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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/29457123/general-anesthesia-with-propofol-for-ovarian-teratoma-excision-associated-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#1
Masami Sato, Hiroaki Yasumoto, Toshiyuki Arai
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder caused by production of anti-NMDAR antibodies that is often associated with ovarian teratoma and exhibits various manifestations including psychiatric symptoms, seizures, hypoventilation, and autonomic nerve instability. Patients with this disorder who receive early surgical tumor resection along with immunotherapy have better outcome than the rest of the patients. To establish an anesthetic plan, it is important to understand the pharmacological interaction between the anesthetic agents and the disabled NMDAR, because NMDAR is one of the major sites of action for commonly-used anesthetic agents...
2018: JA Clin Rep
https://www.readbyqxmd.com/read/29452686/technological-advances-and-changing-indications-for-lumbar-puncture-in-neurological-disorders
#2
REVIEW
Joost M Costerus, Matthijs C Brouwer, Diederik van de Beek
Technological advances have changed the indications for and the way in which lumbar puncture is done. Suspected CNS infection remains the most common indication for lumbar puncture, but new molecular techniques have broadened CSF analysis indications, such as the determination of neuronal autoantibodies in autoimmune encephalitis. New screening techniques have increased sensitvity for pathogen detection and can be used to identify pathogens that were previously unknown to cause CNS infections. Evidence suggests that potential treatments for neurodegenerative diseases, such as Alzheimer's disease, will rely on early detection of the disease with the use of CSF biomarkers...
March 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29444764/clinical-radiological-pathological-correlation-in-an-unusual-case-of-refractory-epilepsy-a-two-year-journey-of-whodunit
#3
Deepak Menon, Ramshekhar N Menon, Chandrasekharan Kesavadas, Anita Mahadevan, Ashalatha Radhakrishnan, Sudheeran Kannoth, Pradeep P Nair, Mathew Abraham, Bejoy Thomas, Sanjeev V Thomas
New-onset refractory focal epilepsy poses significant challenges to the clinician in the absence of specific diagnostic biomarkers. Differential diagnoses based on imaging may be expanded by a veritable spectrum of peri-ictal imaging findings that may mask the underlying substrate. We report a 13-year-old girl who presented with refractory focal seizures of left parieto-occipital origin with cytotoxic gyral oedema noted over the same region on imaging. Despite an initial negative autoantibody profile, the patient was treated with immunosuppression, followed by serial relapses requiring immune-modulation...
February 14, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29443773/anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-acute-toxoplasma-gondii-infection-a-case-report
#4
Xiaotang Cai, Hui Zhou, Yongmei Xie, Dan Yu, Zhiling Wang, Haitao Ren
RATIONALE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been recognized as the most frequent autoimmune encephalitis in children. Several infectious agents have been implicated in anti-NMDA encephalitis. PATIENT CONCERNS: A previously healthy immunocompetent 9-year-old girl first presented with seizures, headaches and vomiting. Cerebrospinal fluid and brain magnetic resonance imaging were normal. After one week onset, the patient gradually developed unexplained personality and behavior changes, accompanied by fever and seizures again...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29441039/metastatic-burned-out-seminoma-causing-neurological-paraneoplastic-syndrome-not-quite-burned-out
#5
Yuval Freifeld, Payal Kapur, Ritika Chitkara, Francesca Lee, Pravin Khemani, Aditya Bagrodia
A 44-year-old man presented with cerebellar ataxia and limbic encephalitis and was ultimately diagnosed with metastatic germ cell neoplasm resulting from a "burned out" primary testicular tumor. The patient had progressive ataxia, leading to a thorough investigation for infectious, autoimmune, metabolic, and malignant causes of acquired cerebellar ataxia that revealed no significant findings. Testicular sonography demonstrated a possible right testicular lesion that was not confirmed on radical inguinal orchiectomy...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29440316/glial-fibrillary-acidic-protein-antibody-positive-meningoencephalomyelitis
#6
Angeliki Zarkali, Oliver Cousins, Dilan Athauda, Samuel Moses, Nicholas Moran, Sreedharan Harikrishnan
Glial fibrillary acidic protein antibody-positive meningoencephalomyelitis is a newly described, possibly under-recognised, severe inflammatory condition of the nervous system. The clinical presentation is variable but most commonly is a combination of meningitis, encephalitis and myelitis; other manifestations may include seizures, psychiatric symptoms and tremor. There is a significant association with malignancies, often occult, and with other autoimmune conditions. Although the disease responds well to corticosteroids acutely, it typically relapses when these are tapered, and so patients need long-term immunosuppression...
February 9, 2018: Practical Neurology
https://www.readbyqxmd.com/read/29439749/nmda-receptor-autoimmunity-in-mania-following-hsv-encephalitis
#7
Graham Blackman, Nicholas Moran, Eli Silber, Christopher Symeon, Franz Brunnhuber, Asif Mazumder, Fatima Jaffer, Thomas Pollak
No abstract text is available yet for this article.
February 14, 2018: Psychological Medicine
https://www.readbyqxmd.com/read/29433947/antiglutamic-acid-decarboxylase-65-gad65-antibody-associated-epilepsy
#8
REVIEW
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
https://www.readbyqxmd.com/read/29426955/uncoupling-the-widespread-occurrence-of-anti-nmdar1-autoantibodies-from-neuropsychiatric-disease-in-a-novel-autoimmune-model
#9
Hong Pan, Bárbara Oliveira, Gesine Saher, Ekrem Dere, Daniel Tapken, Marina Mitjans, Jan Seidel, Janina Wesolowski, Debia Wakhloo, Christina Klein-Schmidt, Anja Ronnenberg, Kerstin Schwabe, Ralf Trippe, Kerstin Mätz-Rensing, Stefan Berghoff, Yazeed Al-Krinawe, Henrik Martens, Martin Begemann, Winfried Stöcker, Franz-Josef Kaup, Reinhard Mischke, Susann Boretius, Klaus-Armin Nave, Joachim K Krauss, Michael Hollmann, Fred Lühder, Hannelore Ehrenreich
Autoantibodies of the IgG class against N-methyl-D-aspartate-receptor subunit-NR1 (NMDAR1-AB) were considered pathognomonic for anti-NMDAR encephalitis. This view has been challenged by the age-dependent seroprevalence (up to >20%) of functional NMDAR1-AB of all immunoglobulin classes found in >5000 individuals, healthy or affected by different diseases. These findings question a merely encephalitogenic role of NMDAR1-AB. Here, we show that NMDAR1-AB belong to the normal autoimmune repertoire of dogs, cats, rats, mice, baboons, and rhesus macaques, and are functional in the NMDAR1 internalization assay based on human IPSC-derived cortical neurons...
February 9, 2018: Molecular Psychiatry
https://www.readbyqxmd.com/read/29417305/anti-nmda-receptor-encephalitis-presenting-as-postpartum-psychosis-a-clinical-description-and-review
#10
Mukku Shiva Shanker Reddy, Harish Thippeswamy, Sundarnag Ganjekar, Madhu Nagappa, Anita Mahadevan, H R Arvinda, Prabha S Chandra, Arun B Taly
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is increasingly being recognised to be associated with protean neuropsychiatric manifestations. Anti-NMDAR encephalitis is considered to be the most common amongst the autoimmune-mediated encephalitic disorders. It is caused by the autoantibodies against GluN1 subunits of N-methyl-D-aspartate (NMDA) receptor and manifests with prominent psychiatric symptoms, especially during the initial phase of illness. Literature anti-NMDAR encephalitis presenting with postpartum psychosis is scant...
February 7, 2018: Archives of Women's Mental Health
https://www.readbyqxmd.com/read/29417255/on-chronic-fatigue-syndrome-and-nosological-categories
#11
Kassem Sharif, Abdulla Watad, Nicola Luigi Bragazzi, Michael Lichtbroun, Mariano Martini, Carlo Perricone, Howard Amital, Yehuda Shoenfeld
Chronic fatigue syndrome (CFS) is a heterogeneous disease which presents with pronounced disabling fatigue, sleep disturbances, and cognitive impairment that negatively affects patients' functional capability. CFS remains a poorly defined entity and its etiology is still in question. CFS is neither a novel diagnosis nor a new medical condition. From as early as the eighteenth century, a constellation of perplexing symptoms was observed that resembled symptoms of CFS. Commencing with "febricula" and ending with CFS, many names for the disease were proposed including neurocirculatory asthenia, atypical poliomyelitis, Royal Free disease, effort syndrome, Akureyri disease, Tapanui disease, chronic Epstein-Barr virus syndrome, and myalgic encephalitis...
February 7, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29406902/autoimmune-and-paraneoplastic-movement-disorders-an-update
#12
REVIEW
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29403279/failure-to-improve-after-ovarian-resection-could-be-a-marker-of-recurrent-ovarian-teratoma-in-anti-nmdar-encephalitis-a-case-report
#13
Yuto Uchida, Daisuke Kato, Yoriko Yamashita, Yasuhiko Ozaki, Noriyuki Matsukawa
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of autoimmune encephalitis that can be paraneoplastic and usually responds to tumor resection and immunotherapy. More than 75% of patients with anti-NMDAR encephalitis fully recover or have only mild sequelae, whereas the remainder experience severe disability. It remains unknown why certain cases have refractory clinical disease courses. We report a case of anti-NMDAR encephalitis with bilateral ovarian teratomas who was refractory to tumor resection and early initiation of immunotherapy...
2018: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29401173/clinical-presentation-of-autoimmune-and-viral-encephalitides
#14
Divyanshu Dubey, Michel Toledano, Andrew McKeon
PURPOSE OF REVIEW: We describe clinical and diagnostic features of various autoimmune and viral encephalitis subtypes. RECENT FINDINGS: Population-based studies have demonstrated both autoimmune and viral causes have similar prevalence and incident rates. Repertoire of autoimmune biomarkers has considerably increased with discovery of many novel neural antibodies including glial fibrillary acidic proteinα-immunoglobulin G. Similarly, with use of next generation sequencing and DNA libraries, many viral causes are being detected which would have been categorized as encephalitis of unknown cause a decade ago...
February 3, 2018: Current Opinion in Critical Care
https://www.readbyqxmd.com/read/29399043/treatment-strategies-for-autoimmune-encephalitis
#15
REVIEW
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
https://www.readbyqxmd.com/read/29397673/limbic-encephalitis-manifesting-as-selective-amnesia-and-seizure-like-activity-a-case-report
#16
So-Yeon Kim, Yoo Hyun Um, Sung Chul Lim, Jong-Hyun Jeong
Limbic encephalitis (LE) is characterized by short-term memory loss, disorientation, agitation, seizures, and histopathological evidence of medial temporal lobe inflammation. Leucine-rich, glioma inactivated 1 (LGI-1) is an auto-antigen associated with LE. We report a 37-year-old male patient with LGI-1-related LE who presented with recurrent episodes of selective amnesia, seizure-like activity, confusion, and personality change. His symptoms were significantly improved with steroid therapy. Thorough differential diagnosis with consideration for autoimmune encephalitis should be in patients with presentation of symptoms, such as memory impairment, personality change and seizure-like activity, especially when other neurological diagnoses are excluded...
February 28, 2018: Clinical Psychopharmacology and Neuroscience: the Official Scientific Journal of the Korean College of Neuropsychopharmacology
https://www.readbyqxmd.com/read/29396172/severe-gabaa-receptor-encephalitis-without-seizures-a-paediatric-case-successfully-treated-with-early-immunomodulation
#17
Marc Nikolaus, Ellen Knierim, Christian Meisel, Jakob Kreye, Harald Prüss, Dirk Schnabel, Tilmann Kallinich
BACKGROUND: Autoimmune-mediated processes are the driving force behind many neurological diseases. Autoimmune encephalitis, a group of syndromes, mediated by or at least associated with autoantibodies against neuronal tissue, have gained increasing importance especially in paediatric neurology. Since the first NMDAR encephalitis was described a growing number of patients with encephalopathy, seizures and psychiatric symptoms were found to suffer from treatable autoimmune disorders. Recently a severe form of encephalitis associated with GABAAR antibodies was described showing extensive MRI abnormalities and refractory seizures...
January 9, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29396169/pediatric-nmdar-encephalitis-a-single-center-observation-study-with-a-closer-look-at-movement-disorders
#18
Tiziana Granata, Sara Matricardi, Francesca Ragona, Elena Freri, Federica Zibordi, Francesca Andreetta, Simona Binelli, Nardo Nardocci
Anti-N-Methyl-d-aspartate-receptor (NMDAR) encephalitis is the most frequent autoimmune encephalitis in pediatric age. This retrospective observational study was aimed at describing the clinical characteristics of the disease in a cohort of children and teenagers. Eighteen patients (10 females and 8 males), with a median age of 12.4 years at symptom onset were enrolled. The clinical presentation of the disease was marked by neurological manifestations in 13 patients and by severe psychiatric and behavioral symptoms in 5...
January 26, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29395322/thymoma-associated-myasthenia-gravis-and-lgi1-encephalitis-with-nephrotic-syndrome-post-thymectomy
#19
Jyh Yung Hor, Thien Thien Lim, Mei Chih Cheng, Yuen Kang Chia, Chee Keong Wong, Su Min Lim, Chun Fai Cheah, Kenny Tan, P E Samuel Easaw, M Isabel Leite
Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose...
January 30, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29393234/lipid-metabolism-in-patients-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#20
Yaqing Shu, Bing Qin, Yue Xu, Xiaobo Sun, Zheng Chen, Jia Wang, Lisheng Peng, Wei Qiu, Zhengqi Lu, Aimin Wu
OBJECTIVE: Lipid metabolism has been implicated in autoimmune disorders, but its relationship with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is unclear. This study examined the association of serum lipids with anti-NMDAR encephalitis. METHODS: Serum lipid profiles and C-reactive protein (CRP) were evaluated in 68 patients with anti-NMDAR encephalitis, and 68 age- and sex-matched healthy controls (CTLs). Follow-up evaluations were conducted 3 months after admission in 32 of the 68 patients...
February 1, 2018: Neuroimmunomodulation
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