Az autoimmun encephalitisek laboratóriumi vizsgálati lehetőségei

2018 ◽  
Vol 159 (3) ◽  
pp. 107-112
Author(s):  
Katalin Böröcz ◽  
Zsófia Hayden ◽  
Viktória Mészáros ◽  
Zsuzsanna Csizmadia ◽  
Kornélia Farkas ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Early Stage ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Immunosuppressive Drugs ◽  
Synaptic Proteins ◽  
Receptor Proteins ◽  
Surface Receptors ◽  
The Past ◽  
Paraneoplastic Limbic Encephalitis

Abstract: Introduction: The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABABR, AMPAR) or synaptic proteins (LGI1, CASPR2). Aim: Our aim is to bring to awareness the increasing number of autoimmune encephalitis patients requiring neurologic, psychiatric and intensive care and to emphasize the significance of detecting various autoantibodies in diagnosing patients. Method: In the past 6 years, our laboratory received 836 autoimmune encephalitis diagnostic test requests from a total of 717 patients. Serum and cerebrospinal fluid (CSF) samples were analysed with indirect immunofluorescence using a BIOCHIP consisting of cell lines transfected with 6 different receptor proteins. Results: IgG autoantibodies against receptor proteins were present in 7.5% of patients. The frequency of positive samples was the following: NMDAR > LGI1 > GABABR > CASPR2. Conclusion: Detecting autoantibodies facilitates the diagnosis of autoimmune encephalitis in an early stage. Patients diagnosed early can be effectively treated with plasmapheresis and immunosuppressive drugs. The efficiency of therapies can be monitored by autoantibody detection. Therefore, the diagnostic immune laboratory plays an important role in proper diagnosis and in the prevention of rapidly progressing symptoms. Orv Hetil. 2018; 159(3): 107–112.

scholarly journals Functional Recovery in Autoimmune Encephalitis: A Prospective Observational Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Thomas Seifert-Held ◽  
Katharina Eberhard ◽  
Christian Lechner ◽  
Stefan Macher ◽  
Harald Hegen ◽  
...  
Keyword(s):  
Functional Recovery ◽  
Treatment Options ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Synaptic Proteins ◽  
Group Iii ◽  
Surface Receptors ◽  
Group I ◽  
Group Ii

BackgroundProspective observations of functional recovery are lacking in patients with autoimmune encephalitis defined by antibodies against synaptic proteins and neuronal cell surface receptors.MethodsAdult patients with a diagnosis of autoimmune encephalitis were included into a prospective registry. At 3, 6 and 12 months of follow-up, the patients’ modified Rankin Scale (mRS) was obtained.ResultsPatients were stratified into three groups according to their antibody (Ab) status: anti-NMDAR-Ab (n=12; group I), anti-LGI1/CASPR2-Ab (n=35; group II), and other antibodies (n=24; group III). A comparably higher proportion of patients in group I received plasma exchange/immunoadsorption and second line immunosuppressive treatments at baseline. A higher proportion of patients in group II presented with seizures. Group III mainly included patients with anti-GABABR-, anti-GAD65- and anti-GlyR-Ab. At baseline, one third of them had cancer. Patients in groups I and III had much higher median mRS scores at 3 months compared to patients in group II. A median mRS of 1 was found at all follow-up time points in group II.ConclusionsThe different dynamics in the recovery of patients with certain autoimmune encephalitides have important implications for clinical trials. The high proportion of patients with significant disability at 3 months after diagnosis in groups I and III points to the need for improving treatment options. More distinct scores rather than the mRS are necessary to differentiate potential neurological improvements in patients with anti-LGI1-/CASPR2-encephalitis.

scholarly journals Th17 lymphocytes drive vascular and neuronal deficits in a mouse model of postinfectious autoimmune encephalitis

2020 ◽  
Vol 117 (12) ◽  
pp. 6708-6716 ◽  
Author(s):  
Maryann P. Platt ◽  
Kevin A. Bolding ◽  
Charlotte R. Wayne ◽  
Sarah Chaudhry ◽  
Tyler Cutforth ◽  
...  
Keyword(s):  
Microglial Activation ◽  
Neural Circuit ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Abrupt Onset ◽  
Synaptic Proteins ◽  
Excitatory Synapses ◽  
Odor Processing ◽  
Th17 Lymphocytes

Antibodies against neuronal receptors and synaptic proteins are associated with a group of ill-defined central nervous system (CNS) autoimmune diseases termed autoimmune encephalitides (AE), which are characterized by abrupt onset of seizures and/or movement and psychiatric symptoms. Basal ganglia encephalitis (BGE), representing a subset of AE syndromes, is triggered in children by repeated group AStreptococcus(GAS) infections that lead to neuropsychiatric symptoms. We have previously shown that multiple GAS infections of mice induce migration of Th17 lymphocytes from the nose into the brain, causing blood–brain barrier (BBB) breakdown, extravasation of autoantibodies into the CNS, and loss of excitatory synapses within the olfactory bulb (OB). Whether these pathologies induce functional olfactory deficits, and the mechanistic role of Th17 lymphocytes, is unknown. Here, we demonstrate that, whereas loss of excitatory synapses in the OB is transient after multiple GAS infections, functional deficits in odor processing persist. Moreover, mice lacking Th17 lymphocytes have reduced BBB leakage, microglial activation, and antibody infiltration into the CNS, and have their olfactory function partially restored. Th17 lymphocytes are therefore critical for selective CNS entry of autoantibodies, microglial activation, and neural circuit impairment during postinfectious BGE.

scholarly journals Apheresis in Autoimmune Encephalitis and Autoimmune Dementia

2020 ◽  
Vol 9 (9) ◽  
pp. 2683 ◽  
Author(s):  
Rosa Rössling ◽  
Harald Prüss
Keyword(s):  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Synaptic Proteins ◽  
First Line ◽  
First Line Therapy ◽  
Neuronal Autoantibodies ◽  
Neurodegenerative Dementia ◽  
Pathogenic Antibodies ◽  
Clinical Conditions

Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease. Underlying autoantibodies can bind to neuronal surfaces and synaptic proteins resulting in psychiatric symptoms, focal neurological signs, autonomic dysfunction and cognitive decline. Early and effective treatment is mandatory to reduce clinical symptoms and to achieve remission. Therapeutic apheresis, involving both plasma exchange (PE) and immunoadsorption (IA), can rapidly remove pathogenic antibodies from the circulation, thus representing an important first-line treatment in AE patients. We here review the most relevant studies regarding therapeutic apheresis in AE, summarizing the outcome for patients and the expanding clinical spectrum of treatment-responsive clinical conditions. For example, patients with slowly progressing cognitive impairment suggesting a neurodegenerative dementia can have underlying autoantibodies and improve with therapeutic apheresis. Findings are encouraging and have led to the first ongoing clinical studies assessing the therapeutic effect of IA in patients with anti-neuronal autoantibodies and the clinical presentation of dementia. Therapeutic apheresis is an established and well tolerated option for first-line therapy in AE and, potentially, other antibody-mediated central nervous system diseases.

scholarly journals Neuroimmunological antibody-mediated encephalitis and implications for diagnosis and therapy in neuropsychiatry

2019 ◽  
Vol 32 (4) ◽  
pp. 177-185
Author(s):  
Joseph E. Marinas ◽  
Dmitriy Matveychuk ◽  
Serdar M. Dursun ◽  
Glen B. Baker
Keyword(s):  
Psychiatric Symptoms ◽  
Dopamine D2 Receptor ◽  
D2 Receptor ◽  
Initial Point ◽  
Neuronal Cell ◽  
Clinical Importance ◽  
Autoimmune Encephalitis ◽  
Surface Proteins ◽  
Response To Treatment ◽  
Synaptic Receptors

AbstractThe past decade has seen a surge of reports and investigations into cases of autoimmune-mediated encephalitis. The increasing recognition of these disorders is especially of relevance to the fields of neurology and psychiatry. Autoimmune encephalitis involves antibodies against synaptic receptors, neuronal cell surface proteins and intracellular targets. These disorders feature prominent symptoms of cognitive impairment and behavioural changes, often associated with the presence of seizures. Early in the clinical course, autoimmune encephalitis may manifest as psychiatric symptoms of psychosis and involve psychiatry as an initial point of contact. Although commonly associated with malignancy, these disorders can present in the absence of an inciting neoplasm. The identification of autoimmune encephalitis is of clinical importance as a large proportion of individuals experience a response to immunotherapy. This review focuses on the current state of knowledge on n-methyl-d-aspartate (NMDA) receptor-associated encephalitis and limbic encephalitis, the latter predominantly involving antibodies against the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor, the γ-aminobutyric acid (GABA)B receptor and leucine-rich glioma-inactivated 1 (LGI1) protein. In addition, we briefly describe anti-dopamine D2 receptor encephalitis. A summary of the literature will focus on common clinical presentations and course, diagnostic approaches and response to treatment. Since a substantial proportion of patients with autoimmune encephalitis exhibit symptoms of psychosis, the relevance of this disorder to theories of psychosis and schizophrenia will also be discussed.

scholarly journals 433 - A possible link between Bipolar Disease and Frontotemporal Dementia

2021 ◽  
Vol 33 (S1) ◽  
pp. 52-53
Author(s):  
B. Jorge ◽  
C. Pedro ◽  
J. Carvalho ◽  
S. Carneiro ◽  
M. Mangas
Keyword(s):  
Frontotemporal Dementia ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Early Stage ◽  
Synaptic Proteins ◽  
Behavioral Disturbances ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Bipolar Disease ◽  
Personality Changes ◽  
Relationship Of

Background:Both neurological and psychiatric symptoms are observed among mental disorders and represent a challenge for the differential diagnosis, specially in old adults presenting behavioral changes. Investigations have documented manic/hypomanic symptoms from behavioral variant frontotemporal dementia(bvFTD), suggesting a relationship of bipolar disease (BD) with bvFTD.Research objective:This work aims to determine the relationship between patients with bipolar disease and behavioral variant frontotemporal dementia.Method:A non-systematic review of the literature is presented. Bibliographic selection was carried out through keyword research in MEDLINE and Google Scholar.Results:An early stage of bvFTD often displays a mix of behavioral disturbances and personality changes. Also, BP is associated with a higher risk of dementia in older adults and with cognitive impairment, where a subset of patients presents a neuroprogressive pattern during the disease course. It was shown a specific type of post-BD dementia with clinical features of bvFTD and cases of patients with marked manic symptoms for the first time in their life had subsequent diagnosis of FTD. Mutations in the progranulin gene (GRN) were the most frequent causes of autosomal dominant FTD and have also been reported in sporadic FTD. Genetic polymorphisms in this gene are also associated with schizophrenia and BD. An hypothetical model of shared mechanisms between bvFTD and BD was proposed, including specific mendelian mutations associated with genetic predisposition (e.g. brain-derived neurotrophic factor-BDNF gene) and environmental factors with an effect on cellular homeostasis (e.g. increased cell deaths, decreased synthesis of synaptic proteins) and an influence over behavioural and cognitive symptoms. Nevertheless, comparison of the executive functions, social cognition profiles and structural neuroimaging of bvFTD and elderly patients with BD showed difference in patterns.Discussion:Although BD is principally considered a neurodevelopment disorder, while FTD is a neurodegenerative disorder, follow-up studies of cognitive deficits, imaging, and genetics in BD patients could elucidate the possible correlation between these major diseases and may have implications for pathogenesis, as well as for treatment.

scholarly journals Clinical Characteristics and Outcome of Neuronal Surface Antibody-Mediated Autoimmune Encephalitis Patients in a National Cohort

2021 ◽  
Vol 12 ◽  
Author(s):  
Zsófia Hayden ◽  
Beáta Bóné ◽  
Gergely Orsi ◽  
Monika Szots ◽  
Ferenc Nagy ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Disease Onset ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Test Results ◽  
Nmdar Encephalitis

Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. In our current multicenter retrospective study, we analyzed the clinical characteristics and outcome of AE patients with positive neuronal cell surface autoantibody test results.Methods: A standard online questionnaire was used to collect demographic and clinical characteristics, laboratory and imaging data, therapy and prognosis of 30 definitive AE patients in four major clinical centers of the region.Results: In our study, 19 patients were positive for anti-NMDAR (63%), 6 patients (20%) for anti-LGI1, 3 patients for anti-GABABR (10%) and 3 patients for anti-Caspr2 (10%) autoantibodies. Most common prodromal symptoms were fever or flu-like symptoms (10/30, 33%). Main clinical features included psychiatric symptoms (83%), epileptic seizures (73%) and memory loss (50%). 19 patients (63%) presented with signs of central nervous system (CNS) inflammation, which occurred more frequently in elder individuals (p = 0.024), although no significant differences were observed in sex, tumor association, time to diagnosis, prognosis and immunotherapy compared to AE patients without CNS inflammatory markers. Anti-NMDAR encephalitis patients were in more severe condition at the disease onset (p = 0.028), although no significant correlation between mRS score, age, sex and immunotherapy was found. 27% of patients (n = 8) with associated tumors had worse outcome (p = 0.045) than patients without tumor. In most cases, immunotherapy led to clinical improvement of AE patients (80%) who achieved a good outcome (mRS ≤ 2; median follow-up 33 months).Conclusion: Our study confirms previous publications describing characteristics of AE patients, however, differences were observed in anti-NMDAR encephalitis that showed no association with ovarian teratoma and occurred more frequently among young males. One-third of AE patients lacked signs of inflammation in both CSF and brain MRI, which emphasizes the importance of clinical symptoms and autoantibody testing in diagnostic workflow for early introduction of immunotherapy, which can lead to favorable outcome in AE patients.

‘Higher then to her no bookes doe reach’

2018 ◽  
Author(s):  
Martha Vandrei
Keyword(s):  
Early Stage ◽  
Deep Understanding ◽  
Contemporary Philosophy ◽  
James I ◽  
Historical Method ◽  
The Past ◽  
Classical World ◽  
Material Evidence ◽  
Do So

This chapter and the following both draw the reader into seventeenth-century understandings of the past, and of Boudica in particular, and makes clear that in a time before disciplines, writers of ‘history’ were erudite commentators, immersed in political thought, the classical world, and contemporary ideas, as well as in drama, poetry, and the law. Chapter 1 shows the subtleties of Boudica’s place in history at this early stage by giving sustained attention to the work of Edmund Bolton (1574/5–c.1634), the first person to analyse the written and material evidence for Boudica’s deeds, and the last to do so in depth before the later nineteenth century. Bolton’s distaste for contemporary philosophy and his loyalty to James I were highly influential in determining the way the antiquary approached Boudica and her rebellion; but equally important was Bolton’s deep understanding of historical method and the strictures this placed on his interpretive latitude.

scholarly journals Autoimmune encephalitis in a South Asian population

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nilanka Wickramasinghe ◽  
Dhanushka Dasanayake ◽  
Neelika Malavige ◽  
Rajiva de Silva ◽  
Thashi Chang
Keyword(s):  
Sri Lanka ◽  
Psychiatric Symptoms ◽  
South Asians ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Asian Population ◽  
South Asian Population ◽  
Clinical Presentations

Abstract Background Autoimmune encephalitis (AE) is now considered a main, potentially curable cause of encephalitis, but remains conspicuously underreported from South Asia. We studied the clinical characteristics in relation to their antibody status and outcomes of patients presenting with AE in Sri Lanka. Methods Patients admitting to government hospitals who were clinically suspected of AE by an on-site neurologist were prospectively recruited over a period of 12 months. Sera and cerebrospinal fluid were tested for NMDAR, AMPAR1, AMPAR2, LGI1, CASPR2, GABARB1/B2 antibodies (Ab) using commercial cell-based assays. Demographic, clinical and laboratory data were compiled into an investigator-administered proforma. Patients were reviewed at 1 year follow up either in person or via telephone. Results One-hundred and forty-two patients from 21 of 25 districts in Sri Lanka (median age = 20.5 years; range 1–86 years; females = 61.3%) were recruited. Of them, 65 (45.8%; median age = 19 years; range 1–86 years; females = 64.6%) fulfilled diagnostic criteria for probable NMDAR-antibody encephalitis (NMDARE) and 6 (4.2%; median age = 44 years; range 28–71 years; females = 83.3%) limbic encephalitis (LE). Abnormal behaviour (95.3%), seizures (81.5%) and movement disorders (69.2%) were the most frequent clinical manifestations of probable NMDARE. NMDAR-antibodies were detectable in 29 (44.6%) and not detectable in 36 in CSF of probable-NMDARE patients. Abnormal EEG was more frequent (p = 0.003) while a worse outcome (OR = 2.78; 95% CI = 0.88–9.09) and deaths (OR = 2.38; 95% CI = 0.67–8.33) were more likely in antibody-negative than antibody-positive probable-NMDARE. Most patients with LE had amnesia (50%) and/or confusion (100%) with agitation (83.3%) and seizures (100%) but none had detectable antibodies to any of the antigens tested. Conclusions NMDARE is the commonest type of AE among South Asians as is the case worldwide. Clinical presentations of NMDARAb-positive and NMDARAb-negative AE patients do not significantly differ but EEG may be a useful marker of an autoimmune basis for psychiatric symptoms.

scholarly journals Anosognosia in People with Cognitive Impairment: Association with Cognitive Deficits and Behavioral Disturbances

2015 ◽  
Vol 5 (1) ◽  
pp. 42-50 ◽  
Author(s):  
Antonella De Carolis ◽  
Virginia Cipollini ◽  
Valentina Corigliano ◽  
Anna Comparelli ◽  
Micaela Sepe-Monti ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Deficits ◽  
Rating Scale ◽  
Motor Behavior ◽  
Psychiatric Symptoms ◽  
Early Stage ◽  
Functional Anatomy ◽  
Night Time ◽  
Behavioral Disturbances ◽  
The Relationship

Aims: To investigate, in a group of subjects at an early stage of cognitive impairment, the relationship between anosognosia and both cognitive and behavioral symptoms by exploring the various domains of insight. Methods: One hundred and eight subjects affected by cognitive impairment were consecutively enrolled. The level of awareness was evaluated by means of the Clinical Insight Rating Scale (CIRS). Psychiatric symptoms were evaluated using the Italian version of the Neuropsychiatric Inventory (NPI), whereas memory (memory index, MI) and executive (executive index, EI) functions were explored using a battery of neuropsychological tests and qualified by means of a single composite cognitive index score for each function. Results: A significant positive correlation between the total NPI score and global anosognosia score was found. Furthermore, both the MI and EI scores were lower in subjects with anosognosia than in those without anosognosia (p < 0.001 and p < 0.007, respectively). When the single domains of the CIRS were considered, anosognosia of reason of visit correlated with the EI score (r = -0.327, p = 0.01) and night-time behavioral disturbances (r = 0.225; p = 0.021); anosognosia of cognitive deficit correlated with depression (r = -0.193; p = 0.049) and the MI score (r = -0.201; p = 0.040); anosognosia of functional deficit correlated with the MI score (r = -0.257; p = 0.008), delusions (r = 0.232; p = 0.015) and aberrant motor behavior (r = 0.289; p = 0.003); anosognosia of disease progression correlated with the MI score (r = -0.236; p = 0.015), agitation (r = 0.247; p = 0.011), aberrant motor behavior (r = 0.351; p = 0.001) and night-time behavioral disturbances (r = 0.216; p = 0.027). Conclusions: Our study suggests that, in the early stage of cognitive impairment, anosognosia is associated with both cognitive deficits and behavioral disorders according to the specific functional anatomy of the symptoms.

Nitric oxide: a new role in intensive care

2020 ◽  
Vol 22 (1) ◽  
pp. 72-79
Author(s):  
Alexandra Lee ◽  
◽  
Warwick Butt ◽  
◽  
◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Potential Role ◽  
Nitric Oxide Donors ◽  
Ischaemia Reperfusion Injury ◽  
Surface Receptors ◽  
The Past ◽  
Ischaemia Reperfusion ◽  
Human Experiments ◽  
Endogenous Nitric Oxide

Inhaled nitric oxide has been used for 30 years to improve oxygenation and decrease pulmonary vascular resistance. In the past 15 years, there has been increased understanding of the role of endogenous nitric oxide on cell surface receptors, mitochondria, and intracellular processes involving calcium and superoxide radicals. This has led to several animal and human experiments revealing a potential role for administered nitric oxide or nitric oxide donors in patients with systemic inflammatory response syndrome or ischaemia–reperfusion injury, and in patients for whom exposure of blood to artificial surfaces has occurred.

Sign in / Sign up

Export Citation Format

Share Document