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lgi-1 antibody

Neşe Ayşit-Altuncu, Canan Ulusoy, Gürkan Öztürk, Erdem Tüzün
Anti-leucine-rich glioma inactivated 1 (anti-LGI1) encephalitis is one of the most frequently encountered forms of autoimmune encephalitis. Many patients with anti-LGI1 encephalitis develop permanent hippocampal neuron loss and chronic neuropsychiatric symptoms, suggesting that LGI antibodies (Ab) might have a neurotoxic action. To investigate this hypothesis, purified serum IgG of three patients with anti-LGI1 encephalitis and six healthy controls were incubated with cultured primary hippocampal neurons obtained from newborn mice...
May 15, 2018: Neuroreport
Ronnyson Susano Grativvol, Mateus Mistieri Simabukuro, Raphael Ribeiro Spera, Wagner Cid Palmeira Cavalcante, Hugo Henrique Soares Araújo, Daniel Sakuno, Leandro Tavares Lucato, Lécio Figueira Pinto, Luiz Henrique Martins Castro, Ricardo Nitrini
No abstract text is available yet for this article.
November 2016: Arquivos de Neuro-psiquiatria
Osamu Watanabe
Antibodies against various neural surface antigens induce cognitive impairments. Anti-VGKC (voltage gated potassium channel) complex antibodies are well known as one of the causative autoantibodies. An anti-VGKC antibody was identified as the autoantibody in acquired neuromyotonia (Isaacs' syndrome), which causes muscle cramps and difficulty in opening the palm of the hands. However, this antibody also tests positive in autoimmune limbic encephalitis, which has a subacute progress and causes poor memory or epilepsy attacks...
April 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Colin Casault, Katayoun Alikhani, Neelan Pillay, Marcus Koch
This is a case of autoimmune encephalitis with features of faciobrachial dystonic seizures (FBDS) pathognomonic for Leucine Rich Glioma inactivated (LGI)1 antibody encephalitis. This voltage-gated potassium channel complex encephalitis is marked by rapid onset dementia, FBDS and hyponatremia, which is sensitive to management with immunotherapy including steroids, IVIG and other agents. In this case report we review the clinical features, imaging and management of this condition.
December 15, 2015: Journal of Neuroimmunology
Yoshiko Murata, Osamu Watanabe, Go Taniguchi, Daichi Sone, Mao Fujioka, Mitsutoshi Okazaki, Eiji Nakagawa, Yutaka Watanabe, Masako Watanabe
Autoimmune epilepsy is an isolated phenotype of autoimmune encephalitis, which may be suspected in patients with unexplained adult-onset seizure disorders or resistance to antiepileptic drugs (AEDs). Antibodies against leucine-rich glioma inactivated subunit 1 of the voltage-gated potassium channel (VGKC) complex, recently termed anti-LGI-1 antibodies, are one of the causes of autoimmune epilepsies. Bizarre symptoms with extremely short duration and high frequency are clues to the possible presence of autoimmune epilepsy with anti-LGI-1 antibodies...
2015: Epilepsy & Behavior Case Reports
E Le Dault, S Lagarde, E Guedj, B Dufournet, C Rey, E Kaphan, G Tanguy, M Bregigeon, E Sagui, C Brosset
INTRODUCTION: Anti-leucine rich glioma inactivated 1 encephalitis is a common and a treatable etiology of autoimmune encephalitis. Its diagnosis is a challenge because the initial diagnostic work-up is often normal. CASE REPORT: A 48-year-old man experienced cognitive and behavioral troubles, facio-brachial dystonic seizures and a syndrome of inappropriate antidiuretic hormone secretion. First line tests excluded infectious, neoplastic, systemic inflammatory, endrocrine or toxic etiologies...
February 2016: La Revue de Médecine Interne
M P Malter, C Frisch, J C Schoene-Bake, C Helmstaedter, K P Wandinger, W Stoecker, H Urbach, R Surges, C E Elger, A V Vincent, C G Bien
In limbic encephalitis (LE) with antibodies (Abs) to the voltage-gated potassium channel complex (VGKC), the Abs are mainly directed to the VGKC-complex proteins, leucine-rich, glioma inactivated 1 protein (LGI1) or contactin-associated protein-like 2 (CASPR-2) or neither. Here, we relate the outcomes of VGKC-LE patients to the presence of Abs to LGI1, CASPR-2 or neither antigen (LGI1/CASPR-2-Ab(-)). Clinical, neuropsychology and MRI data were obtained from patient records for all LE patients from the Bonn Epilepsy Centre positive for VGKC-Abs by radioimmunoprecipitation assay between 2002 and 2011...
September 2014: Journal of Neurology
Osamu Watanabe
Isaacs' syndrome is an antibody-mediated potassium channel disorder. Clinical symptoms of Isaacs' syndrome are characterized by muscle cramp, slow relaxation following muscle contraction, and hyperhidrosis. Hyperexcitability of the peripheral nerve cause these symptoms, which are relieved by administration of Na channel blockers and immunotherapy.The target channel proteins are voltage-gated potassium channels (VGKCs). The suppression of voltage-gated outward K(+) current by antibodies induces hyperexcitability of the peripheral nerve...
2013: Rinshō Shinkeigaku, Clinical Neurology
Brice Nativel, Mery Marimoutou, Vincent G Thon-Hon, Manoj Kumar Gunasekaran, Jessica Andries, Giovédie Stanislas, Cynthia Planesse, Christine Robert Da Silva, Maya Césari, Thomas Iwema, Philippe Gasque, Wildriss Viranaicken
Low-grade inflammation (LGI) is a central phenomenon in the genesis of obesity and insulin-resistance characterized by IL-6 in human serum. Whereas this LGI was initially thought to be mainly attributed to macrophage activation, it is now known that pre-adipocytes and adipocytes secrete several adipokines including IL-6 and participate to LGI and associated pathologies. In macrophages, HMGB1 is a nuclear yet secreted protein and acts as a cytokine to drive the production of inflammatory molecules through RAGE and TLR2/4...
2013: PloS One
Osamu Watanabe
Antibodies to voltage-gated potassium channels(VGKC) were first identified by radioimmunoassay of radioisotope labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain. These antibodies were found only in a proportion of patients with acquired neuromyotonia (Isaacs' syndrome). VGKC antibodies were also detected in Morvan's syndrome and in a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC" antibodies are mainly directed toward associated proteins(for example LGI-1, Caspr-2) that complex with the VGKCs themselves...
May 2013: Nihon Rinsho. Japanese Journal of Clinical Medicine
Osamu Watanabe
Various antibodies are associated with voltage-gated potassium channels (VGKCs). Representative antibodies to VGKCs were first identified by radioimmunoassays using radioisotope-labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain. These antibodies were detected only in a proportion of patients with acquired neuromyotonia (Isaacs' syndrome). VGKC antibodies were also detected in patients with Morvan's syndrome and in those with a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC" antibodies are mainly directed toward associated proteins (for example LGI-1 and CASPR-2) that complex with the VGKCs themselves...
April 2013: Brain and Nerve, Shinkei Kenkyū No Shinpo
Koji Sagane, Hachiro Sugimoto, Akinori Akaike
ADAM metallopeptidase domain 22 (ADAM22) is a neuronal membrane-spanning protein that is a potential receptor for leucine-rich, glioma-inactivated 1 (LGI1), and leucine-rich repeat LGI family, member 4 (LGI4). Several lines of study have shown a direct interaction between ADAM22 and LGI1, a mutation which is responsible for inherited epilepsy in humans. Both ADAM22-deficient mice and claw paw mice, congenitally deficient in LGI4, show hypomyelination in the peripheral nerves, suggesting that these molecules are involved in myelination processes...
April 2010: Journal of Receptor and Signal Transduction Research
Michèle Balage, Julien Averous, Didier Rémond, Cécile Bos, Estelle Pujos-Guillot, Isabelle Papet, Laurent Mosoni, Lydie Combaret, Dominique Dardevet
Aging is characterized by a decline in muscle mass that could be explained by a defect in the regulation of postprandial muscle protein metabolism. This study was undertaken to examine a possible link between the development of low-grade inflammation (LGI) in elderly and the resistance of muscle protein synthesis and degradation pathways to food intake. Fifty-five 20-month-old-rats were studied for 5 months; blood was withdrawn once a month to assess plasma fibrinogen and alpha2-macroglobulin. Animals were then separated into two groups at 25 months old according to their inflammation status: a control non-inflamed (NI, n=24) and a low-grade inflamed group (LGI, n=23)...
April 2010: Journal of Nutritional Biochemistry
Woo-Jae Park, Sang Eun Lee, Nyoun Soo Kwon, Kwang Jin Baek, Dong-Seok Kim, Hye-Young Yun
Leucine-rich glioma inactivated 3 (LGI3) is a member of LGI/epitempin (EPTP) family. The biological function of LGI3 and its association with disease are not known. We previously reported that mouse LGI3 was highly expressed in brain in a developmentally and transcriptionally regulated manner. In this study, we identified syntaxin 1, a SNARE component in exocytosis, as a candidate functional target of LGI3. Western blot analysis of mouse brain extract with LGI3 antibodies detected multiple protein forms (75-, 60-, 35- and 25-kDa)...
October 31, 2008: Neuroscience Letters
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