scholarly journals Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019 ◽  
Author(s):  
Qingyang Luo ◽  
Xianghong Wu ◽  
Wen Huang

Abstract Background: Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation: A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions: Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma. Keywords: Autoimmune encephalitis, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, thymoma, myasthenia gravis.

2019 ◽  
Author(s):  
Qingyang Luo ◽  
Xianghong Wu ◽  
wen huang

Abstract Background Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma.


2021 ◽  
Vol 12 ◽  
Author(s):  
Tian-Yi Zhang ◽  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Qi-Lun Lai ◽  
Chun-Hong Shen ◽  
...  

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis, was first reported by Lai et al. The AMPAR antibodies target against extracellular epitopes of the GluA1 or GluA2 subunits of the receptor. AMPARs are expressed throughout the central nervous system, especially in the hippocampus and other limbic regions. Anti-AMPAR encephalitis was more common in middle-aged women and most patients had an acute or subacute onset. Limbic encephalitis, a classic syndrome of anti-AMPAR encephalitis, was clinically characterized by a subacute disturbance of short-term memory loss, confusion, abnormal behavior and seizure. Magnetic resonance imaging often showed T2/fluid-attenuated inversion-recovery hyperintensities in the bilateral medial temporal lobe. For suspected patients, paired serum and cerebrospinal fluid (CSF) testing with cell-based assay were recommended. CSF specimen was preferred given its higher sensitivity. Most patients with anti-AMPAR encephalitis were complicated with tumors, such as thymoma, small cell lung cancer, breast cancer, and ovarian cancer. First-line treatments included high-dose steroids, intravenous immunoglobulin and plasma exchange. Second-line treatments, including rituximab and cyclophosphamide, can be initiated in patients who were non-reactive to first-line treatment. Most patients with anti-AMPAR encephalitis showed a partial neurologic response to immunotherapy.


2021 ◽  
Vol 8 (5) ◽  
pp. e1053
Author(s):  
Mar Guasp ◽  
Jon Landa ◽  
Eugenia Martinez-Hernandez ◽  
Lidia Sabater ◽  
Takahiro Iizuka ◽  
...  

ObjectiveTo report the clinical, neuroimaging, and antibody associations in patients with autoimmune encephalitis (AE) and thymoma.MethodsA retrospective cohort study of 43 patients was conducted. Antibody determination and immunoprecipitation to characterize novel antigens were performed using reported techniques.ResultsPatients' median age was 52 years (range: 23–88 years). Forty (93%) had neuronal surface antibodies: gamma-aminobutyric acid receptor A (GABAAR) (15), amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) (13), contactin-associated protein-like 2 (CASPR2) (4), leucine-rich, glioma inactivated 1 (LGI1) (3), glycine receptor (GlyR) (3), and unknown antigens (2). Concurrent antibodies against intracellular antigens occurred in 13 (30%; 9 anti–collapsin response mediator protein 5 [CRMP5]) and were more frequent in anti-AMPAR encephalitis (54% vs 20%; p = 0.037). The most common clinical presentation was encephalitis with multiple T2/fluid-attenuated inversion recovery hyperintense lesions in 23 (53%) patients (15 GABAAR, 5 AMPAR, and 1 unknown neuropil antibody), followed by encephalitis with peripheral nerve hyperexcitability in 7 (16%; 4 CASPR2, 2 LGI1, and 1 unknown antibody), limbic encephalitis in 6 (14%; 4 AMPAR, 1 LGI1, and 1 antibody negative), progressive encephalomyelitis with rigidity and myoclonus in 4 (9%; 3 GlyR and 1 AMPAR antibodies), and encephalitis with normal MRI in 3 (7%; AMPAR antibodies). Anti-GABAAR encephalitis was more prevalent in Japanese patients compared with Caucasians and other ethnicities (61% vs 16%; p = 0.003). In anti-AMPAR encephalitis, 3/4 patients with poor and 0/6 with good outcome had concurrent CRMP5 antibodies (p = 0.033). Immunoprecipitation studies identified metabotropic glutamate receptor 3 antibodies that were additionally found in 5 patients (3 with and 2 without encephalitis).ConclusionsAE in patients with thymoma include several clinical-radiologic syndromes that vary according to the associated antibodies. Anti-GABAAR encephalitis was the most frequent AE and occurred more frequently in Japanese patients.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 60 ◽  
Author(s):  
Rashmi Kumar

Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae  globally. Causes are diverse and include viral and non-viral infections of the brain as well as autoimmune processes. In the West, the autoimmune encephalitides are now more common than any single infectious cause, but, in Asia, infectious causes are still more common. In 2006, the World Health Organization coined the term “acute encephalitis syndrome”, which simply means acute onset of fever with convulsions or altered consciousness or both. In 2013, the International Encephalitis Consortium set criteria for diagnosis of encephalitis on basis of clinical and laboratory features. The most important infectious cause in the West is herpes simplex virus, but globally Japanese encephalitis (JE) remains the single largest cause. Etiologic diagnosis is difficult because of the large number of agents that can cause encephalitis. Also, the responsible virus may be detectable only in the brain and is either absent or transiently found in blood or cerebrospinal fluid (CSF). Virological diagnosis is complex, expensive, and time-consuming. Different centres could make their own algorithms for investigation in accordance with the local etiologic scenarios. Magnetic resonance imaging (MRI) and electroencephalography are specific for few agents. Clinically, severity may vary widely. A severe case may manifest with fever, convulsions, coma, neurologic deficits, and death. Autoimmune encephalitis (AIE)  includes two major categories: (i) classic paraneoplastic limbic encephalitis (LE) with autoantibodies against intracellular neuronal antigens (Eg: Hu and Ma2) and (ii) new-type AIE with autoantibodies to neuronal surface or synaptic antigens (Eg: anti-N-methyl-D-aspartate receptor). AIE has prominent psychiatric manifestations: psychosis, aggression, mutism, memory loss, euphoria, or fear. Seizures, cognitive decline, coma, and abnormal movements are common. Symptoms may fluctuate rapidly. Treatment is largely supportive. Specific treatment is available for herpesvirus group and non-viral infections. Various forms of immunotherapy are used for AIE.


2021 ◽  
Vol 22 (23) ◽  
pp. 12697
Author(s):  
Melanie J. Sekeres ◽  
Meenakshie Bradley-Garcia ◽  
Alonso Martinez-Canabal ◽  
Gordon Winocur

A wide range of cognitive deficits, including memory loss associated with hippocampal dysfunction, have been widely reported in cancer survivors who received chemotherapy. Changes in both white matter and gray matter volume have been observed following chemotherapy treatment, with reduced volume in the medial temporal lobe thought to be due in part to reductions in hippocampal neurogenesis. Pre-clinical rodent models confirm that common chemotherapeutic agents used to treat various forms of non-CNS cancers reduce rates of hippocampal neurogenesis and impair performance on hippocampally-mediated learning and memory tasks. We review the pre-clinical rodent literature to identify how various chemotherapeutic drugs affect hippocampal neurogenesis and induce cognitive impairment. We also review factors such as physical exercise and environmental stimulation that may protect against chemotherapy-induced neurogenic suppression and hippocampal neurotoxicity. Finally, we review pharmacological interventions that target the hippocampus and are designed to prevent or reduce the cognitive and neurotoxic side effects of chemotherapy.


2019 ◽  
Vol 70 (7) ◽  
pp. 2330-2334
Author(s):  
Mihaela Ciopec ◽  
Adina Negrea ◽  
Narcis Duteanu ◽  
Corneliu Mircea Davidescu ◽  
Iosif Hulka ◽  
...  

Arsenic content in groundwater�s present a wide range of concentration, ranging from hundreds of micrograms to thousands of micrograms of arsenic per litter, while the maximum permitted arsenic concentration established by World Health Organization (WHO) is 10 mg L-1. According to the WHO all people, regardless of their stage of development and their social economic condition, have the right to have access to adequate drinking water. The most efficient and economic technique used for arsenic removal is represented by adsorption. In order to make this remediation technique more affordable and environmentally friendly is important to new materials with advance adsorbent properties. Novelty of present paper is represented by the usage of a new adsorbent material obtained by physical - chemical modification of Amberlite XAD polymers using crown ethers followed by iron doping, due to well-known affinity of arsenic for iron ions. Present paper aims to test the obtained modified Amberlite polymer for arsenic removal from real groundwater by using adsorption in a fixed bed column, establishing in this way a mechanism for the adsorption process. During experimental work was studied the influence of competing ions from real water into the arsenic adsorption process.


2020 ◽  
Author(s):  
André De Faria Pereira Neto ◽  
Leticia Barbosa ◽  
Rodolfo Paolucci

UNSTRUCTURED Billions of people in the world own a smartphone. It is a low-cost, portable computing device with countless features, among which applications stand out, which are programs or software developed to meet a specific goal. A wide range of applications available ranging from entertainment and personal organization to work and education is available currently. It is a vast and profitable market. Health applications have been a means of intervention for different areas, including chronic diseases, epidemics, and health emergencies. A recently published paper in the journal with the highest impact factor in Digital Health (“Journal of Medical Internet Research”) proposes a classification of health applications. This study performs a critical analysis of this organization and presents other sort criteria. This paper also presents and analyzes the “Meu Info Saúde” (“My Health Info”) app – a pioneering government initiative focused on primary care launched by the Oswaldo Cruz Foundation. The application classification proposal that will be presented builds on the intervention strategies in the health-disease process, namely: “Health Promotion”, “Disease Prevention” and “Care, Treatment and Rehabilitation”, as defined by official documents such as the World Health Organization and the Centers for Disease Control and Prevention. Most applications present in the sample are of private and foreign origin, free to download, but with a display of ads or the sale of products and services. The sampled applications were classified as “Health Promotion”, and some applications have also been categorized as “Disease Prevention” or “Care, Treatment or Rehabilitation” because they have multiple functionalities. The applications identified as “Health Promotion” focused only on individuals’ lifestyle and their increased autonomy and self-care management capacity. From this perspective, the apps analyzed in this paper differ from the “Meu Info-Saúde” application developed at Fiocruz.


2020 ◽  
Author(s):  
Salime Goharinezhad

BACKGROUND World Health Organization declared the vaccine hesitancy as a global public health threat in 2019. Since even a slight reduction in vaccine coverage rates can lead to a decrease in herd immunity, it is imperative to explore the underlying factors affecting vaccine hesitancy. in specific contexts, considering socioeconomic and cultural variation, to ensure interventions targeting hesitancy are well formulated and intervened. OBJECTIVE The main objective of this study is to identify underlying factors affecting vaccine hesitancy in Iran. METHODS A framework qualitative study will be conducted in the west of Tehran province in 2020. Participants in the study will be recruited hesitance-parents who extracted from the SIB system (an electronic health record in Iran) to maximize diversity. Interviews will be analyzed based on ''Determinants of Vaccine Hesitancy Matrix'' which developed by the WHO-SAGE Working Group. RESULTS deep understanding from the context-specific reasons for vaccine hesitancy cause to formulate better strategies to address them. The ultimate goal of this study is to inform future policies to increase the uptake of the vaccine in Iran. CONCLUSIONS This result of study will show variety opinions about vaccination among different types of socioeconomic and demographic households. The wide range of reasons related to vaccine hesitancy imply to more comprehensive, context-specific interventions. Today, the most important intervention issues focus on improving information about effectiveness and safety of vaccines, while other interventions for promoting vaccination is need to addressed.


2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095055
Author(s):  
Yali Wang ◽  
Zhihua Si ◽  
Jingzhe Han ◽  
Shuangqing Cao

Cerebral fat embolism (CFE) syndrome is relatively rare in clinical practice. Currently, there is no uniform standard of magnetic resonance imaging for the diagnosis of the disease. In this report, we present head computed tomography and magnetic resonance images (T2-weighted images, fluid-attenuated inversion recovery images, diffusion-weighted images, and susceptibility-weighted images) in a case of CFE. This report explains the imaging characteristics of CFE and improves the clinician’s understanding of this disease and its etiology.


Author(s):  
Hernâni Marques ◽  
Pedro Cruz-Vicente ◽  
Tiago Rosado ◽  
Mário Barroso ◽  
Luís A. Passarinha ◽  
...  

Environmental tobacco smoke exposure (ETS) and smoking have been described as the most prevalent factors in the development of certain diseases worldwide. According to the World Health Organization, more than 8 million people die every year due to exposure to tobacco, around 7 million due to direct ETS and the remaining due to exposure to second-hand smoke. Both active and second-hand exposure can be measured and controlled using specific biomarkers of tobacco and its derivatives, allowing the development of more efficient public health policies. Exposure to these compounds can be measured using different methods (involving for instance liquid- or gas-chromatographic procedures) in a wide range of biological specimens to estimate the type and degree of tobacco exposure. In recent years, a lot of research has been carried out using different extraction methods and different analytical equipment; this way, liquid–liquid extraction, solid-phase extraction or even miniaturized procedures have been used, followed by chromatographic analysis coupled mainly to mass spectrometric detection. Through this type of methodologies, second-hand smokers can be distinguished from active smokers, and this is also valid for e-cigarettes and vapers, among others, using their specific biomarkers. This review will focus on recent developments in the determination of tobacco smoke biomarkers, including nicotine and other tobacco alkaloids, specific nitrosamines, polycyclic aromatic hydrocarbons, etc. The methods for their detection will be discussed in detail, as well as the potential use of threshold values to distinguish between types of exposure.


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