoclonal bands, but all tests for neurotropic viruses or borrelia antibodies were negative. Presurgical evaluation identified a right mesiotemporal focus. As a tumour was the most likely differential diagnosis, we performed selective amygdalohippocampectomy of the right hemisphere. Subsequent histopathological examination revealed the surprising diagnosis of LE. As a consequence, tumour screening was initiated and a testicular carcinoma with high anti-Ma2-antibody titres was detected. Following surgical and chemotherapeutical treatment, the patient was seizure-free and Ma2-antibodies decreased below detection limits. Conclusion - This case report highlights that LE has to be considered even in patients with atypical clinical presentation, i.e. without neuropsychological deficits, if CSF analysis reveals an inflammatory response. When LE is diagnosed, extensive tumour search is mandatory to detect and treat the paraneoplastic origin of LE. Therapeutic strategies of LE include surgical treatment as well as early immunosuppression.

[Characteristics of long-term persisting strains of tick-borne encephalitis virus in different forms of the chronic process in animals].

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Frolova, T V; Pogodina, V V; Frolova, M P; Karmysheva, V Ia

1982-01-01

The properties of the Vasilchenko strain of tick-borne encephalitis (TBE) virus and its 3 variants isolated at various stages of persistent infection (383, 453, and 535 days) in Macaca rhesus monkeys and Syrian hamsters with different forms of the chronic TBE were studied. The process characterized by chronic focal inflammatory-degenerative changes in the brains of hamsters without the disturbance of motor functions was associated with persistence of different kinds of virus-specific antigens without virulent virus production. Brain explants of this group of hamsters yielded a virus with cytopathogenic properties but not pathogenic for mice. In a chronic disease developing without the initial acute period, a virus was recovered from hamsters which proved to be virulent for mice and to possess the hemagglutinating and high invasive activity. The most virulent strain was isolated from monkeys with continuously progressive chronic encephalitis with steady paralysis of the extremities. This isolate differed from the parental Vasilchenko strain by a high pathogenicity for hamsters by intracerebral and subcutaneous routes, and thermostability at 50 degrees C.

Tick-borne encephalitis virus in horses, Austria, 2011

Czech Academy of Sciences Publication Activity Database

Rushton, J. O.; Lecollinet, S.; Hubálek, Zdeněk; Svobodová, Petra; Lussy, H.; Nowotny, N.

2013-01-01

Roč. 19, č. 4 (2013), s. 635-637 ISSN 1080-6040 Institutional support: RVO:68081766 Keywords : tick-borne encephalitis virus (TBEV) * strains Subject RIV: GJ - Animal Vermins ; Diseases, Veterinary Medicine Impact factor: 7.327, year: 2013

Anti-N-methyl-D-aspartate receptor encephalitis associated with an ovarian teratoma: two cases report and anesthesia considerations.

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Liu, Haiyang; Jian, Minyu; Liang, Fa; Yue, Hongli; Han, Ruquan

2015-10-16

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated syndrome caused by the production of anti-NMDAR receptor antibodies. The syndrome characterised by psychosis, seizures, sleep disorders, hallucinations and short-term memory loss. Ovarian teratoma is the confirmed tumour associated with anti-NMDAR antibodies. The patients with anti-NMDAR encephalitis complicated by ovarian teratoma require surgical treatment under general anesthesia. NMDARs are important targets of many anesthetic drugs. The perioperative management and complications of anti-NMDAR encephalitis, including hypoventilation, paroxysmal sympathetic hyperactivity (PSH) and epilepsy, are challenging for ansthesiologists. This report described two female patients who presented for resection of the ovarian teratoma, they had confirmed anti-NMDAR encephalitis accompanied by ovarian teratoma. Two patients received gamma globulin treatments and the resection of the ovarian teratoma under total intravenous anesthesia. They were recovered and discharged on the 20(th) and 46(th) postoperative day respectively. There is insufficient evidence about the perioperative management, monitoring and anesthesia management of anti-NMDAR encephalitis. This report was based on the consideration that controversial anesthetics that likely act on NMDARs should be avoided. Additionally, BIS monitoring should to be prudently applied in anti-NMDAR encephalitis because of abnormal electric encephalography (EEG). Anesthesiologists must be careful with regard to central ventilation dysfunctions and PSH due to anti-NMDAR encephalitis.

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, ...Alvarez X, Williams K. J Leukoc Biol. 2003 Nov;74(5):650-6. Epub 2003 Aug 11. (.png) (.svg) (.html) (.csml) Show Monocyte/macrophage... traffic in HIV and SIV encephalitis. PubmedID 12960230 Title Monocyte/macrophage tr

Encephalization in tropical teleost fishes and comparison with their mode of life.

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Bauchot, R; Randall, J E; Ridet, J M; Bauchot, M L

1989-01-01

The brains were dissected from a total of 1225 fishes representing 737 species, 310 genera and 113 families of tropical and subtropical teleosts. Each fish was weighed before brain dissection, and each brain weighed after its removal. The encephalization coefficient k was determined for each fish from a quadratic formula; to conveniently compare brain size of one species with that of another, we used an encephalization index so that an encephalization index of 100 is the average for all the species investigated. The encephalization indices for the families of fishes studied varied from 7 for the Moringuidae to 233 for the Coryphaenidae. There is no strong correlation in relative brain size with phylogenetic position. Although there is a general trend for the more highly evolved fishes to have larger brains, this is partially obscured by some high values in certain primitive groups and low ones in the more advanced. Elongate fishes have lower encephalization indices in general. This may in part be related to low phylogenetic position of most elongate species (anguilliform fishes, for example), in part to the greater relative body weight due to the longer vertebral column (and usually more numerous fin rays as as well), and to their usual mode of swimming by lateral undulations of the body (the most primitive type of aquatic locomotion--one in which the spinal cord plays a major role). No difference could be noted in the encephalization indices of herbivorous families of fishes compared to carnivorous ones. Within a genus, among medium to large-size fishes, those species of larger size tend to have lower encephalization indices. This may be related to larger fishes having less to fear of predators. Fishes which in some passive way avoid predation have low indices in general. This is particularly true of benthic species which conceal themselves by flattened form, fleshy protuberances or protective coloration, or which bury in the sediment or take refuge in burrows

Syndromes of rapidly progressive cognitive decline-our experience

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Sadanandavalli Retnaswami Chandra

2017-01-01

Full Text Available Background: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. Aims: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center. Settings and Design: Patients who presented with rapid deterioration of cognitive functions within weeks to 1 year between 2011 and December 2016 were evaluated. Patients and Methods: All patients underwent all mandatory tests for dementia including brain imaging. Complete vasculitis workup, autoimmune encephalitis profile including Voltage Gated Potassium Channel, N-methyl-D-aspartic acid receptor, glutamic acid-decarboxylase, thyroid-peroxidase antibody, cerebrospinal fluid, and other special tests such as duodenal biopsy and paraneoplastic workup were done based on clinical indications. Results and Conclusions: Out of 144 patients 42 had immune-mediated encephalopathy, 18 had Creutzfeldt-Jakob disease, 3 had Vitamin B12 deficiency, 63 had infection with neurocysticercosis, 7 had tuberculosis, 2 had HIV, 1 had herpes simplex encephalitis, 1 had neurosyphilis, 1 Whipples disease, 1 had Subacute Sclerosing Panencephalitis, 1 had Mass lesion, 3 had Frontotemporal dementia, and 3 had small vessel disease. Good majority of these patients have infective and immune-mediated causes and less number belong to degenerative group. Therefore, caution is needed to look for treatable cause as it carries a different treatment options and outcome.

Investigations of Caspr2, an autoantigen of encephalitis and neuromyotonia

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Lancaster, Eric; Huijbers, Maartje GM; Bar, Vered; Boronat, Anna; Wong, Andrew; Martinez-Hernandez, Eugenia; Wilson, Christina; Jacobs, Dina; Lai, Meizan; Walker, Russell W; Graus, Francesc; Bataller, Luis; Illa, Isabel; Markx, Sander; Strauss, Kevin A.; Peles, Elior; Scherer, Steven S; Dalmau, Josep

2010-01-01

Objective To report clinical and immunological investigations of contactin-associated protein-like 2 (Caspr2), an autoantigen of encephalitis and peripheral nerve hyperexcitability (PNH) previously attributed to voltage-gated potassium channels (VGKC). Methods Clinical analysis of patients with encephalitis, PNH, or both. Immunoprecipitation and mass spectrometry were used to identify the antigen and to develop an assay with Caspr2-expressing cells. Immunoabsorption with Caspr2 and comparative immunostaining of brain and peripheral nerve of wild-type and Caspr2-null mice were used to assess antibody specificity. Results Using Caspr2-expressing cells, antibodies were identified in 8 patients but not in 140 patients with several types of autoimmune or viral encephalitis, PNH, or mutations of the Caspr2-encoding gene. Patients’ antibodies reacted with brain and peripheral nerve in a pattern that co-localized with Caspr2. This reactivity was abrogated after immunoabsorption with Caspr2 and was absent in tissues from Caspr2-null mice. Of the 8 patients with Caspr2 antibodies, 7 had encephalopathy or seizures, 5 neuropathy or PNH, and 1 isolated PNH. Three patients had also myasthenia gravis, bulbar weakness, or symptoms that initially suggested motor neuron disease. None of the patients had active cancer; 7 responded to immunotherapy and were healthy or only mildly disabled at last follow-up (median 8 months, range 6–84). Interpretation Caspr2 is an autoantigen of encephalitis and PNH previously attributed to VGKC antibodies. The occurrence of other autoantibodies may result in a complex syndrome that at presentation could be mistaken for a motor neuron disorder. Recognition of this disorder is important because it responds to immunotherapy. PMID:21387375

Comprehensive and Methodical: Diagnostic and Management Approaches to Rapidly Progressive Dementia.

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Mahajan, Supriya; Appleby, Brian S

2017-09-30

Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone. Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer's disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD. Additionally, in the past decade, emerging retrospective data have led to suggested treatments in autoimmune encephalitis that, if instituted early, can protect patients against residual deficits and disease relapse. Summary Here, we provide an integrative clinical and diagnostic treatment approach that is applicable to the various forms of RPD. We have highlighted the clinical features of selected types of RPD that have experienced advances in the last 10-15 years.

Herpes encephalitis is a disease of middle aged and elderly people: polymerase chain reaction for detection of herpes simplex virus in the CSF of 516 patients with encephalitis. The Study Group.

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Koskiniemi, M; Piiparinen, H; Mannonen, L; Rantalaiho, T; Vaheri, A

1996-02-01

To assess the diagnostic potential of the polymerase chain reaction (PCR) in herpes simplex virus (HSV) encephalitis. Samples of CSF from 516 patients with encephalitis were studied for HSV-DNA by PCR. Samples taken one to 29 days from the onset of symptoms from 38 patients (7.4%) were positive, 32 (6.2%) for HSV-1 and six (1.2%) for HSV-2. At follow up, eight of 28 patients studied were still HSV-PCR positive. A diagnostic serum:CSF antibody ratio to HSV but not to other viruses was detected in 25 of the 38 HSV-PCR positive patients thus supporting the initial PCR findings. Patients positive by HSV-PCR were concentrated in the age group > or = 40 years, and especially in patients aged 60-64 years, of whom nine of 24 (37.5%) were positive. The HSV-PCR was negative in all other patients with encephalitis of known or unknown aetiology. This group included 34 patients with a diagnostic serum:CSF antibody ratio to other viruses. A dual infection, HSV and another microbe, was considered possible in seven patients. The HSV-PCR is a rapid and useful diagnostic method during the early phase of encephalitis. It may be useful in monitoring the efficacy of treatment and allowing the recognition of new features in the appearance of herpes encephalitis. The HSV-PCR test and antibody determinations from serum and CSF are complementary methods, which should both be applied in pursuit of clinical laboratory diagnosis of these conditions.

Two Unique Cases with Anti-GluR Antibody-Positive Encephalitis

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Kosuke Matsuzono

2013-01-01

Full Text Available We report two cases of anti-glutamic acid receptor (anti-GluR antibody-positive encephalitis in males with symptoms such as Parkinsonism, urinary retention, and paralytic ileus. Although non-herpetic encephalitis typically shows magnetic resonance imaging (MRI lesions in the limbic system during early stages, the present cases showed MRI lesions during later stages in the bilateral claustrum and pons. In both cases, anti-GluRε2 and δ2 antibodies were later shown to be positive in the cerebrospinal fluid but negative in the serum. Although early detection of anti-GluR antibodies is essential, early treatment may be significantly more important.

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.

Limbic encephalitis and antibodies to Ma2: a paraneoplastic presentation of breast cancer

OpenAIRE

Sutton, I.; Winer, J.; Rowlands, D.; Dalmau, J.

2000-01-01

A patient with atypical medullary breast cancer is described who presented with symptoms of limbic encephalitis. The patient's serum and CSF contained antibodies that reacted with the nervous system and the tumour. These antibodies recognised Ma2, a neuronal protein related to paraneoplastic limbic and brainstem encephalitis in men with testicular tumours. This report highlights the importance of testing for paraneoplastic antineuronal antibodies in cases of unexplained l...

Progressive multifocal leukoencephalopathy limited to the brain stem

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Kastrup, O.; Maschke, M.; Diener, H.C. [Neurologische Universitaetsklinik, University of Essen (Germany); Wanke, I. [Department of Neuroradiology, University of Essen (Germany)

2002-03-01

Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating slow-virus encephalitis caused by the JC polyomavirus in 2-5% of patients with AIDS. MRI typically shows multiple lesions in the cerebral hemispheres. We present a rare case of rapidly evolving and lethal PML with a severe bulbar syndrome and spastic tetraparesis in a patient with AIDS. MRI showed high-signal lesions on T2-weighted images confined to the brain stem, extending from the medulla oblongata to the midbrain. JC virus polymerase chain reaction in cerebrospinal fluid was positive, and neuropathology showed the findings of PML. This case was also notable because of the rapid progression despite improved immune status with antiretroviral therapy. (orig.)

Electroencephalographic and computed X-ray tomographic findings in 99 Japanese cases of herpes simplex encephalitis

International Nuclear Information System (INIS)

Kamei, Satoshi; Takasu, Toshiaki; Tamura, Masato; Otani, Sugishi.

1988-01-01

This is a retrospective analysis of electroencephalograms (EEG) and computed tomograms (CT) obtained from 99 Japanese patients with herpes simplex encephalitis (HSE). Abnormal findings of EEG were seen in 89 patients (99 %). Focal abnormality, which was frequently detected in the first EEG recording, was seen in 68 patients (76 %). Periodic synchronous discharge was observed in 25 patients (28 %). There were abnormal findings on CT in 88 patients (81 %). Low and high density areas were seen in 64 patients (73 %) and 26 patients (37 %), respectively, with the most frequent site being the temporal lobe. Mass effect was demonstrated in 33 patients (37 %). Electroencephalography detected the abnormal findings earlier than CT during the early stage of HSE in many instances. There was concordance between EEG and CT in the detection of HSE lesions in 45 patients (58 %). (Namekawa, K.)

Validation of EncephalApp, Smartphone-Based Stroop Test, for the Diagnosis of Covert Hepatic Encephalopathy.

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Bajaj, Jasmohan S; Heuman, Douglas M; Sterling, Richard K; Sanyal, Arun J; Siddiqui, Muhammad; Matherly, Scott; Luketic, Velimir; Stravitz, R Todd; Fuchs, Michael; Thacker, Leroy R; Gilles, HoChong; White, Melanie B; Unser, Ariel; Hovermale, James; Gavis, Edith; Noble, Nicole A; Wade, James B

2015-10-01

Detection of covert hepatic encephalopathy (CHE) is difficult, but point-of-care testing could increase rates of diagnosis. We aimed to validate the ability of the smartphone app EncephalApp, a streamlined version of Stroop App, to detect CHE. We evaluated face validity, test-retest reliability, and external validity. Patients with cirrhosis (n = 167; 38% with overt HE [OHE]; mean age, 55 years; mean Model for End-Stage Liver Disease score, 12) and controls (n = 114) were each given a paper and pencil cognitive battery (standard) along with EncephalApp. EncephalApp has Off and On states; results measured were OffTime, OnTime, OffTime+OnTime, and number of runs required to complete 5 off and on runs. Thirty-six patients with cirrhosis underwent driving simulation tests, and EncephalApp results were correlated with results. Test-retest reliability was analyzed in a subgroup of patients. The test was performed before and after transjugular intrahepatic portosystemic shunt placement, and before and after correction for hyponatremia, to determine external validity. All patients with cirrhosis performed worse on paper and pencil and EncephalApp tests than controls. Patients with cirrhosis and OHE performed worse than those without OHE. Age-dependent EncephalApp cutoffs (younger or older than 45 years) were set. An OffTime+OnTime value of >190 seconds identified all patients with CHE with an area under the receiver operator characteristic value of 0.91; the area under the receiver operator characteristic value was 0.88 for diagnosis of CHE in those without OHE. EncephalApp times correlated with crashes and illegal turns in driving simulation tests. Test-retest reliability was high (intraclass coefficient, 0.83) among 30 patients retested 1-3 months apart. OffTime+OnTime increased significantly (206 vs 255 seconds, P = .007) among 10 patients retested 33 ± 7 days after transjugular intrahepatic portosystemic shunt placement. OffTime+OnTime decreased significantly (242 vs

Eastern equine encephalitis cases among horses in Brazil between 2005 and 2009.

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de Novaes Oliveira, Rafael; Iamamoto, Keila; Silva, Maria Luana Cristiny Rodrigues; Achkar, Samira Maria; Castilho, Juliana Galera; Ono, Ekaterina Durymanova; Lobo, Renata Spinelli Vaz; Brandão, Paulo Eduardo; Carnieli, Pedro; Carrieri, Maria Luiza; Kotait, Ivanete; Macedo, Carla Isabel

2014-10-01

Eastern equine encephalitis is a viral zoonosis that exhibits complex distribution and epidemiology, and greater importance should be given to this disease by the public-health authorities. In Brazil, although eastern equine encephalitis virus (EEEV) has been identified in vectors and antibodies are sometimes detected in horses and humans, there have been no records of equine encephalitis in horses caused by this virus during the last 24 years. This study describes eighteen cases of eastern equine encephalomyelitis that occurred in six Brazilian states between 2005 and 2009. Viral RNA was identified using semi-nested RT-PCR to detect members of the genus Alphavirus, and by genetic sequencing. The gene encoding NSP1 was partially amplified, and after genetic sequencing, eighteen sequences were generated. All eighteen strains were classified as belonging to lineage III of American EEEV. These findings could be an indication of the importance of this virus in animal and human public health.

Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Adult Patients Requiring Intensive Care.

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de Montmollin, Etienne; Demeret, Sophie; Brulé, Noëlle; Conrad, Marie; Dailler, Frédéric; Lerolle, Nicolas; Navellou, Jean-Christophe; Schwebel, Carole; Alves, Mikaël; Cour, Martin; Engrand, Nicolas; Tonnelier, Jean-Marie; Maury, Eric; Ruckly, Stéphane; Picard, Géraldine; Rogemond, Véronique; Magalhaes, Éric; Sharshar, Tarek; Timsit, Jean-François; Honnorat, Jérôme; Sonneville, Romain

2017-02-15

Encephalitis caused by anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is the leading cause of immune-mediated encephalitis. There are limited data on intensive care unit (ICU) management of these patients. To identify prognostic factors of good neurologic outcome in patients admitted to an ICU with anti-NMDAR encephalitis. This was an observational multicenter study of all consecutive adult patients diagnosed with anti-NMDAR encephalitis at the French National Reference Centre, admitted to an ICU between 2008 and 2014. The primary outcome was a good neurologic outcome at 6 months after ICU admission, defined by a modified Rankin Scale score of 0-2. Seventy-seven patients were included from 52 ICUs. First-line immunotherapy consisted of steroids (n = 61/74; 82%), intravenous immunoglobulins (n = 71/74; 96%), and plasmapheresis (n = 17/74; 23%). Forty-five (61%) patients received second-line immunotherapy (cyclophosphamide, rituximab, or both). At 6 months, 57% of patients had a good neurologic outcome. Independent factors of good neurologic outcome were early (≤8 d after ICU admission) immunotherapy (odds ratio, 16.16; 95% confidence interval, 3.32-78.64; for combined first-line immunotherapy with steroids and intravenous immunoglobulins vs. late immunotherapy), and a low white blood cell count on the first cerebrospinal examination (odds ratio, 9.83 for 50 cells/mm 3 ; 95% confidence interval, 1.07-90.65). Presence of nonneurologic organ failures at ICU admission and occurrence of status epilepticus during ICU stay were not associated with neurologic outcome. The prognosis of adult patients with anti-NMDAR encephalitis requiring intensive care is good, especially when immunotherapy is initiated early, advocating for prompt diagnosis and early aggressive treatment.

[NMDA-GluR Subunit Antibody-Positive Encephalitis: A Clinical Analysis of Five Cases].

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Kaneko, Chikako; Shakespear, Norshalena; Tuchiya, Mario; Kubo, Jin; Yamamoto, Teiji; Katayama, Soichi; Takahashi, Yukitoshi

2016-09-01

Five consecutive cases of anti-NMDA-receptor encephalitis that we encountered were marked by a rapidly fluctuating level of consciousness associated with psychotic and delirious mental states. Opisthotonus, catatonia, and rhythmic and non-rhythmic involuntary movements of the mouth and jaw were also characteristic features of these particular cases. Serious and potentially fatal problems included epilepsia partialis continua, partial and generalized seizures, and respiratory depression, resembling the symptoms of encephalitis lethargica. An epidemic of encephalitis lethargica, also known of Economo encephalitis, occurred around 1917. Magnetic resonance imaging revealed edema of the neocortex in two cases and electroencephalography showed polymorphic and monomorphic delta slowing in the acute stage, although electroencephalographic seizure activity were not apparent. Routine cerebrospinal fluid analyses revealed lymphocyte-dominant pleocytosis in three cases, but antibodies against the NMDA-GluR subunit, GluN2B N-terminal, were at a high level in the fluid. All patients recovered without apparent sequelae. Two patients found to have ovarian teratoma underwent surgery for tumor removal. Treatments included pulse intravenous methylprednisolone, high-dose immunoglobulin, and plasma exchange together with seizure control and respiratory support. However, rituximab and or cyclophosphamide pulse therapy should also be considered for intractable cases, as indicated by recent reports. (Received February 16, 2016; Accepted May 2, 2016; Published September 1, 2016).

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Review and Neuropsychological Case Study.

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Loughan, Ashlee R; Allen, Aislyn; Perna, Robert; Malkin, Mark G

2016-01-01

Anti-N-Methyl-d-Aspartate Receptor (NMDAR) Encephalitis is an autoimmune-mediated encephalitis, which may be associated with a tumor, which occurs when antibodies bind central NMDA receptors. Although typically diagnosed in women, approximately 20% of cases have been males. Due to the challenges with identification, imaging, and diverse symptom presentation, this syndrome is often misdiagnosed. Accurate diagnosis may provide an opportunity for introduction of disease-modifying therapies, which may alter disease trajectory. Moreover, neuropsychology has yet to fully clarify the pattern of impairments expected with this disorder. This manuscript reviews a single case study of a 42-year-old male diagnosed with NMDAR encephalitis. Neuropsychological evaluation was completed subsequent to diagnosis, treatment, and rehabilitation. Ongoing patient complaints, approximately six months post diagnosis, included reduced sustained attention, poor word retrieval, and daily forgetfulness. Adaptive skills were improved following rehabilitation. Direct testing revealed mildly impaired sustained attention, processing speed, oral word fluency, and executive functioning. All other cognitive domains were within estimated premorbid range, low average to average. Neuropsychological deficits were consistent with mild frontal brain dysfunction and continued recovery. This case illustrates the need for medical and psychological practitioners to understand NMDAR encephalitis, its symptom presentation, and related neuropsychological impact; particularly with the potential for misdiagnosis.

Temporal expression profiling of plasma proteins reveals oxidative stress in early stages of Type 1 Diabetes progression

International Nuclear Information System (INIS)

Liu, Chih-Wei; Bramer, Lisa; Computational Modeling); Webb-Robertson, Bobbie-Jo; Computational Modeling); Waugh, Kathleen; Rewers, Marian J.; Zhang, Qibin; Biochemistry)

2017-01-01

We report that blood markers other than islet autoantibodies are greatly needed to indicate the pancreatic beta cell destruction process as early as possible, and more accurately reflect the progression of Type 1 Diabetes Mellitus (T1D). To this end, a longitudinal proteomic profiling of human plasma using TMT-10plex-based LC-MS/MS analysis was performed to track temporal proteomic changes of T1D patients (n = 11) across 9 serial time points, spanning the period of T1D natural progression, in comparison with those of the matching healthy controls (n = 10). To our knowledge, the current study represents the largest (> 2000 proteins measured) longitudinal expression profiles of human plasma proteome in T1D research. By applying statistical trend analysis on the temporal expression patterns between T1D and controls, and Benjamini-Hochberg procedure for multiple-testing correction, 13 protein groups were regarded as having statistically significant differences during the entire follow-up period. Moreover, 16 protein groups, which play pivotal roles in response to oxidative stress, have consistently abnormal expression trend before seroconversion to islet autoimmunity. Importantly, the expression trends of two key reactive oxygen species-decomposing enzymes, Catalase and Superoxide dismutase were verified independently by ELISA.

Diffusion-weighted imaging in the diagnosis of enterovirus 71 encephalitis

International Nuclear Information System (INIS)

Lian, Zhou-yang; Huang, Biao; Liang, Chang-hong; He, Shaoru; Guo, Yuxiong

2012-01-01

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

Diffusion-weighted imaging in the diagnosis of enterovirus 71 encephalitis

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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

A case of suspected 'Bickerstaff's encephalitis' and its CT findings

International Nuclear Information System (INIS)

Moto, Atsufumi; Endo, Shunro; Ohmori, Tomoaki; Oka, Nobuo; Takaku, Akira

1985-01-01

A case of suspected ''Bickerstaff's encephalitis'' was presented with special reference to the CT findings. A 4-year-old girl exhibited fever and nausea after the beginning of December, 1983, followed by unsteady gait on January 1, 1984. She was admitted to another hospital and diagnosed by means of a spinalfluid examination as having aseptic meningitis. Her symptoms subsided transiently, but then she began again to suffer recurrently from unsteady gait on January 27. After that she developed dysarthria and dysphagia, a disturbance of eye movement was observed, and she became lethargic and disoriented. On February 6, she was transferred to our hospital. Plain CT scan on admission revealed a low-density area on the left cerebellar peduncle and the inferior medial part of the left temporal lobe. Moreover, the low-density area was heterogenously enhanced with the contrast medium. Angiography showed no abnormal findings. Considering these CT findings, we suspected a pontine tumor. Four days after her admission, however, her symptoms and signs began rapidly to subside, and also the abnormal findings on the CT scan diminished gradually as the symptoms were relieved. On March 2, she was discharged without any neurological abnormality. (author)

Anti-NMDA receptor encephalitis: clinical characteristics, predictors of outcome and the knowledge gap in southwest China.

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Wang, W; Li, J-M; Hu, F-Y; Wang, R; Hong, Z; He, L; Zhou, D

2016-03-01

The aim was to analyse the clinical profiles and outcomes of patients with anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis in China. A retrospective study of anti-NMDAR encephalitis in China was performed between June 2011 and June 2014. The clinical characteristics and predictors of poor outcome were determined. A total of 51 patients with a definitive diagnosis of anti-NMDAR encephalitis were included in this study. Four of them were surgically confirmed to have a neoplasm. Thirty-two patients, amongst whom 24 were female, presented with psychiatric disorder as the initial symptom, whereas 14 patients, of whom nine were male, presented with seizure as the initial symptom (P = 0.011). Twenty-nine patients (56.86%) were initially misdiagnosed with psychosis, viral encephalitis or other diseases, and 58.8% of the patients experienced at least one type of complication. It typically took 3 weeks before these patients were admitted to our hospital and another 2 weeks before the correct diagnosis was made. Forty-one patients (80%) reached a good outcome; 10 patients (20%) had a poor outcome. Older age, extended hospital stay, memory deficits, decreased consciousness, central hypoventilation, complications and abnormal cerebrospinal fluid results were associated with poor outcome (P < 0.05). Female patients more frequently initially present with psychiatric disorder but male patients more frequently initially present with seizure. Patients with anti-NMDAR encephalitis in China have a lower incidence of neoplasm. Nevertheless, this study reveals several challenges in treating anti-NMDAR encephalitis in China that may contribute to poor outcome. © 2015 EAN.

Dengue Haemorrhagic Encephalitis: Rare Case Report with Review of Literature.

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Kutiyal, Aditya Singh; Malik, Chetanya; Hyanki, Gitika

2017-07-01

Dengue is an endemic arboviral infection prevalent especially in tropical countries including Southern and Southeast Asia. Central Nervous System (CNS) involvement in dengue infection is uncommon. Haemorrhagic encephalitis is a rare presentation in dengue. This is a case of a 58-year-old male who presented with fever, petechial rash and altered sensorium. Dengue serology IgM was reactive and MRI brain was suggestive of haemorrhagic encephalitis. Patient was managed in Intensive Care Unit (ICU) but eventually succumbed to his illness. We report this fatal outcome of a common viral infection with unusual neurological presentation to propose an association between dengue and neurotropism and the need to look at dengue infection beyond its classical features.

Dynamic profiling of different ready-to-drink fermented dairy products: A comparative study using Temporal Check-All-That-Apply (TCATA), Temporal Dominance of Sensations (TDS) and Progressive Profile (PP).

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Esmerino, Erick A; Castura, John C; Ferraz, Juliana P; Tavares Filho, Elson R; Silva, Ramon; Cruz, Adriano G; Freitas, Mônica Q; Bolini, Helena M A

2017-11-01

Despite the several differences in ingredients, processes and nutritional values, dairy foods as yogurts, fermented milks and milk beverages are widely accepted worldwide, and although they have their sensory profiling normally covered by descriptive analyses, the temporal perception involved during the consumption are rarely considered. In this sense, the present work aimed to assess the dynamic sensory profile of three categories of fermented dairy products using different temporal methodologies: Temporal Dominance of Sensations (TDS), Progressive Profiling (PP), Temporal CATA (TCATA), and compare the results obtained. The findings showed that the different sensory characteristics among the products are basically related to their commercial identity. Regarding the methods, all of them collected the variations between samples with great correlation between data. In addition, to detect differences in intensities, TCATA showed to be the most sensitive method in detecting textural changes. When using PP, a balanced experimental design considering the number of attributes, time intervals, and food matrix must be weighed. The findings are of interest to guide sensory and consumer practitioners involved in the dairy production to formulate/reformulate their products and help them choosing the most suitable dynamic method to temporally evaluate them. Copyright © 2017 Elsevier Ltd. All rights reserved.

Estimating the burden of Japanese encephalitis virus and other encephalitides in countries of the mekong region.

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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.

Focal CA3 hippocampal subfield atrophy following LGI1 VGKC-complex antibody limbic encephalitis

OpenAIRE

Miller, T; Chong, T; Aimola Davies, A; Ng, T; Johnson, M; Irani, S; Vincent, A; Husain, M; Jacob, S; Maddison, P; Kennard, C; Gowland, P; Rosenthal, C

2017-01-01

Magnetic resonance imaging has linked chronic voltage-gated potassium channel (VGKC) complex antibody-mediated limbic encephalitis with generalized hippocampal atrophy. However, autoantibodies bind to specific rodent hippocampal subfields. Here, human hippocampal subfield (subiculum, cornu ammonis 1-3, and dentate gyrus) targets of immunomodulation-treated LGI1 VGKC-complex antibody-mediated limbic encephalitis were investigated using in vivo ultra-high resolution (0.39 × 0....

Anti-N-methyl-D-aspartate receptor encephalitis in Taiwan--a comparison between children and adults.

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Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Chou, Min-Liang; Hung, Po-Cheng; Hsieh, Meng-Ying; Chou, I-Jun; Wang, Huei-Shyong

2014-06-01

Since the discovery of antibodies against the N-methyl-D-aspartate receptor in 2007, anti-N-methyl-D-aspartate receptor encephalitis is increasingly recognized worldwide. We compare the clinical features of adults and children with this disorder in Taiwan. Patients admitted to Chang Gung Memorial Hospital and Chang Gung Children's Hospital and those who were referred from other institutions because of unknown encephalitis from 2009 to 2013 were enrolled, and their clinical features were analyzed. Data on cases from a review of the literature were also included in the analysis. Twelve patients (10 females) aged between 7 years and 28 years with anti-N-methyl-D-aspartate receptor encephalitis were identified. Six patients (50%) were Anti-N-methyl-D-aspartate receptor encephalitis is increasingly recognized in Taiwan. It is characterized by its clinical features, predominantly affects females with and/or without an ovarian tumor, and it is a potentially treatable disorder. It is important for neurologists to be familiar with the clinical presentations of the disease in children and young adults. Copyright © 2014 Elsevier Inc. All rights reserved.

Enterovirus 71 Brainstem Encephalitis and Cognitive and Motor Deficits

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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.

Quantification of vector and host competence for Japanese encephalitis virus: a systematic review of the literature

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Japanese encephalitis virus (JEV) is a virus of the Flavivirus genus that may result in encephalitis in human hosts. This vector-borne zoonosis occurs in Eastern and Southeastern Asia and an intentional or inadvertent introduction into the United States (US) will have major public health and economi...

Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease.

Science.gov (United States)

Castle, James; Sakonju, Ai; Dalmau, Josep; Newman-Toker, David E

2006-10-01

A 39-year-old man presented with a history of several months of progressive personality changes, social withdrawal, bradykinesia, mutism, dysphagia, worsening gait, and difficulty with daily living activities. Examination revealed an atypical parkinsonian appearance with incomplete supranuclear ophthalmoplegia and an unusual oculomotor disorder characterized by both low-amplitude, intermittent opsoclonus, and slow, nystagmoid intrusions. Routine laboratory testing, autoimmune and infectious serologies, brain MRI, lumbar puncture, electroencephalogram, whole-body CT scan, paraneoplastic serologies, small bowel biopsy, 18F-fluorodeoxyglucose positron emission tomography CT scan, brain biopsy, and testicular ultrasound. Anti-Ma2 paraneoplastic encephalitis in association with metastatic testicular cancer; initially misdiagnosed as CNS Whipple's disease. Corticosteroids, intravenous immunoglobulins, orchiectomy, muscle relaxants, mycophenolate mofetil, plasmapheresis, and bleomycin, etoposide and platinum chemotherapy.

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.

Hemorrhage listerial encephalitis in a patient with systemic lupus erythematosis: A case report

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Lee, Hyun Gyu; Lee, Ji Young; Lee, Young Jun; Kim, Young Seo; Kim, Hyun Young; Sung, Won Jae [Hanyang University Hospital, Hanyang University College of Medicine, Seoul (Korea, Republic of)

2017-05-15

A 31-year-old female with systemic lupus erythematosus (SLE) presented with fever, headache, seizures and mental status changes. Brain MRI showed T2 hyperintense lesions in the cerebellum and frontal white matter and a lesion in the cerebellum exhibited hemorrhagic changes and peripheral ring enhancement. The MRI features of listerial encephalitis are difficult to differentiate from those of neuropsychiatric SLE and various other diseases. Here, we report a case of hemorrhagic listerial encephalitis in a patient with SLE.

High Grade Glioma Mimicking Voltage Gated Potassium Channel Complex Associated Antibody Limbic Encephalitis

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Dilan Athauda

2014-01-01

Full Text Available Though raised titres of voltage gated potassium channel (VGKC complex antibodies have been occasionally associated with extracranial tumours, mainly presenting as Morvan's Syndrome or neuromyotonia, they have not yet been reported to be associated with an intracranial malignancy. This is especially important as misdiagnosis of these conditions and delay of the appropriate treatment can have important prognostic implications. We describe a patient with a high grade glioma presenting with clinical, radiological, and serological features consistent with the diagnosis of VGKC antibody associated limbic encephalitis (LE. This is the first association between a primary brain tumour and high titre of VGKC complex antibodies. Clinicoradiological progression despite effective immunosuppressive treatment should prompt clinicians to look for alternative diagnoses. Further studies to elucidate a possible association between VGKC complex and other surface antigen antibodies with primary brain tumours should be carried out.

High grade glioma mimicking voltage gated potassium channel complex associated antibody limbic encephalitis.

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Athauda, Dilan; Delamont, R S; Pablo-Fernandez, E De

2014-01-01

Though raised titres of voltage gated potassium channel (VGKC) complex antibodies have been occasionally associated with extracranial tumours, mainly presenting as Morvan's Syndrome or neuromyotonia, they have not yet been reported to be associated with an intracranial malignancy. This is especially important as misdiagnosis of these conditions and delay of the appropriate treatment can have important prognostic implications. We describe a patient with a high grade glioma presenting with clinical, radiological, and serological features consistent with the diagnosis of VGKC antibody associated limbic encephalitis (LE). This is the first association between a primary brain tumour and high titre of VGKC complex antibodies. Clinicoradiological progression despite effective immunosuppressive treatment should prompt clinicians to look for alternative diagnoses. Further studies to elucidate a possible association between VGKC complex and other surface antigen antibodies with primary brain tumours should be carried out.

Influence of Malnutrition on Adverse Outcome in Children with Confirmed or Probable Viral Encephalitis: A Prospective Observational Study

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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.

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

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Kuang-Lin Lin

Full Text Available 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.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 prognosis were analyzed.During the study period, 1038 patients (450 girls, 588 boys were enrolled. Among them, 44.6% (463 had seizures in the acute phase, 33% had status epilepticus, and 26% (251 developed postencephalitic epilepsy. At one year of follow-up, 205 of the 251 patients with postencephalitic epilepsy were receiving antiepileptic drugs while 18% were seizure free even after discontinuing the antiepileptic drugs. Among those with postencephalitic epilepsy, 67% had favorable outcomes and were using <2 anti-epileptic drugs while 15% had intractable seizures and were using ≥ 2 antiepileptic drugs. After benzodiazepines, intravenous phenobarbital was preferred over phenytoin as treatment of postencephalitic seizures in the acute phase. For refractory status epilepticus, high-dose topiramate combined with intravenous high-dose phenobarbital or high-dose lidocaine had less side effects.Children with encephalitis have a high rate of postencephalitic epilepsy. Phenobarbital and clonazepam are the most common drugs used, alone or in combination, for postencephalitic epilepsy.

Acute encephalitis and encephalopathy associated with human parvovirus B19 infection in children.

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Watanabe, Toru; Kawashima, Hideshi

2015-11-08

Reports of neurologic manifestations of human parvovirus B19 (B19) infection have been on the rise. Acute encephalitis and encephalopathy is the most common, accounting for 38.8% of total B19-associated neurological manifestations. To date, 34 children with B19 encephalitis and encephalopathy have been reported, which includes 21 encephalitis and 13 encephalopathy cases. Ten (29%) were immunocompromised and 17 (39%) had underlying diseases. Fever at the onset of disease and rash presented in 44.1% and 20.6% of patients, respectively. Neurological manifestations include alteration of consciousness occurred in all patients, seizures in 15 (44.1%) patients, and focal neurologic signs in 12 (35.3%) patients. Anemia and pleocytosis in cerebrospinal fluid (CSF) occurred in 56.3% and 48.1% of patients, respectively. Serum Anti-B19 IgM (82.6%) and CSF B19 DNA (90%) were positive in the majority of cases. Some patients were treated with intravenous immunoglobulins and/or steroids, although an accurate evaluation of the efficacy of these treatment modalities cannot be determined. Nineteen (57.6%) patients recovered completely, 11 (33.3%) patients had some neurological sequelae and 3 (8.8%) patients died. Although the precise pathogenesis underlying the development of B19 encephalitis and encephalopathy is unclear, direct B19 infection or NS1protein of B19 toxicity in the brain, and immune-mediated brain injuries have been proposed.

Second primary tumor in anti-Ma1/2-positive paraneoplastic limbic encephalitis.

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Leyhe, T; Schüle, R; Schwärzler, F; Gasser, T; Haarmeier, T

2006-05-01

Memory loss can be a symptom of paraneoplastic limbic encephalitis (PLE) a neuropsychiatric disorder associated mostly with small-cell lung cancer and anti-Hu antibodies or with testicular tumors and anti-Ma2 antibodies. We present the case of a patient with temporal coincidence of beginning cognitive decline and diagnosis of a carcinoma of the prostate in whom we diagnosed anti-Ma1/Ma2-positive PLE. The tumor had been completely resected but memory impairment further deteriorated. As the effective treatment of the cancer is considered as the most efficient treatment of a paraneoplastic neurological syndrome (PNS) a second neoplasia was suspected in the patient. By the aid of whole body positron emission tomography with 18-fluorine fluoro-2-deoxy-glucose (FDG-PET) an adenocarcinoma of the cecum could be detected. Two months after surgery anti-Ma antibodies were negative. We conclude that a second neoplasia should be considered, if effective cancer treatment does not lead to improvement or stabilisation of a PNS. Tumor search should be exhaustive and include PET when conventional imaging fails to show a malignancy.

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

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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. Copyright © 2016 Elsevier B.V. All rights reserved.

Limbic encephalitis presenting as a post-partum psychiatric condition.

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Gotkine, Marc; Ben-Hur, Tamir; Vincent, Angela; Vaknin-Dembinsky, Adi

2011-09-15

We describe a woman who presented with a psychiatric disorder post-partum and subsequently developed seizures and cognitive dysfunction prompting further investigation. A diagnosis of limbic encephalitis (LE) was made and antibodies to voltage-gated potassium channel complex (VGKC) detected. These antibodies are found in many non-paraneoplastic patients with LE. Although antibody-mediated conditions tend to present or relapse post-partum, VGKC-LE in the post-partum period has not been described. Case report. Clinical and imaging data were consistent with limbic encephalitis. High titres of anti-VGKC-complex antibodies confirmed the diagnosis of VGKC-LE. The similarities between the psychiatric symptomatology of VGKC-LE and post-partum psychiatric disorders raise the possibility that some instances of post-partum psychiatric conditions are manifestations of immune-mediated, non-paraneoplastic LE. Copyright © 2011 Elsevier B.V. All rights reserved.

Herpes simplex type 2 encephalitis and methotrexate medication: a fortuitous or causative association in a patient with spondyloarthritis?

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Lupo, Julien; Dos Santos, Ophélie; Germi, Raphaele; Baccard-Longère, Monique; Stahl, Jean-Paul; Epaulard, Olivier; Morand, Patrice

2017-01-01

It is unclear whether immunosuppression is a risk factor for herpes encephalitis. Herein, we describe a rare case of herpes simplex virus 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 methotrexate therapy as a risk factor of neurological herpes reactivation and severe disease. The host-related and viral risk factors are also addressed.

[VGKC antibodies associated with limbic encephalitis].

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Soeder, B M; Urbach, H; Elger, C E; Bien, C G; Beyenburg, S

2005-06-01

Since the initial description of limbic encephalitis (LE) in 1960/1968, several subforms of this clinico-neuropathological syndrome have been identified. The best known is paraneoplastic LE. However, non-paraneoplastic forms have been reported, too. Very recently, autoantibodies against voltage-gated potassium channels have been described in association with LE. The diagnostic workup of such a case and the apparently typical good response to long-term immunotherapy of this LE subform are described.

Determinants of tick-borne encephalitis in counties of southern Germany, 2001-2008

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Kiffner Christian

2010-08-01

Full Text Available Abstract Background Tick-borne encephalitis (TBE virus can cause severe symptoms in humans. The incidence of this vector-borne pathogen in humans is characterised by spatial and temporal heterogeneity. To explain the variation in reported human TBE cases per county in southern Germany, we designed a time-lagged, spatially-explicit model that incorporates ecological, environmental, and climatic factors. Results We fitted a logistic regression model to the annual counts of reported human TBE cases in each of 140 counties over an eight year period. The model controlled for spatial autocorrelation and unexplained temporal variation. The occurrence of human TBE was found to be positively correlated with the proportions of broad-leafed, mixed and coniferous forest cover. An index of forest fragmentation was negatively correlated with TBE incidence, suggesting that infection risk is higher in fragmented landscapes. The results contradict previous evidence regarding the relevance of a specific spring-time temperature regime for TBE epidemiology. Hunting bag data of roe deer (Capreolus capreolus in the previous year was positively correlated with human TBE incidence, and hunting bag density of red fox (Vulpes vulpes and red deer (Cervus elaphus in the previous year were negatively correlated with human TBE incidence. Conclusions Our approach suggests that a combination of landscape and climatic variables as well as host-species dynamics influence TBE infection risk in humans. The model was unable to explain some of the temporal variation, specifically the high counts in 2005 and 2006. Factors such as the exposure of humans to infected ticks and forest rodent population dynamics, for which we have no data, are likely to be explanatory factors. Such information is required to identify the determinants of TBE more reliably. Having records of TBE infection sites at a finer scale would also be necessary.

Substantia nigra depigmentation and exposure to encephalitis lethargica.

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Hack, Nawaz; Jicha, Gregory A; Abell, Annalisa; Dean, Dawson; Vitek, Jerrold L; Berger, Joseph R

2012-12-01

Parkinsonism has occasionally been reported as a consequence of infectious diseases. The present study examines the clinical and pathological correlates of parkinsonism across birth cohorts in relation to critical exposure to the encephalitis lethargica epidemic in the early 1900s. The study population consisted of 678 participants in the Nun Study, of whom 432 died and came to autopsy. Qualitative indices of substantia nigra (SN) depigmentation were verified in a subset of 40 randomly selected subjects using quantitative stereological techniques. SN depigmentation, detected neuropathologically, was correlated with clinical parameters of Parkinson disease, age, and birth cohort. SN depigmentation was detected in 57 (13.2%) of the cohort. Although qualitative SN depigmentation correlated modestly with age (p = 0.02), it correlated best with birth cohort (p = 0.009) for women born in the years 1895-1899. Quantitative measures of SN depigmentation were increased in this birth cohort compared to age matched subjects from flanking birth cohorts 1890-1894 and 1900-1904 (p < 0.001). SN depigmentation correlated with speed of 6- and 50-foot walk (p < 0.0001), up and go test (p < 0.0001), and hand coordination (p < 0.0001). Subjects in the birth cohort 1895-1899 would have been in their late teens and 20s at the onset and during the peak of the encephalitis lethargica epidemic. These were precisely the age ranges of persons who were most often affected by the illness. These data suggest the possibility that the coexistence of parkinsonism and SN depigmentation in this birth cohort may have resulted from the yet undetermined infectious agent responsible for encephalitis lethargica. Copyright © 2012 American Neurological Association.

Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report.

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Ju, Weina; Qi, Baochang; Wang, Xu; Yang, Yu

2017-10-01

We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma. A clinical diagnosis of lymphoma and PNS was made. The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days. We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2-associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case.

Managing severe behavioral symptoms of a patient with anti-NMDAR encephalitis: case report and findings in current literature

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Vanina Lima Monteiro

2015-03-01

Full Text Available Objective: Psychiatric symptoms emerge in the early stages of anti-N-methyl-D-aspartate receptor (anti-NMDAR encephalitis, and patients often seek treatment in psychiatric departments before visiting any other general medical services. Numerous articles about anti-NMDAR encephalitis have been published in the scientific community worldwide, but few emphasize the role of psychiatry in symptom management.Case description: We describe the case of a patient with anti- -NMDAR encephalitis seen in our service and discuss the management of behavioral symptoms based on current scientific literature. High doses of atypical antipsychotics and benzodiazepines were used to control agitation, and trazodone was administered to treat insomnia.Comments: Consultation-liaison psychiatry may help the healthcare team adjust the management of neuropsychiatric complications that might affect inpatients with anti-NMDAR encephalitis.

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.

Early pathologic findings and long-term improvement in anti-Ma2-associated encephalitis.

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Blumenthal, D T; Salzman, K L; Digre, K B; Jensen, R L; Dunson, W A; Dalmau, J

2006-07-11

A 67-year-old man sequentially developed anti-Ma2-associated paraneoplastic encephalitis (PNE) and contralateral herpes simplex encephalitis (HSE). Brain biopsy 1 month before HSE revealed extensive infiltrates of T cells, B cells, and plasma cells. Most T cells expressed the cytotoxic granule-associated protein TIA-1 and the membranolytic protein granzyme-B. Although recovery was thought to be unlikely, treatment of the PNE with corticosteroids and resection of the associated lung cancer resulted in dramatic improvement for 21 months.

Nucleoside Inhibitors of Tick-Borne Encephalitis Virus

Czech Academy of Sciences Publication Activity Database

Eyer, L.; Valdés, James J.; Gil, V.A.; Nencka, Radim; Hřebabecký, Hubert; Šála, Michal; Salát, J.; Černý, Jiří; Palus, Martin; De Clercq, E.; Růžek, Daniel

2015-01-01

Roč. 59, č. 9 (2015), s. 5483-5493 ISSN 0066-4804 R&D Projects: GA ČR GAP502/11/2116; GA MŠk(CZ) EE2.3.30.0032 Institutional support: RVO:60077344 ; RVO:61388963 Keywords : tick-borne encephalitis virus * infection * molecular analyses Subject RIV: EE - Microbiology, Virology; CC - Organic Chemistry (UOCHB-X) Impact factor: 4.415, year: 2015

Anesthesia in anti-N-methyl-D-aspartate receptor encephalitis - is general anesthesia a requisite? A case report

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Sook Hui Chaw

Full Text Available Abstract Anti-N-methyl-D-aspartate receptor encephalitis is a recently described neurological disorder and an increasingly recognized cause of psychosis, movement disorders and autonomic dysfunction. We report 20-year-old Chinese female who presented with generalized tonic-clonic seizures, recent memory loss, visual hallucinations and abnormal behavior. Anti-N-methyl-D-aspartate receptor encephalitis was diagnosed and a computed tomography scan of abdomen reviewed a left adnexal tumor. We describe the first such case report of a patient with anti-N-methyl-D-aspartate receptor encephalitis who was given a bilateral transversus abdominis plane block as the sole anesthetic for removal of ovarian tumor. We also discuss the anesthetic issues associated with anti-N-methyl-D-aspartate receptor encephalitis. As discovery of tumor and its removal is the focus of initial treatment in this group of patients, anesthetists will encounter more such cases in the near future.

Pathologically confirmed autoimmune encephalitis in suspected Creutzfeldt-Jakob disease

NARCIS (Netherlands)

Maat, P.; de Beukelaar, J.W.; Jansen, C.; Schuur, M.; van Duijn, C.M.; van Coevorden, M.H.; de Graaff, E.; Titulaer, E.; Rozemuller, A.J.M.; Sillevis Smitt, P.

2015-01-01

Objective: To determine the clinical features and presence in CSF of antineuronal antibodies in patients with pathologically proven autoimmune encephalitis derived from a cohort of patients with suspected Creutzfeldt-Jakob disease (CJD). Methods: The Dutch Surveillance Centre for Prion Diseases

REM Sleep Behavior Disorder and Narcoleptic Features in Anti–Ma2-associated Encephalitis

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Compta, Yaroslau; Iranzo, Alex; Santamaría, Joan; Casamitjana, Roser; Graus, Francesc

2007-01-01

A 69-year-old man with anti-Ma2 paraneoplastic encephalitis presented with subacute onset of severe hypersomnia, memory loss, parkinsonism, and gaze palsy. A brain magnetic resonance imaging study showed bilateral damage in the dorsolateral midbrain, amygdala, and paramedian thalami. Videopolysomnography disclosed rapid eye movement (REM) sleep behavior disorder, and a Multiple Sleep Latency Test showed a mean sleep latency of 7 minutes and 4 sleep-onset REM periods. The level of hypocretin-1 in the cerebrospinal fluid was low (49 pg/mL). This observation illustrates that REM sleep behavior disorder and narcoleptic features are 2 REM-sleep abnormalities that (1) may share the same autoimmune-mediated origin affecting the brainstem, limbic, and diencephalic structures and (2) may occur in the setting of the paraneoplastic anti–Ma2-associated encephalitis. Citation: Compta Y; Iranzo A; Santamaría J et al. REM Sleep Behavior Disorder and Narcoleptic Features in Anti–Ma2-associated Encephalitis. SLEEP 2007;30(6):767-769. PMID:17580598

Changes of MCP-1, FKN, and related cytokines in the serum and cerebrospinal fluid in children with epidemic encephalitis B

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Qian Wang

2017-09-01

Full Text Available Objective: To explore the changes of MCP-1, FKN, and related cytokines in the serum and cerebrospinal fluid (CSF in children with epidemic encephalitis B. Methods: A total of 40 children with epidemic encephalitis B who were admitted in our hospital from June, 2014 to June, 2017 were included in the study and divided into the severe group (n=15 and general group (n=25 according to the severity group. Moreover, 20 children who were suffered from oblique inguinal hernia, perineal adhesion, and cryptorchidism were served as the control group. The serum and CSF specimens were collected 24 h after admission and during the recovery period in children with epidemic encephalitis B. The serum specimen was collected 24 h after admission in the control group, and CSF specimen was collected during the lumbar puncture. ELISA was used to detect CMP-1, FKN, IL-1β, IL-18, and TNF-α levels in the serum and CSF. CMP-1, FKN, IL-1β, IL-18, and TNF-α levels in children with epidemic encephalitis B on the day after admission and 2-3 weeks after admission and in the control group were compared. The changes of CMP-1, FKN, IL-1β, IL-18, and TNF-α in children with severe and general epidemic encephalitis B were observed. Results: CMP-1 and FKN levels in the serum and CSF in children with epidemic encephalitis B in the critical stage were significantly higher than those in the recovery stage and in the control group. The serum CMP-1 and FKN levels in children with epidemic encephalitis B during the recovery stage were not significantly different from those in the control group, while CMP-1 and FKN levels in CSF were significantly higher than those in the control group. CMP-1 and FKN levels in the serum and CSF in children with severe epidemic encephalitis B were significantly higher than those in the general group. IL-1β, TNF-α, and IL-18 levels in the serum and CSF in children with epidemic encephalitis B during the critical stage were significantly higher than

Herpes simplex virus-1 evasion of CD8+ T cell accumulation contributes to viral encephalitis.

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Koyanagi, Naoto; Imai, Takahiko; Shindo, Keiko; Sato, Ayuko; Fujii, Wataru; Ichinohe, Takeshi; Takemura, Naoki; Kakuta, Shigeru; Uematsu, Satoshi; Kiyono, Hiroshi; Maruzuru, Yuhei; Arii, Jun; Kato, Akihisa; Kawaguchi, Yasushi

2017-10-02

Herpes simplex virus-1 (HSV-1) is the most common cause of sporadic viral encephalitis, which can be lethal or result in severe neurological defects even with antiviral therapy. While HSV-1 causes encephalitis in spite of HSV-1-specific humoral and cellular immunity, the mechanism by which HSV-1 evades the immune system in the central nervous system (CNS) remains unknown. Here we describe a strategy by which HSV-1 avoids immune targeting in the CNS. The HSV-1 UL13 kinase promotes evasion of HSV-1-specific CD8+ T cell accumulation in infection sites by downregulating expression of the CD8+ T cell attractant chemokine CXCL9 in the CNS of infected mice, leading to increased HSV-1 mortality due to encephalitis. Direct injection of CXCL9 into the CNS infection site enhanced HSV-1-specific CD8+ T cell accumulation, leading to marked improvements in the survival of infected mice. This previously uncharacterized strategy for HSV-1 evasion of CD8+ T cell accumulation in the CNS has important implications for understanding the pathogenesis and clinical treatment of HSV-1 encephalitis.

Clinical study on antibody-associated limbic encephalitis

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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.

The effect of body size evolution and ecology on encephalization in cave bears and extant relatives.

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Veitschegger, Kristof

2017-06-05

The evolution of larger brain volumes relative to body size in Mammalia is the subject of an extensive amount of research. Early on palaeontologists were interested in the brain of cave bears, Ursus spelaeus, and described its morphology and size. However, until now, it was not possible to compare the absolute or relative brain size in a phylogenetic context due to the lack of an established phylogeny, comparative material, and phylogenetic comparative methods. In recent years, many tools for comparing traits within phylogenies were developed and the phylogenetic position of cave bears was resolved based on nuclear as well as mtDNA. Cave bears exhibit significantly lower encephalization compared to their contemporary relatives and intraspecific brain mass variation remained rather small. Encephalization was correlated with the combined dormancy-diet score. Body size evolution was a main driver in the degree of encephalization in cave bears as it increased in a much higher pace than brain size. In Ursus spelaeus, brain and body size increase over time albeit differently paced. This rate pattern is different in the highest encephalized bear species within the dataset, Ursus malayanus. The brain size in this species increased while body size heavily decreased compared to its ancestral stage. Early on in the evolution of cave bears encephalization decreased making it one of the least encephalized bear species compared to extant and extinct members of Ursidae. The results give reason to suspect that as herbivorous animals, cave bears might have exhibited a physiological buffer strategy to survive the strong seasonality of their environment. Thus, brain size was probably affected by the negative trade-off with adipose tissue as well as diet. The decrease of relative brain size in the herbivorous Ursus spelaeus is the result of a considerable increase in body size possibly in combination with environmental conditions forcing them to rest during winters.

Computed tomography of herpes simplex encephalitis

International Nuclear Information System (INIS)

Rodiek, S.O.; Backmund, H.; Max-Planck-Institut fuer Psychiatrie, Muenchen

1984-01-01

Referring to 9 patients of our own material we report on the pattern of distribution and the development of CT-changes in herpes simplex encephalitis (HSE). Our cases include the outstanding findings of a primarily hemorrhagic HSE and an extensive calcification at the residual stage on the borderline of widespread tissue necrosis on a baby. With respect to literature we receive a quite homogenous picture, reflecting the crucial characteristics of the disease as known from neuropathology. (orig.) [de

Epidemiology of Meningitis and Encephalitis in the United States, 2011-2014.

Science.gov (United States)

Hasbun, Rodrigo; Rosenthal, Ning; Balada-Llasat, J M; Chung, Jessica; Duff, Steve; Bozzette, Samuel; Zimmer, Louise; Ginocchio, Christine C

2017-08-01

Large epidemiological studies evaluating the etiologies, management decisions, and outcomes of adults with meningitis or encephalitis in the United States (US) are lacking. Adult patients (≥18 years) with meningitis or encephalitis by International Classification of Diseases, Ninth Revision codes available in the Premier Healthcare Database during 2011-2014 were analyzed. A total of 26429 patients with meningitis or encephalitis were identified. The median age was 43 years; 53% were female. The most common etiology was enterovirus (13463 [51.6%]), followed by unknown (4944 [21.4%]), bacterial meningitis (3692 [14.1%]), herpes simplex virus (2184 [8.3%]), noninfectious (921 [3.5%]), fungal (720 [2.7%]), arboviruses (291 [1.1%]), and other viruses (214 [0.8%]). Empiric antibiotics, antivirals, and antifungals were administered in 85.8%, 53.4%, and 7.8%, respectively, and varied by etiologies. Adjunctive steroids were utilized in 15.9% of all patients and in 39.3% of patients with pneumococcal meningitis, with an associated decrease in mortality (6.67% vs 12.5%, P = .0245). The median length of stay was 4 days, with the longest duration in those with fungal (13), arboviral (10), and bacterial meningitis (7). Overall inpatient mortality was 2.9% and was higher in those with bacterial (8.2%), fungal (8.2%), or arboviral (8.9%) disease. Overall readmission rate at 30 days was 3.2%; patients with arboviral (12.7%), bacterial (6.7%), and fungal (5.4%) etiologies had higher rates. Viruses are the most common cause of meningitis and encephalitis in the United States and are treated with antibiotic therapy in the majority of cases. Adjunctive steroid treatment is underutilized in pneumococcal meningitis, where it has shown to decrease mortality. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com

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

Infectious meningitis and encephalitis in adults in Denmark

DEFF Research Database (Denmark)

Bodilsen, Jacob; Storgaard, Merete; Larsen, Lykke

2018-01-01

-haemolytic streptococci (n=14). Meningococcal meningitis was rare (n=11). In encephalitis, Herpes simplex virus-1 was most common (n=37) followed by Varicella zoster virus (n=20), while Varicella zoster virus (n=61) was most common in viral meningitis followed by enterovirus (n=50) and Herpes simplex virus-2 (n=46). Case...

Anti-Ma2–associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma

Science.gov (United States)

Ju, Weina; Qi, Baochang; Wang, Xu; Yang, Yu

2017-01-01

Abstract Rationale: We report the rare case of a 74-year-old man with anti-Ma2–associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. Patient concerns: The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma. Diagnosis: A clinical diagnosis of lymphoma and PNS was made. Interventions: The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days. Lessons: We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2–associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case. PMID:28984777

Low serum vitamin D levels and anti-N-methyl-d-aspartate receptor encephalitis: A case-control study.

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Shu, Yaqing; Su, Qingmei; Liao, Siyuan; Lu, Tingting; Li, Rui; Sun, Xiaobo; Qiu, Wei; Yang, Yu; Hu, Xueqiang; Lu, Zhengqi

2017-01-01

Low vitamin D levels are associated with autoimmunity, but the relationship with anti-N-Methyl-d-aspartate receptor (anti-NMDAR) encephalitis is unknown. 25(OH) D levels and clinical and cerebrospinal fluid parameters were evaluated in 30 patients with anti-NMDAR encephalitis and compared with 90 age-, sex-, and season-matched healthy controls. 25(OH)D levels were lower in patients with anti-NMDAR encephalitis compared to controls (43.89 ± 17.91 vs 64.24 ± 24.38 nmol/L, p  30 years, p = 0.002), severe impairment (mRS ≥ 5) (vs mRS D levels were associated with age (r = 0.393, p = 0.032), and mRS (r = -0.417, p = 0.022). Our data showed that serum 25(OH)D levels were reduced in patients with anti-NMDAR encephalitis. Copyright © 2016. Published by Elsevier Ltd.

[Anesthesia in anti-N-methyl-d-aspartate receptor encephalitis - is general anesthesia a requisite? A case report].

Science.gov (United States)

Chaw, Sook Hui; Foo, Li Lian; Chan, Lucy; Wong, Kang Kwong; Abdullah, Suhailah; Lim, Boon Kiong

Anti-N-methyl-d-aspartate receptor encephalitis is a recently described neurological disorder and an increasingly recognized cause of psychosis, movement disorders and autonomic dysfunction. We report 20-year-old Chinese female who presented with generalized tonic-clonic seizures, recent memory loss, visual hallucinations and abnormal behavior. Anti-N-methyl-d-aspartate receptor encephalitis was diagnosed and a computed tomography scan of abdomen reviewed a left adnexal tumor. We describe the first such case report of a patient with anti-N-methyl-d-aspartate receptor encephalitis who was given a bilateral transversus abdominis plane block as the sole anesthetic for removal of ovarian tumor. We also discuss the anesthetic issues associated with anti-N-methyl-d-aspartate receptor encephalitis. As discovery of tumor and its removal is the focus of initial treatment in this group of patients, anesthetists will encounter more such cases in the near future. Copyright © 2015 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Clinical Significance of Human Metapneumovirus in Refractory Status Epilepticus and Encephalitis: Case Report and Review of the Literature

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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.

Full genome sequences and molecular characterization of tick-borne encephalitis virus strains isolated from human patients

Czech Academy of Sciences Publication Activity Database

Formanová, P.; Černý, Jiří; Černá Bolfíková, B.; Valdés, James J.; Kozlová, I.; Dzhioev, Y.; Růžek, Daniel

2015-01-01

Roč. 6, č. 1 (2015), s. 38-46 ISSN 1877-959X R&D Projects: GA ČR GAP502/11/2116; GA ČR GAP302/12/2490 Institutional support: RVO:60077344 Keywords : tick-borne encephalitis virus * tick-borne encephalitis * genome analysis * human patient s Subject RIV: EE - Microbiology, Virology Impact factor: 2.690, year: 2015

Neuronal surface antigen antibodies in limbic encephalitis

Science.gov (United States)

Graus, F; Saiz, A; Lai, M; Bruna, J; López, F; Sabater, L; Blanco, Y; Rey, M J.; Ribalta, T; Dalmau, J

2008-01-01

Objective: To report the frequency and type of antibodies against neuronal surface antigens (NSA-ab) in limbic encephalitis (LE). Methods: Analysis of clinical features, neuropathologic findings, and detection of NSA-ab using immunochemistry on rat tissue and neuronal cultures in a series of 45 patients with paraneoplastic (23) or idiopathic (22) LE. Results: NSA-ab were identified in 29 patients (64%; 12 paraneoplastic, 17 idiopathic). Thirteen patients had voltage-gated potassium channels (VGKC)-ab, 11 novel NSA (nNSA)-ab, and 5 NMDA receptor (NMDAR)-ab. nNSA-ab did not identify a common antigen and were more frequent in paraneoplastic than idiopathic LE (39% vs 9%; p = 0.03). When compared with VGKC-ab or NMDAR-ab, the nNSA associated more frequently with intraneuronal antibodies (11% vs 73%; p = 0.001). Of 12 patients (9 nNSA-ab, 2 VGKC-ab, 1 NMDAR-ab) with paraneoplastic LE and NSA-ab, concomitant intraneuronal antibodies occurred in 9 (75%). None of these 12 patients improved with immunotherapy. The autopsy of three of them showed neuronal loss, microgliosis, and cytotoxic T cell infiltrates in the hippocampus and amygdala. These findings were compatible with a T-cell mediated neuronal damage. In contrast, 13 of 17 (76%) patients with idiopathic LE and NSA-ab (8 VGKC-ab, 4 NMDAR-ab, 1 nNSA-ab) and 1 of 5 (20%) without antibodies had clinical improvement (p = 0.04). Conclusions: In paraneoplastic limbic encephalitis (LE), novel antibodies against neuronal surface antigens (nNSA-ab) occur frequently, coexist with antibodies against intracellular antigens, and these cases are refractory to immunotherapy. In idiopathic LE, the likelihood of improvement is significantly higher in patients with NSA-ab than in those without antibodies. GLOSSARY GAD = glutamic acid decarboxylase; LE = limbic encephalitis; NMDAR = N-methyl-D-aspartate receptor; NSA = neuronal surface antigens; nNSA = novel NSA; SCLC = small-cell lung cancer; VGKC = voltage-gated potassium channels

[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

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.

Chronic Granulomatous Herpes Encephalitis in a Child with Clinically Intractable Epilepsy

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James R. Hackney

2012-01-01

Full Text Available Most patients with herpes simplex virus Type I encephalitis experience an acute, monophasic illness. Chronic encephalitis is much less common, and few late relapses are associated with intractable seizure disorders. A 10-year-old boy was admitted to our institution for intractable epilepsy as part of an evaluation for epilepsy surgery. His history was significant for herpes meningitis at age 4 months. At that time, he presented to an outside hospital with fever for three days, with acyclovir treatment beginning on day 4 of his 40-day hospital course. He later developed infantile spasms and ultimately a mixed seizure disorder. Video electroencephalogram showed a Lennox-Gastaut-type pattern with frequent right frontotemporal spikes. Imaging studies showed an abnormality in the right frontal operculum. Based on these findings, he underwent a right frontal lobectomy. Neuropathology demonstrated chronic granulomatous inflammation with focal necrosis and mineralizations. Scattered lymphocytes, microglial nodules and nonnecrotizing granulomas were present with multinucleated giant cells. Immunohistochemistry for herpes simplex virus showed focal immunoreactivity. After undergoing acyclovir therapy, he returned to baseline with decreased seizure frequency. This rare form of herpes encephalitis has only been reported in children, but the initial presentation of meningitis and the approximate 10-year-time interval in this case are unusual.

Systematic review of rehabilitation intervention outcomes of adult and paediatric patients with infectious encephalitis

Science.gov (United States)

Christie, Shanice; Chan, Vincy; Mollayeva, Tatyana; Colantonio, Angela

2018-01-01

Objective Although a range of rehabilitation interventions have been applied to restore function after infectious encephalitis, there is a lack of literature summarising the benefits of these interventions. This systematic review aims to synthesise current scientific knowledge on outcome measures following rehabilitative interventions among children and adults with infectious encephalitis, with a specific focus on the influence of the age, sex, baseline status and intervention type. Search strategy Five scholarly databases (MEDLINE, Embase, PsycINFO, CINAHL and Cochrane Central Register of Controlled Trials), three sources of grey literature (Google, Google Scholar and Grey Matters) and reference lists of included publications were systematically searched. Literature published before 15 December 2017 and focused on patients with infectious encephalitis in any rehabilitation setting were included. Quality assessment was completed using the Downs and Black rating scale. Results Of the 12 737 reference titles screened, 20 studies were included in this review. All of the studies had sample sizes of less than 25 patients and received a score of less than 15 out of 31 points on the Downs and Black rating scale. Findings showed a variety of interventions has been applied to alleviate sequelae from infectious encephalitis, including using cognitive therapy (nine studies), behavioural therapy (five studies), physical therapy (two studies) or two or more therapies (four studies). There was inconclusive evidence on the effect of sex, age and baseline functional abilities on outcomes. Due to clinical and methodological heterogeneity between studies, meta-analyses were not performed. Conclusion Evidence suggests the potential for a beneficial effect of rehabilitation interventions in patients with infectious encephalitis. Future research is required to identify all effect modifiers and to determine the effect of time in the natural course of recovery. An enhanced set of known

Decreased occipital lobe metabolism by FDG-PET/CT: An anti-NMDA receptor encephalitis biomarker.

Science.gov (United States)

Probasco, John C; Solnes, Lilja; Nalluri, Abhinav; Cohen, Jesse; Jones, Krystyna M; Zan, Elcin; Javadi, Mehrbod S; Venkatesan, Arun

2018-01-01

To compare brain metabolism patterns on fluorodeoxyglucose (FDG)-PET/CT in anti-NMDA receptor and other definite autoimmune encephalitis (AE) and to assess how these patterns differ between anti-NMDA receptor neurologic disability groups. Retrospective review of clinical data and initial dedicated brain FDG-PET/CT studies for neurology inpatients with definite AE, per published consensus criteria, treated at a single academic medical center over a 10-year period. Z-score maps of FDG-PET/CT were made using 3-dimensional stereotactic surface projections in comparison to age group-matched controls. Brain region mean Z scores with magnitudes ≥2.00 were interpreted as significant. Comparisons were made between anti-NMDA receptor and other definite AE patients as well as among patients with anti-NMDA receptor based on modified Rankin Scale (mRS) scores at the time of FDG-PET/CT. The medial occipital lobes were markedly hypometabolic in 6 of 8 patients with anti-NMDA receptor encephalitis and as a group (Z = -4.02, interquartile range [IQR] 2.14) relative to those with definite AE (Z = -2.32, 1.46; p = 0.004). Among patients with anti-NMDA receptor encephalitis, the lateral and medial occipital lobes were markedly hypometabolic for patients with mRS 4-5 (lateral occipital lobe Z = -3.69, IQR 1; medial occipital lobe Z = -4.08, 1) compared with those with mRS 0-3 (lateral occipital lobe Z = -0.83, 2; p occipital lobe Z = -1.07, 2; p = 0.001). Marked medial occipital lobe hypometabolism by dedicated brain FDG-PET/CT may serve as an early biomarker for discriminating anti-NMDA receptor encephalitis from other AE. Resolution of lateral and medial occipital hypometabolism may correlate with improved neurologic status in anti-NMDA receptor encephalitis.

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.

Slowly progressive fluent aphasia

International Nuclear Information System (INIS)

Sakurai, Yasuhisa; Momose, Toshimitsu; Watanabe, Toshiaki; Ishikawa, Takashi; Iwata, Makoto; Bando, Mitsuaki.

1991-01-01

Three patients with slowly progressive fluent aphasia are reported. One of the patients presented with memory disturbance. They were characterized clinically by having selective deficits in vocabulary, which resulted in impairment of confrontation naming, and auditory comprehension. MRI showed an atrophy not only in the left temporal lobe (including the superior, middle and inferior temporal gyri), hippocampus, parahippocampual gyrus, and fusiform gyrus, but also in the left parietal lobe. I-123 IMP SPECT and F-18 FDG PET were used to determine regional cerebral blood flow and regional cerebral metabolic rate, respectively. In addition to the decreased tracer uptake in the left temporal and/or parietal lobe, a decreased uptake was seen in the bilateral basal ganglia, the inner side of the temporal lobe (including the bilateral hippocampus), the right anterior temporal lobe, and the left thalamus. These findings may deny the previous thought that lesions are localized in slowly progressive fluent aphasia. Furthermore, noticeable difficulty in naming, i.e., patients unable to recognize the right answer, are considered attributable to widespread lesions from the whole left temporal lobe, including the hippocampus, to the right temporal lobe. (N.K.)

Limbic Encephalitis in Taiwanese Children and Adolescence: A Single Center Study

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I-Jun Chou

2013-08-01

Conclusion: This study provides evidence for a potential association between antibodies and limbic encephalitis. The presence of antibodies, especially antibodies to GAD, may serve as an indicator for immunotherapy.

Enterovirus 71 encephalomyelitis and Japanese encephalitis can be distinguished by topographic distribution of inflammation and specific intraneuronal detection of viral antigen and RNA.

Science.gov (United States)

Wong, K T; Ng, K Y; Ong, K C; Ng, W F; Shankar, S K; Mahadevan, A; Radotra, B; Su, I J; Lau, G; Ling, A E; Chan, K P; Macorelles, P; Vallet, S; Cardosa, M J; Desai, A; Ravi, V; Nagata, N; Shimizu, H; Takasaki, T

2012-08-01

To investigate if two important epidemic viral encephalitis in children, Enterovirus 71 (EV71) encephalomyelitis and Japanese encephalitis (JE) whose clinical and pathological features may be nonspecific and overlapping, could be distinguished. Tissue sections from the central nervous system of infected cases were examined by light microscopy, immunohistochemistry and in situ hybridization. All 13 cases of EV71 encephalomyelitis collected from Asia and France invariably showed stereotyped distribution of inflammation in the spinal cord, brainstem, hypothalamus, cerebellar dentate nucleus and, to a lesser extent, cerebral cortex and meninges. Anterior pons, corpus striatum, thalamus, temporal lobe, hippocampus and cerebellar cortex were always uninflamed. In contrast, the eight JE cases studied showed inflammation involving most neuronal areas of the central nervous system, including the areas that were uninflamed in EV71 encephalomyelitis. Lesions in both infections were nonspecific, consisting of perivascular and parenchymal infiltration by inflammatory cells, oedematous/necrolytic areas, microglial nodules and neuronophagia. Viral inclusions were absent. Immunohistochemistry and in situ hybridization assays were useful to identify the causative virus, localizing viral antigens and RNA, respectively, almost exclusively to neurones. The stereotyped distribution of inflammatory lesions in EV71 encephalomyelitis appears to be very useful to help distinguish it from JE. © 2011 The Authors. Neuropathology and Applied Neurobiology © 2011 British Neuropathological Society.

Hypersomnia as presenting symptom of anti-Ma2-associated encephalitis: case study.

Science.gov (United States)

Rojas-Marcos, Iñigo; Graus, Francesc; Sanz, Gema; Robledo, Arturo; Diaz-Espejo, Carlos

2007-01-01

We describe a patient who presented with excessive daytime sleepiness (EDS) and was eventually diagnosed with anti-Ma2 encephalitis. Neurological examination disclosed somnolence, left palpebral ptosis, and vertical gaze paresis. A brain MRI showed high signal intensity in the hypothalamus and each hippocampus. Ma2 antibodies were found in the patient's serum, and fiberbronchoscopy disclosed a lung carcinoma. After three months of steroid treatment, the results of the patient's neurological exam became normal. We conclude that anti-Ma2 encephalitis may present with mostly isolated EDS and that it may respond to steroids despite old age and the presence of an untreated lung cancer.

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

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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.

Molecular detection and genotyping of Japanese Encephalitis Virus in mosquitoes during a 2010 outbreak in the Republic of Korea

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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.

Stability and dynamics of spatio-temporal structures. Progress report, September 15, 1993--September 14, 1994

Energy Technology Data Exchange (ETDEWEB)

Riecke, H.

1994-05-01

Goal is to contribute to understanding of localized spatial and spatio-temporal structures far from thermodynamic equilibrium. Here we report on our progress in the study of three classes of systems. (1) We have studied cellular flame structures arising in a circular burner. Using numerical computations we have found a number of traveling-wave structures in which different cells undergo different motion. Most strikingly, we have found a localized wave traveling through the array of steady cells. Results are interpreted using various asymptotic approaches. They are in qualitative agreement with recent experiments. (2) We have continued our investigation of localized waves in binary-mixture convection. Starting from the extended Ginzburg-Landau equations introduced earlier, we have derived equations of motion for interacting fronts connecting the conductive and the convective state. These equations reveal a repulsive interaction between the fronts which implies a new localization mechanism for waves. It is solely due to the long-wavelength mode specific to the extended Ginzburg-Landau equations. The stability properties of the resulting localized waves are in qualitative agreement with very recent experiments. (3) We have extended our investigation of domain structures to include their temporal evolution.

Beyond the temporal pole: limbic memory circuit in the semantic variant of primary progressive aphasia.

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Tan, Rachel H; Wong, Stephanie; Kril, Jillian J; Piguet, Olivier; Hornberger, Michael; Hodges, John R; Halliday, Glenda M

2014-07-01

Despite accruing evidence for relative preservation of episodic memory in the semantic variant of primary progressive aphasia (previously semantic dementia), the neural basis for this remains unclear, particularly in light of their well-established hippocampal involvement. We recently investigated the Papez network of memory structures across pathological subtypes of behavioural variant frontotemporal dementia and demonstrated severe degeneration of all relay nodes, with the anterior thalamus in particular emerging as crucial for intact episodic memory. The present study investigated the status of key components of Papez circuit (hippocampus, mammillary bodies, anterior thalamus, cingulate cortex) and anterior temporal cortex using volumetric and quantitative cell counting methods in pathologically-confirmed cases with semantic variant of primary progressive aphasia (n = 8; 61-83 years; three males), behavioural variant frontotemporal dementia with TDP pathology (n = 9; 53-82 years; six males) and healthy controls (n = 8, 50-86 years; four males). Behavioural variant frontotemporal dementia cases with TDP pathology were selected because of the association between the semantic variant of primary progressive aphasia and TDP pathology. Our findings revealed that the semantic variant of primary progressive aphasia and behavioural variant frontotemporal dementia show similar degrees of anterior thalamic atrophy. The mammillary bodies and hippocampal body and tail were preserved in the semantic variant of primary progressive aphasia but were significantly atrophic in behavioural variant frontotemporal dementia. Importantly, atrophy in the anterior thalamus and mild progressive atrophy in the body of the hippocampus emerged as the main memory circuit regions correlated with increasing dementia severity in the semantic variant of primary progressive aphasia. Quantitation of neuronal populations in the cingulate cortices confirmed the selective loss of anterior cingulate

Validity of the coding for herpes simplex encephalitis in the Danish National Patient Registry

DEFF Research Database (Denmark)

Jørgensen, Laura Krogh; Dalgaard, Lars Skov; Østergaard, Lars Jørgen

2016-01-01

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...... (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 of 75.9% (95% CI: 56.5-89.7), thereby representing the highest PPV. The estimated sensitivity was 95.5%. CONCLUSION: The PPVs of the ICD-10 diagnosis coding for adult HSE in the DNPR were relatively low. Hence...

Detailed immunohistochemical characterization of temporal and spatial progression of Alzheimer's disease-related pathologies in male triple-transgenic mice

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Bowers William J

2008-08-01

Full Text Available Abstract Background Several transgenic animal models genetically predisposed to develop Alzheimer's disease (AD-like pathology have been engineered to facilitate the study of disease pathophysiology and the vetting of potential disease-modifying therapeutics. The triple transgenic mouse model of AD (3xTg-AD harbors three AD-related genetic loci: human PS1M146V, human APPswe, and human tauP301L. These mice develop both amyloid plaques and neurofibrillary tangle-like pathology in a progressive and age-dependent manner, while these pathological hallmarks are predominantly restricted to the hippocampus, amygdala, and the cerebral cortex the main foci of AD neuropathology in humans. This model represents, at present, one of the most advanced preclinical tools available and is being employed ever increasingly in the study of mechanisms underlying AD, yet a detailed regional and temporal assessment of the subtleties of disease-related pathologies has not been reported. Methods and results In this study, we immunohistochemically documented the evolution of AD-related transgene expression, amyloid deposition, tau phosphorylation, astrogliosis, and microglial activation throughout the hippocampus, entorhinal cortex, primary motor cortex, and amygdala over a 26-month period in male 3xTg-AD mice. Intracellular amyloid-beta accumulation is detectable the earliest of AD-related pathologies, followed temporally by phospho-tau, extracellular amyloid-beta, and finally paired helical filament pathology. Pathology appears to be most severe in medial and caudal hippocampus. While astrocytic staining remains relatively constant at all ages and regions assessed, microglial activation appears to progressively increase temporally, especially within the hippocampal formation. Conclusion These data fulfill an unmet need in the ever-widening community of investigators studying 3xTg-AD mice and provide a foundation upon which to design future experiments that seek to

Selective Limbic Blood–Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies

Science.gov (United States)

Tröscher, Anna R.; Klang, Andrea; French, Maria; Quemada-Garrido, Lucía; Kneissl, Sibylle Maria; Bien, Christian G.; Pákozdy, Ákos; Bauer, Jan

2017-01-01

Human leucine-rich glioma-inactivated protein 1 encephalitis (LGI1) is an autoimmune limbic encephalitis in which serum and cerebrospinal fluid contain antibodies targeting LGI1, a protein of the voltage gated potassium channel (VGKC) complex. Recently, we showed that a feline model of limbic encephalitis with LGI1 antibodies, called feline complex partial seizures with orofacial involvement (FEPSO), is highly comparable to human LGI1 encephalitis. In human LGI1 encephalitis, neuropathological investigations are difficult because very little material is available. Taking advantage of this natural animal model to study pathological mechanisms will, therefore, contribute to a better understanding of its human counterpart. Here, we present a brain-wide histopathological analysis of FEPSO. We discovered that blood–brain barrier (BBB) leakage was present not only in all regions of the hippocampus but also in other limbic structures such as the subiculum, amygdale, and piriform lobe. However, in other regions, such as the cerebellum, no leakage was observed. In addition, this brain-region-specific immunoglobulin leakage was associated with the breakdown of endothelial tight junctions. Brain areas affected by BBB dysfunction also revealed immunoglobulin and complement deposition as well as neuronal cell death. These neuropathological findings were supported by magnetic resonance imaging showing signal and volume increase in the amygdala and the piriform lobe. Importantly, we could show that BBB disturbance in LGI1 encephalitis does not depend on T cell infiltrates, which were present brain-wide. This finding points toward another, so far unknown, mechanism of opening the BBB. The limbic predilection sites of immunoglobulin antibody leakage into the brain may explain why most patients with LGI1 antibodies have a limbic phenotype even though LGI1, the target protein, is ubiquitously distributed across the central nervous system. PMID:29093718

Selective Limbic Blood–Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies

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Anna R. Tröscher

2017-10-01

Full Text Available Human leucine-rich glioma-inactivated protein 1 encephalitis (LGI1 is an autoimmune limbic encephalitis in which serum and cerebrospinal fluid contain antibodies targeting LGI1, a protein of the voltage gated potassium channel (VGKC complex. Recently, we showed that a feline model of limbic encephalitis with LGI1 antibodies, called feline complex partial seizures with orofacial involvement (FEPSO, is highly comparable to human LGI1 encephalitis. In human LGI1 encephalitis, neuropathological investigations are difficult because very little material is available. Taking advantage of this natural animal model to study pathological mechanisms will, therefore, contribute to a better understanding of its human counterpart. Here, we present a brain-wide histopathological analysis of FEPSO. We discovered that blood–brain barrier (BBB leakage was present not only in all regions of the hippocampus but also in other limbic structures such as the subiculum, amygdale, and piriform lobe. However, in other regions, such as the cerebellum, no leakage was observed. In addition, this brain-region-specific immunoglobulin leakage was associated with the breakdown of endothelial tight junctions. Brain areas affected by BBB dysfunction also revealed immunoglobulin and complement deposition as well as neuronal cell death. These neuropathological findings were supported by magnetic resonance imaging showing signal and volume increase in the amygdala and the piriform lobe. Importantly, we could show that BBB disturbance in LGI1 encephalitis does not depend on T cell infiltrates, which were present brain-wide. This finding points toward another, so far unknown, mechanism of opening the BBB. The limbic predilection sites of immunoglobulin antibody leakage into the brain may explain why most patients with LGI1 antibodies have a limbic phenotype even though LGI1, the target protein, is ubiquitously distributed across the central nervous system.

Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies.

Science.gov (United States)

Tröscher, Anna R; Klang, Andrea; French, Maria; Quemada-Garrido, Lucía; Kneissl, Sibylle Maria; Bien, Christian G; Pákozdy, Ákos; Bauer, Jan

2017-01-01

Human leucine-rich glioma-inactivated protein 1 encephalitis (LGI1) is an autoimmune limbic encephalitis in which serum and cerebrospinal fluid contain antibodies targeting LGI1, a protein of the voltage gated potassium channel (VGKC) complex. Recently, we showed that a feline model of limbic encephalitis with LGI1 antibodies, called feline complex partial seizures with orofacial involvement (FEPSO), is highly comparable to human LGI1 encephalitis. In human LGI1 encephalitis, neuropathological investigations are difficult because very little material is available. Taking advantage of this natural animal model to study pathological mechanisms will, therefore, contribute to a better understanding of its human counterpart. Here, we present a brain-wide histopathological analysis of FEPSO. We discovered that blood-brain barrier (BBB) leakage was present not only in all regions of the hippocampus but also in other limbic structures such as the subiculum, amygdale, and piriform lobe. However, in other regions, such as the cerebellum, no leakage was observed. In addition, this brain-region-specific immunoglobulin leakage was associated with the breakdown of endothelial tight junctions. Brain areas affected by BBB dysfunction also revealed immunoglobulin and complement deposition as well as neuronal cell death. These neuropathological findings were supported by magnetic resonance imaging showing signal and volume increase in the amygdala and the piriform lobe. Importantly, we could show that BBB disturbance in LGI1 encephalitis does not depend on T cell infiltrates, which were present brain-wide. This finding points toward another, so far unknown, mechanism of opening the BBB. The limbic predilection sites of immunoglobulin antibody leakage into the brain may explain why most patients with LGI1 antibodies have a limbic phenotype even though LGI1, the target protein, is ubiquitously distributed across the central nervous system.

Adolescent with acute psychosis due to anti-N-methyl-D-aspartate receptor encephalitis: successful recovery

OpenAIRE

Jonuskaite, Dovile; Kalibatas, Paulius; Praninskiene, Ruta; Zalubiene, Asta; Jucaite, Aurelija; Cerkauskiene, Rimante

2017-01-01

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a relatively new autoimmune disorder of the central nervous system. We report the first case of anti-NMDAR autoimmune encephalitis combined with anti-voltage-gated potassium channel (anti-VGKC) antibodies in Lithuania in a 16-year-old girl. The patient was admitted to psychiatry unit because of an acute psychotic episode. She was unsuccessfully treated with antipsychotics, and electroconvulsive therapy was initiated because of he...

Measurement of renal blood flow by 131I-labelled sodium iodohippurate imaging in a rat model of herpes encephalitis

International Nuclear Information System (INIS)

Cleator, G.M.; Klapper, P.E.; Lewis, A.G.; Sharma, H.L.; Smith, A.M.; Manchester Univ.

1990-01-01

Renal blood flow was assessed qualitatively over a 30 min period in a rat model of herpes encephalitis by extra-renal scintigraphic imaging following the injection of 131 I-labelled sodium iodohippurate. No significant differences were observed for renal blood flow in either kidney between (or within) infected and control groups. Our data suggest that kidney function is not compromised in this animal model of encephalitis. The results are discussed in the context of the development of a non-invasive protocol for the in vivo diagnosis of herpes encephalitis. (orig.)

Optimization and Evaluation of a PCR Assay for Detecting Toxoplasmic Encephalitis in Patients with AIDS

Science.gov (United States)

Joseph, Priya; Calderón, Maritza M.; Gilman, Robert H.; Quispe, Monica L.; Cok, Jaime; Ticona, Eduardo; Chavez, Victor; Jimenez, Juan A.; Chang, Maria C.; Lopez, Martín J.; Evans, Carlton A.

2002-01-01

Toxoplasma gondii is a common life-threatening opportunistic infection. We used experimental murine T. gondii infection to optimize the PCR for diagnostic use, define its sensitivity, and characterize the time course and tissue distribution of experimental toxoplasmosis. PCR conditions were adjusted until the assay reliably detected quantities of DNA derived from less than a single parasite. Forty-two mice were inoculated intraperitoneally with T. gondii tachyzoites and sacrificed from 6 to 72 h later. Examination of tissues with PCR and histology revealed progression of infection from blood to lung, heart, liver, and brain, with PCR consistently detecting parasites earlier than microscopy and with no false-positive results. We then evaluated the diagnostic value of this PCR assay in human patients. We studied cerebrospinal fluid and serum samples from 12 patients with AIDS and confirmed toxoplasmic encephalitis (defined as positive mouse inoculation and/or all of the Centers for Disease Control clinical diagnostic criteria), 12 human immunodeficiency virus-infected patients with suspected cerebral toxoplasmosis who had neither CDC diagnostic criteria nor positive mouse inoculation, 26 human immunodeficiency virus-infected patients with other opportunistic infections and no signs of cerebral toxoplasmosis, and 18 immunocompetent patients with neurocysticercosis. Eleven of the 12 patients with confirmed toxoplasmosis had positive PCR results in either blood or cerebrospinal fluid samples (6 of 9 blood samples and 8 of 12 cerebrospinal fluid samples). All samples from control patients were negative. This study demonstrates the high sensitivity, specificity, and clinical utility of PCR in the diagnosis of toxoplasmic encephalitis in a resource-poor setting. PMID:12454142

Reducing Vulnerability to the Threat of Japanese Encephalitis in ...

International Development Research Centre (IDRC) Digital Library (Canada)

2016-04-25

Apr 25, 2016 ... The Japanese encephalitis (JE) virus results in between 30,000 to 50,000 reported ... members and health officials to promote sustainable solutions. ... little or no education and have a limited understanding of the disease. ... Tourism is an important driver of economic growth throughout Southeast Asia.

Dual Pathology in Rasmussen's Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature.

Science.gov (United States)

Prayson, Richard A

2012-01-01

Rasmussen's encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology). This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diagnosis of coexistent Rasmussen's encephalitis, marked by chronic inflammation, microglial nodules, and focal cortical atrophy, combined with focal cortical dysplasia (Palmini et al. type IIA, ILAE type IIA). The literature on dual pathology in the setting of Rasmussen's encephalitis is reviewed.

Anti-Ma2 paraneoplastic encephalitis associated with testicular germ cell tumor treated by carboplatin, etoposide and bleomycin.

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Kimura, Masaki; Onozawa, Mizuki; Fujisaki, Akira; Arakawa, Takashi; Takeda, Katsuhiko; Dalmau, Joseph; Hattori, Kazunori

2008-10-01

Anti-Ma2-associated encephalitis is a paraneoplastic disorder that predominantly affects the limbic system, diencephalon and brainstem, and is usually associated with tumors of the testis. We report a 35-year-old man with a right testicular mass who presented with multiple neurological complains, and clinical, serological and radiological features compatible with anti-Ma2-associated encephalitis. After three courses of carboplatin, etoposide and bleomycin for metastatic testicular germ-cell tumor, all elevated tumor markers normalized and the retroperitoneal metastases disappeared, but the neurological disorder deteriorated. To our knowledge, this is the first case in which orchiectomy followed by carboplatin, etoposide and bleomycin for a testicular tumor with anti-Ma2 encephalitis was performed.

Tick-borne encephalitis virus infects human brain microvascular endothelial cells without compromising blood-brain barrier integrity

Czech Academy of Sciences Publication Activity Database

Palus, Martin; Vancová, Marie; Širmarová, J.; Elsterová, Jana; Perner, Jan; Růžek, Daniel

2017-01-01

Roč. 507, JUL (2017), s. 110-122 ISSN 0042-6822 R&D Projects: GA MZd(CZ) NV16-34238A; GA MŠk(CZ) LM2015062; GA TA ČR(CZ) TE01020118 Institutional support: RVO:60077344 Keywords : tick-borne encephalitis * tick-borne encephalitis virus * blood- brain barrier * neuroinfection Subject RIV: EE - Microbiology, Virology OBOR OECD: Virology Impact factor: 3.353, year: 2016

Tick-borne encephalitis.

Science.gov (United States)

Dumpis, U; Crook, D; Oksi, J

1999-04-01

Tick-borne encephalitis (TBE) is a zoonotic arbovirus infection endemic to Russia and Eastern and Central Europe. Despite being a common and serious life-threatening disease for which a mass vaccination program was implemented in Austria, there is only limited reference to this disease in the English-language literature. TBE is transmitted to humans usually by the bite of a tick (either Ixodes persulcatus or Ixodes ricinus); occasionally, cases occur following consumption of infected unpasteurized milk. Transmission is seasonal and occurs in spring and summer, particularly in rural areas favored by the vector. TBE is a serious cause of acute central nervous system disease, which may result in death or long-term neurological sequelae. Effective vaccines are available in a few countries. The risk for travelers of acquiring TBE is increasing with the recent rise in tourism to areas of endemicity during spring and summer.

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...

JST Thesaurus Headwords and Synonyms: Japanese encephalitis virus [MeCab user dictionary for science technology term[Archive

Lifescience Database Archive (English)

Full Text Available MeCab user dictionary for science technology term Japanese encephalitis virus 名詞 一般... * * * * 日本脳炎ウイルス ニホンノウエンウイルス ニホンノーエンウイルス Thesaurus2015 200906080846001441 C LS07 UNKNOWN_2 Japanese encephalitis virus

Prevalence of antibodies to tickborne encephalitis and West Nile flaviviruses and the clinical signs of tickborne encephalitis in dogs in the Czech Republic

Czech Academy of Sciences Publication Activity Database

Klimeš, J.; Juřicová, Zina; Literák, I.; Schánilec, P.; Trachta e Silva, E.

2001-01-01

Roč. 148, č. 1 (2001), s. 17-20 ISSN 0042-4900 Institutional research plan: CEZ:AV0Z6093917 Keywords : tick-borne encephalitis Subject RIV: FN - Epidemiology, Contagious Diseases ; Clinical Immunology Impact factor: 1.145, year: 2001 http://veterinaryrecord.bvapublications.com/cgi/content/abstract/148/1/17

Should there be a standardised approach to the diagnostic workup of suspected adult encephalitis? a case series from Australia

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Williams David

2010-12-01

Full Text Available Abstract Background The clinical diagnosis of encephalitis is often difficult and identification of a causative organism is infrequent. The encephalitis syndrome may herald the emergence of novel pathogens with outbreak potential. Individual treatment and an effective public health response rely on identifying a specific pathogen. In Australia there have been no studies to try to improve the identification rate of encephalitis pathogens. This study aims to review the diagnostic assessment of adult suspected encephalitis cases. Methods A retrospective clinical audit was performed, of all adult encephalitis presentations between July 1998 and December 2007 to the three hospitals with adult neurological services in the Hunter New England area, northern New South Wales, Australia. Case notes were examined for evidence of relevant history taking, clinical features, physical examination, laboratory and neuroradiology investigations, and outcomes. Results A total of 74 cases were included in the case series. Amongst suspected encephalitis cases, presenting symptoms and signs included fever (77.0%, headache (62.1%, altered consciousness (63.5%, lethargy (32.4%, seizures (25.7%, focal neurological deficits (31.1% and photophobia (17.6%. The most common diagnostic laboratory test performed was cerebrospinal fluid (CSF analysis (n = 67, 91%. Herpes virus polymerase chain reaction (n = 53, 71.6% and cryptococcal antigen (n = 46, 62.2% were the antigenic tests most regularly performed on CSF. Neuroradiological procedures employed were computerized tomographic brain scanning (n = 68, 91.9% and magnetic resonance imaging of the brain (n = 35, 47.3%. Thirty-five patients (47.3% had electroencephalograms. The treating clinicians suspected a specific causative organism in 14/74 cases (18.9%, of which nine (12.1% were confirmed by laboratory testing. Conclusions The diagnostic assessment of patients with suspected encephalitis was not standardised. Appropriate

Granulomatous herpes simplex encephalitis in an infant with multicystic encephalopathy: a distinct clinicopathologic entity?

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Schutz, Peter W; Fauth, Clarissa T; Al-Rawahi, Ghada N; Pugash, Denise; White, Valerie A; Stockler, Sylvia; Dunham, Christopher P

2014-04-01

Herpes simplex virus encephalitis can manifest as a range of clinical presentations including classic adult, neonatal, and biphasic chronic-granulomatous herpes encephalitis. We report an infant with granulomatous herpes simplex virus type 2 encephalitis with a subacute course and multicystic encephalopathy. A 2-month-old girl presented with lethargy and hypothermia. Computed tomography scan of the head showed multicystic encephalopathy and calcifications. Cerebrospinal fluid analysis by polymerase chain reaction testing for herpes simplex virus 1 and 2, enterovirus, and cytomegalovirus was negative. Normal cerebrospinal fluid interferon-α levels argued against Aicardi-Goutières syndrome. The patient died 2 weeks after presentation. At autopsy, multicystic encephalopathy was confirmed with bilateral gliosis, granulomatous inflammation with multinucleated giant cells, and calcifications. Bilateral healing necrotizing retinitis suggested a viral etiology, but retina and brain were free of viral inclusions and immunohistochemically negative for herpes simplex virus-2 and cytomegalovirus. However, polymerase chain reaction analysis showed herpes simplex virus-2 DNA in four cerebral paraffin blocks. Subsequent repeat testing of the initial cerebrospinal fluid sample using a different polymerase chain reaction assay was weakly positive for herpes simplex virus-2 DNA. Granulomatous herpes simplex virus encephalitis in infants can present with subacute course and result in multicystic encephalopathy with mineralization and minimal cerebrospinal fluid herpes simplex virus DNA load. Infectious etiologies should be carefully investigated in the differential diagnosis of multicystic encephalopathy with mineralization, in particular if multinucleated giant cells are present. Copyright © 2014 Elsevier Inc. All rights reserved.

Brainstem encephalitis and acute polyneuropathy associated with hepatitis E infection.

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Salim, Omar Jabbar; Davidson, Amy; Li, Kathy; Leach, John Paul; Heath, Craig

2017-09-11

A 59-year-old man presented with feverish illness. His Glasgow Coma Scale was 15, had reduced visual acuity in the left eye with partial left ptosis and mild left hemiparesis with an extensor left plantar. Over 48 hours, he accrued multiple cranial nerves palsies and progressed to a flaccid paralysis necessitating admission to an intensive care unit.Cerebrospinal fluid (CSF) study showed 20 lymphocytes and raised protein. Viral and bacterial PCRs were negative. Samples for Lyme, blood-borne viruses, syphilis and autoantibodies were also negative. MRI brain showed T2 abnormalities within the brainstem. Nerve conduction studies revealed an acute motor and sensory axonal neuropathy pattern of Guillian Barre Syndrome (GBS). The patient was treated for both infective and inflammatory causes of brainstem encephalitis and GBS.Retrospective studies confirmed the presence of hepatitis E virus (HEV) RNA in CSF and serum studies showed positive HEV IgG and IgM prior to intravenous infusion. After 3 months of intensive rehabilitation, the patient was discharged home walking with a frame. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Young woman with a four-year history of epilepsy and progressive focal cortical atrophy — What is the diagnosis?

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S. Pati

2014-01-01

Full Text Available The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood. We report the case of a young woman with a four-year history of epilepsy that progressed rapidly as evidenced by the development of progressive focal cortical atrophy. She underwent biopsy that showed perinatal ischemia and a prominent inflammatory response, including T-cell infiltration and microglial activation. There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke was considered. The possible role of inflammation in the progression of epilepsy caused by a “static” lesion (perinatal stroke is discussed.

Concomitant fractional anisotropy and volumetric abnormalities in temporal lobe epilepsy: cross-sectional evidence for progressive neurologic injury.

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Simon S Keller

Full Text Available BACKGROUND: In patients with temporal lobe epilepsy and associated hippocampal sclerosis (TLEhs there are brain abnormalities extending beyond the presumed epileptogenic zone as revealed separately in conventional magnetic resonance imaging (MRI and MR diffusion tensor imaging (DTI studies. However, little is known about the relation between macroscopic atrophy (revealed by volumetric MRI and microstructural degeneration (inferred by DTI. METHODOLOGY/PRINCIPAL FINDINGS: For 62 patients with unilateral TLEhs and 68 healthy controls, we determined volumes and mean fractional anisotropy (FA of ipsilateral and contralateral brain structures from T1-weighted and DTI data, respectively. We report significant volume atrophy and FA alterations of temporal lobe, subcortical and callosal regions, which were more diffuse and bilateral in patients with left TLEhs relative to right TLEhs. We observed significant relationships between volume loss and mean FA, particularly of the thalamus and putamen bilaterally. When corrected for age, duration of epilepsy was significantly correlated with FA loss of an anatomically plausible route - including ipsilateral parahippocampal gyrus and temporal lobe white matter, the thalamus bilaterally, and posterior regions of the corpus callosum that contain temporal lobe fibres - that may be suggestive of progressive brain degeneration in response to recurrent seizures. CONCLUSIONS/SIGNIFICANCE: Chronic TLEhs is associated with interrelated DTI-derived and volume-derived brain degenerative abnormalities that are influenced by the duration of the disorder and the side of seizure onset. This work confirms previously contradictory findings by employing multi-modal imaging techniques in parallel in a large sample of patients.

Tick-borne encephalitis (TBE): an underestimated risk…still: report of the 14th annual meeting of the International Scientific Working Group on Tick-Borne Encephalitis (ISW-TBE).

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Kunze, Ursula

2012-06-01

Today, the risk of getting tick-borne encephalitis (TBE) is still underestimated in many parts of Europe and worldwide. Therefore, the 14th meeting of the International Scientific Working Group on Tick-Borne Encephalitis (ISW-TBE) - a group of neurologists, general practitioners, clinicians, travel physicians, virologists, pediatricians, and epidemiologists - was held under the title "Tick-borne encephalitis: an underestimated risk…still". Among the discussed issues were: TBE, an underestimated risk in children, a case report in two Dutch travelers, the very emotional report of a tick victim, an overview of the epidemiological situation, investigations to detect new TBE cases in Italy, TBE virus (TBEV) strains circulation in Northern Europe, TBE Program of the European Centre for Disease Prevention and Control (ECDC), efforts to increase the TBE vaccination rate in the Czech Republic, positioning statement of the World Health Organization (WHO), and TBE in dogs. To answer the question raised above: Yes, the risk of getting TBE is underestimated in children and adults, because awareness is still too low. It is still underestimated in several areas of Europe, where, for a lack of human cases, TBEV is thought to be absent. It is underestimated in travelers, because they still do not know enough about the risk, and diagnostic awareness in non-endemic countries is still low. Copyright © 2012. Published by Elsevier GmbH. All rights reserved.

Human parechovirus causes encephalitis with white matter injury in Neonates

NARCIS (Netherlands)

Verboon-Maciolek, Malgorzata A.; Groenendaal, Floris; Hahn, Cecil D.; Hellmann, Jonathan; van Loon, Anton M.; Boivin, Guy; de Vries, Linda S.

Objective: To assess the role of human parechoviruses (HPeVs) as a cause of neonatal cerebral infection and to report neuroimaging findings of newborn infants with encephalitis caused by HPeVs. Methods: Clinical presentation, cranial ultrasonography, magnetic resonance imaging (MRI) findings, and

Paraneoplastic limbic encephalitis and possible narcolepsy in a patient with testicular cancer: case study.

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Landolfi, Joseph C.; Nadkarni, Mangala

2003-01-01

We describe a patient who presented with a clinical syndrome of limbic encephalitis, narcolepsy, and cataplexy. The anti-Ma2 antibody was positive. Although there was no mass on imaging, orchiectomy was performed in this patient, and testicular carcinoma was found. This is the first known case of limbic encephalitis and anti-Ma2 antibody to be associated with cataplexy and possible narcolepsy. Neurological symptoms precede the diagnosis of cancer in 50% of patients with paraneoplastic syndromes, and clinicians are therefore strongly advised to evaluate patients with neurological symptoms for this condition. PMID:12816728

Extreme delta brush evolving into status epilepticus in a patient with anti-NMDA encephalitis

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Aline Herlopian, MD

2017-01-01

Full Text Available Extreme delta brush (EDB is an EEG pattern unique to anti-NMDA encephalitis. It is correlated with seizures and status epilepticus in patients who have a prolonged course of illness. The etiology of the underlying association between EDB and seizures is not understood. We present a patient with anti-NMDA encephalitis who developed status epilepticus evolving from the high frequency activity of the extreme delta brush. This case demonstrates that EDB is not only a marker for a greater propensity for seizures but also directly implicated in seizure generation.

Four cases of gabab receptor encephalitis

DEFF Research Database (Denmark)

Szots, Monika; Blaabjerg, M.; Kondziella, D

2016-01-01

loss of short-term memory, but no epilepsy. Without immunotherapy, his memory spontaneously improved up to mild cognitive impairment in six weeks. GABAbR antibodies persisted in his serum, and 18 months later, FDG-PET detected abnormal mediastinal lymph nodes and small cell lung cancer (SCLC). Another...... in three patients. Only the patient, who spontaneously improved, survived for more than 24 months. In summary, our cases show that (i) GABAbR encephalitis may develop without epilepsy; (ii) the severe short-term memory loss can spontaneously improve; (iii) persistent hyponatremia can be present...

Ion channels in EEG: isolating channel dysfunction in NMDA receptor antibody encephalitis.

Science.gov (United States)

Symmonds, Mkael; Moran, Catherine H; Leite, M Isabel; Buckley, Camilla; Irani, Sarosh R; Stephan, Klaas Enno; Friston, Karl J; Moran, Rosalyn J

2018-04-30

Neurological and psychiatric practice frequently lack diagnostic probes that can assess mechanisms of neuronal communication non-invasively in humans. In N-methyl-d-aspartate (NMDA) receptor antibody encephalitis, functional molecular assays are particularly important given the presence of NMDA antibodies in healthy populations, the multifarious symptomology and the lack of radiological signs. Recent advances in biophysical modelling techniques suggest that inferring cellular-level properties of neural circuits from macroscopic measures of brain activity is possible. Here, we estimated receptor function from EEG in patients with NMDA receptor antibody encephalitis (n = 29) as well as from encephalopathic and neurological patient controls (n = 36). We show that the autoimmune patients exhibit distinct fronto-parietal network changes from which ion channel estimates can be obtained using a microcircuit model. Specifically, a dynamic causal model of EEG data applied to spontaneous brain responses identifies a selective deficit in signalling at NMDA receptors in patients with NMDA receptor antibody encephalitis but not at other ionotropic receptors. Moreover, though these changes are observed across brain regions, these effects predominate at the NMDA receptors of excitatory neurons rather than at inhibitory interneurons. Given that EEG is a ubiquitously available clinical method, our findings suggest a unique re-purposing of EEG data as an assay of brain network dysfunction at the molecular level.

Group A Rotavirus Associated with Encephalitis in Red Fox.

Science.gov (United States)

Busi, Chiara; Martella, Vito; Papetti, Alice; Sabelli, Cristiano; Lelli, Davide; Alborali, G Loris; Gibelli, Lucia; Gelmetti, Daniela; Lavazza, Antonio; Cordioli, Paolo; Boniotti, M Beatrice

2017-09-01

In 2011, a group A rotavirus was isolated from the brain of a fox with encephalitis and neurologic signs, detected by rabies surveillance in Italy. Intracerebral inoculation of fox brain homogenates into mice was fatal. Genome sequencing revealed a heterologous rotavirus of avian origin, which could provide a model for investigating rotavirus neurovirulence.

Delays in initiation of acyclovir therapy in herpes simplex encephalitis.

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Hughes, Peter S; Jackson, Alan C

2012-09-01

Diagnosis of herpes simplex encephalitis (HSE) is based on clinical findings, MRI, and detection of herpes simplex virus (HSV) DNA in cerebrospinal fluid (CSF) using polymerase chain reaction amplification. Delays in starting treatment are associated with poorer clinical outcomes. We assessed the timing of initiation of acyclovir therapy in HSE. Inpatient databases from seven hospitals in Winnipeg, Manitoba were used to identify individuals diagnosed with encephalitis and HSE from 2004 to 2009. The time taken to initiate therapy with acyclovir and the reasons for delays were determined. Seventy-seven patients were identified; 69 (90%) received acyclovir; in the others a non-HSV infection was strongly suspected. Thirteen patients were subsequently confirmed to have HSE. Acyclovir was initiated a median of 21 hours (3-407) after presentation in encephalitis cases, and a median of 11 hours (3-118) in HSE. The most common reason for delay was a failure to consider HSE in the differential diagnosis, despite suggestive clinical features. Where therapy was delayed in HSE patients, the decision to begin acyclovir was prompted by transfer of the patient to a different service (55%), recommendations by consultants (18%), imaging results (18%), and CSF pleocytosis (9%). Delays in initiating acyclovir for HSE are common, and are most often due to a failure to consider HSE in a timely fashion on presentation. In order to improve patient outcomes, physicians should be more vigilant for HSE, and begin acyclovir therapy expeditiously on the basis of clinical suspicion rather than waiting for confirmatory tests.

Autoimmune encephalitis associated with voltage-gated potassium channels-complex and leucine-rich glioma-inactivated 1 antibodies - a national cohort study

DEFF Research Database (Denmark)

Celicanin, M; Blaabjerg, Morten; Maersk-Moller, C

2017-01-01

BACKGROUND AND PURPOSE: The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2...... antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016. METHODS: We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment. All magnetic resonance imaging......-Barré syndrome, Creutzfeldt-Jakob disease, neuromyotonia and anti-N-methyl-D-aspartate receptor encephalitis. Magnetic resonance imaging abnormalities were demonstrated in 69% of the LGI1-positive patients. Two patients with normal magnetic resonance imaging demonstrated temporal lobe hypermetabolism using (18...

[Venezuelan equine encephalitis. 1995 outbreak: clinical profile of the case with neurologic involvement].

Science.gov (United States)

Molina, O M; Morales, M C; Soto, I D; Peña, J A; Haack, R S; Cardozo, D P; Cardozo, J J

Venezuelan equine encephalitis virus has caused periodic epidemics and epizootics in the American continent since the 1920s. Such events have been profusely documented from the epidemiologic point of view, however, reports concerning the clinical features of this disease are rather scarce. To analyze the clinical characteristics evidenced by Venezuelan equine encephalitis patients from Zulia state (western Venezuela) studied during the outbreak that occurred in Colombia and Venezuela in 1995. These cases, classified as complicated, were hospitalized at the Hospital Universitario de Maracaibo, state of Zulia, Venezuela. The clinical charts of 313 Venezuelan equine encephalitis patients hospitalized during the period January 1st 1995-March 31st 1996 were reviewed. These cases accounted for 2.82% of 11,072 patients that were medically assisted during the outbreak. The following variables were analyzed: age, gender, signs and symptoms, contact history, complications and evolution. Intracranial hypertension signs became eloquent in 55.9% of these patients. Neurologic complications were represented by two cases of cerebellitis, two cases of meningoencephalitis and one case of encephalomyelitis. The mortality rate was 1.7%. Our results corroborate the benign evolutionary profile that is typical of this entity.

Western equine encephalitis with rapid onset of parkinsonism.

Science.gov (United States)

Schultz, D R; Barthal, J S; Garrett, G

1977-11-01

A patient with confirmed western equine encephalitis had the rapid onset of postencephalitic parkinsonian sequelae. This observation corroborates similar previous but rare reports. Response to therapy with levodopa, dopa decarboxylase inhibitor, and trihexyphenidyl was dramatic. However, remission maintained for 12 months without medication suggests that the parkinsonism would have remitted spontaneously. In either case, this has not previously been reported with the western equine togavirus.

Anti-N-methyl-D-aspartate receptor encephalitis with serum anti-thyroid antibodies and IgM antibodies against Epstein-Barr virus viral capsid antigen: a case report and one year follow-up

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Xu Chun-Ling

2011-11-01

Full Text Available Abstract Background Anti-N-methyl-D-aspartate receptor encephalitis is an increasingly common autoimmune disorder mediated by antibodies to certain subunit of the N-methyl-D-aspartate receptor. Recent literatures have described anti-thyroid and infectious serology in this encephalitis but without follow-up. Case presentation A 17-year-old Chinese female patient presented with psychiatric symptoms, memory deficits, behavioral problems and seizures. She then progressed through unresponsiveness, dyskinesias, autonomic instability and central hypoventilation during treatment. Her conventional blood work on admission showed high titers of IgG antibodies to thyroglobulin, thyroid peroxidase and IgM antibodies to Epstein-Barr virus viral capsid antigen. An immature ovarian teratoma was found and removal of the tumor resulted in a full recovery. The final diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was made by the identification of anti-N-methyl-D-aspartate receptor antibodies in her cerebral spinal fluid. Pathology studies of the teratoma revealed N-methyl-D-aspartate receptor subunit 1 positive ectopic immature nervous tissue and Epstein-Barr virus latent infection. She was discharged with symptoms free, but titers of anti-thyroid peroxidase and anti-thyroglobulin antibodies remained elevated. One year after discharge, her serum remained positive for anti-thyroid peroxidase and anti-N-methyl-D-aspartate receptor antibodies, but negative for anti-thyroglobulin antibodies and IgM against Epstein-Barr virus viral capsid antigen. Conclusions Persistent high titers of anti-thyroid peroxidase antibodies from admission to discharge and until one year later in this patient may suggest a propensity to autoimmunity in anti- N-methyl-D-aspartate receptor encephalitis and support the idea that neuronal and thyroid autoimmunities represent a pathogenic spectrum. Enduring anti-N-methyl-D-aspartate receptor antibodies from admission to one year

Encephalitis treatment – a case report with long-term follow-up of EBV PCR in cerebrospinal fluid

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Zarlasht F

2017-10-01

Full Text Available Fnu Zarlasht,1 Mashal Salehi,2 Mohammad Abu-Hishmeh,3 Muzammil Khan2 1Department of Medicine, Lourdes Hospital, Binghamton, NY, USA, 2Department of Medicine, NYC Health + Hospital/Harlem, Columbia University, NY, USA, 3Department of Medicine, Lincoln Medical and Mental Health Centre, Bronx NY, USA Background: Epstein–Barr virus (EBV has been found to cause infectious mononucleosis multiple times, but has been associated rarely with EBV encephalitis. Also, whenever it is diagnosed, it is always treated symptomatically.Case report: A case of confirmed EBV encephalitis is presented, which was treated with antiviral therapy resulting in complete clearance of the virus in cerebrospinal fluid and minimal neurologic symptoms after hospital discharge.Conclusion: The Infectious Diseases Society of America guidelines state that intravenous acyclovir is not recommended for EBV-related encephalitis. But we reviewed the literature and found similar cases, and we believe that antiviral therapy should be recommended for EBV encephalitis because it is a potentially fatal disease and if left untreated, can lead to raised intracranial pressure, craniotomy and even death. Keywords: Epstein–Barr virus, intravenous, human immune deficiency virus, HIV

Scrub Typhus Leading to Acute Encephalitis Syndrome, Assam, India.

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Khan, Siraj A; Bora, Trishna; Laskar, Basanta; Khan, Abdul M; Dutta, Prafulla

2017-01-01

To determine the contribution of Orientia tsutsugamushi, the agent of scrub typhus, as a cause of acute encephalitis syndrome (AES) in Assam, India, we conducted a retrospective study of hospital patients with symptoms of AES during 2013-2015. Our findings suggest that O. tsutsugamushi infection leads to AES and the resulting illness and death.

Microscopic dysgerminoma associated with anti-Ma2 paraneoplastic encephalitis in a patient with gonadal dysgenesis.

Science.gov (United States)

Abdulkader, Marwah M; Yousef, Mohammad M; Abdelhadi, Mohamad K; Amr, Samir S; Alabsi, Eyad S; Al-Abbadi, Mousa A

2013-05-01

We present a 27-yr-old female with gonadal dysgenesis (46, XY), who presented to our hospital with poor consciousness, aphasia, restlessness, and visual hallucination. Physical examination revealed normal breast development and normal external female genetalia. Computed tomography scan of the head and neck revealed the presence of brain edema, hydrocephalous, and a localized hypodense lesion in the hypothalamus. Her serum was positive for the anti-Ma2, which is associated with paraneoplastic encephalitis syndrome. Computed tomography of the abdomen revealed the presence of a 7.5×5.3×3.0 cm solid pelvic mass. Interestingly, a single microscopic focus of dysgerminoma was identified in a background of stromal fibrosis and focal dystrophic calcifications. No ovarian stroma or testicular tissue was identified. To our knowledge, this is the first case of gonadal dysgenesis presenting with anti-Ma2 paraneoplastic encephalitis with dysgerminoma. A discussion about paraneoplastic encephalitis with a microscopic dysgerminoma associated with anti-Ma2 antibody is presented.

Paraneoplastic Encephalitis Associated with Anti-Ma2 Antibodies and Mesothelioma-Like Poorly Differentiated Lung Cancer

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Can Ebru Bekircan

2009-06-01

Full Text Available We report a case of paraneoplastic encephalitis associated with anti-Ma2 antibodies. Medical history and thorax computed tomog- raphy findings suggested malignant mesothelioma. Pleural biopsy results were compatible with high-grade neoplastic infiltration. Alt- hough the biopsy could not differentiate the type of neoplasm, mesothelioma was considered a strong possibility in this poorly dif- ferentiated lung carcinoma. To the best of our knowledge this is the first case report of paraneoplastic encephalitis associated with anti-Ma2 antibodies and mesothelioma

Acute psychosis followed by fever: Malignant neuroleptic syndrome or viral encephalitis?

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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.

Sequential MRI, SPECT and PET in respiratory syncytial virus encephalitis

International Nuclear Information System (INIS)

Hirayama, K.; Sakazaki, Hiromi; Murakami, Seiko; Yonezawa, Sumiko; Fujimoto, Keiji; Seto, Toshiyuki; Tanaka, Katsuji; Hattori, Hideji; Matsuoka, Osamu; Murata, Ryosuke

1999-01-01

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.)

Tick-borne encephalitis virus infection of cultured mouse macrophages

Czech Academy of Sciences Publication Activity Database

Ahantarig, A.; Růžek, Daniel; Vancová, Marie; Janowitz, A.; Šťastná, Hana; Tesařová, Martina; Grubhoffer, Libor

2009-01-01

Roč. 52, č. 5 (2009), s. 283-290 ISSN 0300-5526 R&D Projects: GA ČR GA524/06/1479; GA MŠk(CZ) LC06009 Institutional research plan: CEZ:AV0Z60220518 Keywords : tick-borne encephalitis * macrophage s * electron microscopy Subject RIV: GJ - Animal Vermins ; Diseases, Veterinary Medicine Impact factor: 1.106, year: 2009

Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome Associated With Anti-N-methyl-D-aspartate Receptor Encephalitis.

Science.gov (United States)

Player, Brittany; Harmelink, Matthew; Bordini, Brett; Weisgerber, Michael; Girolami, Michael; Croix, Michael

2015-11-01

The full clinical spectrum of anti-N-methyl-D-aspartate receptor encephalitis is unknown in the pediatric population. We describe a previously healthy 4-year-old girl presenting with opsoclonus-myoclonus together with ataxia who had NR1-specific, anti-N-methyl-D-aspartate receptor antibodies in the cerebral spinal fluid. The presence of NR1-specific, anti-N-methyl-D-aspartate receptor antibodies in the setting of opsoclonus-myoclonus and ataxia syndrome may represent an expansion of the clinical presentations of anti-N-methyl-D-aspartate receptor encephalitis. Copyright © 2015 Elsevier Inc. All rights reserved.

Anti-N-methyl-D-aspartate receptor encephalitis associated with intracranial Angiostrongylus cantonensis infection: a case report.

Science.gov (United States)

Peng, Yu; Liu, Xiaojia; Pan, Suyue; Xie, Zuoshan; Wang, Honghao

2017-04-01

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently described paraneoplastic syndrome with prominent neuropsychiatric symptoms. Many of these cases are associated with neoplasma especially teratoma. In addition, a few of cases with anti-NMDAR antibodies triggered by viral infection have been reported, but never by parasitic infection. Here, we report a novel case of NMDA receptor encephalitis in a 51-year-old male related to the development of anti-NMDAR antibodies triggered by Angiostrongylus cantonensis infection.

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...

Anti-N-methyl-d-aspartate receptor encephalitis in children of Central South China: Clinical features, treatment, influencing factors, and outcomes.

Science.gov (United States)

Wang, Ying; Zhang, Weixi; Yin, Jinghua; Lu, Qianjin; Yin, Fei; He, Fang; Peng, Jing

2017-11-15

We analyzed the clinical manifestations of children with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis in Central South China and the factors influencing the effectiveness of treatment. A retrospective study of children (0-14years old) with anti-NMDAR encephalitis in Central South China was carried out from March 2014 to November 2016. Demographics, clinical features, treatment, outcome, and the factors influencing the effectiveness of treatment were reviewed. Fifty-one patients with anti-NMDAR encephalitis were enrolled (age from 4months to 14years old; median age, 8years; 30 females). Forty-five patients (88%) presented with psychiatric symptoms, 40 (78%) with dyskinesia and movement disorders, 39 (77%) with sleep disturbances, 34 (67%) with seizures, 30 (59%) with a decreased level of consciousness (Glasgow scoreanti-NMDAR encephalitis in Central South China. Patients with decreased consciousness, PICU stay and autonomic instability were more likely to have no or limited response to first-line immunotherapy and to require second-line or even more aggressive immunotherapy. Children with anti-NMDAR encephalitis in China have a much lower incidence of tumors, lower mortality rates, and a lower proportion of lethal autonomic instability than adults. Copyright © 2017 Elsevier B.V. All rights reserved.

Affinity (tropism) of caprine arthritis encephalitis virus for brain cells ...

African Journals Online (AJOL)

In this study, explant cultures prepared from the brain of new-born goat-kid were infected with. Caprine Arthritis 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 (RTPCR) and transmission ...

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...

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.

Dual Pathology in Rasmussen’s Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature

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Richard A. Prayson

2012-01-01

Full Text Available Rasmussen’s encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology. This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diagnosis of coexistent Rasmussen’s encephalitis, marked by chronic inflammation, microglial nodules, and focal cortical atrophy, combined with focal cortical dysplasia (Palmini et al. type IIA, ILAE type IIA. The literature on dual pathology in the setting of Rasmussen’s encephalitis is reviewed.

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...

Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis.

Science.gov (United States)

Schmitt, Sarah E; Pargeon, Kimberly; Frechette, Eric S; Hirsch, Lawrence J; Dalmau, Josep; Friedman, Daniel

2012-09-11

To determine continuous EEG (cEEG) patterns that may be unique to anti-NMDA receptor (NMDAR) encephalitis in a series of adult patients with this disorder. We evaluated the clinical and EEG data of 23 hospitalized adult patients with anti-NMDAR encephalitis who underwent cEEG monitoring between January 2005 and February 2011 at 2 large academic medical centers. Twenty-three patients with anti-NMDAR encephalitis underwent a median of 7 (range 1-123) days of cEEG monitoring. The median length of hospitalization was 44 (range 2-200) days. Personality or behavioral changes (100%), movement disorders (82.6%), and seizures (78.3%) were the most common symptoms. Seven of 23 patients (30.4%) had a unique electrographic pattern, which we named "extreme delta brush" because of its resemblance to waveforms seen in premature infants. The presence of extreme delta brush was associated with a more prolonged hospitalization (mean 128.3 ± 47.5 vs 43.2 ± 39.0 days, p = 0.008) and increased days of cEEG monitoring (mean 27.6 ± 42.3 vs 6.2 ± 5.6 days, p = 0.012). The modified Rankin Scale score showed a trend toward worse scores in patients with the extreme delta brush pattern (mean 4.0 ± 0.8 vs 3.1 ± 1.1, p = 0.089). Extreme delta brush is a novel 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, its presence should raise consideration of this syndrome.

Japanese encephalitis in a 114-month-old cow: pathological investigation of the affected cow and genetic characterization of Japanese encephalitis virus isolate.

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Kako, Naomi; Suzuki, Seiji; Sugie, Norie; Kato, Tomoko; Yanase, Tohru; Yamakawa, Makoto; Shirafuji, Hiroaki

2014-03-11

Japanese encephalitis virus (JEV) is classified into the genus Flavivirus in the family Flaviviridae. JEV can cause febrile illness and encephalitis mainly in humans and horses, and occasionally in cattle. In late September 2010, a 114-month-old cow showed neurological symptoms similar to the symptoms observed in previous bovine cases of Japanese encephalitis (JE); therefore, we conducted virological and pathological tests on the cow. As a result, JEV was isolated from the cerebrum of the affected cow. We determined the complete genome sequence of the JEV isolate, which we named JEV/Bo/Aichi/1/2010, including the envelope (E) gene region and 3' untranslated region (3'UTR). Our phylogenetic analyses of the E region and complete genome showed that the isolate belongs to JEV genotype 1 (G1). The isolate, JEV/Bo/Aichi/1/2010, was most closely related to several JEV G1 isolates in Toyama Prefecture, Japan in 2007-2009 by the phylogenetic analysis of the E region. In addition, the nucleotide alignment revealed that the deletion in the 3'UTR was the same between JEV/Bo/Aichi/1/2010 and several other JEV G1 isolates identified in Toyama Prefecture in 2008-2009. A hemagglutination inhibition (HI) test was conducted for the detection of anti-JEV antibodies in the affected cow, and the test detected 2-mercaptoethanol (2-ME)-sensitive HI antibodies against JEV in the serum of the affected cow. The histopathological investigation revealed nonsuppurative encephalomyelitis in the affected cow, and the immunohistochemical assay detected JEV antigen in the cerebrum. We diagnosed the case as JE of a cow based on the findings of nonsuppurative encephalomyelitis observed in the central nervous system, JEV antigen detected in the cerebrum, JEV isolated from the cerebrum, and 2-ME-sensitive HI antibodies against JEV detected in the serum. This is the first reported case of JE in a cow over 24 months old.

Cerebral (18)FluoroDeoxy-Glucose Positron Emission Tomography in paediatric anti N-methyl-D-aspartate receptor encephalitis: A case series.

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Lagarde, Stanislas; Lepine, Anne; Caietta, Emilie; Pelletier, Florence; Boucraut, José; Chabrol, Brigitte; Milh, Mathieu; Guedj, Eric

2016-05-01

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a frequent and severe cause of encephalitis in children with potential efficient treatment (immunotherapy). Suggestive clinical features are behavioural troubles, seizures and movement disorders. Prompt diagnosis and treatment initiation are needed to guarantee favourable outcome. Nevertheless, diagnosis may be challenging because of the classical ancillary test (magnetic resonance imaging (MRI), electroencephalogram, standard cerebro-spinal fluid analysis) have limited sensitivity. Currently, immunological analyses are needed for the diagnostic confirmation. In adult patients, some studies suggested a potential role of cerebral (18)FluoroDeoxy-Glucose Positron Emission Tomography (FDG-PET) in the evaluation of anti-NMDAR encephalitis. Nevertheless, almost no data exist in paediatric population. We report retrospectively clinical, ancillary tests and cerebral FDG-PET data in 6 young patients (median age=10.5 years, 4 girls) with immunologically confirmed anti-NMDAR encephalitis. Our patients presented classical clinical features of anti-NMDAR encephalitis with severe course (notably four patients had normal MRI). Our series shows the feasibility and the good sensitivity of cerebral FDG-PET (6/6 patients with brain metabolism alteration) in paediatric population. We report some particular features in this population: extensive, symmetric cortical hypometabolism especially in posterior areas; asymmetric anterior focus of hypermetabolism; and basal ganglia hypermetabolism. We found also a good correlation between the clinical severity and the cerebral metabolism changes. Moreover, serial cerebral FDG-PET showed parallel brain metabolism and clinical improvement. Our study reveals the existence of specific patterns of brain metabolism alteration in anti-NMDAR encephalitis in paediatric population. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

MRI and associated clinical characteristics of EV71-induced brainstem encephalitis in children with hand-foot-mouth disease

International Nuclear Information System (INIS)

Zeng, Hongwu; Gan, Yungen; Wen, Feiqiu; Huang, Wenxian

2012-01-01

This study was conducted to investigate MRI and associated clinical characteristics of brainstem encephalitis induced by enterovirus 71 (EV71) in children with hand-foot-mouth disease (HFMD). We analyzed clinical and imaging data from 42 HFMD cases with EV71-induced brainstem encephalitis. All patients underwent plain and enhanced MRI cranial scans and were placed into one of two groups according to MRI enhancement results, an enhanced group or a nonenhanced group. Thirty-two cases were positive on MRI exam. The primary location of the lesion for brainstem encephalitis was the dorsal pons and medulla oblongata (32 cases), followed by the cerebellar dentate nucleus (8 cases), midbrain (5 cases), and thalamus (2 cases). Plain T1-weighted images showed isointense or hypointense signals, and T2-weighted images showed isointense and hyperintense signals. Enhanced MRI scans showed that 12 cases had slight to moderate enhancement; 4 of these were normal on plain scan. The time from MRI examination to disease onset was statistically different between the enhanced (n = 12) and nonenhanced (n = 21) groups with a mean of 7.67 days (SD = 1.07) vs 11.95 days (SD = 5.33), respectively. The most common neurological symptoms for brainstem encephalitis were myoclonus and tremor. The greater the area of affected brain, the more severe the clinical symptoms were. The locations of EV71-induced HFMD-associated brainstem encephalitis lesions are relatively specific. Enhanced MRI scans could also identify the lesions missed by early plain scans. MRI scans can provide important information for clinical evaluation and treatment. (orig.)

MRI and associated clinical characteristics of EV71-induced brainstem encephalitis in children with hand-foot-mouth disease

Energy Technology Data Exchange (ETDEWEB)

Zeng, Hongwu; Gan, Yungen [Shenzhen Children' s Hospital, Department of Radiology, Shenzhen (China); Wen, Feiqiu [Shenzhen Children' s Hospital, Department of Neurology, Shenzhen (China); Huang, Wenxian [Shenzhen Children' s Hospital, Department of Respiratory, Shenzhen (China)

2012-06-15

This study was conducted to investigate MRI and associated clinical characteristics of brainstem encephalitis induced by enterovirus 71 (EV71) in children with hand-foot-mouth disease (HFMD). We analyzed clinical and imaging data from 42 HFMD cases with EV71-induced brainstem encephalitis. All patients underwent plain and enhanced MRI cranial scans and were placed into one of two groups according to MRI enhancement results, an enhanced group or a nonenhanced group. Thirty-two cases were positive on MRI exam. The primary location of the lesion for brainstem encephalitis was the dorsal pons and medulla oblongata (32 cases), followed by the cerebellar dentate nucleus (8 cases), midbrain (5 cases), and thalamus (2 cases). Plain T1-weighted images showed isointense or hypointense signals, and T2-weighted images showed isointense and hyperintense signals. Enhanced MRI scans showed that 12 cases had slight to moderate enhancement; 4 of these were normal on plain scan. The time from MRI examination to disease onset was statistically different between the enhanced (n = 12) and nonenhanced (n = 21) groups with a mean of 7.67 days (SD = 1.07) vs 11.95 days (SD = 5.33), respectively. The most common neurological symptoms for brainstem encephalitis were myoclonus and tremor. The greater the area of affected brain, the more severe the clinical symptoms were. The locations of EV71-induced HFMD-associated brainstem encephalitis lesions are relatively specific. Enhanced MRI scans could also identify the lesions missed by early plain scans. MRI scans can provide important information for clinical evaluation and treatment. (orig.)

Seasonal Patterns of Japanese Encephalitis and Associated Meteorological Factors in Taiwan.

Science.gov (United States)

Lin, Che-Liang; Chang, Hsiao-Ling; Lin, Chuan-Yao; Chen, Kow-Tong

2017-10-29

The persistent transmission of Japanese encephalitis virus (JEV) in Taiwan necessitates exploring the risk factors of occurrence of Japanese encephalitis (JE). The purpose of this study was to assess the relationship between meteorological factors and the incidence of JE in Taiwan. We collected data for cases of JE reported to the Taiwan Centers for Disease Control (Taiwan CDC) from 2000 to 2014. Meteorological data were obtained from the Taiwan Central Weather Bureau. The relationships between weather variability and the incidence of JE in Taiwan were determined via Poisson regression analysis and a case-crossover methodology. During the 15-year study period, a total of 379 cases of JE were reported. The incidence of JE showed significant seasonality, with the majority of cases occurring in summertime (for oscillation, p Taiwan. Therefore, these factors could be regarded as warning signals indicating the need to implement preventive measures.

Aciclovir-induced acute kidney injury in patients with 'suspected viral encephalitis' encountered on a liaison neurology service.

Science.gov (United States)

Bogdanova-Mihaylova, Petya; Burke, David; O'Dwyer, John P; Bradley, David; Williams, Jennifer A; Cronin, Simon J; Smyth, Shane; Murphy, Raymond P; Murphy, Sinead M; Wall, Catherine; McCabe, Dominick J H

2018-01-06

Patients with 'suspected viral encephalitis' are frequently empirically treated with intravenous aciclovir. Increasing urea and creatinine are 'common', but rapidly progressive renal failure is reported to be 'very rare'. To describe the clinical course and outcome of cases of aciclovir-induced acute kidney injury (AKI) encountered by the Liaison Neurology Service at AMNCH and to highlight the importance of surveillance and urgent treatment of this iatrogenic complication. Retrospectively and prospectively collected data from the Liaison Neurology Service at AMNCH on patients who received IV aciclovir for suspected viral encephalitis and developed AKI were analysed. Aciclovir-induced AKI was defined by a consultant nephrologist in all cases as a rise in serum creatinine of > 26 μmol/L in 48 h or by ≥ 1.5 times the baseline value. Renal function, haematocrit, and fluid balance were monitored following AKI onset. Data from 10 patients were analysed. Median time to AKI onset was 3.5 days (range: 1-6 days). Aciclovir was stopped or the dose adjusted. All patients recovered with IV normal saline, aiming for a urine output > 100-150 ml/h. The interval between first rise in creatinine and return to normal levels varied between 5 and 19 days. Liaison neurologists and general physicians need to be aware that aciclovir may cause AKI attributed to distal intra-tubular crystal nephropathy. Daily fluid balance and renal function monitoring are essential because AKI may arise even with intensive pre-hydration. Prognosis is good if identified early and actively treated.

Isolation, preliminary characterization, and full-genome analyses of tick-borne encephalitis virus from Mongolia.

Science.gov (United States)

Frey, Stefan; Mossbrugger, Ilona; Altantuul, Damdin; Battsetseg, Jigjav; Davaadorj, Rendoo; Tserennorov, Damdindorj; Buyanjargal, Tsoodol; Otgonbaatar, Dashdavaa; Zöller, Lothar; Speck, Stephanie; Wölfel, Roman; Dobler, Gerhard; Essbauer, Sandra

2012-12-01

Tick-borne encephalitis virus (TBEV) causes one of the most important inflammatory diseases of the central nervous system, namely severe encephalitis in Europe and Asia. Since the 1980s tick-borne encephalitis is known in Mongolia with increasing numbers of human cases reported during the last years. So far, however, data on TBEV strains are still sparse. We herein report the isolation of a TBEV strain from Ixodes persulcatus ticks collected in Mongolia in 2010. Phylogenetic analysis of the E-gene classified this isolate as Siberian subtype of TBEV. The Mongolian TBEV strain showed differences in virus titers, plaque sizes, and growth properties in two human neuronal cell-lines. In addition, the 10,242 nucleotide long open-reading frame and the corresponding polyprotein sequence were revealed. The isolate grouped in the genetic subclade of the Siberian subtype. The strain Zausaev (AF527415) and Vasilchenko (AF069066) had 97 and 94 % identity on the nucleotide level. In summary, we herein describe first detailed data regarding TBEV from Mongolia. Further investigations of TBEV in Mongolia and adjacent areas are needed to understand the intricate dispersal of this virus.

Vaccination against Louping Ill Virus Protects Goats from Experimental Challenge with Spanish Goat Encephalitis Virus.

Science.gov (United States)

Salinas, L M; Casais, R; García Marín, J F; Dalton, K P; Royo, L J; Del Cerro, A; Gayo, E; Dagleish, M P; Alberdi, P; Juste, R A; de la Fuente, J; Balseiro, A

2017-05-01

Spanish goat encephalitis virus (SGEV) is a recently described member of the genus Flavivirus belonging to the tick-borne encephalitis group of viruses, and is closely related to louping ill virus (LIV). Naturally acquired disease in goats results in severe, acute encephalitis and 100% mortality. Eighteen goats were challenged subcutaneously with SGEV; nine were vaccinated previously against LIV and nine were not. None of the vaccinated goats showed any clinical signs of disease or histological lesions, but all of the non-vaccinated goats developed pyrexia and 5/9 developed neurological clinical signs, primarily tremors in the neck and ataxia. All non-vaccinated animals developed histological lesions restricted to the central nervous system and consistent with a lymphocytic meningomyeloencephalitis. Vaccinated goats had significantly (P goats throughout the experiment, but increased rapidly and were significantly (P goats against LIV confers highly effective protection against SGEV; this is probably mediated by IgG and prevents an increase in viral RNA load in serum such that vaccinated animals would not be an effective reservoir of the virus. Copyright © 2017 Elsevier Ltd. All rights reserved.

West Nile Virus Encephalitis in a Barbary Macaque (Macaca sylvanus)

Science.gov (United States)

Barker, Ian K.; Crawshaw, Graham J.; Bertelsen, Mads F.; Drebot, Michael A.; Andonova, Maya

2004-01-01

An aged Barbary ape (Macaca sylvanus) at the Toronto Zoo became infected with naturally acquired West Nile virus (WNV) encephalitis that caused neurologic signs, which, associated with other medical problems, led to euthanasia. The diagnosis was based on immunohistochemical assay of brain lesions, reverse transcriptase–polymerase chain reaction, and virus isolation. PMID:15200866

Affinity (tropism) of caprine arthritis encephalitis virus for brain cells

African Journals Online (AJOL)

STORAGESEVER

2008-11-19

Nov 19, 2008 ... Full Length Research Paper. Affinity (tropism) of caprine arthritis encephalitis virus for brain cells. Adebayo, I. A.1*, Awoniyi, T. A. M. 1 and Olaleye, O. D.2. 1Department of Animal Production and Health, Animal Parasitology and Microbiology Research Unit, Federal University of Technology, P M B 704, ...

A Case with Probable Herpes Simplex Encephalitis Characterized by Specific Emotional and Behavioral Disorders and Gogi (Word-Meaning Aphasia-Like Syndrome with Neologism and Neologistic Kanji Processing

Directory of Open Access Journals (Sweden)

I. Jibiki

1992-01-01

Full Text Available A right-handed male patient with probable herpes simplex encephalitis is presented because of the rarity of the clinicial picture. Brain X-ray CT scans showed lesions located in the bilateral fronto-temporal regions primarily involving the left lower temporal lobe. The clinical picture following the acute phase of the disease was characterized by specific emotional and behavioral disorders, i.e. oral tendency, hyperactivity, thoughtless talkativeness, random speech and exhilaration, which were partly compatible with the Klüver-Bucy syndrome. Furthermore, this case was characterized by Gogi (word-meaning aphasia-like transcortical sensory aphasia and neologism produced saliently when naming objects and peculiar neologistic kanji processing in writing to dictation and oral reading. Both the neologism and neologistic kanji processing varied in quantity in parallel with the specific emotional and behavioral disorders. The relationships of these clinical features to lesional sites demonstrated by X-ray CT are discussed.

Fulminant course in a patient with anti-N-methyl-D-aspartate receptor encephalitis with bilateral ovarian teratomas: A case report and literature review.

Science.gov (United States)

Lee, Kuo-Wei; Liou, Li-Min; Wu, Meng-Ni

2018-04-01

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune disorder that can be controlled and reversed by immunotherapy. The presentation of NMDA receptor encephalitis varies, but NMDA receptor encephalitis is seldom reported in patients with both bilateral teratomas and preexisting brain injury. A 28-year-old female with a history of traumatic intracranial hemorrhage presented acute psychosis, seizure, involuntary movement, and conscious disturbance with a fulminant course. Anti-NMDA receptor antibody was identified in both serum and cerebrospinal fluid, confirming the diagnosis of anti-NMDA receptor encephalitis. Bilateral teratomas were also identified during tumor survey. DIAGNOSES:: anti-N-methyl-D-aspartate receptor encephalitis. Tumor resection and immunotherapy were performed early during the course. The patient responded well to tumor resection and immunotherapy. Compared with other reports in the literature, her symptoms rapidly improved without further relapse. This case report demonstrates that bilateral teratomas may be related to high anybody titers and that the preexisting head injury may be responsible for lowering the threshold of neurological deficits. Early diagnosis and therapy are crucial for a good prognosis in such patients.

Crystal structure of the Japanese encephalitis virus envelope protein.

Science.gov (United States)

Luca, Vincent C; AbiMansour, Jad; Nelson, Christopher A; Fremont, Daved H

2012-02-01

Japanese encephalitis virus (JEV) is the leading global cause of viral encephalitis. The JEV envelope protein (E) facilitates cellular attachment and membrane fusion and is the primary target of neutralizing antibodies. We have determined the 2.1-Å resolution crystal structure of the JEV E ectodomain refolded from bacterial inclusion bodies. The E protein possesses the three domains characteristic of flavivirus envelopes and epitope mapping of neutralizing antibodies onto the structure reveals determinants that correspond to the domain I lateral ridge, fusion loop, domain III lateral ridge, and domain I-II hinge. While monomeric in solution, JEV E assembles as an antiparallel dimer in the crystal lattice organized in a highly similar fashion as seen in cryo-electron microscopy models of mature flavivirus virions. The dimer interface, however, is remarkably small and lacks many of the domain II contacts observed in other flavivirus E homodimers. In addition, uniquely conserved histidines within the JEV serocomplex suggest that pH-mediated structural transitions may be aided by lateral interactions outside the dimer interface in the icosahedral virion. Our results suggest that variation in dimer structure and stability may significantly influence the assembly, receptor interaction, and uncoating of virions.

CT features of fibrous dysplasia of the temporal bone

International Nuclear Information System (INIS)

Charrada-Ben Farhat, L.; Bourkhis, S.; Ben Yaacoub, I.; Dali, N.; Askri, A.; Hendaoui, L.

2006-01-01

Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. The temporal bone is rarely involved. In this location, complications such as facial deformity, conductive hearing loss and facial peripheral neural involvement can occur. Positive diagnosis can be established with computerized tomography which also enables assessment of extension and detection of complications. We report a case of a 27-year-old man with extensive fibrous dysplasia of the right temporal bone presenting with conductive hearing loss secondary to progressive stenosis of the external auditory canal. Computerized tomography of the temporal region was performed. (authors)

Severe human parechovirus type 3 myocarditis and encephalitis in an adolescent with hypogammaglobulinemia

Directory of Open Access Journals (Sweden)

Stacey K. Mardekian

2015-07-01

Full Text Available Human parechovirus (HPeV belongs to the Picornaviridae family of RNA viruses. HPeV infections can be asymptomatic, lead to mild respiratory and/or gastrointestinal symptoms, or less frequently cause severe diseases such as sepsis, meningitis, encephalitis, and myocarditis. Severe neurological HPeV infections occur most commonly in infants and neonates. There are currently 16 recognized types of HPeV. HPeV type 3 (HPeV3 has been the predominant type associated with severe central nervous system disease in neonates and newborns since its discovery in 1999. Although HPeV-related infections have been reported in adults, symptomatic HPeV3 infections in adolescents and adults are uncommon. A case of severe HPeV3 myocarditis and encephalitis in an adolescent is described.

Parasite distribution and early-stage encephalitis in Sarcocystis calchasi infections in domestic pigeons (Columba livia f. domestica).

Science.gov (United States)

Maier, Kristina; Olias, Philipp; Enderlein, Dirk; Klopfleisch, Robert; Mayr, Sylvia L; Gruber, Achim D; Lierz, Michael

2015-01-01

Pigeon protozoal encephalitis is a biphasic, neurologic disease of domestic pigeons (Columba livia f. domestica) caused by the apicomplexan parasite Sarcocystis calchasi. Despite severe inflammatory lesions of the brain, associated parasitic stages have only rarely been identified and the cause of the lesions is still unclear. The aim of this study was therefore to characterize the tissue distribution of S. calchasi within pigeons between the two clinical phases and during the occurrence of neurological signs. For this purpose, a semi-quantitative real-time polymerase chain reaction (PCR) was developed. Forty-five domestic pigeons were infected orally (via a cannula into the crop) with 200 S. calchasi sporocysts and euthanized in groups of three pigeons at intervals of 2 to 10 days over a period of 61 days. Tissue samples including brain and skeletal muscle were examined by histology, immunohistochemistry, and PCR. Schizonts were detected in the liver of one pigeon at day 10 post infection. A mild encephalitis was detected at day 20 post infection, around 4 weeks before the onset of neurological signs. At the same time, immature sarcocysts were present in the skeletal muscle. In seven pigeons a few sarcocysts were identified in the brain, but not associated with any lesion. These results suggest that the encephalitis is induced at a very early stage of the S. calchasi lifecycle rather than in the chronic phase of pigeon protozoal encephalitis. Despite the increasing severity of lesions in the central nervous system, the amount of sarcocysts did not increase. This supports the hypothesis of a delayed-type hypersensitivity response as the cause of the encephalitis. The study also demonstrated that S. calchasi DNA is detectable in tissues negative by histological methods, indicating a higher sensitivity of the real-time PCR.

Von Willebrand Factor Gene Variants Associate with Herpes simplex Encephalitis

Czech Academy of Sciences Publication Activity Database

Abdelmagid, N.; Bereczky-Veress, B.; Atanur, S.; Musilová, Alena; Zídek, Václav; Saba, L.; Warnecke, A.; Khademi, M.; Studahl, M.; Aurelius, E.; Hjalmarsson, A.; Garcia-Dias, A.; Denis, C. V.; Bergström, T.; Sköldenberg, B.; Kockum, I.; Aitman, T.; Hübner, N.; Olsson, T.; Pravenec, Michal; Diez, M.

2016-01-01

Roč. 11, č. 5 (2016), e0155832 E-ISSN 1932-6203 R&D Projects: GA MŠk(CZ) 7E10067; GA MŠk(CZ) LL1204 Institutional support: RVO:67985823 Keywords : Von Willebrand Factor gene * Herpes simplex encephalitis * rat * humans Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.806, year: 2016

[Early-summer meningo-encephalitis (ESME) and ESME-vaccination: status 2000].

Science.gov (United States)

Kunze, U; Bernhard, G; Böhm, G; Groman, E

2000-01-01

Tick-borne encephalitis (TBE) is a public health problem very well under control in Austria because of a vaccination programme using a safe, efficient and well tolerated vaccine and a carefully designed social marketing concept. The Austrian vaccine underwent another technological updating and is now marketed under a new brand name (TicoVac) on the basis of an EU registration. A second product is also available (Encepur), but some limitations of use have to be taken into account. To improve the epidemiological situation even further (only 41 hospital cases in 1999) special attention has to be given to the age group 50 years and older as this is the segment of the population where the majority of cases is observed. TBE is a growing international health problem as awareness increases and cases are identified in many European countries, even in regions where TBE so far was not diagnosed. An "International Scientific Working-group on Tick-borne encephalitis (ISW-TBE)" was established to coordinate research and public health activities.

Acute hemorrhagic encephalitis: An unusual presentation of dengue viral infection

International Nuclear Information System (INIS)

Nadarajah, Jeyaseelan; Madhusudhan, Kumble Seetharama; Yadav, Ajay Kumar; Gupta, Arun Kumar; Vikram, Naval Kumar

2015-01-01

Dengue is a common viral infection worldwide with presentation varying from clinically silent infection to dengue fever, dengue hemorrhagic fever, and severe fulminant dengue shock syndrome. Neurological manifestation usually results from multisystem dysfunction secondary to vascular leak. Presentation as hemorrhagic encephalitis is very rare. Here we present the case of a 13-year-old female admitted with generalized tonic clonic seizures. Plain computed tomography (CT) scan of head revealed hypodensities in bilateral deep gray matter nuclei and right posterior parietal lobe without any hemorrhage. Cerebrospinal fluid (CSF) and serology were positive for IgM and IgG antibodies to dengue viral antigen. Contrast-enhanced magnetic resonance imaging (MRI) revealed multifocal T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in bilateral cerebral parenchyma including basal ganglia. No hemorrhage was seen. She was managed with steroids. As her clinical condition deteriorated, after being stable for 2 days, repeat MRI was done which revealed development of hemorrhage within the lesions, and diagnosis of acute hemorrhagic encephalitis of dengue viral etiology was made

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents.

Science.gov (United States)

Florance, Nicole R; Davis, Rebecca L; Lam, Christopher; Szperka, Christina; Zhou, Lei; Ahmad, Saba; Campen, Cynthia J; Moss, Heather; Peter, Nadja; Gleichman, Amy J; Glaser, Carol A; Lynch, David R; Rosenfeld, Myrna R; Dalmau, Josep

2009-07-01

To report the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients ELISA) using HEK293 cells ectopically expressing NR1. Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were 18 years old, 31% in girls < or =18 years old (p = 0.05), and 9% in girls < or =14 years old (p = 0.008). None of the male patients had tumors. Of 32 patients < or =18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03). Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children. Ann Neurol 2009;66:11-18.

Fatal tick-borne encephalitis in an immunosuppressed 12-year-old patient

Czech Academy of Sciences Publication Activity Database

Chmelík, V.; Chrdle, A.; Růžek, Daniel

2016-01-01

Roč. 74, 1 January (2016), s. 73-74 ISSN 1386-6532 R&D Projects: GA ČR GAP502/11/2116 Institutional support: RVO:60077344 Keywords : Tick-borne encephalitis * immunosuppressed patient * fatal case * haemophagocytic lymphohistiocytosis Subject RIV: EE - Microbiology, Virology Impact factor: 3.051, year: 2016

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

Science.gov (United States)

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. © The Author(s) 2015.

Voltage-Gated Potassium Channel Antibodies in Slow-Progression Motor Neuron Disease.

Science.gov (United States)

Godani, Massimiliano; Zoccarato, Marco; Beronio, Alessandro; Zuliani, Luigi; Benedetti, Luana; Giometto, Bruno; Del Sette, Massimo; Raggio, Elisa; Baldi, Roberta; Vincent, Angela

2017-01-01

The spectrum of autoimmune neurological diseases associated with voltage-gated potassium channel (VGKC)-complex antibodies (Abs) ranges from peripheral nerve disorders to limbic encephalitis. Recently, low titers of VGKC-complex Abs have also been reported in neurodegenerative disorders, but their clinical relevance is unknown. The aim of the study was to explore the prevalence of VGKC-complex Abs in slow-progression motor neuron disease (MND). We compared 11 patients affected by slow-progression MND with 9 patients presenting typical progression illness. Sera were tested for VGKC-complex Abs by radioimmunoassay. The distribution of VGKC-complex Abs was analyzed with the Mann-Whitney U test. The statistical analysis showed a significant difference between the mean values in the study and control groups. A case with long-survival MND harboring VGKC-complex Abs and treated with intravenous immunoglobulins is described. Although VGKC-complex Abs are not likely to be pathogenic, these results could reflect the coexistence of an immunological activation in patients with slow disease progression. © 2016 S. Karger AG, Basel.

Diffusion-weighted imaging findings on MRI as the sole radiographic findings in a child with proven herpes simplex encephalitis

Energy Technology Data Exchange (ETDEWEB)

Obeid, Makram; Franklin, Jeremy; Shrestha, Shraddha; Johnson, Lara; Hurst, Daniel [Texas Tech University Health Sciences Center, Department of Pediatrics, Lubbock, TX (United States); Quattromani, Frank [Texas Tech University Health Sciences Center, Department of Radiology, Lubbock, TX (United States)

2007-11-15

We present a case of herpes simplex encephalitis in an 8-year-old girl, in whom hyperintensity was detected on diffusion-weighted imaging (DWI) while conventional MRI sequences were normal 1 week after the onset of neurological symptoms. This case is rare in that a child beyond the neonatal period with focal herpes simplex encephalitis had an abnormal DWI sequence as the only MRI finding. (orig.)

Successful combination immunotherapy of anti-gamma aminobutyric acid (GABA)A receptor antibody-positive encephalitis with extensive multifocal brain lesions.

Science.gov (United States)

Fukami, Yuki; Okada, Hiroaki; Yoshida, Mari; Yamaguchi, Keiji

2017-08-31

A 78-year old woman who presented with akinetic mutism was admitted to our hospital. Brain MRI showed multifocal increased T 2 /FLAIR signal with extensive cortical-subcortical involvement. We suspected autoimmune encephalitis and the patient received methylprednisolone pulse. Her conscious level gradually recovered, but later relapsed again and presented with refractory status epilepticus. We treated her with intravenous immunoglobulin, plasma exchange and pulsed cyclophosphamide, with satisfactory response. A brain biopsy showed perivascular lymphocytic infiltrates and reactive gliosis. Anti-gamma aminobutyric acid (GABA) A receptor antibodies test came back to be positive after her recovery, and the diagnosis of anti-GABA A receptor antibody-positive encephalitis was made. This is a very rare case where brain biopsies were performed in a patient with anti-GABA A receptor antibody-positive encephalitis.

REM sleep behavior disorder and narcoleptic features in anti-Ma2-associated encephalitis.

Science.gov (United States)

Compta, Yaroslau; Iranzo, Alex; Santamaría, Joan; Casamitjana, Roser; Graus, Francesc

2007-06-01

A 69-year-old man with anti-Ma2 paraneoplastic encephalitis presented with subacute onset of severe hypersomnia, memory loss, parkinsonism, and gaze palsy. A brain magnetic resonance imaging study showed bilateral damage in the dorsolateral midbrain, amygdala, and paramedian thalami. Videopolysomnography disclosed rapid eye movement (REM) sleep behavior disorder, and a Multiple Sleep Latency Test showed a mean sleep latency of 7 minutes and 4 sleep-onset REM periods. The level of hypocretin-1 in the cerebrospinal fluid was low (49 pg/mL). This observation illustrates that REM sleep behavior disorder and narcoleptic features are 2 REM-sleep abnormalities that (1) may share the same autoimmune-mediated origin affecting the brainstem, limbic, and diencephalic structures and (2) may occur in the setting of the paraneoplastic anti-Ma2-associated encephalitis.

EEG Differences in Two Clinically Similar Rapid Dementias: Voltage-Gated Potassium Channel Complex-Associated Autoimmune Encephalitis and Creutzfeldt-Jakob Disease.

Science.gov (United States)

Freund, Brin; Probasco, John C; Cervenka, Mackenzie C; Sutter, Raoul; Kaplan, Peter W

2018-05-01

Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex-associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015. More patients in the VGKC AE group had seizures (12/17) than those with CJD (3/14; P = .008). Serum sodium levels were lower in those with VGKC AE ( P = .001). Cerebrospinal fluid (CSF) white blood cell count was higher in VGKC AE ( P = .008). CSF protein 14-3-3 ( P = .018) was more commonly detected in CJD, and tau levels were higher in those with CJD ( P VGKC AE, and electroencephalography can aid in their diagnoses. Performing serial EEGs better delineates these conditions.

Cortical laminar necrosis in dengue encephalitis-a case report.

Science.gov (United States)

Garg, Ravindra Kumar; Rizvi, Imran; Ingole, Rajan; Jain, Amita; Malhotra, Hardeep Singh; Kumar, Neeraj; Batra, Dhruv

2017-04-20

Dengue encephalitis is a rare neurological manifestation of dengue fever. Its clinical presentation is similar to other viral encephalitides and encephalopathy. No single specific finding on magnetic resonance imaging of dengue encephalitis has yet been documented. They are highly variable and atypical. A 15-year boy presented with fever, the headache and altered sensorium of 12-day duration. On neurological examination, his Glasgow Coma Scale score was 10 (E3M4V3). There was no focal neurological deficit. Laboratory evaluation revealed leukopenia and marked thrombocytopenia. Dengue virus IgM antibody was positive both in serum and cerebrospinal fluid. Magnetic resonance imaging of the brain revealed signal changes in bilateral parietooccipital and left frontal regions (left hemisphere more involved than the right hemisphere). There was gyriform enhancement bilateral parietooccipital regions consistent with cortical laminar necrosis. Bilaterally diffuse subcortical white matter was also involved and subtle T2 hyperintensity involving both basal ganglia was noted. Gradient echo sequence revealed presence of hemorrhage in the subcortical white matter. Patient was treated conservatively and received platelet transfusion. Patient became fully conscious after 7 days. In a patient with highly suggestive dengue e\\ephalitis, we describe an unusual magnetic resonance imaging finding. This report is possibly the first instance of cortical laminar necrosis in such a setting.

Abrogated inflammatory response promotes neurogenesis in a murine model of Japanese encephalitis.

Directory of Open Access Journals (Sweden)

Sulagna Das

2011-03-01

Full Text Available 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.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.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.

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

Energy Technology Data Exchange (ETDEWEB)

Urbach, H.; Mader, I. [University Medical Center Freiburg, Department of Neuroradiology, Freiburg (Germany); Rauer, S.; Baumgartner, A. [University Medical Center Freiburg, Department of Neurology, Freiburg (Germany); Paus, S. [University Medical Center, Department of Neurology, Bonn (Germany); Wagner, J. [University Medical Center, Department of Epileptology, Bonn (Germany); Malter, M.P. [University of Cologne, Department of Neurology, Cologne (Germany); Pruess, H. [Charite - Universitaetsmedizin Berlin, Department of Neurology, Berlin (Germany); Lewerenz, J.; Kassubek, J. [Ulm University, Department of Neurology, Ulm (Germany); Hegen, H.; Auer, M.; Deisenhammer, F. [University Innsbruck, Department of Neurology, Innsbruck (Austria); Ufer, F. [University Medical Center, Department of Neurology, Hamburg (Germany); Bien, C.G. [Epilepsy Centre Bethel, Bielefeld-Bethel (Germany)

2015-12-15

Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). SWMB is a newly described MRI sign rather specific for VGKC-LE. (orig.)

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

International Nuclear Information System (INIS)

Urbach, H.; Mader, I.; Rauer, S.; Baumgartner, A.; Paus, S.; Wagner, J.; Malter, M.P.; Pruess, H.; Lewerenz, J.; Kassubek, J.; Hegen, H.; Auer, M.; Deisenhammer, F.; Ufer, F.; Bien, C.G.

2015-01-01

Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). SWMB is a newly described MRI sign rather specific for VGKC-LE. (orig.)

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis.

Science.gov (United States)

Urbach, H; Rauer, S; Mader, I; Paus, S; Wagner, J; Malter, M P; Prüss, H; Lewerenz, J; Kassubek, J; Hegen, H; Auer, M; Deisenhammer, F; Ufer, F; Bien, C G; Baumgartner, A

2015-12-01

Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). SWMB is a newly described MRI sign rather specific for VGKC-LE.

Serological and molecular epidemiology of Japanese encephalitis virus infections in swine herds in China, 2006-2012.

Science.gov (United States)

Chai, Chunxia; Wang, Qiao; Cao, Sanjie; Zhao, Qin; Wen, Yiping; Huang, Xiaobo; Wen, Xintian; Yan, Qiguai; Ma, Xiaoping; Wu, Rui

2018-01-31

Japanese encephalitis virus (JEV) is a mosquito-borne, zoonotic flavivirus causing viral encephalitis in humans and reproductive disorder in swine. JEV is prevalent throughout China in human; however, spatiotemporal analysis of JEV in Chinese swine herds has not been reported previously. Herein, we present serological and molecular epidemiological results and estimates of prevalence of JEV infections among swine herds in various regions of China. The results suggest that JEV infections are widespread and genotype I and III strains co-exist in the same regions. Therefore, there is an urgent need to monitor JEV infection status among swine herds in China.

Hypothermia due to limbic system involvement and longitudinal myelitis in a case of Japanese encephalitis: a case report from India

Directory of Open Access Journals (Sweden)

Narayanan S

2017-04-01

Full Text Available Santhosh Narayanan,1 NK Thulaseedharan,1 Gomathy Subramaniam,2 Geetha Panarkandy,1 VK Shameer,1 Arathi Narayanan1 1Department of General Medicine, 2Department of Radiodiagnosis, Government Medical College, Kozhikode, Kerala, India Abstract: Japanese encephalitis (JE is an infectious encephalitis prevalent in Asia. It usually presents with fever, headache, convulsions and extrapyramidal symptoms. Limbic system involvement and hypothermia though common in autoimmune encephalitis have never been reported in JE. We report a case of an 18-year-old girl with no previous comorbidities who presented to us with a history of fever and headache for 1 week duration. She developed bilateral lateral rectus palsy and asymmetric flaccid weakness of all four limbs, after 2 days of admission, which was followed by altered sensorium and intermittent hypothermia. Neuroimaging revealed longitudinal myelitis extending from pons till the L1 level along with bilateral thalamic hemorrhage in brain. Cerebrospinal fluid (CSF was positive for IgM antibody to JE virus. She was treated with supportive measures, but she developed intractable hypothermia and seizures and succumbed to illness after 2 weeks of admission. Keywords: Japanese encephalitis, hypothermia, limbic system

Contrast enhancement of the gyri in herpes simplex encephalitis

International Nuclear Information System (INIS)

Schumacher, K.A.; Langer, M.; Langer, R.

1982-01-01

A case of herpes simplex encephalitis was examined by computer tomography. Both cerebral hemispheres showed contrast enhancement of the gyri. The cause of this is considered. The increased contrast medium accumulation in the affected areas is probably due to the marked vascular proliferation which can be demonstrated anatomically, and to the rapid escape of contrast from the capillaries into the interstitial spaces. The findings of other authors, which differ somewhat, are discussed. (orig.) [de

Limbic encephalitis presenting as a post-partum psychiatric condition.

OpenAIRE

Gotkine, Marc; Ben-Hur, Tamir; Vincent, Angela; Vaknin-Dembinsky, Adi

2011-01-01

OBJECTIVE: We describe a woman who presented with a psychiatric disorder post-partum and subsequently developed seizures and cognitive dysfunction prompting further investigation. A diagnosis of limbic encephalitis (LE) was made and antibodies to voltage-gated potassium channel complex (VGKC) detected. These antibodies are found in many non-paraneoplastic patients with LE. Although antibody-mediated conditions tend to present or relapse post-partum, VGKC-LE in the post-partum period has not b...

Development of Isaacs' syndrome following complete recovery of voltage-gated potassium channel antibody-associated limbic encephalitis.

Science.gov (United States)

Takahashi, Hirokatsu; Mori, Masahiro; Sekiguchi, Yukari; Misawa, Sonoko; Sawai, Setsu; Hattori, Takamichi; Kuwabara, Satoshi

2008-12-15

Autoantibodies against voltage-gated potassium channels (VGKC-Abs) are associated with acquired neuromyotonia (Isaacs' syndrome) and related disorders such as Morvan's syndrome and some cases of limbic encephalitis. The mechanisms underlying the various phenotypes induced by VGKC-Abs are not fully understood. Recently, we reported a case of LE with VGKC-Abs accompanied by severe intestinal pseudo-obstruction and thymoma. Thymectomy and immunosuppressive therapy induced dramatic clinical improvement of LE symptoms, and VGKC-Abs titers decreased from 1254 pM to 549 pM (normal>100 pM). Seventeen months later, the patient developed progressive generalized muscle cramping, paresthesias in his lower extremities, excessive sweating, and severe constipation. There was no recurrence of the LE. Electromyography showed fasciculation potentials and myokymic discharges, and the plasma VGKC-Abs titer was again elevated to 879 pM. Here we report a case of Isaacs' syndrome after complete remission of LE with VGKC-Abs that may provide an insight into a possible link among VGKC-Abs associated syndromes.

Assessment of listing and categorisation of animal diseases within the framework of the Animal Health Law (Regulation (EU) No 2016/429): Japanese encephalitis (JE)

DEFF Research Database (Denmark)

EFSA Panel on Animal Health and Welfare; More, Simon J.; Bøtner, Anette

2017-01-01

Japanese encephalitis (JE) has been assessed according to the criteria of the Animal Health Law (AHL), in particular criteria of Article 7 on disease profile and impacts, Article 5 on the eligibility of Japanese encephalitis to be listed, Article 9 for the categorisation of Japanese encephalitis......, if no consensus was reached before, also at collective level. The output is composed of the categorical answer, and for the questions where no consensus was reached, the different supporting views are reported. Details on the methodology used for this assessment are explained in a separate opinion. According...

Lymphocyte subset contents in cerebrospinal fluid of children with viral encephalitis

Directory of Open Access Journals (Sweden)

An-Ran Xu

2016-06-01

Full Text Available Objective: To study the lymphocyte subset contents in cerebrospinal fluid of children with viral encephalitis and their correlation with disease. Methods: Children with viral encephalitis were selected as VE group, children excluded of central nervous system infection by lumbar puncture or children without central nervous system diseases but receiving surgery with spinal anesthesia were selected as control group, and then cerebrospinal fluid and serum were collected to detect lymphocyte subset contents, nerve injury molecule contents as well as inflammatory response indicators and oxidative stress response indicators. Results: CD3+, CD3+CD4+, CD4/CD8 and CD16+CD56+ in cerebrospinal fluid of VE group were lower than those of control group, and both CD3+CD8+ and CD19+ were higher than those of control group; CD3+, CD3+CD4+, CD4/CD8 and CD16+CD56+ in cerebrospinal fluid of children with abnormal MRI were lower than those of children with normal MRI, and both CD3+CD8+ and CD19+ were higher than those of children with normal MRI; NSE, MBP, S-100 and NPT contents in cerebrospinal fluid and serum of VE group were significantly higher than those of control group and had good correlation with lymphocyte subset contents; MMP9, TNF-α and IL-6 contents in cerebrospinal fluid of VE group were significantly higher than those of control group, and SOD and GSH-Px contents were significantly lower than those of control group and had good correlation with lymphocyte subset contents. Conclusions: CD4+/CD8+T lymphocyte ratio and NK cell content decrease, and B lymphocyte content increases in cerebrospinal fluid of children with viral encephalitis, and lymphocyte subset contents have inhibitory effect on MRI manifestation, degree of inflammatory response and oxidative stress response.

Rodents as Sentinels for the Prevalence of Tick-Borne Encephalitis Virus

Czech Academy of Sciences Publication Activity Database

Achazi, K.; Růžek, Daniel; Donoso-Mantke, O.; Schlegel, M.; Ali, H. S.; Wenk, M.; Schmidt-Chanasit, J.; Ohlmeyer, L.; Ruhe, F.; Vor, T.; Kiffner, Ch.; Kallies, R.; Ulrich, R. G.; Niedrig, M.

2011-01-01

Roč. 11, č. 6 (2011), 641-647 ISSN 1530-3667 R&D Projects: GA ČR GPP302/10/P438; GA MŠk(CZ) LC06009 Institutional research plan: CEZ:AV0Z60220518 Keywords : Distribution * Monitoring * Rodents * Tick-borne encephalitis * Zoonosis Subject RIV: EE - Microbiology, Virology Impact factor: 2.437, year: 2011

A purified inactivated Japanese encephalitis virus vaccine made in Vero cells.

Science.gov (United States)

Srivastava, A K; Putnak, J R; Lee, S H; Hong, S P; Moon, S B; Barvir, D A; Zhao, B; Olson, R A; Kim, S O; Yoo, W D; Towle, A C; Vaughn, D W; Innis, B L; Eckels, K H

2001-08-14

A second generation, purified, inactivated vaccine (PIV) against Japanese encephalitis (JE) virus was produced and tested in mice where it was found to be highly immunogenic and protective. The JE-PIV was made from an attenuated strain of JE virus propagated in certified Vero cells, purified, and inactivated with formalin. Its manufacture followed current GMP guidelines for the production of biologicals. The manufacturing process was efficient in generating a high yield of virus, essentially free of contaminating host cell proteins and nucleic acids. The PIV was formulated with aluminum hydroxide and administered to mice by subcutaneous inoculation. Vaccinated animals developed high-titered JE virus neutralizing antibodies in a dose dependent fashion after two injections. The vaccine protected mice against morbidity and mortality after challenge with live, virulent, JE virus. Compared with the existing licensed mouse brain-derived vaccine, JE-Vax, the Vero cell-derived JE-PIV was more immunogenic and as effective as preventing encephalitis in mice. The JE-PIV is currently being tested for safety and immunogenicity in volunteers.

Potential Sympatric Vectors and Mammalian Hosts of Venezuelan Equine Encephalitis Virus in Southern Mexico.

Science.gov (United States)

Sotomayor-Bonilla, Jesús; Abella-Medrano, Carlos Antonio; Chaves, Andrea; Álvarez-Mendizábal, Paulina; Rico-Chávez, Óscar; Ibáñez-Bernal, Sergio; Rostal, Melinda K; Ojeda-Flores, Rafael; Barbachano-Guerrero, Arturo; Gutiérrez-Espeleta, Gustavo; Aguirre, A Alonso; Daszak, Peter; Suzán, Gerardo

2017-07-01

Arboviruses are important zoonotic agents with complex transmission cycles and are not well understood because they may involve many vectors and hosts. We studied sympatric wild mammals and hematophagous mosquitoes having the potential to act as hosts and vectors in two areas of southern Mexico. Mosquitoes, bats, and rodents were captured in Calakmul (Campeche) and Montes Azules (Chiapas), between November 2010 and August 2011. Spleen samples from 146 bats and 14 rodents were tested for molecular evidence of Venezuelan equine encephalitis virus (VEEV), eastern equine encephalitis virus (EEEV), western equine encephalitis virus (WEEV), and West Nile virus (WNV) using PCR protocols. Bat ( Artibeus lituratus , Carollia sowelli , Glossophaga soricina , and Sturnira parvidens) and rodent ( Sigmodon hispidus and Oryzomys alfaroi ) species were positive for VEEV. No individuals were positive for WNV, EEEV, or WEEV. A total of 1,298 mosquitoes were collected at the same sites, and five of the mosquito species collected were known VEEV vectors (Aedes fulvus, Mansonia indubitans, Psorophora ferox, Psorophora cilipes, and Psorophora confinnis). This survey simultaneously presents the first molecular evidence, to our knowledge, of VEEV in bats and rodents from southern Mexico and the identification of potential sympatric vectors. Studies investigating sympatric nonhuman hosts, vectors, and arboviruses must be expanded to determine arboviral dynamics in complex systems in which outbreaks of emerging and reemerging zoonoses are continuously occurring.

Brote de encefalitis de San Luis en el Área Metropolitana Buenos Aires Outbreack of St. Luis encephalitis in the Metropolitan Buenos Aires Area

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Alfredo Seijo

2011-06-01

Full Text Available Se describen los hallazgos epidemiológicos y clínicos de 13 enfermos con diagnóstico de infección por virus de la encefalitis de San Luis, con transmisión entre enero y marzo de 2010, en el Area Metropolitana Buenos Aires (AMBA. Los 13 enfermos, promedio de edad 38 años, tuvieron un comienzo agudo caracterizado por hipertermia y cefalea. Entre los días dos y diez de iniciados los síntomas, 7/13 enfermos tuvieron signos y síntomas de compromiso neurológico caracterizado por meningitis sin signos encefálicos en 1/7. En 6/7 los hallazgos más frecuentes fueron: rigidez de nuca, desorientación temporoespacial, fotofobia, confusión y alteración del lenguaje. Dos resonancias magnéticas y un electroencefalograma presentaron signos de afectación de lóbulos temporales. El líquido cefalorraquídeo se caracterizó por pleocitosis con predominio de células mononucleadas, glucorraquia normal y discreto aumento de proteínas. No hubo casos fatales. En 6/13 pacientes la sospecha clínica inicial fue dengue. Por la agrupación espacial y temporal de los casos puede considerarse un brote epidémico, el primero conocido en el AMBA, ya que no se había notificado previamente la circulación epidémica del virus.We describe the epidemiological and clinical findings of 13 patients with diagnosis of infection by St. Louis encephalitis virus, transmitted between January and March 2010 in Buenos Aires Metropolitan Area (AMBA. Thirteen patients, average age 38 years, had an acute onset with hyperthermia and headache. Between days two and ten of the onset of symptoms, 7/13 patients had signs and symptoms of neurological involvement. This was characterized by meningitis without encephalic sings in 1/7 and in 6/7 the most frequent findings were: stiff neck, disorientation, photophobia, confusion and language impairment. Two MR and one EEG revealed signs of involvement of temporal lobes. The cerebrospinal fluid showed pleocytosis with predominance of

Adult herpes simplex encephalitis: fifteen years' experience.

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Riera-Mestre, Antoni; Gubieras, Laura; Martínez-Yelamos, Sergio; Cabellos, Carmen; Fernández-Viladrich, Pedro

2009-03-01

Herpes simplex encephalitis (HSE) is the most frequent cause of sporadic necrotizing encephalitis in adults. The aim of this study is to describe the characteristics of HSE and the factors influencing its outcome. Retrospective study of patients diagnosed with HSE in a tertiary care teaching hospital over a 15-year period. Diagnosis was based on a consistent clinical profile for HSE, plus either a PCR-positive CSF HSV study or consistent brain neuroimaging findings. Patients were divided into 2 groups according to the modified Rankin Scale: good outcome (Grades =3). Thirty-five patients were included. Mean age was 53.9 years. More than half presented febricula or fever, headache, disorientation, behavioral changes, decreased level of consciousness, or neurological deficit. CSF glucose concentration was normal in all patients and WBC count was normal in 8 (23%). PCR for HSV was positive in 92% and cranial MRI was suggestive of HSE in 100% of patients. Mortality was 8.6%. In relation to outcome, age (OR=1.079; 95% CI, 1.023-1.138) and serum albumin level at admission (OR=0.87; 95% CI, 0.794-0.954) were independent prognostic factors at discharge. At 6 months, days of fever after initiation of acyclovir therapy (OR=1.219; 95% CI, 1.046-1.422) and serum albumin level at admission (OR=0.917; 95% CI, 0.87-0.967) were independent prognostic factors. Normal brain MRI or detection of low CSF glucose concentration requires consideration of diagnoses other than HSE. Age, serum albumin level at admission, and days of fever after initiation of acyclovir therapy were independent prognostic factors of the disease.

PET/CT in the evaluation of anti-NMDA-receptor encephalitis: what we need to know as a NM physician

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Padma, S.; Shanmuga Sundaram, P.; Marmattom, Bobby Varkey

2011-01-01

Anti N-methyl-D-aspartate receptor encephalitis (ANMDARE), also known as limbic encephalitis (LE), is a treatable rare disorder characterized by personality changes, irritability, depression, seizures, memory loss and sometimes dementia. It is classified under paraneoplastic syndrome (PNS) and produces antibodies against NR1 and NR2 subunits of glutamate aspartate receptor. It is thought to be closely related with malignancies like small cell lung cancer, ovarian teratoma and Hodgkin's lymphoma, apart from testis, breast and rarely gastric malignancies. Non-paraneoplastic encephalitis cases are the ones with no detectable malignancy and may be triggered by severe infection. As nuclear medicine physicians, we must be aware of the diverse presentation of ANMDARE or LE and should include a whole body positron emission tomography/computed tomography (PET/CT) and not just brain PET/CT during imaging. We describe the first case of PET/CT in an idiopathic ANMDARE Indian adolescent girl. (author)

Influence of Sex and Age on Natural Resistance to St. Louis Encephalitis Virus Infection in Mice

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Andersen, Arthur A.; Hanson, Robert P.

1974-01-01

A difference was observed in susceptibility of adult male and female mice to St. Louis encephalitis (SLE) virus as measured by the death rate after intravenous challenge. Female mice that had susceptibility similar to that of males at 2 months of age had increased resistance to SLE virus at 3 and 4 months of age. The increased resistance occurred after sexual maturity, indicating that the resistance factor possibly was related to an aging process in the female. The susceptibility of male mice remained unchanged over the 2- to 4-month period. Neither pregnancy nor castration had any effect on resistance of adult mice to St. Louis encephalitis virus. PMID:4857422

Topographic changes of ictal hyperperfusion during progression of clinical seizures

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Shin, Won Chul; Hong, Seung Bong; Tae, Woo Suk; Shon, Young Min; Seo, Dae Won; Kim, Byoung Joon; Hong, Seung Chyul; Kim, Sang Eun [School of Medicine, Sungkyunkwan, Seoul (Korea, Republic of)

2001-12-01

To investigate ictal hyperperfusion patterns during semiologic progression of seizures, we performed SPECT subtraction in 50 patients with temporal lobe epilepsy (TLE). The patients were categorized into five groups according to semiologic progression during ictal SPECT (group-1 having only aura; group-2 having motionless staring with or without aura; group-3 having motionless staring and then automatism with or without aure; group-4 having motionless staring and then automatism with or withour aura; group-4 having motionless staring and then dystonic posturing with or without aura and automatism; group-5 having motionless staring, automatism, then head version and generalized seizures with or without aura and dystonic posturing). In group-1, three patients showed ipsilateral temporal hyperperfusion and two had bilateral temporal hyperperfusion with ipsilateral predominance. In group-2, three (42.9%) patients showed bilateral temporal hyperperfusion with unilateral predominance and four (57.1%) revealed insular hyperperfusion of epileptic side. In group-3, 15 patients (88.2%) showed bilateral temporal hyperperfusion with unilateral predominance and 12 (70.6%) insular hyperperfusion. In group-4, 11 patients (84.6%) showed basal ganglia hyperperfusion on the opposite hemisphere to the side of the dystonic posturing. In group-5, there were multiple hyperperfusion areas in the frontal, temporal and basal ganglia regions. However, the injection times of radiotracer in five groups were relatively short and similar. The semiologic progression in TLE seizures were related to the propagation of hyperperfusion from ipsilateral temporal lobe to contralateral temporal lobe, insula, basal ganglia, and frontal lobe. Not only the radiotracer injection time but also semiologic progression after the injection was significant in determining hyperperfusion pattern of ictal SPECT.

Autoimmune encephalitis with anti-leucine-rich glioma-inactivated 1 or anti-contactin-associated protein-like 2 antibodies (formerly called voltage-gated potassium channel-complex antibodies).

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Bastiaansen, Anna E M; van Sonderen, Agnes; Titulaer, Maarten J

2017-06-01

Twenty years since the discovery of voltage-gated potassium channel (VGKC)-related autoimmunity; it is currently known that the antibodies are not directed at the VGKC itself but to two closely associated proteins, anti-leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2). Antibodies to LGI1 and Caspr2 give well-described clinical phenotypes. Anti-LGI1 encephalitis patients mostly have limbic symptoms, and anti-Caspr2 patients have variable syndromes with both central and peripheral symptoms. A large group of patients with heterogeneous symptoms are VGKC positive but do not have antibodies against LGI1 or Caspr2. The clinical relevance of VGKC positivity in these 'double-negative' patients is questionable. This review focusses on these three essentially different subgroups. The clinical phenotypes of anti-LGI1 encephalitis and anti-Caspr2 encephalitis have been described in more detail including data on treatment and long-term follow-up. A specific human leukocyte antigen (HLA) association was found in nontumor anti-LGI1 encephalitis, but not clearly in those with tumors. There has been increasing interest in the VGKC patients without LGI1/Caspr2 antibodies questioning its relevance in clinical practice. Anti-LGI1 encephalitis and anti-Caspr2 encephalitis are separate clinical entities. Early recognition and treatment is necessary and rewarding. The term VGKC-complex antibodies, lumping patients with anti-LGI1, anti-Caspr2 antibodies or lacking both, should be considered obsolete.

What should you know about limbic encephalitis?

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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.

Neuropsychological and FDG-PET profiles in VGKC autoimmune limbic encephalitis.

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Dodich, Alessandra; Cerami, Chiara; Iannaccone, Sandro; Marcone, Alessandra; Alongi, Pierpaolo; Crespi, Chiara; Canessa, Nicola; Andreetta, Francesca; Falini, Andrea; Cappa, Stefano F; Perani, Daniela

2016-10-01

Limbic encephalitis (LE) is characterized by an acute or subacute onset with memory impairments, confusional state, behavioral disorders, variably associated with seizures and dystonic movements. It is due to inflammatory processes that selectively affect the medial temporal lobe structures. Voltage-gate potassium channel (VGKC) autoantibodies are frequently observed. In this study, we assessed at the individual level FDG-PET brain metabolic dysfunctions and neuropsychological profiles in three autoimmune LE cases seropositive for neuronal VGKC-complex autoantibodies. LGI1 and CASPR2 potassium channel complex autoantibody subtyping was performed. Cognitive abilities were evaluated with an in-depth neuropsychological battery focused on episodic memory and affective recognition/processing skills. FDG-PET data were analyzed at single-subject level according to a standardized and validated voxel-based Statistical Parametric Mapping (SPM) method. Patients showed severe episodic memory and fear recognition deficits at the neuropsychological assessment. No disorder of mentalizing processing was present. Variable patterns of increases and decreases of brain glucose metabolism emerged in the limbic structures, highlighting the pathology-driven selective vulnerability of this system. Additional involvement of cortical and subcortical regions, particularly in the sensorimotor system and basal ganglia, was found. Episodic memory and fear recognition deficits characterize the cognitive profile of LE. Commonalities and differences may occur in the brain metabolic patterns. Single-subject voxel-based analysis of FDG-PET imaging could be useful in the early detection of the metabolic correlates of cognitive and non-cognitive deficits characterizing LE condition. Copyright © 2016 Elsevier Inc. All rights reserved.

Progression of CT scan findings in Rett syndrome

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Suzuki, Hisaharu; Hirayama, Yoshito; Sakuragawa, Norio; Arima, Masataka (National Center of Neurology and Psychiatry, Kodaira, Tokyo (Japan))

1989-07-01

Progression of the lesions revealed by CT scan was observed in five girls with Rett syndrome. The most distinct and common finding was progressive dilatation of Sylvian fissures, frontal extracerebral space, interhemispheric fissure, and sulci mainly in frontal lobe. It may indicate progressive lesion in the frontal and the temporal lobes. In addition, dilatation of the anterior horns of the lateral ventricles and the third ventricle was noted in some cases. Brainstem and cerebellum were small at any age with some morphological development as the patients became elder. Neither malformations nor abnormalities in density were found in any case. It is concluded that the main lesion of Rett syndrome on CT scan is progressive and localized in the frontal and the temporal lobes. (author).

Serological and molecular epidemiology of Japanese encephalitis virus infections in swine herds in China, 2006–2012

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Chai, Chunxia; Wang, Qiao; Cao, Sanjie; Zhao, Qin; Wen, Yiping; Huang, Xiaobo; Wen, Xintian; Yan, Qiguai; Ma, Xiaoping

2018-01-01

Japanese encephalitis virus (JEV) is a mosquito-borne, zoonotic flavivirus causing viral encephalitis in humans and reproductive disorder in swine. JEV is prevalent throughout China in human; however, spatiotemporal analysis of JEV in Chinese swine herds has not been reported previously. Herein, we present serological and molecular epidemiological results and estimates of prevalence of JEV infections among swine herds in various regions of China. The results suggest that JEV infections are widespread and genotype I and III strains co-exist in the same regions. Therefore, there is an urgent need to monitor JEV infection status among swine herds in China. PMID:28693301

Tick-borne encephalitis: a disease neglected by travel medicine.

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Haditsch, Martin; Kunze, Ursula

2013-01-01

Tick-borne encephalitis (TBE) is a vector-borne disease that is primarily transmitted to humans by infected ticks and causes infection of the central nervous system. Clinical presentations range from meningitis to encephalitis with or without myelitis, and infection may result in death or long-term neurological sequelae. TBE is endemic in regions of at least 27 European as well as in some Asian countries. Infection and disease, however, can be averted successfully by tick-bite prevention and active vaccination. The risk of infection has shifted from daily life and occupational exposure to leisure-time activities, including travelling. Outdoor activities during the tick season with contact with nature increase the risk of tick bites. Although the number of travel-associated cases is unknown, it is certainly under-estimated because there is hardly any awareness of TBE in non-endemic countries. Therefore, the majority of cases remain undiagnosed, also because of the lack of diagnostic serology, as there is no routine screening for TBE in non-endemic regions. Because of the increasing number of travellers from TBE non-endemic to endemic regions, and in view of the fact that TBE was included in the list of notifiable diseases in the European Union in September 2012, this disease needs to become an important issue in travel medicine. Copyright © 2013 Elsevier Ltd. All rights reserved.

European Aedes albopictus and Culex pipiens Are Competent Vectors for Japanese Encephalitis Virus.

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Mélissanne de Wispelaere

2017-01-01

Full Text Available 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.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.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.

Clinically significant response to zolpidem in disorders of consciousness secondary to anti-N-methyl-D-aspartate receptor encephalitis in a teenager: a case report.

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Appu, Merveen; Noetzel, Michael

2014-03-01

Anti-N-methyl-d-aspartate receptor encephalitis has been associated with a prolonged neuropsychiatric phase that may last for months to years. We report the case of a 16-year-old girl who was diagnosed with anti-N-methyl-d-aspartate receptor encephalitis resulting from left ovarian mature teratoma 2 weeks after presentation with psychosis. Following tumor removal and immunotherapy, recovery from a minimally conscious state was accelerated significantly by zolpidem that was used for her sleep disturbance. Our patient was discharged home 8 weeks after admission with marked improvement in her neurological function. Zolpidem has been reported to improve arousal in disorders of consciousness but there are no previous reports of its benefit among patients with anti-N-methyl-d-aspartate receptor encephalitis. Zolpidem would be a reasonable consideration as an adjunctive treatment in anti-N-methyl-d-aspartate receptor encephalitis after tumor removal and immunotherapy to accelerate recovery and rehabilitation. Copyright © 2014 Elsevier Inc. All rights reserved.

A unique combination of autoimmune limbic encephalitis, type 1 diabetes, and Stiff person syndrome associated with GAD-65 antibody

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Chandra Mohan Sharma

2016-01-01

Full Text Available Antibodies to GAD-65 have been implicated in the pathogenesis of type 1 diabetes , limbic encephalitis and Stiff person syndrome, however these diseases rarely occur concurrently. We intend to present a rare case of 35 year old female who was recently diagnosed as having type 1 diabetes presented with 1½ month history of recurrent seizures, subacute onset gait ataxia, dysathria, psychiatric disturbance and cognitive decline. No tumor was found on imaging and the classic paraneoplastic panel was negative. Cerebrospinal fluid and blood was positive for GAD-65 antibodies.Patient showed significant improvement with immunomodulatory therapy. Association of GAD-65 antibodies has been found with various disorders including type 1 diabetes, limbic encephalitis, Stiff person syndrome,cerebellar ataxia and palatal myoclonus.This case presents with unique combination of type 1 diabetes, Stiff person syndrome and limbic encephalitis associated with GAD-65 antibodies that is responsive to immunotherapy. It also highlights the emerging concept of autoimmunity in the causation of various disorders and there associations.

MRI findings in the diagnosis and monitoring of rasmussen's encephalitis Achados de RM no diagnóstico e monitorização da encefalite de Rasmussen

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Andréia V. Faria

2009-09-01

Full Text Available Rasmussen's encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. Magnetic resonance (MR findings, associated with clinical data and electroencephalogram (EEG, may indicate the diagnosis and could be an indicative of prognosis. We studied 5 patients with Rasmussen's encephalitis, assessing clinical history and MR images. All patients had refractory focal seizures with a predominant motor component associated with hemispheric atrophy, that was proportional to severity of disease and neurological deficits in these patients. Gray and white matter abnormal signal on T2 MR images were found in patients who had hemiparesis. It was not related to the duration of the disease but to aggressiveness. MR proton spectroscopy in severe disease showed lactate and choline increase and decreased NAA, reflecting neuronal and axonal loss, gliosis and elevated membrane turnover and recent - crisis (not controlled. MR studies, in addition to help in diagnosis, may be useful for monitoring metabolic changes and progression of disease in Rasmussen's encephalitis.A encefalite de Rasmussen é uma devastadora síndrome com disfunção cerebral multifocal e convulsões focais. Achados de ressonância magnética (RM, associados aos dados clínicos e de eletrencefalograma (EEG, podem indicar o diagnóstico e podem ser indicativos de prognóstico. Foram estudados 5 pacientes com encefalite de Rasmussen, avaliando a história clínica e imagens de RM. Todos os pacientes apresentavam crises epilépticas focais refratárias com componente predominantemente motor associadas à atrofia hemisférica, que foi proporcional à gravidade da doença e déficits neurológicos nestes pacientes. Alteração da intensidade de sinal nas substâncias branca e cinzenta, nas sequências ponderadas em T2, foram encontradas nos pacientes com hemiparesia. Ela não estava relacionada com a duração da doença, mas à severidade. A espectroscopia de pr

Extended Clinical Spectrum of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Children: A Case Series.

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Goenka, Ajay; Jain, Vivek; Nariai, Hiroki; Spiro, Alfred; Steinschneider, Mitchell

2017-07-01

There is a wide spectrum of clinical manifestations in children with anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis from two different health care settings. We describe our experience with 13 patients (median age, 7 years; range, 5 months to 19 years) presenting to tertiary referral centers in India and the United States. Initial manifestations were neurological (seizures or movement disorders) in eight patients, and psychiatric (e.g., emotional lability and hallucination) in five patients. Symptoms during the clinical course included seizures in ten patients, movement disorders (dyskinesia and choreiform movements) in 11 patients, and behavioral changes (aggressiveness and insomnia) in ten patients. Concomitant infections (herpes simplex virus 1, tuberculous meningitis, and influenza A) were present in three patients. Analysis of the cerebrospinal fluid in all except two cases preceded by infection (herpes simplex virus encephalitis and tuberculous meningitis) was unremarkable. Treatment included intravenous immunoglobulin/methylprednisolone (11 patients), rituximab (eight patients), plasmapheresis (two patients), and cyclophosphamide (two patients). Six patients recovered completely. Two patients had mild residual neurological deficits, whereas four had severe residual neurological deficits. Two patients had profound autonomic instability, which was the cause of death for one of them. Two patients relapsed at two and six months after the initial recovery. We describe the differences and similarities of clinical presentation, test results, and response to treatment of children with anti-N-methyl-d-aspartate receptor encephalitis from India and the United States. Included is a description of one of the youngest patients with anti-N-methyl-d-aspartate receptor encephalitis (five months) and the first patient to be reported in association with tuberculous meningitis. Copyright © 2017 Elsevier Inc. All rights reserved.

West Nile Virus Encephalitis in a Patient with Neuroendocrine Carcinoma

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Romina Deldar

2016-01-01

Full Text Available Importance. Oftentimes, when patients with metastatic cancer present with acute encephalopathy, it is suspected to be secondary to their underlying malignancy. However, there are multiple causes of delirium such as central nervous system (CNS infections, electrolyte abnormalities, and drug adverse reactions. Because West Nile Virus (WNV neuroinvasive disease has a high mortality rate in immunosuppressed patients, a high index of suspicion is required in patients who present with fever, altered mental status, and other neurological symptoms. Observations. Our case report details a single patient with brain metastases who presented with unexplained fever, encephalopathy, and new-onset tremors. Initially, it was assumed that his symptoms were due to his underlying malignancy or seizures. However, because his unexplained fevers persisted, lumbar puncture was pursued. Cerebrospinal fluid analysis included WNV polymerase chain reaction and serologies were ordered which eventually led to diagnosis of WNV encephalitis. Conclusions and Relevance. Patients with metastatic cancer who present with encephalopathy are often evaluated with assumption that malignancy is the underlying etiology. This can lead to delays in diagnosis and possible mistreatment. Our case highlights the importance of maintaining a broad differential diagnosis and an important diagnostic consideration of WNV encephalitis in patients with cancer.

Embodiment of activity progress: The temporalities of service evaluation

DEFF Research Database (Denmark)

Oshima, Sae

2017-01-01

their satisfaction. Yet, an efficient progress of this activity is crucial, as there are often subsequent customers waiting. My analysis shows that this dilemma of taking enough time without taking too much time is managed by the participants’ embodiment of valid activity progress, which is realized through...... their (sometimes asynchronous) mobilization of multimodal resources. I argue that such activity organization helps participants not only to embody the meaningful (versus wasted) consumption of time, but also to secure the customer’s enhanced appreciation of the service outcome....

Increasing trend of Japanese encephalitis cases in West Bengal, India - a threat to paediatric population

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Debjani Taraphdar

2012-10-01

Full Text Available Objective: To detect the Japanese encephalitis virus (JEV as the etiologic agent from the acute encephalitis syndrome (AES cases mainly amongst the children and young adults from vaccinated and non-vaccinated districts of West Bengal. Methods: For the detection of JEV, a total of 828 sera were referred from vaccinated and non vaccinated districts of West Bengal during 2005-2011. Japanese encephalitis (JE positive cases were confirmed by ELISA and RT-PCR method. Results: Out of 828 cases, 245 samples were positive by ELISA method and 46 samples were positive by RT-PCR method. Out of 291 total positive cases, 162 (55.6% were below 20 years of age. Initially in 2005, JE cases were highest amongst the children and young adults (0-20 years. After vaccination, although the JE cases declined gradually in the vaccinated districts, but again from 2010, JE cases from the said age group showed an increasing trend from those districts. JE cases were also reported from other endemic zones of this state, which were still non-vaccinated. Conclusions: In West Bengal, JE cases are still predominated among children and young adults till the year 2011. Mass scale vaccination programme and investigation on the circulating strains are essentially required to find out the reasons of increasing tendency of JE cases in this state.

[Characteristics of clinical features of tick-borne encephalitis in Crimea].

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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.

Anti–N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents

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Florance, Nicole R.; Davis, Rebecca L.; Lam, Christopher; Szperka, Christina; Zhou, Lei; Ahmad, Saba; Campen, Cynthia J.; Moss, Heather; Peter, Nadja; Gleichman, Amy J.; Glaser, Carol A.; Lynch, David R.; Rosenfeld, Myrna R.; Dalmau, Josep

2010-01-01

Objective To report the clinical features of anti–N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients ≤ 18 years old. Methods Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1. Results Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were ≤18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women >18 years old, 31% in girls ≤18 years old (p = 0.05), and 9% in girls ≤14 years old ( p = 0.008). None of the male patients had tumors. Of 32 patients ≤18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03). Interpretation Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children. PMID:19670433

Unusual amnesia in a patient with VGKC-Ab limbic encephalitis: a case study.

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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. Copyright © 2010 Elsevier Srl. All rights reserved.

Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies.

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Modoni, Anna; Masciullo, Marcella; Spinelli, Pietro; Marra, Camillo; Tartaglione, Tommaso; Andreetta, Francesca; Tonali, Pietro; Silvestri, Gabriella

2009-03-01

To describe a case of acute nonherpetic limbic encephalitis (LE) with negative testing for antibodies directed against onconeuronal and cell membrane antigens, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, that showed a dramatic response to immune therapy. A 30-year-old woman manifested generalized seizures, altered consciousness, and memory impairment shortly after a prodromal viral illness. Few days later the patient developed a drug-resistant epileptic status. Electroencephalograph showed bitemporal slowing and paroxysmal slow wave bursts. Brain magnetic resonance imaging showed bilateral swelling in the medial temporal lobes. Cerebrospinal fluid analysis ruled out viral etiologies. A diagnostic search for cancer, including serum testing for known onconeuronal antibodies proved negative. Screening for cell membrane antigen antibodies, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, was also negative. Suspecting an autoimmune etiology, we started an immunomodulatory treatment with intravenous immunoglobulin followed by a short course of oral prednisone, obtaining a full clinical recovery. Our report confirms previous observations of "seronegative" autoimmune LE, suggesting the presence of other, still unknown central nervous system antigens representing a target of a postinfectious, autoimmune response in these patients. Moreover, it emphasizes the importance of early recognition and treatment of acute autoimmune LE, to reduce the risk of intensive care unit-related complications and the occurrence of permanent cognitive or behavioral defects.

MRI findings in eastern equine encephalitis: the "parenthesis" sign.

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Nickerson, Joshua P; Kannabiran, Suma; Burbank, Heather N

2016-01-01

Two patients with eastern equine encephalitis (EEE) presented to a tertiary referral center. Both subjects' brain magnetic resonance imaging showed T2/FLAIR (fluid-attenuated inversion recovery) hyperintensities including linear areas of hyperintensity in the external and internal capsules with sparing of the lentiform nuclei. Single case reports of imaging findings in EEE exist with nonspecific patterns of abnormality. We propose that this "( ) parentheses sign" on T2 or FLAIR imaging may distinguish EEE from other processes. Copyright © 2015 Elsevier Inc. All rights reserved.

General Temporal Knowledge for Planning and Data Mining

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Morris, Robert; Khatib, Lina

2001-01-01

We consider the architecture of systems that combine temporal planning and plan execution and introduce a layer of temporal reasoning that potential1y improves both the communication between humans and such systems, and the performance of the temporal planner itself. In particular, this additional layer simultaneously supports more flexibility in specifying and maintaining temporal constraints on plans within an uncertain and changing execution environment, and the ability to understand and trace the progress of plan execution. It is shown how a representation based on single set of abstractions of temporal information can be used to characterize the reasoning underlying plan generation and execution interpretation. The complexity of such reasoning is discussed.

Anti-Ma-associated encephalitis due to dysgerminoma in a woman with Swyer syndrome.

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Al-Thubaiti, Ibtisam; Al-Hayek, Kefah; Binfalah, Mohammed

2013-04-09

Paraneoplastic limbic encephalitis (PLE)/diencephalitis associated with anti-Ma2 antibodies was linked to testicular cancer and non-small-cell lung cancer (non-SCLC).(1,2) We report a case of anti-Ma-associated PLE/diencephalitis due to dysgerminoma in a woman with gonadal dysgenesis, or Swyer syndrome.

Tick-borne Encephalitis Associated with Consumption of Raw Goat Milk, Slovenia, 2012

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Hudopisk, Neda; Korva, Miša; Janet, Evgen; Simetinger, Marjana; Grgič-Vitek, Marta; Gubenšek, Jakob; Natek, Vladimir; Kraigher, Alenka; Strle, Franc

2013-01-01

Tick-borne encephalitis (TBE) developed in 3 persons in Slovenia who drank raw milk; a fourth person, who had been vaccinated against TBE, remained healthy. TBE virus RNA was detected in serum and milk of the source goat. Persons in TBE-endemic areas should be encouraged to drink only boiled/pasteurized milk and to be vaccinated. PMID:23697658

Mice with different susceptibility to tick-borne encephalitis virus infection show selective neutralizing antibody response and inflammatory reaction in the central nervous system

Czech Academy of Sciences Publication Activity Database

Palus, Martin; Vojtíšková, Jarmila; Salát, Jiří; Kopecký, Jan; Grubhoffer, Libor; Lipoldová, Marie; Demant, P.; Růžek, Daniel

2013-01-01

Roč. 10, JUN 2013 (2013), s. 77 E-ISSN 1742-2094 R&D Projects: GA ČR GAP502/11/2116 Grant - others:GA MŠk(CZ) ED0006/01/01 Institutional support: RVO:60077344 ; RVO:68378050 Keywords : Tick-borne encephalitis * Flavivirus encephalitis * Neuroinflammation * Antibody production Subject RIV: EC - Immunology; EB - Genetics ; Molecular Biology (UMG-J) Impact factor: 4.902, year: 2013

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

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

An Ecological Survey of Mosquitoes and the Distribution of Japanese Encephalitis Virus in Ishikawa Prefecture, Japan, between 2010 and 2014.

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Murakami, Manabu; Hori, Kiyoe; Kitagawa, Yoko; Oikawa, Yosaburo; Kamimura, Kiyoshi; Takegami, Tsutomu

2017-07-24

Japanese encephalitis virus (JEV) is a flavivirus, responsible for over 30,000 annual cases of encephalitis worldwide, with a mortality rate of approximately 30%. Therefore, it is important to examine the distribution of mosquitos carrying JEV in the fields, even though recently, the number of Japanese encephalitis cases has been approximately 5 per year in Japan. We report the seasonal dynamics of mosquitoes between 2010 and 2014 in Ishikawa Prefecture, Japan. We collected 39,308 female adult mosquitoes, 98.2% of which were classified as Culex tritaeniorhynchus Giles. We identified JEV genomic RNA belonging to genotype 1 from the homogenate of Cx. tritaeniorhynchus, collected during our study using reverse transcription-PCR and nucleotide sequencing techniques. Our results indicate that mosquito vectors for JEV are distributed not only in areas in Ishikawa, but also throughout Japan, and the results suggest that we must be careful regarding JEV outbreaks in Japan in the future.

Host genetic risk factors for West Nile virus infection and disease progression.

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Abigail W Bigham

Full Text Available West Nile virus (WNV, a category B pathogen endemic in parts of Africa, Asia and Europe, emerged in North America in 1999, and spread rapidly across the continental U.S. Outcomes of infection with WNV range from asymptomatic to severe neuroinvasive disease manifested as encephalitis, paralysis, and/or death. Neuroinvasive WNV disease occurs in less than one percent of cases, and although host genetic factors are thought to influence risk for symptomatic disease, the identity of these factors remains largely unknown. We tested 360 common haplotype tagging and/or functional SNPs in 86 genes that encode key regulators of immune function in 753 individuals infected with WNV including: 422 symptomatic WNV cases and 331 cases with asymptomatic infections. After applying a Bonferroni correction for multiple tests and controlling for population stratification, SNPs in IRF3 (OR 0.54, p = 0.035 and MX1, (OR 0.19, p = 0.014 were associated with symptomatic WNV infection and a single SNP in OAS1 (OR 9.79, p = 0.003 was associated with increased risk for West Nile encephalitis and paralysis (WNE/P. Together, these results suggest that genetic variation in the interferon response pathway is associated with both risk for symptomatic WNV infection and WNV disease progression.

Chandipura virus infection causing encephalitis in a tribal population of Odisha in eastern India.

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Dwibedi, Bhagirathi; Sabat, Jyotsnamayee; Hazra, Rupenangshu K; Kumar, Anu; Dinesh, Diwakar Singh; Kar, Shantanu K

2015-01-01

The sudden death of 10 children in a tribal village of Kandhamal district, Odisha in eastern India led to this investigation. We conducted a door-to-door survey to identify cases. Antibodies for Chandipura, Japanese encephalitis, dengue, chikungunya and West Nile viruses were tested by ELISA in probable cases. Chandipura virus RNA was tested from both human blood samples and sand flies by reverse transcriptase polymerase chain reaction. We conducted vector surveys in domestic and peridomestic areas, and collected sand flies. Entomological investigations revealed the presence of Phlebotomus argentipes and Sergentomiya sp. Thirty-five patients presented with fever, 12 of them had altered sensorium including 4 who had convulsions. The blood samples of 21 patients were tested; four samples revealed Chandipura virusspecific IgM antibody. Chandipura virus infection causing encephalitis affected this tribal population in eastern India at 1212 m above sea level. Copyright 2015, NMJI.

Efficacy of eastern equine encephalitis immunization in whooping cranes.

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Olsen, G H; Turell, M J; Pagac, B B

1997-04-01

An epizootic of eastern equine encephalitis (EEE) at the Patuxent Wildlife Research Center (PWRC), Laurel, Maryland (USA), in 1989 provided an opportunity to determine if EEE immunization protected whooping cranes (Grus americana). Based on seroconversion of 31% of sympatric hatch-year sandhill cranes, Grus canadensis, and a previous 35% case fatality rate in whooping cranes, 17 (37%) of the 46 susceptible whooping cranes should have been exposed to virus and six should have died. As there were no deaths in these birds, the EEE vaccination program appeared to be efficacious in this whooping crane population.

Herpes simplex encephalitis with onset of acute headache simulating subarachnoid hemorrhage

International Nuclear Information System (INIS)

Oana, Katsumaro; Tomita, Yukio; Kubo, Naohiko; Kanaya, Haruyuki.

1983-01-01

On examination in our clinic, he showed alert consciousness, with nuchal rigidity and left weakness. A lumbar puncture showed an opening pressure of 125 mm H 2 O, xanthochromic in nature, and the cerebrospinal fluid contained 40 white cells per cubic millimeter, mostly lymphocytes, though the total protein and glucose contents were normal. Blood, general, and chemical examinations showed a normal white-cell count and increasing titers of GPT (420), GOT (203), and LDH (723). A computed-tomographic scan of the brain on the day of admission revealed bilateral frontal and right temporal abnormal low-density areas, greater on the right side, and contrast enhancement in the bilateral frontal and right paraventricular regions. Cerebral angiography demonstrated a marked hypervascularity in the bilateral frontal regions in the arterial phase. A diagnosis of herpes simplex encephalitis was made on the basis of the clinical course and the angiographic and computed-tomographicscan findings. On the day of admission, the patient vomited once. On the third hospital day, he complained of a headache and became somnolent. After that, a mild fever continued for two weeks. On the eleventh hospital day, he occasionally vomited after a headache. On the fifteenth hospital day, a repeat spinal tap was performed with an opening pressure of 150 mm H 2 O. The total white blood cell count was 25/cu mm. On the twenty-fourth hospital day, the headache and vomiting disappeared, and the fever also subsided. He was discharged about two months later with minimal mental abnormality. (J.P.N)

Transplantation of organs from deceased donors with meningitis and encephalitis: a UK registry analysis.

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Trotter, Patrick B; Robb, Matthew; Hulme, William; Summers, Dominic M; Watson, Christopher J E; Bradley, J Andrew; Neuberger, James

2016-12-01

Deceased organ donors, where the cause of death is meningitis or encephalitis, are a potential concern because of the risks of transmission of a potentially fatal infection to recipients. Using the UK Transplant Registry, a retrospective cohort analysis of deceased organ donors in the UK was undertaken to better understand the extent to which organs from deceased donors with meningitis and/or encephalitis (M/E) (of both known and unknown cause) have been used for transplantation, and to determine the associated recipient outcomes. Between 2003 and 2015, 258 deceased donors with M/E were identified and the causative agent was known in 188 (72.9%). These donors provided 899 solid organs for transplantation (455 kidneys and 444 other organs). The only recorded case of disease transmission was from a donor with encephalitis of unknown cause at time of transplantation who transmitted a fatal nematode infection to 2 kidney transplant recipients. A further 3 patients (2 liver and 1 heart recipient) died within 30 days of transplantation from a neurological cause (cerebrovascular accident) with no suggestion of disease transmission. Overall, patient and graft survival in recipients of organs from donors with M/E were similar to those for all other types of deceased organ donor. Donors dying with M/E represent a valuable source of organs for transplantation. The risk of disease transmission is low but, where the causative agent is unknown, caution is required. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Focal CA3 hippocampal subfield atrophy following LGI1 VGKC-complex antibody limbic encephalitis.

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Miller, Thomas D; Chong, Trevor T-J; Aimola Davies, Anne M; Ng, Tammy W C; Johnson, Michael R; Irani, Sarosh R; Vincent, Angela; Husain, Masud; Jacob, Saiju; Maddison, Paul; Kennard, Christopher; Gowland, Penny A; Rosenthal, Clive R

2017-05-01

Magnetic resonance imaging has linked chronic voltage-gated potassium channel (VGKC) complex antibody-mediated limbic encephalitis with generalized hippocampal atrophy. However, autoantibodies bind to specific rodent hippocampal subfields. Here, human hippocampal subfield (subiculum, cornu ammonis 1-3, and dentate gyrus) targets of immunomodulation-treated LGI1 VGKC-complex antibody-mediated limbic encephalitis were investigated using in vivo ultra-high resolution (0.39 × 0.39 × 1.0 mm3) 7.0 T magnetic resonance imaging [n = 18 patients, 17 patients (94%) positive for LGI1 antibody and one patient negative for LGI1/CASPR2 but positive for VGKC-complex antibodies, mean age: 64.0 ± 2.55 years, median 4 years post-limbic encephalitis onset; n = 18 controls]. First, hippocampal subfield quantitative morphometry indicated significant volume loss confined to bilateral CA3 [F(1,34) = 16.87, P 3 months from symptom onset) were associated with CA3 atrophy. Third, whole-brain voxel-by-voxel morphometry revealed no significant grey matter loss. Fourth, CA3 subfield atrophy was associated with severe episodic but not semantic amnesia for postmorbid autobiographical events that was predicted by variability in CA3 volume. The results raise important questions about the links with histopathology, the impact of the observed focal atrophy on other CA3-mediated reconstructive and episodic mechanisms, and the role of potential antibody-mediated pathogenicity as part of the pathophysiology cascade in humans. © The Author (2017). Published by Oxford University Press on behalf of the Guarantors of Brain.

Manipulation of host factors optimizes the pathogenesis of western equine encephalitis virus infections in mice for antiviral drug development

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Blakely, Pennelope K.; Delekta, Phillip C.; Miller, David J.; Irani, David N.

2014-01-01

While alphaviruses spread naturally via mosquito vectors, some can also be transmitted as aerosols making them potential bioterrorism agents. One such pathogen, western equine encephalitis virus (WEEV), causes fatal human encephalitis via multiple routes of infection and thus presumably via multiple mechanisms. Although WEEV also produces acute encephalitis in non-human primates, a small animal model that recapitulates features of human disease would be useful for both pathogenesis studies and to evaluate candidate antiviral therapies. We have optimized conditions to infect mice with a low passage isolate of WEEV, thereby allowing detailed investigation of virus tropism, replication, neuroinvasion, and neurovirulence. We find that host factors strongly influence disease outcome, and in particular that age, gender and genetic background all have significant effects on disease susceptibility independent of virus tropism or replication within the central nervous system. Our data show that experimental variables can be adjusted in mice to recapitulate disease features known to occur in both non-human primates and humans, thus aiding further study of WEEV pathogenesis and providing a realistic therapeutic window for antiviral drug delivery. PMID:25361697

Combined CMV- and HSV-1 brainstem encephalitis restricted to medulla oblongata.

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Katchanov, J; Branding, G; Stocker, H

2014-04-15

We report a very rare case of a combined CMV- and HSV-1 isolated brainstem encephalitis restricted to medulla oblongata in a patient with advanced HIV disease. Neither limbic nor general ventricular involvement was detected on neuroimaging. The case highlights the importance of testing for HSV-1 and CMV in HIV-infected patients presenting with an isolated brainstem syndrome. Copyright © 2014 Elsevier B.V. All rights reserved.

Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

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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.

Randomized, controlled trial of oral ribavirin for Japanese encephalitis in children in Uttar Pradesh, India.

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Kumar, Rashmi; Tripathi, Piyush; Baranwal, Madan; Singh, Sudhakar; Tripathi, Sanjeev; Banerjee, Gopa

2009-02-15

Japanese encephalitis is associated with high rates of mortality and disabling sequelae. To date, no specific antiviral has proven to be of benefit for this condition. We attempted to determine the efficacy of oral ribavirin treatment for reducing early mortality among children with Japanese encephalitis in Uttar Pradesh, India. Children (age, 6 months to 15 years) who had been hospitalized with acute febrile encephalopathy (a syrup through nasogastric tube or by mouth. The primary outcome was early mortality; secondary outcome measures were early (at hospital discharge; normal or nearly normal, independent functioning, dependent, vegetative state, or death) outcome, time to resolution of fever, time to resumption of oral feeding, duration of hospitalization, and late outcome (> or =3 months after hospital discharge). The study was double-blind, and analysis was by intention to treat. A total of 153 patients were enrolled during a 3-year period; 70 patients received ribavirin, and 83 received placebo. There was no statistically significant difference between the 2 groups in the early mortality rate: 19 (27.1%) of 70 ribavirin recipients and 21 (25.3%) of 83 placebo recipients died (odds ratio, 1.10; 95% confidence interval, 0.5-2.4). No statistically significant differences in secondary outcome measures were found. For the dosage schedule used in our study, oral ribavirin has no effect in reducing early mortality associated with Japanese encephalitis. ClinicalTrials.gov identifier: NCT00216268 .

Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica.

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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. Copyright © 2015 Elsevier Ltd. All rights reserved.

Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review.

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Ben Achour, Nedia; Ben Younes, Thouraya; Rebai, Ibtihel; Ben Ahmed, Melika; Kraoua, Ichraf; Ben Youssef-Turki, Ilhem

2018-05-01

Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal. Repeat screening for antineuronal antibodies showed negative results for anti-NMDA receptor antibodies but positive results for anti-GAD65 with a low positivity of anti-Ma2 antibodies. Although a transient improvement was noticed after immunomodulatory treatment, the patient developed severe intractable autonomic imbalance including dysrythmia, alternating bradycardia/tachycardia, hypotension/hypertension, hypothermia/hyperthermia and hyperhidrosis. She deceased six months after onset. Our report intends to raise awareness of autoimmune encephalitis with anti-GAD65 antibodies which may involve extralimbic brain regions and manifest with fatal dysautonomia. We highlight the need for prompt diagnosis and aggressive management for this underdiagnosed entity in children. Copyright © 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Unilateral temporal myositis heralding polymyositis: ultrasonographic and elastographic findings. Case report.

Science.gov (United States)

Damian, Laura; Botar Jid, Carolina; Rogojan, Liliana; Dinu, Cristian; Maniu, Alma; Fodor, Daniela; Rednic, Simona; Simon, Siao-Pin

2016-03-01

Temporal myositis is a rare inflammatory disease of the temporal muscle. We report a case of unilateral temporal myositis, in which a polymyositis was diagnosed two years thereafter. Although focal myositis may rarely herald polymyositis, isolated temporal myositis preceding inflammatory myopathies has not been described, to our knowledge. In the setting of a temporal pain and swelling, ultrasonography may help in diagnosis, biopsy guidance, disease extension, and progression assessment. Further studies are necessary to establish the role of elastography in differentiating between muscle inflammation and hypertrophy.

Creutzfeldt-Jakob Disease-Like Periodic Sharp Wave Complexes in Voltage-Gated Potassium Channel-Complex Antibodies Encephalitis: A Case Report.

Science.gov (United States)

Savard, Martin; Irani, Sarosh R; Guillemette, Annie; Gosselin-Lefebvre, Stéphanie; Geschwind, Michael; Jansen, Gerard H; Gould, Peter V; Laforce, Robert

2016-02-01

Voltage-gated potassium channel-complex antibodies (VGKC-cAbs) encephalitis, a treatable autoantibody encephalopathy, has been previously reported to clinically mimic sporadic Creutzfeldt-Jakob disease. Among available clinical clues to distinguish them, periodic sharp wave complexes, a typical finding in sporadic Creutzfeldt-Jakob disease, have never been reported in association with VGKC-cAbs encephalitis. A 76-year-old man was transferred to a tertiary neurology center with a clinical history of 6-month weight loss, cognitive disturbance, and nonspecific generalized weakness. He had two seizures the month before transfer and then evolved to severe encephalopathy, requiring mechanical ventilation. Periodic sharp wave complexes every 1 to 2 seconds over slowed background were found on EEG, and MRI showed cerebellar and bifrontal cortical T2/FLAIR/DWI hypersignal without restricted diffusion on ADC mapping. Pancorporal positron emission tomography scan was negative. An immunotherapy trial did not improve the patient condition. Therefore, he died after life support withdrawal. Brain autopsy revealed mononuclear neocortex infiltrate without significant spongiosis, and the anti-VGKC test showed a seropositivity of 336 pmol/L (normal, 0-31), 3 month after the patient deceased. This is the first reported case of VGKC-cAbs encephalitis associated with periodic sharp wave complexes on EEG, which further confuse the differential diagnosis with sporadic Creutzfeldt-Jakob disease. However, the cortical DWI hypersignal without restriction seems to remain a way to discriminate these two entities appropriately, when present. These clues are of paramount importance because VGKC-cAbs encephalitis is a treatable disease.

A case of limbic encephalitis presenting as a paraneoplastic manifestation of limited stage small cell lung cancer: a case report

Directory of Open Access Journals (Sweden)

Butt Mohammad

2010-12-01

Full Text Available Abstract Introduction The differential diagnosis of altered mental status and behavioral change is very extensive. Paraneoplastic limbic encephalitis is a rare cause of cognitive impairment, which should be considered in the differential diagnosis. Case presentation A 64-year-old British Caucasian woman presented to our hospital with a 12-week history of confusion and short-term memory loss. She was hyponatremic with a serum sodium level of 128mmol/L. Moreover, there was evidence of left hilar prominence on the chest radiograph. A thoracic computed tomography scan showed left hilar opacity with confluent lymphadenopathy. A percutaneous biopsy confirmed a diagnosis of small cell lung cancer. There was no radiological evidence of brain metastasis on the computed tomography scan. In view of continued cognitive impairment, which was felt to be disproportionate to hyponatremia, a magnetic resonance imaging scan of the brain was undertaken. It showed hyperintense signals from both hippocampi, highly suggestive of limbic encephalitis presenting as a paraneoplastic manifestation of small cell lung cancer. She had a significant radiological and clinical response following chemotherapy and radiotherapy. Conclusion This case highlights the importance of considering paraneoplastic syndromes in patients with neurological symptoms in the context of lung malignancy. If initial investigations fail to reveal the cause of cognitive impairment in a patient with malignancy, magnetic resonance imaging may be invaluable in the diagnosis of limbic encephalitis. The clinical presentation, diagnostic techniques and management of paraneoplastic limbic encephalitis are discussed in this case report.

Necrotizing Encephalitis Caused by Disseminated Aspergillus Infection after Orthotopic Liver Transplantation

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Luis E. Barrera-Herrera

2015-01-01

Full Text Available Liver transplantation is the only available treatment for some patients with end-stage liver disease. Despite reduction in mortality rates due to advances related to surgical techniques, intensive medical management and immunosuppressive therapy, invasive fungal infections remain a serious complication in orthotopic liver transplantation. We report the case of an 18-year-old male diagnosed with autoimmune cirrhosis in 2009 who was assessed and listed for liver transplantation for massive variceal hemorrhage. One year after listing a successful orthotopic liver transplantation was performed. Uneventful early recovery was achieved; however, he developed pulmonary and neurological Aspergillus infection 23 and 40 days after surgery, respectively. Antibiotic therapy with voriconazole and amphotericin was started early, with no major response. Neuroimaging revealed multiple right frontal and right parietal lesions with perilesional edema; surgical management of the brain abscesses was performed. A biopsy with periodic acid-Schiff and Gomori stains revealed areas with mycotic microorganisms morphologically consistent with Aspergillus, later confirmed by culture. The patient developed necrotizing encephalitis secondary to aspergillosis and died. Necrotizing encephalitis as a clinical presentation of Aspergillus infection in an orthotopic liver transplant is not common, and even with adequate management, early diagnosis and prompt antifungal treatment, mortality rates remain high.

Anti-leucine rich glioma inactivated 1 protein and anti-N-methyl-D-aspartate receptor encephalitis show distinct patterns of brain glucose metabolism in 18F-fluoro-2-deoxy-d-glucose positron emission tomography.

Science.gov (United States)

Wegner, Florian; Wilke, Florian; Raab, Peter; Tayeb, Said Ben; Boeck, Anna-Lena; Haense, Cathleen; Trebst, Corinna; Voss, Elke; Schrader, Christoph; Logemann, Frank; Ahrens, Jörg; Leffler, Andreas; Rodriguez-Raecke, Rea; Dengler, Reinhard; Geworski, Lilli; Bengel, Frank M; Berding, Georg; Stangel, Martin; Nabavi, Elham

2014-06-20

Pathogenic autoantibodies targeting the recently identified leucine rich glioma inactivated 1 protein and the subunit 1 of the N-methyl-D-aspartate receptor induce autoimmune encephalitis. A comparison of brain metabolic patterns in 18F-fluoro-2-deoxy-d-glucose positron emission tomography of anti-leucine rich glioma inactivated 1 protein and anti-N-methyl-D-aspartate receptor encephalitis patients has not been performed yet and shall be helpful in differentiating these two most common forms of autoimmune encephalitis. The brain 18F-fluoro-2-deoxy-d-glucose uptake from whole-body positron emission tomography of six anti-N-methyl-D-aspartate receptor encephalitis patients and four patients with anti-leucine rich glioma inactivated 1 protein encephalitis admitted to Hannover Medical School between 2008 and 2012 was retrospectively analyzed and compared to matched controls. Group analysis of anti-N-methyl-D-aspartate encephalitis patients demonstrated regionally limited hypermetabolism in frontotemporal areas contrasting an extensive hypometabolism in parietal lobes, whereas the anti-leucine rich glioma inactivated 1 protein syndrome was characterized by hypermetabolism in cerebellar, basal ganglia, occipital and precentral areas and minor frontomesial hypometabolism. This retrospective 18F-fluoro-2-deoxy-d-glucose positron emission tomography study provides novel evidence for distinct brain metabolic patterns in patients with anti-leucine rich glioma inactivated 1 protein and anti-N-methyl-D-aspartate receptor encephalitis.

Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies.

Science.gov (United States)

Dalmau, Josep; Gleichman, Amy J; Hughes, Ethan G; Rossi, Jeffrey E; Peng, Xiaoyu; Lai, Meizan; Dessain, Scott K; Rosenfeld, Myrna R; Balice-Gordon, Rita; Lynch, David R

2008-12-01

A severe form of encephalitis associated with antibodies against NR1-NR2 heteromers of the NMDA receptor was recently identified. We aimed to analyse the clinical and immunological features of patients with the disorder and examine the effects of antibodies against NMDA receptors in neuronal cultures. We describe the clinical characteristics of 100 patients with encephalitis and NR1-NR2 antibodies. HEK293 cells ectopically expressing single or assembled NR1-NR2 subunits were used to determine the epitope targeted by the antibodies. Antibody titres were measured with ELISA. The effect of antibodies on neuronal cultures was determined by quantitative analysis of NMDA-receptor clusters. Median age of patients was 23 years (range 5-76 years); 91 were women. All patients presented with psychiatric symptoms or memory problems; 76 had seizures, 88 unresponsiveness (decreased consciousness), 86 dyskinesias, 69 autonomic instability, and 66 hypoventilation. 58 (59%) of 98 patients for whom results of oncological assessments were available had tumours, most commonly ovarian teratoma. Patients who received early tumour treatment (usually with immunotherapy) had better outcome (p=0.004) and fewer neurological relapses (p=0.009) than the rest of the patients. 75 patients recovered or had mild deficits and 25 had severe deficits or died. Improvement was associated with a decrease of serum antibody titres. The main epitope targeted by the antibodies is in the extracellular N-terminal domain of the NR1 subunit. Patients' antibodies decreased the numbers of cell-surface NMDA receptors and NMDA-receptor clusters in postsynaptic dendrites, an effect that could be reversed by antibody removal. A well-defined set of clinical characteristics are associated with anti-NMDA-receptor encephalitis. The pathogenesis of the disorder seems to be mediated by antibodies.

Reversible Dementia: Two Nursing Home Patients With Voltage-Gated Potassium Channel Antibody-Associated Limbic Encephalitis

NARCIS (Netherlands)

Reintjes, W.; Romijn, M.D.M.; den Hollander, D.; ter Bruggen, J.P.; van Marum, R.J.

2015-01-01

Voltage-gated potassium channel antibody-associated limbic encephalitis (VGKC-LE) is a rare disease that is a diagnostic and therapeutic challenge for medical practitioners. Two patients with VGKC-LE, both developing dementia are presented. Following treatment, both patients showed remarkable

Ixodes ricinus tick saliva modulates tick-borne encephalitis virus infection of dendritic cells

Czech Academy of Sciences Publication Activity Database

Fialová, Anna; Cimburek, Zdeněk; Iezzi, G.; Kopecký, Jan

2010-01-01

Roč. 12, č. 7 (2010), s. 580-585 ISSN 1286-4579 R&D Projects: GA AV ČR IAA600960811 Institutional research plan: CEZ:AV0Z60220518; CEZ:AV0Z50200510 Keywords : Tick-borne encephalitis virus * Dendritic cell * Tick saliva * Ixodes ricinus Subject RIV: EC - Immunology Impact factor: 2.726, year: 2010

Radiation injury to the temporal bone

International Nuclear Information System (INIS)

Guida, R.A.; Finn, D.G.; Buchalter, I.H.; Brookler, K.H.; Kimmelman, C.P.

1990-01-01

Osteoradionecrosis of the temporal bone is an unusual sequela of radiation therapy to the head and neck. Symptoms occur many years after the radiation is administered, and progression of the disease is insidious. Hearing loss (sensorineural, conductive, or mixed), otalgia, otorrhea, and even gross tissue extrusion herald this condition. Later, intracranial complications such as meningitis, temporal lobe or cerebellar abscess, and cranial neuropathies may occur. Reported here are five cases of this rare malady representing varying degrees of the disease process. They include a case of radiation-induced necrosis of the tympanic ring with persistent squamous debris in the external auditory canal and middle ear. Another case demonstrates the progression of radiation otitis media to mastoiditis with bony sequestration. Further progression of the disease process is seen in a third case that evolved into multiple cranial neuropathies from skull base destruction. Treatment includes systemic antibiotics, local wound care, and debridement in cases of localized tissue involvement. More extensive debridement with removal of sequestrations, abscess drainage, reconstruction with vascularized tissue from regional flaps, and mastoid obliteration may be warranted for severe cases. Hyperbaric oxygen therapy has provided limited benefit

Autoimmune encephalitis associated with voltage-gated potassium channels-complex and leucine-rich glioma-inactivated 1 antibodies

DEFF Research Database (Denmark)

Celicanin, Marko; Blaabjerg, M; Maersk-Moller, C

2017-01-01

BACKGROUND AND PURPOSE: The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2......, electroencephalography and (18) F-fluorodeoxyglucose positron emission tomography scans were re-evaluated by experts in the field. RESULTS: A total of 28/192 patients tested positive for VGKC-complex antibodies by radioimmunoassay and indirect immunofluorescence; 17 had antibodies to LGI1 and 6/7 of the available....... CONCLUSIONS: Patients diagnosed with anti-LGI1 autoimmune encephalitis increased significantly from 2009 to 2014, probably due to increased awareness. In contrast to seropositive anti-VGKC-complex patients, all anti-LGI1-positive patients presented with a classical limbic encephalitis. The majority...

A rat model for embolic encephalitis

DEFF Research Database (Denmark)

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

2011-01-01

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...... a most interesting organ in sepsis patients. However, symptoms of brain infection may be confused with systemic responses and gross neuropathologic lesions may be absent. Brain infection in sepsis patients is therefore prone to misclassification or diagnostic delay, and when the diagnosis is made...... 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-brain...

[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.

Discovery of a novel compound with anti-venezuelan equine encephalitis virus activity that targets the nonstructural protein 2.

Directory of Open Access Journals (Sweden)

Dong-Hoon Chung

2014-06-01

Full Text Available Alphaviruses present serious health threats as emerging and re-emerging viruses. Venezuelan equine encephalitis virus (VEEV, a New World alphavirus, can cause encephalitis in humans and horses, but there are no therapeutics for treatment. To date, compounds reported as anti-VEEV or anti-alphavirus inhibitors have shown moderate activity. To discover new classes of anti-VEEV inhibitors with novel viral targets, we used a high-throughput screen based on the measurement of cell protection from live VEEV TC-83-induced cytopathic effect to screen a 340,000 compound library. Of those, we identified five novel anti-VEEV compounds and chose a quinazolinone compound, CID15997213 (IC50 = 0.84 µM, for further characterization. The antiviral effect of CID15997213 was alphavirus-specific, inhibiting VEEV and Western equine encephalitis virus, but not Eastern equine encephalitis virus. In vitro assays confirmed inhibition of viral RNA, protein, and progeny synthesis. No antiviral activity was detected against a select group of RNA viruses. We found mutations conferring the resistance to the compound in the N-terminal domain of nsP2 and confirmed the target residues using a reverse genetic approach. Time of addition studies showed that the compound inhibits the middle stage of replication when viral genome replication is most active. In mice, the compound showed complete protection from lethal VEEV disease at 50 mg/kg/day. Collectively, these results reveal a potent anti-VEEV compound that uniquely targets the viral nsP2 N-terminal domain. While the function of nsP2 has yet to be characterized, our studies suggest that the protein might play a critical role in viral replication, and further, may represent an innovative opportunity to develop therapeutic interventions for alphavirus infection.

A case of limbic encephalitis with small cell lung carcinoma in which the cognitive function improved and redeteriorated during tumor therapy

International Nuclear Information System (INIS)

Fujimoto, So; Yamaguchi, Yasuhiro; Gotanda, Hiroshi

2010-01-01

We report the findings regarding a 70-year-old man with paraneoplastic limbic encephalitis. He presented with a chief complaint of inability to recall any events. He had been well until one month before admission, and then he abruptly began to show progressive amnesia. At admission, the patient's score on the Revised Hasegawa Dementia Scale (HDS-R) showed a decline to 13/30, thus indicating the existence of severe disorientation and an impaired memory. The brain CT and electroencephalogram (EEG) showed no specific abnormalities and an analysis of cerebrospinal fluid showed only a mild increase in the total protein level. A chest X-ray film revealed a mass in the right hilum, while a histological analysis of the biopsied specimen finally established a diagnosis of small cell lung carcinoma. The fluorodeoxyglucose (FDG)-positron emission tomography (PET) and the enhanced brain MRI showed a single small metastatic lesion in the cerebellum. After the 1st course of chemotherapy and whole brain radiation, cognitive function, especially the short-term memory, remarkably improved and the HDS-R score increased to 21/30. However, the tumor again increased in size during the 3rd and 4th courses of chemotherapy. Interestingly, cognitive function also worsened again and the score of HDS-R declined to 15/30, 20 weeks after the start of chemotherapy. Limbic encephalitis can be associated with malignant tumors, such as small cell lung carcinoma, and some reported cases have shown a cognitive improvement after tumor therapy. In our case, we also observed a reworsening of the cognitive function in association with the acquired chemoresistence. (author)

Primary progressive apraxia of speech: clinical features and acoustic and neurologic correlates.

Science.gov (United States)

Duffy, Joseph R; Strand, Edythe A; Clark, Heather; Machulda, Mary; Whitwell, Jennifer L; Josephs, Keith A

2015-05-01

This study summarizes 2 illustrative cases of a neurodegenerative speech disorder, primary progressive apraxia of speech (AOS), as a vehicle for providing an overview of the disorder and an approach to describing and quantifying its perceptual features and some of its temporal acoustic attributes. Two individuals with primary progressive AOS underwent speech-language and neurologic evaluations on 2 occasions, ranging from 2.0 to 7.5 years postonset. Performance on several tests, tasks, and rating scales, as well as several acoustic measures, were compared over time within and between cases. Acoustic measures were compared with performance of control speakers. Both patients initially presented with AOS as the only or predominant sign of disease and without aphasia or dysarthria. The presenting features and temporal progression were captured in an AOS Rating Scale, an Articulation Error Score, and temporal acoustic measures of utterance duration, syllable rates per second, rates of speechlike alternating motion and sequential motion, and a pairwise variability index measure. AOS can be the predominant manifestation of neurodegenerative disease. Clinical ratings of its attributes and acoustic measures of some of its temporal characteristics can support its diagnosis and help quantify its salient characteristics and progression over time.

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.

Alexander the Great and West Nile virus encephalitis.

Science.gov (United States)

Marr, John S; Calisher, Charles H

2003-12-01

Alexander the Great died in Babylon in 323 BC. His death at age 32 followed a 2-week febrile illness. Speculated causes of death have included poisoning; assassination, and a number of infectious diseases. One incident, mentioned by Plutarch but not considered by previous investigators, may shed light on the cause of Alexander's death. The incident, which occurred as he entered Babylon, involved a flock of ravens exhibiting unusual behavior and subsequently dying at his feet. The inexplicable behavior of ravens is reminiscent of avian illness and death weeks before the first human cases of West Nile virus infection were identified in the United States. We posit that Alexander may have died of West Nile virus encephalitis.

Case of herpes simplex encephalitis(HSE) with a thalamic lesion

Energy Technology Data Exchange (ETDEWEB)

Fujimori, K; Koike, R; Yuasa, T; Miyatake, T; Ito, J

1987-02-01

A case of herpes simplex encephalitis (HSE) with thalamic involvement was reported. The patient, a 27-year-old man, was admitted because of abnormal behavior and fever. He exhibited a disturbance of consciousness, meningial signs, and hyperreflexia. A CT scan of the head revealed diffuse brain edema. Acute encephalitis, especially HSE, was suspected, and so the intravenous administration of acyclovir and steroid therapy were started. The titer of herpes simplex Type 1 virus, as measured by CF and ELISA, was found to have increased amounts of serum and cerebrospinal fluid. 5 days after the onset, his consciousness worsened. He could not tell his name and scarely opened his eyes upon pain stimulation. A CT scan at this time showed low-density lesions in the left thalamus, cingulate gyrus, and the posterior portion of the putamen. About 5 days later, his consciousness level was increased, but he was mute. This symptom was thought to be thalamic aphasia, which might be correlative with the low-density lesions shown in the left thalamus by the CT scan. About 30 days after the onset of the disease, his speech became normal, and a CT scan at 51 hospital days showed no abnormality. The etiology of low-density lesions of the left thalamus in the CT scan is speculated to be as follows: firstly, vascular damage of circulation disturbance, and secondly a special affinity of herpes simplex Type 1 virus to the thalamus.

Structure-activity relationships of nucleoside analogues for inhibition of tick-borne encephalitis virus

Czech Academy of Sciences Publication Activity Database

Eyer, L.; Šmídková, Markéta; Nencka, Radim; Neča, J.; Kastl, T.; Palus, Martin; De Clercq, E.; Růžek, Daniel

2016-01-01

Roč. 133, Sep (2016), s. 119-129 ISSN 0166-3542 R&D Projects: GA MZd(CZ) NV16-34238A; GA ČR(CZ) GA16-20054S Institutional support: RVO:61388963 ; RVO:60077344 Keywords : structure-activity relationship * tick-borne encephalitis * nucleoside inhibitor * antiviral activity * cytotoxicity Subject RIV: CC - Organic Chemistry; EE - Microbiology, Virology (BC-A) Impact factor: 4.271, year: 2016

Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis

International Nuclear Information System (INIS)

Kim, Ho Kyun; Lee, Hui Joong

2013-01-01

White matter hyper intensities (WMHI) on MRI are not rare in patients with progressive systemic sclerosis (PSS). In this presentation, WMHI were developed in both middle cerebellar peduncles and temporal white matter in a patient with PSS, and regressed after medication of high dose steroid. However, new lesions were developed in the subcortices of both precentral gyri, and progressed rapidly to tumefactive hyperintensity on MRI. We report an unusual relapsing and progressive tumefactive demyelinating form of central nervous system involvement in PSS.

[N-methyl-D-aspartate receptor antibody encephalitis: value of immunomodulatory therapy].

Science.gov (United States)

Le Moigno, L; Ternant, D; Paintaud, G; Thibault, G; Cloarec, S; Tardieu, M; Lagrue, E; Castelnau, P

2014-06-01

Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is little known to pediatricians and likely underdiagnosed. The child's vital and cognitive prognosis is at stake. The use of immunomodulatory drugs, such as rituximab has led to spectacular results, but many questions remain about its mode of action in this type of pathology. We report the case of a 6-year-old girl with no medical history, admitted for status epilepticus preceded by behavior symptoms and sleep disorders. Gradually, the child became bedridden, mute, and animated by predominantly orofacial dyskinesia. Examinations were normal (cerebrospinal fluid [CSF] analysis, brain MRI). The diagnosis was established by the presence of NMDA-R antibodies in the CSF. After exclusion of a tumor-associated syndrome, treatment was started initially by intravenous immunoglobulins, then by plasma exchange, and finally rituximab. The patient was cured with rituximab despite an unusually early recovery of the B-cell pool. Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is a severe but potentially reversible neurologic disorder only recently described, even in childhood. It may be reversible without sequelae if diagnosed and treated early. The use of immunomodulatory therapy, such as rituximab seemingly improves the outcome. Immunological monitoring is needed to better understand its mechanism of action in autoimmune diseases of the nervous system in childhood. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

An outbreak of post-vaccinal suspected distemper-like encephalitis in farmed ferrets (Mustela putorius furo).

Science.gov (United States)

Gill, J M; Hartley, W J; Hodgkinson, N L

1988-12-01

Two outbreaks of an encephalitis apparently induced by an attenuated live distemper vaccine occurred in a large ferret breeding establishment in New Zealand. Approximately 350 of 6,000 young ferrets 16-22 weeks old died. Many were found dead with no premonitory signs, others showed severe neurological signs. Some with central nervous system (C.N.S.) signs recovered. Pathological examination showed no gross abnormalities except for a few with mild conjunctivitis, rhinitis and lung emphysema. Microscopically there was a moderate to massive non-inflammatory necrosis of hippocampal nerve cell bodies. In those animals which survived for several days with CNS signs there was also a mild to moderately severe non-supportive encephalitis, and in some of these distinct neuronal intranuclear and intracytoplasmic eosinophilic inclusion bodies were seen. Some ferrets also had a bronchiolitis with intracytoplasmic eosinophilic inclusion bodies in bronchiolar epithelium. All these lesions suggest that a distemper like condition was involved. About half of the ferrets also had a mild to severe inflammatory myocardial necrosis.

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

The occurrence of Ixodes ricinus ticks and important tick-borne pathogens in areas with high tick-borne encephalitis prevalence in different altitudinal levels of the Czech Republic. Part I. Ixodes ricinus ticks and tick-borne encephalitis virus

Czech Academy of Sciences Publication Activity Database

Daniel, M.; Danielová, V.; Kříž, B.; Růžek, Daniel; Fialová, A.; Malý, M.; Materna, J.; Pejčoch, M.; Erhart, Jan

2016-01-01

Roč. 65, č. 2 (2016), s. 118-128 ISSN 1210-7913 Institutional support: RVO:60077344 Keywords : Ixodes ricinus * tick-borne encephalitis virus * occurence * altitude * region * season Subject RIV: GJ - Animal Vermins ; Diseases, Veterinary Medicine Impact factor: 0.500, year: 2016

Immunogenicity of immunostimulating complexes of Japanese encephalitis virus in experimental animals

International Nuclear Information System (INIS)

Yeolekar, L.R.; Banerjee, K.

1996-01-01

Immunogenicity of immunostimulating complexes (ISCOMs) of Japanese encephalitis (JE) virus were studied in mice, rabbits and monkeys. Two doses of JE ISCOMs elicited a strong immune response in mice with an uniform distribution in IgG subclasses. Different time intervals between the two doses of ISCOMs led to similar titers of antibodies. Rabbits and monkeys immunized with ISCOMs developed strong neutralizing immune, response. Mice immunized with ISCOMs demonstrated cell-mediated immunity as evident by T cell proliferation and macrophage migration inhibition assays. (author)

Herpes Simplex Type 2 Encephalitis After Craniotomy: Case Report and Literature Review.

Science.gov (United States)

Berger, Assaf; Shahar, Tal; Margalit, Nevo

2016-04-01

Herpes simplex encephalitis (HSE) after neurosurgical procedures is extremely uncommon, and the few published case reports mainly described herpes simplex virus type 1 (HSV-1) as being culpable. We present a rare case of HSV-2 encephalitis after craniotomy and describe its pathophysiology and optimal management. A 70-year-old woman underwent an elective resection of a recurrent left sphenoid wing meningioma and clipping of a left middle cerebral artery aneurysm, the latter having been found incidentally. She returned to our department with clinical findings suggestive of meningitis 12 days after the operation. Her lack of response to empiric antibiotic treatment, taken together with the lymphocyte-predominant initial cerebrospinal fluid obtained by lumbar puncture and the electroencephalographic indications of encephalopathy, led to the suspicion of a diagnosis of HSE, which was later confirmed by a polymerase chain reaction test positive for HSV-2. The patient was then successfully treated with intravenous acyclovir for 2 weeks followed by another week of oral acyclovir treatment before being discharged. The present case stresses the importance of recognizing the relatively rare entity of HSE after craniotomy. Timely correct diagnosis will expedite the initiation of appropriate treatment. Copyright © 2016 Elsevier Inc. All rights reserved.