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Clinical Medicine (London, England) Mar 2018Encephalitis, inflammation of the brain, is most commonly caused by a viral infection (especially herpes simplex virus [HSV] type 1 in the UK) although autoimmune... (Review)
Review
Encephalitis, inflammation of the brain, is most commonly caused by a viral infection (especially herpes simplex virus [HSV] type 1 in the UK) although autoimmune causes, such as N-methyl D-aspartate receptor (NMDAR) antibody encephalitis, are increasingly recognised. Most patients present with a change in consciousness level and may have fever, seizures, movement disorder or focal neurological deficits. Diagnosis hinges crucially on lumbar puncture and cerebrospinal fluid (CSF) examination, but imaging and electroencephalography (EEG) may also be helpful. Treatment of HSV encephalitis with aciclovir dramatically improves outcome, but the optimal management of autoimmune encephalitis is still uncertain. Many patients with encephalitis are left with residual physical or neuropsychological deficits which require long-term multidisciplinary management. Here we review assessment of patients with suspected encephalitis, general aspects of management and areas of ongoing research.
Topics: Acute Disease; Biomedical Research; Brain; Encephalitis; Humans
PubMed: 29626021
DOI: 10.7861/clinmedicine.18-2-155 -
The New England Journal of Medicine Mar 2018
Review
Topics: Adult; Antigens, Surface; Autoantibodies; Autoimmune Diseases of the Nervous System; Child; Encephalitis; Female; Genetic Predisposition to Disease; Humans; Magnetic Resonance Imaging; Male; Neurons
PubMed: 29490181
DOI: 10.1056/NEJMra1708712 -
The Lancet. Neurology Feb 2014Rasmussen's encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and... (Review)
Review
Rasmussen's encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. Neuropathological and immunological studies support the notion that Rasmussen's encephalitis is probably driven by a T-cell response to one or more antigenic epitopes, with potential additional contribution by autoantibodies. Careful analysis of the association between histopathology and clinical presentation suggests that initial damage to the brain is mediated by T cells and microglia, suggesting a window for treatment if Rasmussen's encephalitis can be diagnosed early. Advances in neuroimaging suggest that progression of the inflammatory process seen with MRI might be a good biomarker in Rasmussen's encephalitis. For many patients, families, and doctors, choosing the right time to move from medical management to surgery is a real therapeutic dilemma. Cerebral hemispherectomy remains the only cure for seizures, but there are inevitable functional compromises. Decisions of whether or when surgery should be undertaken are challenging in the absence of a dense neurological deficit, and vary by institutional experience. Further, the optimum time for surgery, to give the best language and cognitive outcome, is not yet well understood. Immunomodulatory treatments seem to slow rather than halt disease progression in Rasmussen's encephalitis, without changing the eventual outcome.
Topics: Encephalitis; Humans
PubMed: 24457189
DOI: 10.1016/S1474-4422(13)70260-6 -
Continuum (Minneapolis, Minn.) Aug 2021This article reviews infections of the brain parenchyma and includes an overview of the epidemiology, pathogenesis, diagnostic approach, and management of infectious... (Review)
Review
PURPOSE OF REVIEW
This article reviews infections of the brain parenchyma and includes an overview of the epidemiology, pathogenesis, diagnostic approach, and management of infectious encephalitis and brain abscess.
RECENT FINDINGS
The epidemiology of infectious encephalitis and brain abscess has changed in recent years. Vaccination has reduced the incidence of certain viruses associated with encephalitis, while a decrease in fulminant otogenic infections has led to fewer brain abscesses associated with otitis media. However, changes in climate and human population density and distribution have enabled the emergence of newer pathogens and expanded the geographic range of others, and greater adoption of intensive immunosuppressive regimens for autoimmune conditions has increased the risk of opportunistic infections of the brain. The widespread use of early neuroimaging, along with improved diagnostic methodologies for pathogen detection, newer antimicrobial therapies with better brain penetration, and less invasive neurosurgical techniques, has resulted in better outcomes for patients with infectious encephalitis and brain abscess. Novel technologies including metagenomic next-generation sequencing are increasingly being applied to these conditions in an effort to improve diagnosis. Nevertheless, both infectious encephalitis and brain abscess continue to be associated with substantial mortality.
SUMMARY
Infectious encephalitis and brain abscess can present as neurologic emergencies and require rapid assessment, thorough and appropriate diagnostic testing, and early initiation of empiric therapies directed against infectious agents. Close clinical follow-up, proper interpretation of diagnostic results, and appropriate tailoring of therapeutic agents are essential to optimizing outcomes. Diagnosis and management of parenchymal brain infections are complex and often best achieved with a multidisciplinary care team involving neurologists, neurosurgeons, neuroradiologists, infectious disease physicians, and pathologists.
Topics: Brain; Brain Abscess; Encephalitis; Humans; Neuroimaging; Neurosurgical Procedures
PubMed: 34623096
DOI: 10.1212/CON.0000000000001006 -
Seminars in Neurology Dec 2015Meningitis is an inflammatory syndrome involving the meninges that classically manifests with headache and nuchal rigidity and is diagnosed by cerebrospinal fluid... (Review)
Review
Meningitis is an inflammatory syndrome involving the meninges that classically manifests with headache and nuchal rigidity and is diagnosed by cerebrospinal fluid examination. In contrast, encephalitis refers to inflammation of the brain parenchyma itself and often results in focal neurologic deficits or seizures. In this article, the authors review the differential diagnosis of meningitis and encephalitis, with an emphasis on infectious etiologies. The recommended practical clinical approach focuses on early high-yield diagnostic testing and empiric antimicrobial administration, given the high morbidity associated with these diseases and the time-sensitive nature of treatment initiation. If the initial workup does not yield a diagnosis, further etiology-specific testing based upon risk factors and clinical characteristics should be pursued. Effective treatment is available for many causes of meningitis and encephalitis, and when possible should address both the primary disease process as well as potential complications.
Topics: Diagnosis, Differential; Encephalitis; Humans; Meningitis
PubMed: 26595861
DOI: 10.1055/s-0035-1564686 -
Lancet (London, England) Feb 2019Encephalitis is a condition of inflammation of the brain parenchyma, occurs as a result of infectious or autoimmune causes, and can lead to encephalopathy, seizures,...
Encephalitis is a condition of inflammation of the brain parenchyma, occurs as a result of infectious or autoimmune causes, and can lead to encephalopathy, seizures, focal neurological deficits, neurological disability, and death. Viral causes account for the largest proportion, but in the last decade there has been growing recognition of anti-neuronal antibody syndromes. This Seminar focuses on the diagnosis and management of acute encephalitis in adults. Although viral and autoimmune causes are highlighted because of their prominent roles in encephalitis, other infectious pathogens are also considered. The role of cerebrospinal fluid studies, MRI, and novel diagnostic modalities (eg, next-generation sequencing) are discussed. Management approaches, including treatment of acute neurological complications and the use of immune suppressive and modulatory drugs for cases of suspected or confirmed autoimmune cause, are covered. Additionally, we discuss the remaining challenges in the diagnosis, management, and prognosis of encephalitis.
Topics: Acute Disease; Adult; Brain; Encephalitis; Humans; Immunocompromised Host; Seizures
PubMed: 30782344
DOI: 10.1016/S0140-6736(18)32526-1 -
Seminars in Neurology Apr 2020Rasmussen encephalitis (RE) is a rare, devastating, progressive pediatric epilepsy. First described 60 years ago, RE continues to present challenges in diagnosis and... (Review)
Review
Rasmussen encephalitis (RE) is a rare, devastating, progressive pediatric epilepsy. First described 60 years ago, RE continues to present challenges in diagnosis and management. RE causes a unilateral focal epilepsy in children that typically becomes medically refractory, results in significant hemiparesis, and causes progressive cognitive decline. The etiology is a cell-mediated immune attack on one cerebral hemisphere, though the inciting antigen remains unknown. While the underlying histopathology is unilateral and RE is described as "unihemispheric," studies have demonstrated (1) atrophy of the unaffected hemisphere, (2) electroencephalographic abnormalities (slowing and spikes) in the unaffected hemisphere, and (3) cognitive decline referable to the unaffected hemisphere. These secondary contralateral effects likely reflect the impact of uncontrolled epileptic activity (i.e., epileptic encephalopathy). Hemispheric disconnection (HD) renders 70 to 80% of patients seizure free. While it has the potential to limit the influence of seizures and abnormal electrical activity emanating from the pathological hemisphere, HD entails hemiparesis and hemianopia, as well as aphasia for patients with dominant HD. With the recent expansion of available immunomodulatory therapies, there has been interest in identifying an alternative to HD, though evidence for disease modification is limited to date. We review what is known and what remains unknown about RE.
Topics: Child; Encephalitis; Epilepsies, Partial; Humans
PubMed: 32185790
DOI: 10.1055/s-0040-1708504 -
Arquivos de Neuro-psiquiatria Jan 2018Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The... (Review)
Review
Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Diagnosis, Differential; Encephalitis; Female; Hashimoto Disease; Humans; Immunotherapy; Male
PubMed: 29364393
DOI: 10.1590/0004-282X20170176 -
Clinical Infectious Diseases : An... Aug 2008Guidelines for the diagnosis and treatment of patients with encephalitis were prepared by an Expert Panel of the Infectious Diseases Society of America. The guidelines...
Guidelines for the diagnosis and treatment of patients with encephalitis were prepared by an Expert Panel of the Infectious Diseases Society of America. The guidelines are intended for use by health care providers who care for patients with encephalitis. The guideline includes data on the epidemiology, clinical features, diagnosis, and treatment of many viral, bacterial, fungal, protozoal, and helminthic etiologies of encephalitis and provides information on when specific etiologic agents should be considered in individual patients with encephalitis.
Topics: Anti-Infective Agents; Encephalitis; Evidence-Based Medicine; Humans
PubMed: 18582201
DOI: 10.1086/589747 -
Current Neurology and Neuroscience... Dec 2011Rhombencephalitis (RE) is a syndrome of multiple causes and multiple outcomes. Most authors now use the terms "rhombencephalitis" and "brainstem encephalitis"... (Review)
Review
Rhombencephalitis (RE) is a syndrome of multiple causes and multiple outcomes. Most authors now use the terms "rhombencephalitis" and "brainstem encephalitis" interchangeably even though anatomically they are slightly different. The etiologic categories of RE include infections, autoimmune diseases, and paraneoplastic syndromes (PNS). Listeria is the most common cause of infectious RE. Listeria RE primary occurs in healthy young adults. It usually occurs as a biphasic time course with a flu-like syndrome followed by brainstem dysfunction; 75% of patients have a cerebrospinal fluid (CSF) pleocytosis, and almost 100% have an abnormal brain MRI scan. Positive CSF and blood cultures are the most specific for diagnosis. Treatment primarily is with ampicillin. Enterovirus 71 is probably the second most common infectious cause of RE; however, 95% of cases have occurred in the Asian-Pacific region and there is no specific treatment. Herpes simplex virus (HSV) is the third most common infectious cause of RE, and about 80% of cases are caused by HSV1 and 20% by HSV2. About 50% only had involvement of the brainstem whereas the other 50% also had supratentorial involvement of the temporal and frontal lobes. Mortality with acyclovir treatment was 22% versus those not on acyclovir 75%. Epstein-Barr virus (EBV) and human herpesvirus 6 (HHV6) have caused a few cases. The most common autoimmune etiology is Behçet disease. Over 90% of those with Behçet RE had abnormal MRI scans and 94% had a CSF pleocytosis. Treatment is with corticosteroids and immunosuppressive agents, but only 25% have complete recovery. Paraneoplastic causes are the third category of RE. Brain MRIs are usually normal; there is usually a CSF pleocytosis but the protein is usually normal. Often anti-neuronal antibodies can be found. Prognosis is poor and treatment is only partially beneficial. Because Listeria and HSV are the most common treatable acute causes of RE, we recommend empiric therapy with ampicillin and acyclovir for all cases after samples have been obtained from CSF and blood for cultures and the polymerase chain reaction (PCR). Antibiotics can be changed based upon MRI, culture results, PCR results, and antibody studies.
Topics: Autoimmune Diseases; Brain Stem; Central Nervous System Infections; Encephalitis; Humans; Magnetic Resonance Imaging; Paraneoplastic Syndromes, Nervous System
PubMed: 21956758
DOI: 10.1007/s11910-011-0228-5