Manifestations Of Encephalitis Research Articles

Sequential Infection with Influenza A Virus Followed by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Leads to More Severe Disease and Encephalitis in a Mouse Model of COVID-19.

COVID-19 is a spectrum of clinical symptoms in humans caused by infection with SARS-CoV-2. The coalescence of SARS-CoV-2 with seasonal respiratory viruses, particularly influenza viruses, is a global health concern. To understand this, transgenic mice expressing the human ACE2 receptor (K18-hACE2) were infected with influenza A virus (IAV) followed by SARS-CoV-2 and the host response and effect on virus biology was compared to K18-hACE2 mice infected with IAV or SARS-CoV-2 alone. The sequentially infected mice showed reduced SARS-CoV-2 RNA synthesis, yet exhibited more rapid weight loss, more severe lung damage and a prolongation of the innate response compared to the singly infected or control mice. Sequential infection also exacerbated the extrapulmonary encephalitic manifestations associated with SARS-CoV-2 infection. Conversely, prior infection with a commercially available, multivalent live-attenuated influenza vaccine (Fluenz Tetra) elicited the same reduction in SARS-CoV-2 RNA synthesis, albeit without the associated increase in disease severity. This suggests that the innate immune response stimulated by IAV inhibits SARS-CoV-2. Interestingly, infection with an attenuated, apathogenic influenza vaccine does not result in an aberrant immune response and enhanced disease severity. Taken together, the data suggest coinfection ('twinfection') is deleterious and mitigation steps should be instituted as part of the comprehensive public health and management strategy of COVID-19.

CLINICAL AND SUB-CLINICAL CHARACTERISTICS OF MENINGITIS CAUSED BY VARICELLA ZOSTER VIRUS

Encephalitis - meningitis today is still a serious public health problem with a high mortality rate.Objectives: To describe the clinical and subclinical characteristics of encephalitis-meningitis caused by Varicella zoster virus (VZV) in people from 15 years old.Material and methods: A retrospective descriptive research on 40 patients with encephalitis-meningitis diagnosed based on positive VZV PCR results in cerebrospinal fluid treated at the National Hospital of Tropical Diseases and Bach Mai Hospital from January 2020 to June 2023.Results: Common clinical manifestations of VZV meningitis are headache (90.6%), fever (59.4%), and nausea (43.8%). Common manifestations of encephalitis are behavioral and language disorders (75%), and fever (50%). In the cerebrospinal fluid, the median number of white blood cells in the two meningitis and encephalitis groups were 235.5 (100 - 440) cells/mL and 157 (46 - 497) cells/mL, respectively, of which white blood cells were lymphocytes predominate, protein of the two meningitis and encephalitis groups are 1.0 (0.5 - 1.6) G/L and 2.1 (0.9 - 5.7) G/L, respectively. We can find out brain damage on CT or MRI in the temporal lobe, frontal lobe, and insula lobe. In addition, demyelinating lesions occur in 25%.

HHV-7 Encephalitis: The Rare Infection and Its Outcome on a Child (P11-10.006)

<h3>Objective:</h3> The primary aims of this study were to relate HHV-7 infection with clinical manifestations of encephalitis, transient hip synovitis and hepatic changes in childhood and to evidence the effectiveness of early diagnosis and treatment in patients with encephalitis. <h3>Background:</h3> A 2-year-old male patient reported persistent high fever, coughing and rhinorrhea starting 1 week ago. They sought outpatient pediatric care and was prescribed ceftriaxone and prednisolone, with no satisfactory response. The symptoms evolved to vomiting and dehydration. During the physical exam, he showed irritation, with constant cervical stiffness and hyperextension, photophobia, and convergent strabismus. On arrival, he had been taking ceftriaxone, acyclovir and desloratadine. Laboratory tests were very altered in leukocytes, platelets, PCR, AST, ALT and GGT. During the ophthalmologic examination, a paresis of cranial nerve VI on the right was identified as secondary to a probable meningitis. After 21 days of treatment with acyclovir, the patient had a good prognosis, however, he had claudication upon movement of the left hip. <h3>Design/Methods:</h3> NA <h3>Results:</h3> The CSF results were colorless, cloudy, pH 8, Glucose 58, Leukocytes 40, Proteins 81. To confirm this observation, the molecular panel detected the presence of HHV-7. The imaging tests helped and added to the results of the blood count and CSF. Cranial MRI showed a hyperintensity in the FLAIR sequence of the perimesencephalic subarachnoid space and small accumulation of liquid. In addition, the abdomen CT found a bit of fluid in the hip region. <h3>Conclusions:</h3> Encephalitis is an inflammation of the brain parenchyma that leads to neurological dysfunction caused by infection or autoimmunity. In this occasion, the patient had a rare case of HHV-7 encephalitis. Basically, the treatment was carried out with acyclovir and ketoprofen, obtaining a progressive improvement of the clinical picture. Consequently, further research on the complications of HHV-7 is essential to develop a good characterization of this disease. <b>Disclosure:</b> Mr. Andrade has nothing to disclose. Miss MARQUES has nothing to disclose. Dr. Mota has nothing to disclose.

Histophilus somni disease conditions with simultaneous infections by ovine gammaherpesvirus 2 in cattle herds from Southern Brazil.

This report investigated the cause of cattle mortality in two farms in Southern Brazil. The tissues of one animal from each farm (animals #1 and #2) respectively were used inpathological and molecular investigations to determine the possible cause of death. The principal pathological findings observed in animal #1 were pulmonary, myocardial, and encephalitic hemorrhages with vasculitis, and lymphoplasmacytic interstitial pneumonia with proliferative vascular lesions (PVL). The main pathological findings observed in animal #2 were purulent bronchopneumonia, hemorrhagic myocarditis, and lymphoplasmacytic interstitial pneumonia with PVL. An immunohistochemical assay detected intralesional antigens of a malignant catarrhal fever virus (MCFV) from multiple tissues of animal #2 while PCR confirmed that the MCFV amplified was ovine gammaherpesvirus 2 (OvGHV2), genus Macavirus, subfamily Gammaherpesvirinae; OvGHV2 was also amplified from multiple tissues of animal #1. Furthermore, PCR assays amplified Histophilus somni DNA from multiple fragments of both animals. However, the nucleic acids of Mannheimia haemolytica, Pasteurella multocida, Mycoplasma bovis, bovine respiratory syncytial virus, bovine alphaherpesvirus virus 1 and 5, bovine coronavirus,and bovine parainfluenza virus 3 were not amplified from any of the tissues analyzed, suggesting that these pathogens did not participate in the development of the lesions herein described. These findings demonstrated that both animals were concomitantly infected by H. somni and OvGHV2 and developed the septicemic and encephalitic manifestations of H. somni. Furthermore, theinterstitial pneumonia observed in cow #2 was more likely associated with infection by OvGHV2.

Encephalomyelitis associated with coronavirus disease 2019: a case report

BackgroundDespite a considerable number of articles regarding neurological manifestations associated with severe acute respiratory syndrome coronavirus 2 infection, reports on transverse myelitis and encephalitis are scarce.Case presentationWe report a 35-year-old Asian Arab female presenting with longitudinally extensive transverse myelitis within 3 weeks after being diagnosed with mild coronavirus disease 2019 infection. Administration of high-dose methylprednisolone led to significant clinical improvement. However, 2 days after discharge, the patient was readmitted with encephalitis manifestations, consisting of fever and loss of consciousness, along with deterioration in myelitis symptoms. Severe acute respiratory syndrome coronavirus 2 antibody was detected in cerebrospinal fluid, but DNA of severe acute respiratory syndrome coronavirus 2 was not found. Clinical recovery was achieved after the administration of intravenous immunoglobulin.ConclusionLongitudinally extensive transverse myelitis can be a neurological manifestation of coronavirus disease 2019 and can be followed by encephalomyelitis episodes. High-dose steroids and intravenous immunoglobulin as an immunomodulator are possible effective treatment options.

Prognostic Factor for Subacute Encephalitis with First-Episode Psychosis

Introduction: Although symptomatic manifestations in encephalitis vary, they typically include seizures, memory deficit(s), and altered consciousness. Psychosis also occurs as an initial manifestation. In clinical practice, clinicians often encounter the question of whether first-episode psychosis (FEP) originates from encephalitis itself or if encephalitis presenting with FEP develops concurrently. The prognosis of FEP among patients with overall encephalitis, including autoimmune encephalitis, remains uncertain. Methods: We examined a prognostic factor in patients with encephalitis who had both FEP and CSF pleocytosis. A total of 36 patients who presented with FEP were enrolled. A score of ≥3 and ≤2 on the modified Rankin scale were defined as poor and good outcomes, respectively. A total of 13 independent variables were analyzed by the multivariate logistic regression analysis. Results: Significant variables on univariate logistic regression analysis included female sex (OR 5.571, 95% CI: 1.297–23.934; p = 0.021) and the use of mechanical ventilation during the acute stage (OR 7.286, 95% CI: 1.508–35.211; p = 0.013). On multivariate logistic regression analysis, the use of mechanical ventilation during the acute stage (OR 5.446, 95% CI: 1.044–28.615; p = 0.044) was significantly associated with poor outcomes. Conclusions: The use of mechanical ventilation is a poor prognostic factor of subacute encephalitis with FEP, and female sex may be a risk factor for unfavorable development of the disease.

Paraneoplastic encephalitis associated with renal cell carcinoma.

Paraneoplastic encephalitis is autoimmune encephalitis accompanied by tumors. Renal cell carcinoma (RCC) is a kidney cancer originating from various types of renal cells and rarely has been associated with paraneoplastic neurologic manifestation. We identified a case series of paraneoplastic encephalitis-associated RCC. From a prospective institutional cohort, we identified autoimmune encephalitis patients with RCC. The association between RCC and encephalitis was determined by the following criteria: (1) possible autoimmune encephalitis according to the operational autoimmune encephalitis diagnostic criteria and (2) RCC simultaneously diagnosed with neurological manifestation of encephalitis. A total of three patients presented encephalitis accompanied by RCC. Two patients had clear cell RCC, and one had chromophobe RCC. All patients showed limbic encephalitis with cognitive decline, memory impairment, or seizure. Brain magnetic resonance imaging showed T2 hyperintensities at the mesial temporal lobe in two patients with clear cell RCC but no remarkable findings in one patient with chromophobe RCC. While one patient who had early surgery within one month of RCC onset had a favorable response to the treatment, the other two patients showed a partial response and a detrimental result. Paraneoplastic encephalitis associated with RCC presented as limbic encephalitis and was responsive to immunotherapy combined with tumor resection. As our cases identified, RCC should be considered as a cause of seronegative autoimmune encephalitis.

A case of pseudotumorous lesion of the CNS caused by herpes simplex virus type 1 in a patient with secondary immunodeficiency

Pseudotumorcerebri is a general term for a number of non‑neoplastic CNS lesions of various origins that resemble neoplasia clinically and/or radiologically, often cause serious diagnostic mistakes and prescribing unreasonable treatment that may harm the patient. There are currently three types of pseudotumors of the brain, including inflammatory granulomatous lesions, idiopathic or symptomatic intracranial hypertension, and large demyelinating foci in the white matter of the cerebral hemispheres.This scientific publication describes the medical history of a patient with pseudotumor granulomatous temporal lobe encephalitis HSV‑1/2‑etiology, which developed against the background of secondary cellular immunodeficiency caused by long‑term use of methylprednisolone for rheumatoid arthritis. Initially, a misdiagnosis of brain glioma was made due to the similarity of clinical and radiological manifestations of encephalitis with brain tumor. Diagnostic error was identified only during neurosurgery to remove the probable tumor. Histological and immunohistochemical analysis of a brain tissue sample from a pseudotumorous lesion obtained by intraoperative biopsy showed signs of granulomatous macrophage inflammation with moderate microcystosis and a small amount of tissue detritus. The diagnosis of HSV‑1/2 neuroinfection was verified on the basis of PCR of cerebrospinal fluid obtained during neurosurgery. The patient was discontinued previously planned radiation therapy, which was categorically contraindicated in this case, and was prescribed treatment with acyclovir to inhibit the reproductive activity of the virus and dialysable extract of blood leukocytes to eliminate secondary immunodeficiency. Neurosurgical intervention was found to be unjustified. Methylprednisolone was discontinued, and the patient was transferred to an aminoquinolone series drug for the treatment of rheumatoid arthritis. Only after performing these interventions it was possible to achieve a significant improvement in the patient's condition.

Epidemic Alphaviruses: Ecology, Emergence and Outbreaks.

Over the past century, the emergence/reemergence of arthropod-borne zoonotic agents has been a growing public health concern. In particular, agents from the genus Alphavirus pose a significant risk to both animal and human health. Human alphaviral disease presents with either arthritogenic or encephalitic manifestations and is associated with significant morbidity and/or mortality. Unfortunately, there are presently no vaccines or antiviral measures approved for human use. The present review examines the ecology, epidemiology, disease, past outbreaks, and potential to cause contemporary outbreaks for several alphavirus pathogens.

Comparative characteristic of laboratory indicators for encephalitis, disseminated encephalomyelitis and multiple sclerosis in children

Background. The similarity of the clinical manifestations of encephalitis (E), disseminated encephalomyelitis (DEM) and multiple sclerosis (MS) in children, the complexity of predicting the nature of the course and outcome of diseases determines the search for additional diagnostic and prognosis criteria.Objective: a comparative characteristic of laboratory indicators for E, DEM and RS in children to assess their diagnostic and prognostic value.Materials and methods. Fifty six children (14 children with E, 14 – with DEM, 28 – with MS) and 16 children of the control group (with acute respiratory virus infection) at the age from 10 to 17 years were examined. Laboratory methods included standard studies of cerebrospinal fluid (protein, cytosis), determination of concentrations of albumin and immunoglobulin G (IgG) in cerebrospinal fluid and serum with subsequent calculation of protein indices (albumin, immunoglobulin, intrathecal immunoglobulin G indexes).Results. With all nosological forms in the serum there were no significant differences between albumin and IgG from the control group, whereas in the cerebrospinal fluid an excess of the concentration of IgG was detected at a normal level of albumin. An increase in the albumin index was found only in E, whereas the immunoglobulin index was significantly higher than the norm in all groups of patients. A significant spread of the intrathecal IgG index (from 0.1 to 11.9) was found, which determined the analysis of clinical and laboratory parameters by subgroups, depending on its magnitude. The maximum incidence of increased intrathecal IgG synthesis was detected in children with MS (with exacerbation of MS – 74 %, in remission – 67 %), whereas in E and DEM, an increase was observed in about half of the patients surveyed and associated with a more favorable course of the disease.Conclusion. The data obtained make it possible to assert that, despite the commonness of some pathogenetic mechanisms, there are differences in the degree of impaired permeability of the blood brain barrier, the intensity of the systemic and intrathecal humoral immune response in E, DEM and RS, which may determine the features of their course and the outcome of the disease, including the transformation of the DEM in the RS.

Evidence of Chikungunya Virus Disease in Pakistan Since 2015 With Patients Demonstrating Involvement of the Central Nervous System.

Several arboviruses are endemic to and co-circulate in Pakistan. In recent years, Pakistan has observed a rise in arboviral infections. A cross-sectional study for arboviral diseases, which included screening for Chikungunya virus (CHIKV), was initiated in 2015 to determine which pathogens were causing disease in patients presenting to health care services. Exposure to CHIKV was verified via detection of viral nucleic acids or virus-specific IgM with virus-specific neutralizing antibodies. Out of 997 enrolled patients presenting with clinical features suggestive of arboviral disease, 102 patients were positive for CHIKV IgM antibodies and 60 patients were positive for CHIKV nucleic acids or neutralizing antibodies. The data presented here show that CHIKV has been circulating in Pakistan since April of 2015. CHIKV infections were detected in study subjects up to the conclusion of our enrollment period in July 2017. Syndromic and clinical data show that arthralgia was associated with CHIKV as was rash, fever greater than 38°C, and lymphopenia. Neurological symptoms were reported in 49% of CHIKV suspect patients and in 46.6% of confirmed infections. Acute disseminated encephalomyelitis was diagnosed in 5% of confirmed infection and various manifestation of encephalitis diagnosed in an additional 16.6% of patients with confirmed CHIKV infections. CHIKV-exposed patients were just as likely to present with neurological symptoms and encephalitis as patients with West Nile Virus infections but were 4.57 times more likely to have lymphopenia. This proportion of neurological symptoms may be a complicating factor in countries where WNV and/or JEV co-circulate with CHIKV.

Herpes Simplex Virus Type 1 Encephalitis: A Descriptive, Cross Sectional Study, 2011 - 2016, Iran

Background: Encephalitis may arise from infectious or non-infectious etiologies. Infections, predominantly viruses, are the most common causes of encephalitis. Herpes simplex virus (HSV) is the most prevalent cause of viral encephalitis in the developed countries. HSV encephalitis occurs equally in both genders and most cases are caused by HSV type 1 (HSV-1). Methods: The current descriptive, cross sectional study was conducted on patients admitted with HSV-1 encephalitis from 2011 to 2016 to Imam-Khomeini hospital complex in Tehran, Iran. The patients with clinical diagnosis of encephalitis whose cerebrospinal fluid (CSF) HSV-1 polymerase chain reaction (PCR) was positive were included. All clinical, laboratory, and imaging characteristics of patients along with their outcomes were evaluated and analyzed. Results: In the current study, of 12 cases with encephalitis manifestations and positive CSF HSV-1 PCR results, 5 were male and 7 were female with mean age of 42 years. Fever and headache were the most common complaints. Headache was more common in the cases admitted earlier (P value = 0.001). The patients were studied by brain magnetic resonance imaging (MRI) and the findings confirmed encephalitis in all the cases. Five cases had unilateral temporal lobe lesions and 6 cases had bilateral lesions. There was a clear relationship between bilateral temporal lobe lesions and higher protein level in CSF (P value = 0.001); 25% of patients (3 cases) needed stay in the intensive care unit (ICU). Three patients died and 5 patients developed disabilities. Conclusions: HSV encephalitis is a devastating disease. Clinical findings along CSF analysis and neuroimaging may result in the appropriate diagnosis. Despite appropriate treatment, undesired outcomes are common.

Autoimmune encephalitis associated with two antibodies

•In patients presenting with clinical manifestations of encephalitis without clinical or laboratory signs of infection, an autoimmune etiology should be suspected.•Antibodies for various neural antigens may coexist, thus a complete and clinically-guided autoimmune panel must be done in suspected cases of autoimmune encephalitis.•Tumor resection, if applicable, combined with high dose steroids and immunotherapy are effective treatment strategies for autoimmune encephalitis with coexisting antibodies.

Autoimmune and Epilepsy

The recent discovery of autoimmune antibodies to the neuronal cell surface membrane and extra- or intra-cellular proteins, such as NMDAR and LGI1, shed light on a proposed new etiology of epilepsy, namely, "autoimmune epilepsy". A large part of this entity most likely belongs to a forme fruste of autoimmune (limbic) encephalitis. Seizures are usually subacute in onset and refractory to antiepileptic medications. Patients occasionally manifest multiple seizure semiologies, such as autonomic or faciobrachial dystonic seizures. They may develop other symptoms of autoimmune encephalitis to variable degrees. Clinical diagnosis of autoimmune epilepsy should be considered even before the results of antibody testing, when objective evidence of CNS inflammation, such as abnormal MRI (amygdala/hippocampal enlargement), FDG-PET (hypermetabolism), EEG (bitemporal spikes or subclinical seizure patterns) and/or CSF findings, are present. Early intensive immunotherapy could cure seizures and prevent the full-blown clinical manifestation of encephalitis. Some patients present with relatively mild clinical symptoms and/or MRI findings, and can have a smoldering course lasting years. Establishment of the biomarkers of the ongoing CNS inflammation, especially for the smoldering and the suspected autoantibody-negative cases, is warranted for tailored immunotherapy for both the subacute and chronic phases of the disease.

Bacillus Calmette-Guérin Confers Neuroprotection in a Murine Model of Japanese Encephalitis

Objective: Japanese encephalitis (JE) is a debilitating disease caused by infection with the JE virus (JEV; family: Flaviviridae), which leaves neurological sequelae in survivors but more often leads to mortality. Neurodegeneration caused by inflammation is the primary pathology behind the clinical manifestation of encephalitis caused by JEV. Bacillus Calmette-Guérin (BCG) has been used in immunoprophylaxis for tuberculosis and in the adjuvant therapy of many malignancies, and has exhibited neuroprotective activities in experimental models of Parkinson and Alzheimer disease. This study aimed at assessing the neuroprotective role of BCG in a murine model of JE. Methods: Suckling mice were inoculated with 10⁶ CFU of BCG and at 18 days postinoculation were challenged with 100 LD₅₀ of JEV. PBS-inoculated mice were used as controls. Mice were sacrificed on days 2, 4, 6, and 8. Brain tissue was homogenized for RNA extraction. One-step real-time RT-PCR was performed to assess the relative gene expressions of TNF-α, IL-6, and iNOS. Results: The BCG-inoculated (BCG+JEV) group exhibited a significant delay in the presentation of neuropathological symptoms, longer survival, and a downregulation in the expression of TNF-α, IL-6, and iNOS on days 2, 4, and 6 post-JEV challenge compared to the JEV group. Conclusion: These findings indicate that the administration of BCG offers neuroprotection in the murine model of JE. BCG should therefore be further investigated as an adjuvant in the management of JE. BCG is an accepted vaccine for tuberculosis in many countries that are endemic for JEV. This approach may have a significant impact on the public health burden in these countries.

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

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

Chapter 13 - Autoimmune epilepsy

Seizures are a common manifestation of autoimmune limbic encephalitis and multifocal paraneoplastic disorders. Accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of encephalitis. The autoimmune epilepsies are immunologically mediated disorders in which recurrent seizures are a primary and persistent clinical feature. When other etiologies have been excluded, an autoimmune etiology is suggested in a patient with epilepsy upon detection of neural autoantibodies and/or the presence of inflammatory changes on cerebrospinal fluid (CSF) or magnetic resonance imaging. In such patients, immunotherapy may be highly effective, depending on the particular autoimmune epilepsy syndrome present. In this chapter, several autoimmune epilepsy syndromes are discussed. First, epilepsies secondary to other primary autoimmune disorders will be discussed, and then those associated with antibodies that are likely to be pathogenic, such as voltage-gated potassium channel-complex and N-methyl-d-aspartate receptor, gamma-aminobutyric acid A and B receptor antibodies. For each syndrome, the typical clinical, imaging, electroencephaloram, CSF, and serologic features, and pathophysiology and treatment are described. Finally, suggested guidelines for the recognition, evaluation, and treatment of autoimmune epilepsy syndromes are provided.

Japanese encephalitis virus invasion of cell: allies and alleys.

The mosquito-borne flavivirus, Japanese encephalitis virus (JEV), is the leading cause of virus-induced encephalitis globally and a major public health concern of several countries in Southeast Asia, with the potential to become a global pathogen. The virus is neurotropic, and the disease ranges from mild fever to severe hemorrhagic and encephalitic manifestations and death. The early steps of the virus life cycle, binding, and entry into the cell are crucial determinants of infection and are potential targets for the development of antiviral therapies. JEV can infect multiple cell types; however, the key receptor molecule(s) still remains elusive. JEV also has the capacity to utilize multiple endocytic pathways for entry into cells of different lineages. This review not only gives a comprehensive update on what is known about the virus attachment and receptor system (allies) and the endocytic pathways (alleys) exploited by the virus to gain entry into the cell and establish infection but also discusses crucial unresolved issues. We also highlight common themes and key differences between JEV and other flaviviruses in these contexts.

Autoimmune Epilepsy

Seizures are recognized as a common manifestation of autoimmune limbic encephalitis and multifocal paraneoplastic disorders, but accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of encephalitis. Autoimmune encephalitis and epilepsy have been linked to neural-specific autoantibodies targeting both intracellular and plasma membrane antigens. The detection of these antibodies can serve as a diagnostic marker directing physicians toward specific cancers and can assist in therapeutic decision-making, but are not necessary to establish the diagnosis. Response to an immunotherapy trial can support the diagnosis and help establish prognosis. Early recognition is important because expedited diagnosis can facilitate recovery. In this review, the authors summarize the clinical presentation, pathophysiology, and management of autoimmune epilepsies for which neural antigen-specific autoantibodies serve as diagnostic aids.

G65(P) Neuropsychiatric presentation of antibody negative limbic encephalitis

Autoimmune encephalitis is an increasingly recognised cause for a combination of symptoms of seizures, disturbance of memory, behaviour and cognition. Other manifestations include acute onset movement disorders, unexplained encephalopathy and refractory status epilepticus. N-methyl aspartate (NMDA) encephalitis and voltage gated potassium channel mediated (VGKC) limbic encephalitis are the two commonest causes of autoimmune encephalitis. These children initially present to general paediatricians therefore it is vital to consider this in the differential diagnosis of infective encephalitis as prompt recognition, investigation and immune therapy determines longterm outcome. Early recognition and treatment can potentially halt temporal lobe atrophy and improve outcome. We report a 14 year old girl who presented with symptomatic, intermittent memory loss, behaviour disturbance, fatigue and cognitive change with limbic encephalitis. Initial investigations revealed negative PCR for HSV, EBV. Brain imaging detected mesial right temporal high signal area in FLAIR sequences. EEG showed epileptic inter-ictal focal abnormality over right frontal mesial temporal region. NMDA, VGKC, Anti GAD, Hu, Ma, CV2, CRMP5 and thyroid antibodies were negative. Screening for tumour with baseline abdominal ultrasound and CXR were negative. Baseline WISC testing prior to starting medications revealed her immediate memory for verbal information was in the low average range (verbal immediate index score:82,12th percentile) and her time delayed recall verbal information was in the impaired range (verbal delayed index score:72, 3rd percentile). She was treated with iv methylprednisolone followed by tapering dose of oral prednisolone. Repeat WISC assessment is due in 6 months. Limbic encephalitis should be considered in children with prominent neuropsychiatric manifestation of encephalitis. Her future MRI reports will determine the 3 month and 6 month outcome for this teenager. Longterm surveillance for tumour detection is recommended in autoimmune encephalitis.