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https://www.readbyqxmd.com/read/29145168/frontal-infraslow-activity-marks-the-motor-spasms-of-anti-lgi1-encephalitis
#1
Richard Wennberg, Claude Steriade, Robert Chen, Danielle Andrade
OBJECTIVE: The clinical and electrographic features of seizures in anti-LGI1 encephalitis are distinct from those seen in other autoimmune encephalitides or non-encephalitic epilepsies. One electroclinical phenomenon specific to the condition consists of lateralized motor spasms, known as faciobrachial dystonic seizures (FBDS). An electrodecremental pattern overriding a "DC shift" has been described as the EEG correlate of these spasms. We sought to further characterize this pre-spasm infraslow activity (ISA)...
October 28, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29141829/cerebral-cortical-encephalitis-followed-by-recurrent-cns-demyelination-in-a-patient-with-concomitant-anti-mog-and-anti-nmda-receptor-antibodies
#2
Lei Zhou, Jingzi ZhangBao, Haiqing Li, Xiaoyang Li, Yongheng Huang, Min Wang, Chongbo Zhao, Jiahong Lu, Chuanzhen Lu, Yuxin Li, Chao Quan
We report the case of a patient who initially presented with fever, headache and seizure. MRI revealed a fluid attenuation inversion recovery (FLAIR) high-intensity lesion involving the right temporal, parietal and occipital cortex. Afterwards, the patient developed three recurrent episodes, manifested as brainstem encephalitis, optic neuritis and ADEM-like illness successively, indicating demyelination. Both of his serum anti-MOG and CSF anti-NMDAR antibodies were proved positive by transfected cell based assays...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29110638/application-of-the-2016-diagnostic-approach-for-autoimmune-encephalitis-from-lancet-neurology-to-chinese-patients
#3
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/29108750/new-spectrum-of-the-neurologic-consequences-of-zika
#4
EDITORIAL
Marco T Medina, Marco Medina-Montoya
Zika virus infection represents a new neuropathological agent with association to a wide spectrum of neurological complications: a) Congenital Zika Syndrome by affecting the neural stem cells of the human fetal brain; b) Guillain-Barré Syndrome by an autoimmune response against peripheral myelin and/or axonal components or probable direct inflammatory reaction; c) Encephalitis/meningoencephalitis and myelitis by a direct viral inflammatory process on the central nervous system; d) Sensory neuropathy by infecting directly the peripheral neurons and causing substantial cell death and pathogenic transcriptional dysregulation; e) Acute Disseminated Encephalomyelitis and optic neuropathy; f) Seizures and Epilepsy and g) childhood arterial ischemic stroke by probable inflammatory reaction and endothelial injury...
November 1, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29103423/autoimmune-epilepsies
#5
Anusha K Yeshokumar, Carlos A Pardo
Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, or other focal neurological deficits, or all of these. Traditional neurological diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29103007/anti-lgi1-encephalitis-causing-faciobrachial-dystonic-seizures
#6
Stamatios Zouras, Jeffrey W Stephens, Srinivasa Rao Abburu, Chika Emelle
Anti-leucine-richglioma inactivated protein 1 (LGI1) encephalitis has an autoimmune origin and can be reversed with immunotherapy. It is obvious that identifying and treating this condition early is of paramount importance. We present the case of a 69-year-old man who was admitted to hospital with faciobrachial dystonic seizures and was found to have antibodies to LGI1. His symptoms started approximately 3 months prior admission to the hospital. There had also been some subtle cognitive impairment. He was treated with two courses of intravenous immunoglobulin and commenced on prednisolone 50 mg daily and clonazepam 500 µg at night...
November 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29093718/selective-limbic-blood-brain-barrier-breakdown-in-a-feline-model-of-limbic-encephalitis-with-lgi1-antibodies
#7
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/29070756/a-case-of-anti-mog-antibody-positive-multiphasic-disseminated-encephalomyelitis-co-occurring-with-unilateral-cerebral-cortical-encephalitis
#8
Naoya Fukushima, Miki Suzuki, Ryo Ogawa, Kitami Hayashi, Jun-Ichi Takanashi, Takashi Ohashi
A 20-year-old woman first developed acute disseminated encephalomyelitis (ADEM) at 11 years of age. At 17 years of age, she was hospitalized due to generalized seizure and diagnosed with encephalitis. Brain MRI revealed a FLAIR-hyperintense lesion in the unilateral cerebral cortex. At 18 years of age, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was detected. At 20 years of age, she was admitted to our hospital, diagnosed with multifocal disseminated encephalomyelitis (MDEM). MDEM has been observed in patients that are seropositive for the anti-MOG antibody...
October 26, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29057855/a-rare-case-of-autoimmune-limbic-encephalitis-an-uncharted-territory
#9
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/29055902/lgi1-caspr2-and-related-antibodies-a-molecular-evolution-of-the-phenotypes
#10
REVIEW
Sophie N M Binks, Christopher J Klein, Patrick Waters, Sean J Pittock, Sarosh R Irani
Recent biochemical observations have helped redefine antigenic components within the voltage-gated potassium channel (VGKC) complex. The related autoantibodies may be now divided into likely pathogenic entities, which target the extracellular domains of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2), and species that target intracellular neuronal components and are likely non-pathogenic. This distinction has enhanced clinical practice as direct determination of LGI1 and CASPR2 antibodies offers optimal sensitivity and specificity...
October 21, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29053037/possible-autoantibody-negative-limbic-encephalitis-after-anterior-temporal-lobectomy-for-hippocampal-sclerosis
#11
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/29042902/unusual-case-of-posterior-reversible-encephalopathy-syndrome-in-a-patient-with-anti-glomerular-basement-membrane-antibody-glomerulonephritis-a-case-report-and-review-of-the-literature
#12
Boram Cha, Dae Young Kim, Hyunil Jang, Seun Deuk Hwang, Huck Jei Choi, Moon-Jae Kim
Posterior reversible encephalopathy syndrome (PRES) is characterized by a clinical and radiological entity with the sudden onset of seizures, headache, altered consciousness, and visual disturbances in patients with the findings of reversible vasogenic subcortical edema without infarction. Hypertension, renal disease, and autoimmune disease are co-morbid conditions of PRES. Nevertheless, there have only been a few case reports of PRES in a patient with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN)...
September 2017: Electrolyte & Blood Pressure: E & BP
https://www.readbyqxmd.com/read/29042524/-autoimmune-and-epilepsy
#13
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/29040795/case-presentation-of-anti-nmda-receptor-encephalitis-in-a-4-year-old-boy
#14
Daniel A Gbadero, Emmanuel O Adegbite, Jean-Baptist LePichon, Tina M Slusher
We report a case of a 4-year-old boy from Oyo, Nigeria, presenting with prolonged seizures and coma with the subsequent development of oro-lingual-facial dyskinesia with frequent tongue thrusting, dysconjugate gaze and choreoathetoid movements of the limbs because of autoimmune encephalitis consistent with anti-N-methyl-D-aspartate (anti-NMDAR) encephalitis.
October 6, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/29018476/recurrent-and-prolonged-infections-in-a-child-with-a-homozygous-ifih1-nonsense-mutation
#15
Maha Zaki, Michaela Thoenes, Amit Kawalia, Peter Nürnberg, Rolf Kaiser, Raoul Heller, Hanno J Bolz
In an Egyptian girl born to consanguineous parents, whole-exome sequencing (WES) identified a homozygous mutation in PHGDH, c.1273G>A (p.Val425Met), indicating 3-phosphoglycerate dehydrogenase deficiency. This diagnosis was compatible with the patient's microcephaly, severe psychomotor retardation, seizures and cataracts. However, she additionally suffered from recurrent (at least monthly) episodes of prolonged and severe chest infections requiring hospitalization, suggesting a secondary, predisposing and potentially Mendelian, condition...
2017: Frontiers in Genetics
https://www.readbyqxmd.com/read/28988523/immunopathology-in-drug-resistant-mesial-temporal-lobe-epilepsy-with-different-types-of-hippocampal-sclerosis
#16
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/28982961/mechanisms-in-endocrinology-maternal-thyroid-dysfunction-during-pregnancy-and-behavioural-and-psychiatric-disorders-of-children-a-systematic-review
#17
REVIEW
Dagnachew Muluye Fetene, Kim S Betts, Rosa Alati
BACKGROUND: Maternal thyroid dysfunction during pregnancy may lead to persistent neurodevelopmental disorders in the offspring appearing in later life. This study aimed to review the available evidence concerning the relationship between maternal thyroid status during pregnancy and offspring behavioural and psychiatric disorders. METHODS: Systematic electronic database searches were conducted using PubMed, Embase, PsycNET, Scopus, Google Scholar and Cochrane library...
November 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28979520/status-epilepticus-and-anti-nmda-receptor-encephalitis-after-resection-of-an-ovarian-teratoma
#18
Amritpal S Jandu, Peter M Odor, Steven D Vidgeon
Anti-N-methyl-D-aspartate receptor encephalitis is a recently recognised autoimmune, paraneoplastic syndrome that typically presents with psychiatric disturbance, reduced conscious level and seizures. The disorder has been previously associated with ovarian teratomas. We present the case of a 35-year-old female, with a previous surgical history for resection of an ovarian teratoma, who later developed status epilepticus and anti-N-methyl-D-aspartate receptor encephalitis requiring intensive care management...
November 2016: J Intensive Care Soc
https://www.readbyqxmd.com/read/28974152/encephalitis-with-antibodies-against-the-gabab-receptor-seizures-as-the-most-common-presentation-at-admission
#19
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/28951835/effect-of-plasmapheresis-on-serum-levels-of-clobazam-levetiracetam-and-topiramate
#20
To Harmony Hau Man, Chang Richard Shek-Kwan, Chan Angel On-Kei, Chan Phoebe Wing Lam
A 27-year-old man with a diagnosis of new onset refractory status epilepticus (NORSE) was treated with five anti-seizure drugs (ASDs) including clobazam, levetiracetam and topiramate. He received plasma exchange (PE) for presumed autoimmune etiology. Serum ASD levels were serially monitored in two sessions. Levels of clobazam, levetiracetam and topiramate were significant reduced by PE. Serum clobazam level dropped down to at least 85% and 75% of the baseline during and after the procedure respectively; levetiracetam dropped down to 83% and 83%; and topiramate dropped to 86% and 79%...
2017: Epilepsy & Behavior Case Reports
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