Clinical relevance of positive voltage-gated potassium channel (VGKC)-complex antibodies: experience from a tertiary referral centre. While many cases of autoimmune encephalitis are indistinguishable from each other or viral encephalitis, there may be clues to specific autoimmune etiologies (Table 1). The term ‘limbic encephalitis’ (LE) describes the condition when limbic areas of the brain are inflamed (swollen) and consequently not functioning properly. Howe CL, et al. NMDAR and other cell surface antibody tests are most sensitive and specific with CSF. The Lancet Infections Diseases. The illness usually begins with less-specific symptoms such as fever, headache, stiff neck, vomiting and anorexia. Chancey C, Grinev A, Volkova E, Rios M. The global ecology and epidemiology of West Nile virus. Isaacs syndrome presents with muscle spasms, cramps and fasciculations due to peripheral nerve hyper-excitability.11,12 Morvan syndrome (Morvan's fibrilllary chorea) consists of peripheral nerve hyper-excitability with encephalitis and severe insomnia. Since these antibodies are both much rarer than other autoantibodies to the NMDA receptor, the status epilepticus in the setting of autoimmune encephalitis probably occurs more frequently with NMDAR antibodies overall. Background Olfactory dysfunction in MS patients is reported in the literature. 2018; doi:10.1002/ana.25131. The differential diagnosis of ADEM includes acute meningitis, acute viral encephalitis, autoimmune encephalitis and acute multiple sclerosis. Zachary KC. GAD65 as a prototypic autoantigen. people may have after encephalitis, why rehabilitation/support from These patients may be treated for longer periods of time with second line therapy, especially rituximab, but the optimal duration of treatment has not been established. Patients who worsen after infectious encephalitis should therefore be carefully evaluated for both infectious and autoimmune etiologies. Das Wort "disseminata" bezeichnet wiederum das verstreute Vorkommen dieser Entzündungen. Cross SA, Salomao DR, Parisi JE, Kryzer TJ, Bradley EA, Mines JA, et al. Viral encephalitis in adults. Encephalitis. Fatigue or weakness. Yamamoto W, Ogusa E, Matsumoto K, Maruta A, Ishigatsubo Y, Kanamori H. Human herpesvirus-6 encephalopathy after hematopoietic stem cell transplantation and class I human leukocyte antigen. Ziel der Untersuchung war es, diffusionsgewichtete und kontrastverstärkte Untersuchungen bezüglich der Erkennung aktiver Läsionen zu vergleichen. Bernal F, Shams'ili S, Rojas I, Sanchez-Valle R, Saiz A, Dalmau J, et al. Repeating CSF cytology, awareness of known tumors, and screening for malignancy may all help lead to recognition of these causes. If you have found this information helpful, please consider making a donation to help us continue our life-saving work in the future. LGI1/CASPR2-antibody encephalitis is an autoimmune encephalitis in which antibodies target LGI1 (leucine-rich glioma inactivated 1) or CASPR2 (contactin-associated protein 2). Clinical analysis of anti-Ma2-associated encephalitis. Autoimmune encephalitis may be divided into several groups of diseases: those with pathogenic antibodies to cell surface proteins, those with antibodies to intracellular synaptic proteins, T-cell diseases associated with antibodies to intracellular antigens, and those associated with other autoimmune disorders. Cancers are relatively rare in this disorder, and some of these may be chance events in this older age group. Seminars in Neurology. The emergency care team will likely include specialists in infectious diseases, the brain and nervous system (neurologist). Saunders Elsevier; 2020. http://www.clinicalkey.com. Fallon BA, Levin ES, Schweitzer PJ, Hardesty D. Inflammation and central nervous system Lyme disease. Anti-Ri (ANNA-2) associate with diverse syndromes including cerebellar degeneration and encephalitis. Mayo Clinic; 2022. Differentiation of these diseases is not easy, certainly in the early stages. Check out these best-sellers and special offers on books and newsletters from Mayo Clinic Press. Our Support Service is here to help you with any questions about encephalitis, recovery and rehabilitation or emotional support. Thomas AR. Have you had unprotected sex with a new or long-term sexual partner? 1087843. This risk be can reduced with use of a GnRH agonist in women,82 or addressed with egg/sperm collection. Materials and Methods OB and olfactory brain volume was assessed within 34 MS . Greene M, Lai Y, Baella N, Dalmau J, Lancaster E. Antibodies to Delta/notch-like epidermal growth factor-related receptor in patients with anti-Tr, paraneoplastic cerebellar degeneration, and Hodgkin lymphoma. Kennzeichnend sind im Gewebe verteilte (örtlich disseminierte) herdförmige ZNS -Läsionen, die primär durch Demyelinisierung von Nervenfasern und durch sekundäre axonale Schäden entstehen. ; The leading cause of severe encephalitis is the herpes simplex virus. Cell surface/synaptic antibodies are generally found in the spinal fluid only in patients with the relevant neurological disorder and are not found incidentally. Nakae Y, Ikeda S, Yamamoto R, Tanaka F, Johkura K. Hemichorea in a thymoma patient without anti-CRMP-5 antibody. Steroids may be beneficial in a range of autoimmune disorders but could potentially create problems with the diagnosis of certain disorders such as CNS lymphoma. Date created November 2001; Date updated: October 2021; Review date: October 2024. Neurologic complications of gastric bypass surgery for morbid obesity. EEG is useful in patients with autoimmune or infectious encephalitis for excluding subclinical seizures, for prognosis, and sometimes for suggesting particular diagnoses. Yamamoto S, Takahashi S, Tanaka R, Okayama A, Araki A, Katano H, et al. Encephalitis is a rare condition that is most often caused by viruses (viral encephalitis). Gluckman SJ. Due to its relatively favorable safety profile, rituximab is more often used as monotherapy in children. Magnetic resonance (MR) imaging is one of the best methods in diagnosis of multiple sclerosis, particularly in disclosure of active demyelinating lesions. Ancillary testing with MRI, EEG, and lumbar puncture may further support a diagnosis of encephalitis and potentially suggest particular causes. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. However, GAD65 may co-exist with other autoantibodies, such as GABA-B-R, so may or may not represent the most relevant pathophysiological mechanism in some patients with multiple antibodies. If you would like more information on the source material the author used to write this document please contact the Encephalitis Society. der Multiplen Sklerose lassen sich Entzündungen in Gehirn und Rückenmark an mehreren Stellen nachweisen. Dubey D, et al. Wir von Navigator-Medizin.de meinen ja. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Evaluation should begin with a detailed history and physical examination to detect clues to specific causes. The antibodies themselves are not directly pathogenic. Received 2015 Oct 1; Revised 2015 Oct 2; Accepted 2015 Oct 3. Leypoldt F, Titulaer MJ, Aguilar E, Walther J, Bönstrup M, Havemeister S, et al. The precise mixture of symptoms varies from patient to patient. Intravenous fluids to ensure proper hydration and levels of essential minerals. Dalmau J, Furneaux HM, Rosenblum MK, Graus F, Posner JB. Ohkawa T, Fukata Y, Yamasaki M, Miyazaki T, Yokoi N, Takashima H, et al. Inclusion in an NLM database does not imply endorsement of, or agreement with, This may include surgery, radiation, chemotherapy or a combination of treatments. Antibodies to inhibitory synaptic proteins in neurological syndromes associated with glutamic acid decarboxylase autoimmunity. However, opportunistic CNS infections after autoimmune encephalitis are probably very rare compared to worsening of the autoimmune disease. GAD65 antibodies have diverse clinical correlates, including SPS, cerebellar degeneration, epilepsy, and type 1 diabetes.43 In the context of encephalitis, especially with epilepsy, a CSF GAD65 response is evidence of an autoimmune etiology. The illness has been poorly understood and a variety of terminologies are used to describe it, these including post-viral, post-infectious or para-infectious encephalitis. Optic neuritis has also been reported.77,78,79, Anti-Ma2 (PMNA-2) are found most often in young men with germ cell tumors. Symptoms include fever, headache, and altered mental status, often accompanied by seizures or focal . The expanding field of IgG4-mediated neurological autoimmune disorders. Journal of Clinical Neurology (Seoul, Korea), http://creativecommons.org/licenses/by-nc/3.0/, Most characteristic of GABA-B-R and GABA-A-R but NMDAR is much more common; may occur in other types as well, Neuromyotonia, muscle spasms, fasciculations, Stiff-person syndrome and/or exaggerated startle, GAD65, GlyR, Amphiphysin (with GAD65 being most common in stiff person/stiff limb and GlyR in PERM, and Amphiphysin in women with breast cancer), CNS (myoclonus, startle, delirium) and gastrointestinal hyper-excitability, Ma2, Hu, Miller-Fisher, Bickerstaff (but also infections like Sarcoidosis, Lyme, TB), GAD65, PCA-1 (Yo), ANNA-1 (Hu), DNER (Tr), mGluR1, VGCC, Consider infectious causes of encephalitis in visited region, Opportunistic infections, risk depending on CD4 count, Opportunistic infections (CMV, VZV, HSV1, 6, 7); if recently transplanted, consider infection from donor, Consider specific paraneoplastic syndromes based on tumor, but also lymphomatous/carcinomatous tumor involvement, Consider relapse of initial encephalitis, secondary autoimmune causes, and (if immunosuppressed) opportunistic infections, A common cause in both healthy and immune-compromised patients, with particular predilection for the temporal lobes, Once a leading cause in East Asia, but declining due to vaccination programs, Other, non-polio, strains may also be neurotropic and it is a relatively common cause of encephalitis, Rare cause in immune compromised patients, 10-15% of untreated patients have neurological symptoms, Widely distributed mosquito-born flavivirus, Latex agglutination antigen test, culture, More often presents with meningitis in patients with AIDS and other immune-compromised states, Culture, biopsy, antigen ELISA and other methods, Disseminated CNS aspergillosus is mostly in immune compromised (transplant patients), and pathology usually involves basal ganglia and/or thalami, Culture, biopsy (ideally for nasal involvement), May affect both immunocompromised and immune intact persons, In one study the second most common cause of infectious temporal lobe encephalitis behind HSV, Rhombencephalitis and meningitis are the two main manifestations, Classically, a common cause of brain lesions in patients with AIDS. For instance, West Nile encephalitis has been reported only once in Korea, in a patient who had recently traveled to Guinea.20 Travel to areas endemic for malaria or Lyme disease may be similarly relevant. Download PDF Acute disseminated encephalomyelitis (ADEM), By Professor Clive Hawkins, Professor of Clinical Neurology, Consultant Neurologist, University Hospital of North Staffordshire and reviewed by Dr Sarosh Irani, John Radcliffe Hospital, Oxford and Dr. Arun Venkatesan, Johns Hopkins Hospital, Baltimore, MD, USA. HHS Vulnerability Disclosure, Help Paraneoplastic cerebellar degeneration. Sonia TroweFachärztin für Dermatologie und Venerologie, Dr. med. Advanced brain imaging with PET or SPECT has shown diverse areas of regional hyper- or hypo-metabolism in patients with NMDAR, LGI1, Caspr2 or other autoantibodies.47 These studies have not reached the point where any particular form of encephalitis can be distinguished from another, so I do not generally rely on these studies to rule in or rule out autoimmune causes in my practice. Autoantibodies to two important inhibitory receptors in the brain, GABA-B and GABA-A receptors (at high titer) convey a high risk of severe seizures and intractable status epilepticus.8,9 GAD65 antibodies may present with epilepsy, perhaps also with memory impairment, but with few other symptoms to suggest an autoimmune etiology. Multiple Sklerose (MS, Encephalomyelitis disseminata) ist eine chronische Entzündung des Nervensystems. 2016;263:277. When this is suspected, imaging of the cerebral vessels, search for other evidence of vasculitis (such as serologies for lupus and other rheumatologic diseases) may be useful. Some cases of Isaacs syndrome are associated with autoantibodies to Caspr2 or other, often undefined, members of the voltage-gated potassium channel (VGKC) complex.13,14 Caspr2 antibodies are even more likely in patients with Morvan syndrome, especially patients with thymoma, who may have multiple autoimmune disorders during their disease course. Herpes simplex virus-1 encephalitis can trigger anti-NMDA receptor encephalitis: case report. Introduction. [1] [2] Encephalomyelitis disseminata, a synonym for multiple sclerosis. Accessed April 5, 2022. Infectious Disease Clinics of North America. This content does not have an Arabic version. Titulaer MJ, Dalmau J. Graus F, Keime-Guibert F, Reñe R, Benyahia B, Ribalta T, Ascaso C, et al. Seizures may occur at any point during the disease course of anti-NMDAR encephalitis, including at presentation.49 The extreme delta brush pattern may be observed in patients with anti-NMDAR encephalitis, most often in patients who are comatose.50 This distinctive EEG pattern should prompt testing for NMDAR antibodies. Although mGluR1 and mGluR5 are close homologs, the antibodies do not cross react and the clinical syndromes are distinct. In rare cases it can be caused by bacteria or even fungi. What are the priority areas of research for the Encephalitis Society? Treatment for mild encephalitis usually consists of: Bed rest. Patients may relapse and should receive appropriate follow-up care from a physician familiar with the diseases. The risk of relapse in anti-NMDAR encephalitis in approximately 12% over two years (but continues beyond that) and is highest in untreated patients, intermediate in patients who had only first-line therapy, and lowest in patients treated with second-line therapies.84 Relapsed patients are usually treated with second-line therapies, possibly after first line therapies. Registered in England and Wales No. Epidemiology of Japanese encephalitis in South Korea, 2007-2010. Symptoms may fluctuate rapidly. Hart IK, Waters C, Vincent A, Newland C, Beeson D, Pongs O, et al. The median age is about 60 years . Before Patients with anti-NMDAR encephalitis and other forms of autoimmune encephalitis may also have prolonged periods of unresponsiveness and abnormal behaviors that are not due to seizures, so in these cases prolonged EEG monitoring may be very helpful. Lancaster E, Martinez-Hernandez E, Titulaer MJ, Boulos M, Weaver S, Antoine JC, et al. But if these antibodies are not found immune therapy should be considered. In other types of autoimmune encephalitis, the risk of relapse is less clearly established. The duration of these symptoms is variable. Anti-LGI1 (Leucine-rich, glioma-inactivated 1) encephalitis accounts for most cases of encephalitis previously attributed to VGKC antibodies14,41 (The VGKC test, as described above, detects most LGI1 cases and also most Caspr2 cases). Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, et al. Autoantibodies to epilepsy-related LGI1 in limbic encephalitis neutralize LGI1-ADAM22 interaction and reduce synaptic AMPA receptors. Accessed April 5, 2022. Understanding the pathophysiology of these diseases is helpful in using diagnostic testing and choosing appropriate therapies. AskMayoExpert. The classical presentation of encephalitis consists of a subacute (days to a few weeks) progressive decrease in the level of consciousness, often with fluctuations, and altered cognition. These may include: Some people with autoimmune encephalitis need long-term treatment with immunosuppressive medicines. Human herpesvirus 6 (HHV-6), and less often HHV-7, may cause encephalitis and their reactivation may be an important cause of encephalitis in transplant patients.23,24,25 HHV-6 and HHV-7 are diagnosed by PCR, although this may not be positive initially.26 There are no approved treatments, but ganciclovir, foscarnet, and cidofovir have been used.27 HHV-6 may be more likely in cases whether the donor and/or recipient of a stem cell transplant carry a particular HLA type (HLA-B*40:06).28. Saylor D, Thakur K, Venkatesan A. Juhasz-Pocsine K, Rudnicki SA, Archer RL, Harik SI. Thus a low titer serum VGKC result without corresponding evidence of LGI1 or Caspr2 antibodies, ideally in the CSF, should not be taken as definitive evidence of autoimmune encephalitis. Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA. Prichard MN, Whitley RJ. Most people with infectious encephalitis have flu-like symptoms, such as: Headache. An important recent advance has been the recognition that patients with HSV encephalitis may rarely develop anti-NMDAR encephalitis several weeks later as a post-infectious complication.85 In these patients CSF from the initial attack has no NMDAR antibodies with positive PCR for HSV, but CSF from the second attack now has NMDAR antibodies with negative PCR for HSV. Murinson BB, Guarnaccia JB. [1] Similarly, antibodies to Homer-3, which organizes mGluR1 at synapses, have been reported in a single patient. Da die Entzündungs- bzw. med. Although there are not randomized treatment trials, protocols have been proposed for anti-NMDAR encephalitis,1 and these approaches have been applied to other diseases in the cell-surface/synaptic autoantibody category. Acute disseminated encephalomyelitis (ADEM) Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory condition that affects the brain and spinal cord. A variegated squirrel bornavirus associated with fatal human encephalitis. Advertising revenue supports our not-for-profit mission. In patients with HSV encephalitis, EEG may predict prognosis in addition to helping exclude non-convulsive seizures; normal EEG correlates with good outcomes independent of other prognostic factors.48. Infections may be detected by PCR, culture or other less invasive methods. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. The Encephalitis Society is the operating name of the Encephalitis Support Group which is a registered Charity and Company Limited by Guarantee. The cancer associations are variable, and the prognosis tends to be much better. Lupus may affect diverse areas of the nervous system, causing neuropathy, vasculitis, myelitis, venous sinus thrombosis, stroke, and other manifestations.15 Neuropsychiatric lupus may manifest with seizures, psychosis, or neurovascular disease. Recently long-term studies of patients with ADEM have shown that a small number later develop multiple sclerosis, although this area remains controversial. Singh TD, et al. Ganz genau genommen muss es sogar Encephalomyelitis disseminata heißen. The antibodies target a family of intracellular proteins. These may include azathioprine (Imuran, Azasan), mycophenolate mofetil (CellCept), rituximab (Rituxan) or tocilizumab (Actemra). West Nile encephalitis is a type of infectious encephalitis caused by West Nile virus. Journal of Neurology. Long-term sequelae of West Nile virus-related illness: A systematic review. Fay AJ, Noetzel MJ, Mar SS. Detailed laboratory studies involving measurement of anti-brain antibodies and of cellular immune responses to specific brain antigens suggest that these patients have developed an allergic immune response attack against their own brain constituents, and this is an ‘autoimmune’ response. Vasculitis affecting the CNS may rarely present with symptoms resembling encephalitis. Hampton CW, Galperin D, Alvarez E, West MS. Encephalomyeloneuropathy with CRMP-5 antibodies in a patient with a primary mediastinal serinoma. Human herpesvirus-6 infection-associated acute encephalopathy without skin rash. Selten verursacht eine Kunjin-Infektion eine Enzephalitis. In some cases fascio-brachial dystonic seizures precede other symptoms of the disease by months. Daroff RB, et al. It is typical for patients with autoimmune encephalitis to have testing for various infectious etiologies and they are frequently covered with antibiotic and/or antiviral therapies (such as acyclovir) empirically as infectious causes are excluded. Autoimmune encephalitis therefore should enter into the differential diagnosis of any case of suspected neuroleptic malignant syndrome (Patients with anti-NMDAR encephalitis may be particularly sensitive to strong dopamine antagonists, and our group attempts to avoid using these medications). Caspr2 and LGI1 each associate with VGKCs. Basal ganglia encephalitis has also been reported with D2R antibodies, although this may be very rare.6 Sydenham chorea is a well-recognized autoimmune movement disorder thought to be triggered by streptococcal infections and should be considered in children with this presentation.7. Dale RC, Merheb V, Pillai S, Wang D, Cantrill L, Murphy TK, et al. Registered Office as above. professionals and families are important and what options for 3) Treatment with steroids, rituximab, or cyclophosphamide could complicate tumor diagnosis in the case of tumors like lymphoma. 9th ed. This content does not have an English version. Encephalitis is a serious neurological condition and unfortunately, despite improvements in specific and more supportive treatments such as excellent intensive care management, encephalitis still has a high mortality (death) rate. Responses to treatment are similar in children and adults, with about half failing first line therapies. Ariño H, Höftberger R, Gresa-Arribas N, Martínez-Hernández E, Armangue T, Kruer MC, et al. 2016;6:28699. Myoclonus, hyponatremia, and fascio-brachial dystonic seizures are common. High doses of steroids (drugs used to reduce inflammation) can often lead to a rapid resolution of symptoms with an excellent prognosis.
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