Burden of encephalitis-associated hospitalizations in the United States, 1998–2010

Vora, Neil M.; Holman, Robert C.; Mehal, Jason M.; Steiner, Claudia; Blanton, Jesse D.; Sejvar, James J.

doi:10.1212/wnl.0000000000000086

Cited by 213 publications

(200 citation statements)

References 29 publications

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“…Given the emergence of West Nile virus neuroinvasive disease in the USA and the recognition of immune-mediated etiologies, such as anti-NMDAR encephalitis, Vora et al [158] estimated the burden of encephalitis-associated hospitalizations from 1998 to 2010, updating a previous study [159]. They reported a mean length of hospital stay of 11.2 days with a median inpatient charge of $48,852 for encephalitis-related hospitalizations and $58,082 for encephalitis related to herpes, in 2010.…”

Section: Discussionmentioning

confidence: 99%

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

2016

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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 lifesaving 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.

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Section: Discussionmentioning

confidence: 99%

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

2016

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“…In these syndromes the antibodies target cytoplasmic or nuclear proteins, the pathogenic mechanisms are believed to be mediated by cytotoxic T-cells (instead of B cells), and the symptoms are frequently irreversible. 2 Keeping in mind these 2 different groups of disorders and the large number of patients with encephalopathies for which the cause was (and still is 3 ) unknown, some of us wondered whether a subgroup of CNS diseases could be mediated by antibodies against cell surface or synaptic proteins in a manner similar to the myasthenic syndromes. In my experience, the answer started to be revealed in December 2003 after we saw a young woman with encephalitis of unknown cause who had been in the intensive care unit for several weeks.…”

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confidence: 99%

NMDA receptor encephalitis and other antibody-mediated disorders of the synapse

2016

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Investigations during the last 10 years have revealed a group of disorders mediated by antibodies against ion channels and synaptic receptors, which cause both neurologic and psychiatric symptoms. In this review, I discuss the process of discovery and immunologic triggers of these disorders, and use anti-NMDA receptor encephalitis to emphasize the importance of understanding the underlying physiopathologic mechanisms in those diseases. A better knowledge of these mechanisms reveals points of convergence with other disorders (e.g., schizophrenia), suggests treatment strategies beyond immunotherapy, and is helping us understand how memories are formed and retrieved. Neurology ® 2016;87:2471-2482 GLOSSARY BBB 5 blood-brain barrier; EphB2 5 Ephrin type B2 receptor; FLAIR 5 fluid-attenuated inversion recovery; HSE 5 herpes simplex encephalitis; IgG 5 immunoglobulin G; LEMS 5 Lambert-Eaton myasthenic syndrome; LTP 5 long-term potentiation; NMDAR 5 NMDA receptor.

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“…Studies from different countries and a recent meta-analysis showed that in about 40% of patients with encephalitis the cause is never identified. 1,2 Without reliable biomarkers, a response to empiric immunotherapy is frequently used to support that the disorder is immune-mediated, but a lack of response does not rule out an immune-mediated pathogenesis. For example, approximately 40% of patients with anti-NMDA receptor (NMDAR) encephalitis fail first-line immunotherapy (steroids, plasma exchange, or IV immunoglobulin [IVIg]) and require second-line therapies (rituximab or cyclophosphamide).…”

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Human neurexin-3α antibodies associate with encephalitis and alter synapse development

et al. 2016

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Objective: To report a novel autoimmune encephalitis in which the antibodies target neurexin-3a, a cell adhesion molecule involved in the development and function of synapses.Methods: Five patients with encephalitis and antibodies with a similar pattern of brain reactivity were selected. Antigen precipitation and determination of antibody effects on cultured rat embryonic neurons were performed with reported techniques.Results: Immunoprecipitation and cell-based assays identified neurexin-3a as the autoantigen of patients' antibodies. All 5 patients (median age 44 years, range 23-50; 4 female) presented with prodromal fever, headache, or gastrointestinal symptoms, followed by confusion, seizures, and decreased level of consciousness. Two developed mild orofacial dyskinesias, 3 needed respiratory support, and 4 had findings suggesting propensity to autoimmunity. CSF was abnormal in all patients (4 pleocytosis, 1 elevated immunoglobulin G [IgG] index), and brain MRI was abnormal in 1 (increased fluid-attenuated inversion recovery/T2 in temporal lobes). All received steroids, 1 IV immunoglobulin, and 1 cyclophosphamide; 3 partially recovered, 1 died of sepsis while recovering, and 1 had a rapid progression to death. At autopsy, edema but no inflammatory cells were identified. Cultures of neurons exposed during days in vitro (div) 7-17 to patients' IgG showed a decrease of neurexin-3a clusters as well as the total number of synapses. No reduction of synapses occurred in mature neurons (div 18) exposed for 48 hours to patients' IgG. Neuronal survival, dendritic morphology, and spine density were unaffected. Conclusion:Neurexin-3a autoantibodies associate with a severe but potentially treatable encephalitis in which the antibodies cause a decrease of neurexin-3a and alter synapse development. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Studies from different countries and a recent meta-analysis showed that in about 40% of patients with encephalitis the cause is never identified.1,2 Without reliable biomarkers, a response to empiric immunotherapy is frequently used to support that the disorder is immune-mediated, but a lack of response does not rule out an immune-mediated pathogenesis. For example, approximately 40% of patients with anti-NMDA receptor (NMDAR) encephalitis fail first-line immunotherapy (steroids, plasma exchange, or IV immunoglobulin [IVIg]) and require second-line therapies (rituximab or cyclophosphamide).3,4 However, second-line therapies are rarely used in encephalitis of unclear cause unless evidence of autoimmunity is provided. In this setting, the demonstration of autoantibodies to neuronal cell

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Burden of encephalitis-associated hospitalizations in the United States, 1998–2010

Cited by 213 publications

References 29 publications

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

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

NMDA receptor encephalitis and other antibody-mediated disorders of the synapse

Human neurexin-3α antibodies associate with encephalitis and alter synapse development

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