scholarly journals Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum

2020 ◽  
Vol 63 (8) ◽  
pp. 291-300 ◽  
Author(s):  
Joo Hee Seo ◽  
Yun-Jin Lee ◽  
Ki Hyeong Lee ◽  
Elakkat Gireesh ◽  
Holly Skinner ◽  
...  

Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

Author(s):  
Andrew McKeon

Autoimmune encephalitis clinically encapsulates a spectrum of disorders including limbic encephalitis, and other autoimmune CNS disorders, which often have a paraneoplastic cause. Unlike multiple sclerosis, autoimmune encephalitides are unified by well-characterized neural-specific IgG biomarkers detectable in serum or CSF. Diagnostic laboratory, in vitro and neuropathological studies have demonstrated two broad groups. The first, characterizable by the detection of neuronal nuclear, cytoplasmic, or nucleolar antibodies (such as ANNA-1, aka anti-Hu), likely have a cytotoxic T cell mediated pathogenesis. The second, characterizable by the detection of antibodies targeting plasma membrane antigens, such as NMDA receptor, are likely mediated (at least in part) by pathogenic antibody.


2020 ◽  
Vol 33 (2) ◽  
pp. 73-84 ◽  
Author(s):  
Jeffrey Cummings

Reasons for the Study: The Neuropsychiatric Inventory (NPI) has been used for studies of neuropsychiatric symptoms in neurodegenerative disorders for the past 25 years. This article reviews the history of the development and application of the NPI. Main Findings: The NPI consists of 10 (or 12) items that are assayed with questions, subquestions, and ratings of frequency and severity. The NPI has been shown to be valid and reliable. The NPI has been translated into approximately 40 languages; it has 4 of versions designed for different clinical applications. The NPI studies show contrasting profiles of behavioral symptoms in different neurologic disorders. The NPI has been used in approximately 350 clinical trials. In economic studies, the NPI captures the cost of behavioral symptoms in dementias. Principle Conclusions: The NPI is a useful instrument for capturing behavioral changes in Alzheimer disease and other neurodegenerative disorders.


2020 ◽  
Author(s):  
Keisuke Ueda ◽  
Kevin J. Black

Tic disorders are the most common movement disorders in a pediatric practice. Moreover, tic disorders are frequently associated with comorbid neuropsychiatric symptoms. Therefore, understanding tic disorders is vital for general practitioners to deliver appropriate care to patients with tics. However, misunderstanding and misconceptions of tic disorders still exist among the general population and in health care providers. For the past decade, many studies have been conducted to elucidate tic disorders. This article aims to provide an overview about tics and tic disorders, and recent information on tic disorders including history, definition, diagnosis, epidemiology, etiology, diagnostic approach, comorbidities, treatment and management, and differential diagnosis.


Author(s):  
Elias Manca

AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neuropsychiatric symptoms can range from relatively mild manifestations, such as headache, to more severe complications, such as psychosis. The evaluation of the presence of the autoantibodies in the serum of these patients is the most helpful diagnostic tool for the assessment of the disease. The scientific progresses achieved in the last decades helped researchers and physicians to discover some of autoepitopes targeted by the autoantibodies, although the majority of them have not been identified yet. Additionally, the central nervous system is full of epitopes that cannot be found elsewhere in the human body, for this reason, autoantibodies that selectively target these epitopes might be used for the differential diagnosis between patients with and without the neuropsychiatric symptoms. In this review, the most relevant data is reported with regard to mechanisms implicated in the production of autoantibodies and the most important autoantibodies found among patients with systemic lupus erythematosus with and without the neuropsychiatric manifestations.


2018 ◽  
Vol 115 (33) ◽  
pp. 8252-8259 ◽  
Author(s):  
Will Steffen ◽  
Johan Rockström ◽  
Katherine Richardson ◽  
Timothy M. Lenton ◽  
Carl Folke ◽  
...  

We explore the risk that self-reinforcing feedbacks could push the Earth System toward a planetary threshold that, if crossed, could prevent stabilization of the climate at intermediate temperature rises and cause continued warming on a “Hothouse Earth” pathway even as human emissions are reduced. Crossing the threshold would lead to a much higher global average temperature than any interglacial in the past 1.2 million years and to sea levels significantly higher than at any time in the Holocene. We examine the evidence that such a threshold might exist and where it might be. If the threshold is crossed, the resulting trajectory would likely cause serious disruptions to ecosystems, society, and economies. Collective human action is required to steer the Earth System away from a potential threshold and stabilize it in a habitable interglacial-like state. Such action entails stewardship of the entire Earth System—biosphere, climate, and societies—and could include decarbonization of the global economy, enhancement of biosphere carbon sinks, behavioral changes, technological innovations, new governance arrangements, and transformed social values.


1986 ◽  
Vol 113 (2) ◽  
pp. 255-260 ◽  
Author(s):  
Andrzej Gardas ◽  
Kathleen L. Rives

Abstract. A sensitive and specific enzyme-linked immunosorbent assay (ELISA) for the detection of autoantibodies reacting with thyroid plasma membrane antigens has been established. Autoantibodies reacting with thyroid plasma membrane antigens were detected by the ELISA in 95% of untreated hyperthyroid Graves', 68% of antithyroid drug-treated Graves' up to four months of the therapy, in 62% of Hashimoto's thyroiditis and in 8.9% of toxic nodular goitre. The ELISA was negative in 100% healthy blood donors, 100% non-toxic nodular goitre, in 12 patients with rheumatoid arthritis, 18 patients with scleroderma and 94% of patients with systemic lupus erythematosus. The mean value of autoantibodies titre was higher in untreated hyperthyroid Graves' (1:84 000) and lowest in positive patients with autoimmune disease of non-thyroid origin (1:4000). The cross-reactivity of antimicrosomal antigen antibodies was below 10%; there was no influence of antithyroglobulin antibodies on the ELISA; and most of the autoantibodies react with plasma membrane antigens different from the TSH binding sites.


Author(s):  
Виталий Вячеславович Аксёнов ◽  
Николай Михайлович Агарков ◽  
Александра Игоревна Сурнина

Заболеваемость раком яичников в России в последнее время остается на высоком уровне. В мире более ста тысяч женщин умирают вследствие протекания данного заболевания. За последнее десятилетие заболеваемость острым эндометритом также неуклонно возрастает. Острый эндометрит обладает полиморфизмом симптомов, лабораторных и ультразвуковых изменений и вследствие этого тяжело поддается диагностике и дифференциальной диагностике. В условиях стационара обследованы 100 пациенток с раком яичников II-III стадии и 90 пациенток с диагнозом острого эндометрита. Им выполнялось ультразвуковое исследование. Полученные результаты подвергались обработке и математико-статистическому анализу, включающему расчёт показателей дезинтеграции, сетевое моделирование, математическое ранжирование. Изучение ультразвуковых изменений кровотока в маточных и яичниковых сосудах и венах у 100 заболевших раком яичников и 90 заболевших острым эндометритом дал возможность выделить ведущие дифференциально-диагностические аспекты. Характеристики дезинтеграции, в одном ряду со средними значениями локального кровотока, объективизируют дифференциацию рака яичников и острого эндометрита. В согласовании с дифференциально-диагностической значимостью ультразвуковых характеристик артериального кровотока в маточных и яичниковых сосудах построена сетевая модель дифференциальной диагностики рака яичников и острого эндометрита по более приоритетным переменам, собственно, что разрешает уменьшить размер и время обследования пациента и постановки верного диагноза The incidence of ovarian cancer in Russia has recently remained at a high level. In the world, more than a hundred thousand women die as a result of the course of this disease. The incidence of acute endometritis has also been steadily increasing over the past decade. Acute endometritis has a polymorphism of symptoms, laboratory and ultrasound changes and, as a result, is difficult to diagnose and differential diagnosis. 100 patients with stage II-III ovarian cancer and 90 patients with acute endometritis were examined in the hospital. They performed an ultrasound examination. The obtained results were processed and subjected to mathematical and statistical analysis, including the calculation of disintegration indicators, network modeling, and mathematical ranking. The study of ultrasound changes in blood flow in the uterine and ovarian vessels and veins in 100 patients with ovarian cancer and 90 patients with acute endometritis made it possible to identify the leading differential diagnostic aspects. The characteristics of disintegration, along with the average values of local blood flow, objectify the differentiation of ovarian cancer and acute endometritis. In accordance with the differential diagnostic significance of the ultrasound characteristics of arterial blood flow in the uterine and ovarian vessels, a network model for the differential diagnosis of ovarian cancer and acute endometritis is constructed according to higher priority changes, which actually allows reducing the size and time of the patient's examination and making the correct diagnosis


2013 ◽  
Vol 7 (2) ◽  
pp. 197-205 ◽  
Author(s):  
Corina Satler ◽  
Carlos Tomaz

ABSTRACT Anosognosia, impairment insight and unawareness of deficits are used as equivalent terms in this study. Objective: To investigate the relationship between the presence of anosognosia symptoms and cognitive domains, functional abilities, and neuropsychiatric symptoms in patients with probable Alzheimer's disease (pAD) and elderly controls (EC). Methods: Twenty-one pAD (14 women) and twenty-two EC (16 women) were submitted to a neuropsychological battery of tests assessing global cognitive status, and specific cognitive functions: memory, executive and attention functions, verbal fluency and visuoconstructive abilities. Additionally, functional abilities (FAQ) and neuropsychiatric symptoms (NPI) were measured. Results: The linear regression statistical test found general anosognosia to be associated with subjective memory complaints, age and Arithmetic-DRS in the EC group. On the other hand, cognitive and functional abilities scores (Arithmetic- DRS, IQCODE and FAQ) were the best predictors in pAD patients, particularly for behavioral awareness. Conclusion: These results indicated that different variables are associated with self-awareness for pAD patients and EC, but for both groups executive functions appear to play an important role, contributing particularly to awareness of behavioral changes.


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