Abstract. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. This article describes the main types of autoimmune limbic encephalitis and its mimic disorders, and emphasizes their major imaging features. This study is aimed at investigating the lymphocyte subsets of cerebrospinal fluid (CSF) to provide possible differential diagnostic values and better understand the pathophysiological mechanism underlying autoimmune encephalitis (AE) and infectious lymphocytic encephalitis. Two months thereafter, he began losing previously acquired developmental and language . Brain Abscess. Autoimmune encephalitis as a differential diagnosis of schizophreniform psychosis: clinical symptomatology, pathophysiology, diagnostic approach, and therapeutic considerations Eur Arch Psychiatry Clin Neurosci. The lesions of encephalitis are sometimes spotty and scattered, and sometimes generalized. . Suspected infections should also be reported to the local health department. 65(3):257-67. Cavernous Sinus Syndromes. When an infection of the CNS is considered in the differential diagnosis, . Background. Due to the fact that herpetic rashes on the skin in such patients are rare, carrying out a differential diagnosis causes significant difficulties. Conditions to be ruled out by appropriate investigations include: The extreme difficulty of differential diagnosis on the basis of clinical observation was indicated by the wide range of diagnoses made in these cases before the invading organism was identified by laboratory studies. This report describes a case of a 13-year-old boy who at 3.5 years of age was bitten by a tick. Differential Diagnoses. 3,8 Imaging usually facilitates diagnosis, as stroke has typical imaging features at different stages and follows typical topographic patterns. The following conditions may be considered in the differential diagnoses of herpes simplex encephalitis (HSE) Myoclonus Management of increased intracranial pressure (ICP) in the neuro intensive . Encephalitis. Limbic encephalitis — Limbic encephalitis refers to an inflammatory process localized to structures of the limbic system (eg, hippocampus, amygdala, hypothalamus, cingulate gyrus . Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe form of encephalitis associated with antibodies against NR1 and NR2 subunits of the NMDA receptor and occurs primarily in women.It is characterised by psychotic symptoms, motor changes such as catatonia, seizure-like activity and dyskinesias. Cytomegalovirus (CMV) is a double-stranded DNA virus in the herpes virus family that can cause disseminated or localized end-organ disease in HIV-infected patients with advanced immunosuppression. At times the spread of the disease in the central nervous system may be slow and at other times rapid. May help in the differential diagnosis of encephalitis v encephalopathy. The diagnosis of anti-NMDAR encephalitis utilizes history, examination, and specific assays. Postcontrast T1W image (c) shows no contrast enhancement We review here clues that help in the differential diagnosis with infectious encephalitis. In patients with relapse of HSVE, the latency period can range from weeks to years after primary infection, and multiple relapses are rare. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Once . There are more than 100 pathogens known to cause infectious encephalitis. Image: Coronal T2-weighted MR image shows high signal in the temporal lobes including hippocampal formations and parahippogampal gyrae, insulae, and right inferior frontal gyrus. Suspected infections should also be reported to the local health department. Because a high proportion of encephalitis patients have an infectious etiology, and these . Differential Diagnosis Complete Differential Diagnosis of the Causes of Encephalitis 2 neurological symptoms of jev infection may be similar to those caused by other … The clinical challenge is distinguishing causes of encephalopathy, including septic, metabolic, toxic and others, from patients who have encephalitis and therefore need specific treatments. Acute Subdural Hematoma Management in the ED. . The diagnosis of toxoplasmosis was considered before chagasic encephalitis, given the relative frequency of these diagnoses in the United States, T. gondii seropositivity, and the preliminary pathological interpretation. Moreover, recent investigations indicate that viral encephalitis (e.g., herpes simplex) can trigger synaptic autoimmunity. 12 . Encephalitis is defined as inflammation of the brain parenchyma associated with neurologic dysfunction. The lesions of encephalitis are sometimes spotty and scattered, and sometimes generalized. The 1952 outbreak of encephalitis in California; differential diagnosis. Benign Neonatal Convulsions. Its etiology is unknown. Landau-Kleffner syndrome (LKS) is a rare childhood neurological condition that causes developmental regression, loss of language skills and abnormal electroencephalogram (EEG) patterns. 2014;27(3):361-368. Cardioembolic Stroke. 1-5 Encephalitis in the pediatric population may result in substantial morbidity, disability, and even mortality. From: Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders, 2018. . 2004; 17(5):413-20 (ISSN: 0951-7375) Cunha BA. . For example, a patient presenting with 3 days of fever and rapidly deteriorating mental status cul-minating in seizure activity would raise suspicion for an encephalitis. We present the case of a 41 year-old male, diagnosed with syphilitic encephalitis, in whom cerebral magnetic resonance imaging demonstrated preponderant involvement of bilateral temporal lobes, for this point of view raising differential diagnostic concerns with Herpes virus encephalitis. Differential diagnosis to be considered is HSV encephalitis. We test these Graus criteria on our cohort for . Secondary forms are based on a clearly identifiable organic cause, in terms of either an etiological or a relevant pathogenetic factor. Brain Neoplasms. Background: The differential diagnosis of autoimmune and infectious encephalitis is notoriously difficult. However, existing criteria for autoimmune encephalitis are too reliant on . For 60% of acute febrile encephalitis cases, the cause is unknown. Once . Encephalitis is defined as inflammation of the brain parenchyma associated with neurologic dysfunction. CD8 ratios and B lymphocytes in CSF may be useful for the differential diagnosis for the AE with infectious CNS disease (see Table 2, Figure 1). ↵ * If one of the first 3 criteria is not met, a diagnosis of definite limbic encephalitis can be made only with the detection of antibodies against cell-surface, synaptic, or onconeural proteins.. ↵ † 18 Fluorodeoxyglucose (18 F-FDG) PET can be used to fulfil this criterion. 65(3):257-67. There are >100 different etiologies that can lead to encephalitis in children; however, most researchers have found that the majority (50%- 70%) of patients lack an identified etiology. The diagnosis of acute encephalitis is suspected in a febrile patient who presents with altered consciousness and signs of diffuse cerebral dysfunction. These antibodies are directed against structures located on the neuronal cell surface: glutamate receptors (types NMDA and AMPA), GABABreceptors, as well as the voltage-gated potassium channel-associated proteins LGI1 and CASPR2. Sheriff H, Messenger S, Preas CP, Shahkarami M, et al. 11.Sechi G, Serra A. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. . Bacterial . Rasmussen encephalitis, also known as chronic focal encephalitis, is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. HSV-1 encephalitis is a rare but previously reported complication after neurosurgery procedures. Autoimmune encephalitis is rare and has various clinical manifestations, which may hamper the correct diagnosis. Serious, complex, and potentially fatal disorder with non-infectious and infectious causes. Confusion is a common presentation to the acute medical unit and has a wide differential diagnosis ().The clinical challenge is distinguishing causes of encephalopathy, including septic, metabolic, toxic and others, from patients who have encephalitis and therefore need specific treatments. Yao K, Honarmand S, Espinosa A, Akhyani N, Glaser C, Jacobson S. Detection of human herpesvirus-6 in cerebrospinal fluid of patients with encephalitis. The clinical manifestations and diagnosis of viral encephalitis in children will be discussed here. Ann Neurol . T2W (a), T2 fluid-attenuated inversion recovery (b) images shows hyperintensity in bilateral posterior thalami (arrows). The clinical manifestations of the disorder include complex psychiatric symptoms, seizures, movement disorders, cognitive dysfunction, and autonomic instability. 1-7 The majority of stroke mimics are due to seizures, migraines, tumors and toxic-metabolic disturbances. ObjectiveTo identify the clinical and radiological features that should raise suspicion for the autoimmune encephalitis (AE)-like presentation of . . For this study, we compare the presenting clinical symptoms and paraclinical test results of autoimmune and infectious encephalitis patients. japanese encephalitis virus (jev) is the leading cause of viral neurological disease and disability in children under 15 years in asia, with an estimated 68,000 cases annually, 20-30% of which are fatal. Results from studies from the past 5 years suggest that 18 F-FDG-PET imaging might be more sensitive than MRI to . Bacterial encephalitis caused by spirochetes may present with similar features but requires different treatment. Central Nervous System Complications in HIV. . Yao K, Honarmand S, Espinosa A, Akhyani N, Glaser C, Jacobson S. Detection of human herpesvirus-6 in cerebrospinal fluid of patients with encephalitis. DTV encephalitis should be considered in the differential diagnosis of encephalitis in geographic areas that are endemic for Lyme disease. Confusion is a common presentation to the acute medical unit and has a wide differential diagnosis ( Box 2 ). Basilar Artery Thrombosis. Encephalitis should be considered when patients present with encephalopathy (altered mental status) for ≥24 hours and at least one of the following: fever >38 o C, seizures, focal neurologic findings, cerebrospinal fluid (CSF) pleocytosis, focal electroencephalogram or epileptiform activity, or abnormal neuroimaging [ 1, 2 ]. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a new diagnosis, as recent as 2007, that develops as a result of autoantibodies to the NMDA receptor. 2009 Mar. which will frame the workup and differential diagnosis. . Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. . At times the spread of the disease in the central nervous system may be slow and at other times rapid. Therefore diverse signs and symptoms are seen. Free full text . - Differential diagnosis of paraneoplastic encephalitis . Autoimmuneencephalitis refers to a group of conditions that occur when the body's immune systemmistakenly attacks healthy brain cells, leading to inflammation of the brain. PubMed CrossRef. Tick-borne encephalitis must be differentiated form other tick-borne diseases as well as infections induced by the different subtypes of tick-borne encephalitis virus (TBEV). Infective endocarditis is the infection and inflammation of the endocardium and the heart valves. Some EEG changes . Encephalitis is a pathological state of brain parenchymal dysfunction leading to an altered state of consciousness or focal neurological signs. General imaging differential considerations include: Dyke-Davidoff-Masson syndrome; Sturge Weber syndrome; unilateral megalencephaly; hemiconvulsion-hemiplegia . Differential Diagnosis III: Infective Endocarditis. Delirium Tremens (DTs) . Acute Disseminated Encephalomyelitis. Primary schizophreniform psychoses are thought to be caused by complex gene-environment interactions. In all these disorders, immunotherapy is usually effective. Viral infections to consider in the differential diagnoses of Japanese encephalitis include the following: Arboviral diseases (West Nile virus, Murray Valley encephalitis) Nipah virus infection. 1 approximately 30-50% of survivors have long-term sequelae. PubMed CrossRef. There are also behavioural changes, autonomic dysfunction and impaired consciousness. Figure 3 A 15-year-old female patient with Japanese encephalitis presented with bilateral visual disturbances. Encephalitis is a pathological state of brain parenchymal dysfunction leading to an altered state of consciousness or focal neurological signs. Signs and symptoms of a brain abscess may include fever, headache, nausea, vomiting, neck pain and stiffness, and nuchal rigidity as well as altered awareness, altered arousal, seizures, motor and sensory deficits . Therefore, the pediatrician should be familiar with the clinical symptoms, signs, laboratory features, neuroimaging changes, immunological characteristics, and differential diagnosis of this disease. Curr Opin Neurol. For a long time, they were considered the "gold standard" for the diagnosis of herpetic encephalitis in biopsies. DIFFERENTIAL DIAGNOSIS OF VIRAL ENCEPHALITIS The characteristic presentation of viral encephalitis usually consists of fever, headache, and clouding of consciousness together with seizures and focal neurology in some cases. From: Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders, 2018. . Differential Diagnoses Acute Hypoglycemia Brain Abscess in Emergency Medicine Cat Scratch Disease (Cat Scratch Fever) Emergent Management of Subarachnoid Hemorrhage Herpes Simplex Encephalitis. TBEV also shares a common disease vector with many other tick-borne diseases , therefore a healthcare provider must recognize the potential for multiple co-infections. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. The secondary or "symptomatic" forms of psychosis have reentered the focus stimulated by the discovery of autoantibody (Ab)-associated autoimmune . The criteria for AE were based on a Lancet paper of "A Clinical Approach to Diagnosis of Autoimmune Encephalitis" and "Chinese Expert Consensus for the Diagnosis and Treatment of . Currently, PCR is considered as the most reliable method of clinical diagnosis. It is important to keep HSV status in mind during treatment planning and in the differential diagnoses . In areas with greater risk of Eastern equine encephalitis virus transmission, EEE should be considered by health-care professionals in any case with aseptic meningitis or encephalitis. Recently, several novel autoantibodies have been identified which are closely associated with different subtypes of autoimmune encephalitis. These factors highlight the difficulty of accurate diagnosis of chagasic encephalitis and that more common confounders are . Due to the fact that herpetic rashes on the skin in such patients are rare, carrying out a differential diagnosis causes significant difficulties. Clinical assessment and investigations. An estimated 9% to 30% of patients with suspected stroke and 2.8% to 17% of patients treated with IV-tPA have stroke mimics. Differential Diagnosis. Some of the patient's symptoms were consistent with brain abscess, so that was included as a differential diagnosis. Autoantibodies directed against different synaptic proteins or receptors in patients with autoimmune encephalitis have recently been described and could indicate a differential diagnosis of infectious encephalitis. Confusion is a common presentation to the acute medical unit and has a wide differential diagnosis ().The clinical challenge is distinguishing causes of encephalopathy, including septic, metabolic, toxic and others, from patients who have encephalitis and therefore need specific treatments. The term limbic encephalitis refers to an inflammatory process of the limbic system including, the medial temporal lobes, amygdala, and cingulate gyri, resulting in severe memory deficits, behavioral changes, psychiatric symptoms and temporal lobe seizures. Aseptic Meningitis. The main differential to distinguish is encephalopathy secondary to metabolic or toxic disturbances. 2020 Oct;270(7):803-818. doi: 10.1007/s00406-020-01113-2. A clinical algorithm for the diagnosis of autoimmune encephalitis has recently been published. DOI 10.5001/omj.2014.15 Received: 17 Oct 2013/Accepted: 02 Dec 2013 Acknowledgements The authors reported no conflict of interests and no funding was received for this work. [Differential diagnosis of encephalitis due to anti-NMDA receptor antibodies] Anti-NMDAR encephalitis should be considered in young individuals with subacute presentation of psychiatric symptoms, abnormal movements, and autonomic dysfunction. Differential diagnosis. The radiologist plays an active role as imaging is an important tool for early diagnosis and excluding differential diagnoses, which can modify the progression of disease. Conditions to consider in the differential diagnosis of California encephalitis include the following: Other arbovirus encephalitides [ 6] Herpes simplex encephalitis Varicella zoster encephalitis. For a long time, they were considered the "gold standard" for the diagnosis of herpetic encephalitis in biopsies. 2007;6(5):442-455. 29 Additionally, it is important for providers to keep in mind the many encephalitis mimics and work diligently to rule out other causes. Serious, complex, and potentially fatal disorder with non-infectious and infectious causes. Summary: Therefore diverse signs and symptoms are seen. Deer tick virus (DTV) belongs to the group of tick-borne encephalitis RNA viruses in the flavivirus family. Curr Opin Infect Dis. Neuroimaging plays a key role in the management of patients with suspected limbic encephalitis by supporting diagnosis and excluding differential possibilities. [Medline] . Lancet Neurol. In areas with greater risk of Eastern equine encephalitis virus transmission, EEE should be considered by health-care professionals in any case with aseptic meningitis or encephalitis. Because of the large number of individuals infected in the United States, an expanded spectrum of the disease has been recognized. Open in a . Ann Neurol . Autoimmune etiologies associated with neural autoantibodies are becoming increasingly recognized making the diagnosis challenging.