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immunoglobulin intravenous in encephalitis

C G Bien
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy...
March 13, 2018: Der Nervenarzt
Anita Lwanga, David O Kamson, Tiffany E Wilkins, Vinny Sharma, Jefree J Schulte, James Miller, Ikram Hassan, Ricardo R Lastra
N-methyl-D-aspartate receptor encephalitis (NMDARe) is one of 13 autoimmune-mediated encephalitides that have been discovered over the last decade. This case report describes the course of a 26-year-old female who presented with new-onset seizures and insomnia, complicated by encephalitis. The initial workup ruled out common causes of encephalitis, while a transvaginal ultrasound (TVUS), and computed tomography (CT) scans of the chest, abdomen, and pelvis did not identify a mass. Based on the suspicion that she may have autoimmune encephalitis, the patient was treated with intravenous immunoglobulins and plasma exchange, but continued to deteriorate...
January 1, 2018: Neuroradiology Journal
Junyang Xia, Xiaoming Yin, Mingqin Zhu, Jie Cao, Xiaonan Song
RATIONALE: Autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Antineuronal antibodies have been discovered in patients' serum and cerebrospinal fluid (CSF). However, AE with multiple antineuronal antibodies is rare. To date, there are no published reports of AE with both anti-γ-aminobutyric acid B receptor (GABABR) and anticollapsin response-mediator protein 5 (CV2) antibodies...
January 2018: Medicine (Baltimore)
Hsiuying Wang
Treatments for the anti-NMDA receptor encephalitis usually include steroids, intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab, cyclophosphamide and tumor resection. We aimed to compare the efficacy of the treatments including intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab or cyclophosphamide for male anti-NMDA receptor encephalitis patients without tumor and to discuss potential biomarkers for this disease. The Fisher exact test and the contingency table analysis were used to analyze the treatment efficacy for both male and female these patients...
February 21, 2018: Current Medicinal Chemistry
Choong Yi Fong, Hnin Wint Wint Aung, Arie Khairani, Chin Seng Gan, Nortina Shahrizaila, Khean Jin Goh
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP)...
February 16, 2018: Brain & Development
Ahmad Daif, Rimas V Lukas, Naoum P Issa, Adil Javed, Stephen VanHaerents, Anthony T Reder, James X Tao, Peter Warnke, Sandra Rose, Vernon L Towle, Shasha Wu
Glutamic acid decarboxylase (GAD) antibody-associated encephalitis causes both acute seizures and chronic epilepsy with predominantly temporal lobe onset. This condition is challenging in diagnosis and management, and the incidence of GAD antibody (Ab)-related epilepsy could be much higher than commonly believed. Imaging and CSF evidence of inflammation along with typical clinical presentations, such as adult onset temporal lobe epilepsy (TLE) with unexplained etiology, should prompt testing for the diagnostic antibodies...
February 9, 2018: Epilepsy & Behavior: E&B
Neil M Vora, Lillian A Orciari, J Bradford Bertumen, Inger Damon, James A Ellison, Vance G Fowler, Richard Franka, Brett W Petersen, P S Satheshkumar, Stephen M Schexnayder, Todd G Smith, Ryan M Wallace, Susan Weinstein, Carl Williams, Pamela Yager, Michael Niezgoda
Rabies is an acute encephalitis that is nearly always fatal. It is caused by infection with viruses of the genus Lyssavirus, the most common of which is Rabies lyssavirus. The Council of State and Territorial Epidemiologists (CSTE) defines a confirmed human rabies case as an illness compatible with rabies that meets at least one of five different laboratory criteria.* Four of these criteria do not depend on the patient's rabies vaccination status; however, the remaining criterion, "identification of Lyssavirus-specific antibody (i...
February 9, 2018: MMWR. Morbidity and Mortality Weekly Report
Siyuan Fan, Yan Xu, Haitao Ren, Hongzhi Guan, Feng Feng, Xuehui Gao, Ding Ding, Fang Fang, Guangliang Shan, Tianjia Guan, Yao Zhang, Yi Dai, Ming Yao, Bin Peng, Yicheng Zhu, Liying Cui
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports...
January 30, 2018: Multiple Sclerosis and related Disorders
Yong-Won Shin, Soon-Tae Lee, Kyung-Il Park, Keun-Hwa Jung, Ki-Young Jung, Sang Kun Lee, Kon Chu
Autoimmune encephalitis is one of the most rapidly growing research topics in neurology. Along with discoveries of novel antibodies associated with the disease, clinical experience and outcomes with diverse immunotherapeutic agents in the treatment of autoimmune encephalitis are accumulating. Retrospective observations indicate that early aggressive treatment is associated with better functional outcomes and fewer relapses. Immune response to first-line immunotherapeutic agents (corticosteroids, intravenous immunoglobulin, plasma exchange, and immunoadsorption) is fair, but approximately half or more of patients are administered second-line immunotherapy (rituximab and cyclophosphamide)...
2018: Therapeutic Advances in Neurological Disorders
Ali Nikkhah
Kawasaki disease (KD) is an inflammatory vasculitis. KD is classified into two groups based on clinical characteristics criteria, namely classic and incomplete. Cerebral vascular abnormality, especially arterial ischemic stroke (AIS) is very rare and unusual in KD. Here, we report a 4-year-old boy who was referred to our tertiary pediatric center with abrupt right hemiparesis and aphasia. At admission time, he had febrile illness and was toxic. On physical examination, we found unilateral left submandibular lymphadenopathy...
January 2018: Iranian Journal of Medical Sciences
Najma Shaheen, Francis Mussai
A boy with central nervous system relapse of Burkitt leukemia developed fever and neurological symptoms and cognitive impairment. He had received multi-drug chemotherapy including rituximab. Enterovirus (EV) was detected in cerebrospinal fluid by polymerase chain reaction, and magnetic resonance imaging findings were consistent with viral infection. The patient was treated with intravenous immunoglobulin and within 1 month cleared his EV. Rituximab can cause a profound B-cell deficiency predisposing patients to infections including EV encephalitis...
January 8, 2018: Journal of Pediatric Hematology/oncology
Chuan Li, Chengwu Liu, Feng Lin, Lunxu Liu
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified neuroautoimmune disorder characterized by antibodies against the heteromeric NR1-NR2 receptor complex. To our best knowledge, anti-NMDAR encephalitis combined with mediastinal teratoma is extremely rare with only three cases been reported in literature worldwide. In this article, we present an 18 years old female diagnosed as anti-NMDAR encephalitis caused by a large mature mediastinal teratoma whose initial manifestations were acute headache and seizures...
December 2017: Journal of Thoracic Disease
Nitish Chourasia, Michael W Watkins, Jeremy E Lankford, Joseph S Kass, Ankur Kamdar
BACKGROUND: Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that often affects women of childbearing age, and maternal-fetal transfer of anti-NMDAR antibodies during pregnancy has been documented in both symptomatic and asymptomatic women. The effects of these antibodies on the fetus, however, are incompletely understood. PATIENT DESCRIPTION: This term infant exhibited depressed respiratory effort, poor feeding, and abnormal movements after birth...
November 22, 2017: Pediatric Neurology
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
Christopher A Ovens, Angelo Jayamanne, Andrew Duggins
BACKGROUND: Gamma-aminobutyric acid-B receptor autoantibodies are becoming an increasingly recognized contributor to the spectrum of autoimmune limbic encephalitis. They are classically associated with seizures and behavioral disturbance, and may coexist with other autoantibodies. Many are paraneoplastic, most commonly associated with small cell lung cancer. Until now there have been no reports of cardiac dysrhythmias in these patients. CASE PRESENTATION: A 65-year-old Caucasian man presented with multiple seizures, dysarthria and behavioral disturbance of unclear etiology, with associated asystolic cardiac arrest...
December 29, 2017: Journal of Medical Case Reports
Xiaoliang Liu, Kaiyu Zhou, Dan Yu, Xiaotang Cai, Yimin Hua, Hui Zhou, Chuan Wang
INTRODUCTION: The X-linked hyper-immunoglobulin M syndrome (XHIGM) is an uncommon primary combined immunodeficiency disease caused by CD40L gene mutations. A delayed or missed diagnosis of XHIGM is common and concerning, owing to atypical immunoglobulin profile and phenotype of some patients, low recognition, and limited knowledge of clinicians on XHIGM in some underdeveloped areas. Opportunistic infections are a prominent clinical feature of XHIGM. However, toxoplasma encephalitis occurs sporadically and is extremely rare in patients with XHIGM...
December 2017: Medicine (Baltimore)
Magdalena Figlerowicz, Paweł Kemnitz, Anna Mania, Katarzyna Mazur-Melewska, Emila Tomczak, Karolina Kuls, Katarzyna Jończyk-Potoczna, Francesc Graus, Wojciech Służewski
Recent studies have indicated that some cases of nonparaneoplastic autoimmune encephalitis in children can be caused by a systemic autoimmune disorder that generates autoantibodies to cell membrane proteins. We describe the clinical features of a 10-year-old girl with autoimmune encephalitis with autoantibodies against the GABAA receptor in whom type 1 diabetes mellitus developed during the course of the disease. The diagnosis was based on the progressive course of disease, pleocytosis in the cerebrospinal fluid (CSF), inflammatory changes in the brain and autoantibodies against the GABAA receptor detected in serum (absent in CSF)...
December 12, 2017: Clinical Neurology and Neurosurgery
Ayse B Ergul, Yasemin Altuner Torun, Umut Altug, Fatma T Mutlu, Serkan F Celik, Ahmet S Guven
Here, we report a case of hemophilia A with myocarditis, encephalopathy, and spontaneous intramedullary hemorrhage. A 14-month-old male infant presented with loss of consciousness, generalized tonic-clonic convulsions, and cardiac failure. The neurological examination was normal. Myocarditis was diagnosed. After administration of fresh frozen plasma, the aPTT did not return to normal. The factor VIII (FVIII) level was 10.2% the normal level, and the patient was diagnosed with hemophilia A. The cerebrospinal fluid (CSF) evaluation was unremarkable, with the exception of elevated CSF protein levels...
December 12, 2017: Journal of Pediatric Hematology/oncology
Kenneth E Remy, Jason W Custer, Joshua Cappell, Cortney B Foster, Nan A Garber, L Kyle Walker, Liliana Simon, Dayanand Bagdure
Purpose: Anti- N -methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series. Methods: We performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies...
2017: Frontiers in Pediatrics
Robert Perna, Lindsey Harik, Ana Arenivas
Limbic encephalitis (LE) is a rare neurological disorder characterized by inflammation of the brain caused by autoimmunity or infection. LE has been a difficult to define and diagnose disorder due to the insidious and nonspecific (e.g., irritability, low mood, short-term memory complaints) presentation of early symptoms, as well as inconsistent findings on neuroimaging, lumbar puncture serum analysis, and electroencephalogram. Seizures, memory problems, and psychiatric disturbance are among the earliest and most prominent clinical features...
November 29, 2017: Applied Neuropsychology. Child
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