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https://www.readbyqxmd.com/read/29237452/molecular-cloning-and-characterization-of-the-family-of-feline-leucine-rich-glioma-inactivated-lgi-genes-and-mutational-analysis-in-familial-spontaneous-epileptic-cats
#1
Yoshihiko Yu, Daisuke Hasegawa, Aki Fujiwara-Igarashi, Yuji Hamamoto, Shunta Mizoguchi, Takayuki Kuwabara, Michio Fujita
BACKGROUND: Leucine-rich glioma-inactivated (LGI) proteins play a critical role in synaptic transmission. Dysfunction of these genes and encoded proteins is associated with neurological disorders such as genetic epilepsy or autoimmune limbic encephalitis in animals and human. Familial spontaneous epileptic cats (FSECs) are the only feline strain and animal model of familial temporal lobe epilepsy. The seizure semiology of FSECs comprises recurrent limbic seizures with or without evolution into generalized epileptic seizures, while cats with antibodies against voltage-gated potassium channel complexed/LGI1 show limbic encephalitis and recurrent limbic seizures...
December 13, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/29189554/paraneoplastic-lambert-eaton-myasthenic-syndrome-with-limbic-encephalitis-clinical-correlation-with-the-coexistence-of-anti-vgcc-and-anti-gabab-receptor-antibodies
#2
Jonathan J Cho, James P Wymer
OBJECTIVE: To characterize Lambert-Eaton myasthenic syndrome and limbic encephalitis with coexistent voltage-gated calcium channel (VGCC) antibody and γ-aminobutyric acid (GABA) B receptor antibody. METHODS: Case study. RESULTS: A 57-year-old man presented with 6 months of weakness, unsteadiness, and vision difficulties. Examination revealed proximal weakness and diminished reflexes. Electrodiagnostic study revealed low-amplitude motor potentials and facilitation on high-frequency stimulation...
December 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/29185868/three-case-studies-of-nonparaneoplastic-limbic-encephalitis-nple-in-young-adult-males-onset-course-and-recovery-following-rehabilitation-services
#3
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
https://www.readbyqxmd.com/read/29171405/seizure-semiology-of-anti-lgi1-antibody-encephalitis
#4
Nicholas J Beimer, Linda M Selwa
Limbic encephalitis associated with anti-LGI1 antibody (LGI1 encephalitis) presents with a variety of features, the most prominent of which include seizures and progressive disturbance of memory and behaviour. Although varied in semiology, recognition of the pattern of seizures in LGI1 encephalitis is important, as early diagnosis and definitive treatment may prevent subsequent development of cognitive impairment. We present a patient with LGI1 encephalitis and "faciobrachial dystonic seizures-plus", which began as classic faciobrachial dystonic seizures and progressed to focal seizures with impaired awareness, dacrystic/gelastic-like outbursts, ictal speech, manual automatisms, and autonomic signs (tachycardia)...
November 24, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29158368/anti-hu-associated-autoimmune-limbic-encephalitis-in-a-patient-with-pd-1-inhibitor-responsive-myxoid-chondrosarcoma
#5
Kyriakos P Papadopoulos, Rebecca S Romero, Gabriela Gonzalez, James E Dix, Israel Lowy, Matthew Fury
Autoimmune encephalitis is an uncommon complication of immune checkpoint inhibitor therapy. This article reports a case of fatal anti-Hu-associated autoimmune limbic encephalitis presenting within 8 weeks following anti-PD1 therapy in a patient with myxoid chondrosarcoma and pre-existing anti-Hu antibodies. Although tumor reduction occurred in response to PD-1 inhibitor therapy, the patient had a rapidly progressive decline in neurologic function despite initial stabilization with immunosuppression. Considering the increasing use of immune checkpoint inhibitors for the treatment of various malignancies, an increase in the occurrence of neurologic adverse events is likely, requiring prompt intervention and enhanced pharmacovigilance in malignancies associated with onconeuronal antibodies...
November 20, 2017: Oncologist
https://www.readbyqxmd.com/read/29154389/latent-autoimmune-diabetes-and-limbic-encephalitis-with-antibodies-against-glutamic-acid-decarboxylase
#6
Stoyan Popkirov, Seena Sebastian, Fatme Seval Ismail, Jörg Wellmer
Antibodies against glutamic acid decarboxylase (GAD) are a hallmark of type 1 diabetes and its late-onset variant, latent autoimmune diabetes of the adult (LADA).(1) While cerebrovascular disease and dementia are common causes of cognitive dysfunction and neurological deficits in diabetic patients,(2,3) rare autoimmune disorders of the central nervous system such as stiff-person syndrom or limbic encephalits can also occur on the basis of shared GAD autoimmunity.(4,5).
November 20, 2017: Journal of Diabetes
https://www.readbyqxmd.com/read/29151536/bilateral-hippocampal-infarction-mimicking-limbic-encephalitis
#7
Satoshi Hosoki, Hitoshi Satoi, Sadayuki Matsumoto
No abstract text is available yet for this article.
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29114371/sarcoidosis-limbic-encephalitis-a-case-report
#8
Moussa Toudou-Daouda, Hamid Assadeck, Boubacar Efared
No abstract text is available yet for this article.
July 6, 2017: Iranian Journal of Neurology
https://www.readbyqxmd.com/read/29110638/application-of-the-2016-diagnostic-approach-for-autoimmune-encephalitis-from-lancet-neurology-to-chinese-patients
#9
Lin Li, Lin Sun, Rong Du, Yuanchu Zheng, Feifei Dai, Qiuying Ma, Jiawei Wang
BACKGROUND: A unified clinical approach to diagnose autoimmune encephalitis was published in Lancet Neurology in 2016. Purpose of our study is to examine the feasibility and reasonability of the 2016 "A clinical approach to diagnosis of autoimmune encephalitis" in China with a retrospective study. METHODS: We retrospectively collected 95 cases of autoimmune encephalitis and non autoimmune encephalitis cases with detailed clinical data from Beijing Tongren Hospital and the China National Knowledge Infrastructure (CNKI)...
November 6, 2017: BMC Neurology
https://www.readbyqxmd.com/read/29093718/selective-limbic-blood-brain-barrier-breakdown-in-a-feline-model-of-limbic-encephalitis-with-lgi1-antibodies
#10
Anna R Tröscher, Andrea Klang, Maria French, Lucía Quemada-Garrido, Sibylle Maria Kneissl, Christian G Bien, Ákos Pákozdy, Jan Bauer
Human leucine-rich glioma-inactivated protein 1 encephalitis (LGI1) is an autoimmune limbic encephalitis in which serum and cerebrospinal fluid contain antibodies targeting LGI1, a protein of the voltage gated potassium channel (VGKC) complex. Recently, we showed that a feline model of limbic encephalitis with LGI1 antibodies, called feline complex partial seizures with orofacial involvement (FEPSO), is highly comparable to human LGI1 encephalitis. In human LGI1 encephalitis, neuropathological investigations are difficult because very little material is available...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29057855/a-rare-case-of-autoimmune-limbic-encephalitis-an-uncharted-territory
#11
Hatim Ibrahim, Abdulelah N Al Jasser, Sonia A Khan, Kalthoum G Tlili
Autoimmune encephalitis is rare. Several auto- antibodies are described in autoimmune encephalitis. We describe a case of autoimmune limbic encephalitis associated with positive voltage gated potassium channel (VGKC) antibodies and positive leucine-rich glioma inactivated protein 1 antibodies (LGI1). A 33-year-old Saudi housewife, she presented with 2 months history of cognitive deterioration and recurrent left facio-brachial dystonic seizures followed by generalized tonic clonic seizures. At times the seizures are preceded by rising epigastric aura and shortness of breath...
October 2017: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29053037/possible-autoantibody-negative-limbic-encephalitis-after-anterior-temporal-lobectomy-for-hippocampal-sclerosis
#12
Sara Casciato, Alfredo D'Aniello, Addolorata Mascia, Pier Paolo Quarato, Marco De Risi, Liliana G Grammaldo, Vincenzo Esposito, Marco Zoccarato, Giancarlo Di Gennaro
Amnestic syndromes are acknowledged to be associated to bilateral hippocampal damage. We briefly report the case of a young man who underwent anterior left temporal lobectomy for a medically refractory temporal lobe epilepsy due to hippocampal sclerosis with an excellent seizure and neuropsychological outcome. Approximately 10 years later, he presented with a subacute severe global amnesia and MRI findings of a damage involving the contralateral mesial temporal lobe structures. A diagnosis of a possible autoimmune encephalitis was made...
October 20, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29050866/seizure-semiology-in-leucine-rich-glioma-inactivated-protein-1-antibody-associated-limbic-encephalitis
#13
Chao Chen, Xiu Wang, Chao Zhang, Tao Cui, Wei-Xiong Shi, Hong-Zhi Guan, Hai-Tao Ren, Xiao-Qiu Shao
OBJECTIVE: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE). METHODS: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated. RESULTS: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8)...
October 16, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29042524/-autoimmune-and-epilepsy
#14
Riki Matsumoto, Mitsuhiro Sakamoto, Akio Ikeda
The recent discovery of autoimmune antibodies to the neuronal cell surface membrane and extra- or intra-cellular proteins, such as NMDAR and LGI1, shed light on a proposed new etiology of epilepsy, namely, "autoimmune epilepsy". A large part of this entity most likely belongs to a forme fruste of autoimmune (limbic) encephalitis. Seizures are usually subacute in onset and refractory to antiepileptic medications. Patients occasionally manifest multiple seizure semiologies, such as autonomic or faciobrachial dystonic seizures...
October 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28988523/immunopathology-in-drug-resistant-mesial-temporal-lobe-epilepsy-with-different-types-of-hippocampal-sclerosis
#15
F Irsel Tezer, Aysegul Firat, Erdem Tuzun, Isik Unal, Figen Soylemezoglu, Burcak Bilginer, Figen Kaymaz, Kader K Oguz, Serap Saygi
PURPOSE: There is evidence that autoimmunity has a specific role in temporal lobe seizures of limbic encephalitis patients. Our aim in this study was to investigate any histopathological clues of autoimmune process in refractory temporal lobe epilepsy (TLE) patients with different pathologically proven hippocampal sclerosis (HS) types. METHODS: 22 patients who had undergone to epilepsy surgery due to mesial TLE-HS were included. The sera of patients are tested for neuronal antibodies to NMDAR, LGI1, CASPR2, AMPAR, GABABR and GAD...
October 7, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28987176/immune-mediated-disorders
#16
Romana Höftberger, Hans Lassmann
Paraneoplastic and autoimmune encephalitis comprise a group of immune-mediated disorders that are associated with different immune effector mechanisms. Classic paraneoplastic neurologic syndromes are triggered by an antitumor immune response. The disease is considered to result from a T-cell response; in addition, patients harbour high titers of autoantibodies against intracellular antigens that are considered as epiphenomenon but are useful diagnostic markers. Neuropathology consists of T-cell-dominated inflammation, marked neuronal loss, and microglial activation with upregulation of HLA-DR...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28984777/anti-ma2-associated-limbic-encephalitis-with-coexisting-chronic-inflammatory-demyelinating-polyneuropathy-in-a-patient-with-non-hodgkin-lymphoma-a-case-report
#17
Weina Ju, Baochang Qi, Xu Wang, Yu Yang
RATIONALE: We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. PATIENT CONCERNS: The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28974152/encephalitis-with-antibodies-against-the-gabab-receptor-seizures-as-the-most-common-presentation-at-admission
#18
Xueping Chen, Fan Liu, Jin-Mei Li, Xiao-Qi Xie, Qiong Wang, Dong Zhou, Huifang Shang
OBJECTIVE: Autoimmune encephalitis associated with antibodies against gamma-aminobutyric-acid B receptor (GABABR) has not been described in detail in Chinese patients. METHODS: Patients with anti-GABABR encephalitis treated between January 2013 and December 2015 were analyzed in terms of clinical characteristics, laboratory findings, tumor presence, autoantibody patterns, treatment response and outcomes. RESULTS: Eleven patients were identified (male, N = 8; female, N = 3), with the median age of 51 years...
November 2017: Neurological Research
https://www.readbyqxmd.com/read/28965295/comprehensive-and-methodical-diagnostic-and-management-approaches-to-rapidly-progressive-dementia
#19
REVIEW
Supriya Mahajan, Brian S Appleby
Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone. Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer's disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD...
September 30, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28954337/-clinical-analysis-of-paraneoplastic-neurological-syndrome-associated-with-thymoma
#20
H S Liu, H T Ren, L X Zhou, S Q Li, B Peng, L Y Cui, H Z Guan
Objectives: To investigate the clinical features, diagnosis and treatment of antibody mediated paraneoplastic neurological syndrome associated with thymoma. Methods: From 2012 to 2017, the paraneoplastic antibody and neuron antibody were tested from both blood and cerebrospinal fluid (CSF) in consecutive patients clinically suspected with neurological paraneoplastic syndromes/unknown encephalitis in Peking Union Medical College Hospital.The clinical data, lab results, electrophysiological examinations, imaging features, treatment and clinical prognosis were collected...
September 19, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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