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Autoimmune neurology

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https://www.readbyqxmd.com/read/28644255/periodic-paralysis-and-encephalopathy-as-initial-manifestations-of-graves-disease-case-report-and-review-of-the-literature
#1
Theocharis Tsironis, Athanasios Tychalas, Dimitrios Kiourtidis, Jannis Kountouras, Georgia Xiromerisiou, Jobst Rudolf, Georgia Deretzi
BACKGROUND: Thyrotoxic periodic paralysis (TPP) is an uncommon complication of Graves' disease, characterized by the triad of acute hypokalemia without total body potassium deficit, episodic muscle paralysis, and thyrotoxicosis. Graves' encephalopathy is an extremely rare form of encephalopathy associated with autoimmune thyroid disease (EAATD), characterized by neuropsychiatric symptoms, increased antithyroid antibodies and cerebrospinal fluid protein concentration, nonspecific electroencephalogram abnormalities, and cortico-responsiveness...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#2
Hussein Algahtani, Bader Shirah, Muhammad Amin, Eyad Altarazi, Hashem Almarzouki
Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28641772/susac-syndrome-a-differential-diagnosis-of-white-matter-lesions
#3
Mariano Marrodan, Jorge Correale, Lucas Alessandro, Mariela Amaya, Maria Eugenia Fracaro, Alejandro Alfredo Köhler, Marcela Fiol
INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28638382/expanding-role-of-t-cells-in-human-autoimmune-diseases-of-the-central-nervous-system
#4
REVIEW
Deepti Pilli, Alicia Zou, Fiona Tea, Russell C Dale, Fabienne Brilot
It is being increasingly recognized that a dysregulation of the immune system plays a vital role in neurological disorders and shapes the treatment of the disease. Aberrant T cell responses, in particular, are key in driving autoimmunity and have been traditionally associated with multiple sclerosis. Yet, it is evident that there are other neurological diseases in which autoreactive T cells have an active role in pathogenesis. In this review, we report on the recent progress in profiling and assessing the functionality of autoreactive T cells in central nervous system (CNS) autoimmune disorders that are currently postulated to be primarily T cell driven...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28636961/myeloid-cells-in-the-central-nervous-system
#5
REVIEW
Jasmin Herz, Anthony J Filiano, Ashtyn Smith, Nir Yogev, Jonathan Kipnis
The central nervous system (CNS) and its meningeal coverings accommodate a diverse myeloid compartment that includes parenchymal microglia and perivascular macrophages, as well as choroid plexus and meningeal macrophages, dendritic cells, and granulocytes. These myeloid populations enjoy an intimate relationship with the CNS, where they play an essential role in both health and disease. Although the importance of these cells is clearly recognized, their exact function in the CNS continues to be explored. Here, we review the subsets of myeloid cells that inhabit the parenchyma, meninges, and choroid plexus and discuss their roles in CNS homeostasis...
June 20, 2017: Immunity
https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#6
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28630891/frequency-of-neurological-disorders-in-bullous-pemphigoid-patients-a-cross-sectional-study
#7
Sheida Khosravani, Farhad Handjani, Reyhaneh Alimohammadi, Nasrin Saki
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disorder which occurs mostly in the elderly. Several studies have reported an association between BP and neurological disorders (ND). OBJECTIVE: The purpose of this study was to evaluate the association between BP and neurological disorders in Iranian patients. METHODS: In this cross-sectional study, 87 patients with BP were enrolled. They were compared to 184 controls...
2017: International Scholarly Research Notices
https://www.readbyqxmd.com/read/28626408/pd-1-checkpoint-inhibitor-associated-autoimmune-encephalitis
#8
Stephanie Schneider, Silke Potthast, Paul Komminoth, Guido Schwegler, Steffen Böhm
OBJECTIVE: To report first-hand narrative experience of autoimmune encephalitis and to briefly review currently available evidence of autoimmune encephalitis in cancer patients treated with immune checkpoint inhibitors. SETTING: A case study is presented on the management of a patient who developed autoimmune encephalitis during nivolumab monotherapy occurring after 28 weeks on anti-PD-1 monotherapy (nivolumab 3 mg/kg every 2 weeks) for non-small cell lung cancer...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28620980/case-of-autoimmune-autonomic-ganglionopathy-manifesting-anhidrosis
#9
Asuka Yoshifuku, Koichi Yoneda, Yusuke Sakiyama, Osamu Higuchi, Shunya Nakane, Takuro Kanekura
Autoimmune autonomic ganglionopathy (AAG), clinically characterized by gastrointestinal dysmotility, orthostatic hypotension and tonic pupils, is an idiopathic acquired disorder of the autonomic nervous system elicited by antibodies against ganglionic acetylcholine receptor (gAChR). We encountered a 60-year-old man who presented with severe anhidrosis, difficulty in thermoregulation, orthostatic hypotension, gastrointestinal dysmotility, tonic pupils and ptosis. Histologically, an anhidrotic skin sample was normal...
June 16, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28620692/the-enteric-nervous-system-is-a-potential-autoimmune-target-in-multiple-sclerosis
#10
Marie Wunsch, Samir Jabari, Barbara Voussen, Michael Enders, Shanthi Srinivasan, François Cossais, Thilo Wedel, Martina Boettner, Anna Schwarz, Linda Weyer, Oktay Göcer, Michael Schroeter, Mathias Maeurer, Matthias Woenckhaus, Karolin Pollok, Helena Radbruch, Luisa Klotz, Claus-Jürgen Scholz, Joachim Nickel, Andreas Friebe, Klaus Addicks, Süleyman Ergün, Paul V Lehmann, Stefanie Kuerten
Multiple sclerosis (MS) is a chronic autoimmune disease of the central nervous system (CNS) in young adults that has serious negative socioeconomic effects. In addition to symptoms caused by CNS pathology, the majority of MS patients frequently exhibit gastrointestinal dysfunction, which was previously either explained by the presence of spinal cord lesions or not directly linked to the autoimmune etiology of the disease. Here, we studied the enteric nervous system (ENS) in a B cell- and antibody-dependent mouse model of MS by immunohistochemistry and electron microscopy at different stages of the disease...
June 15, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28614301/microglia-dependent-synapse-loss-in-type-i-interferon-mediated-lupus
#11
Allison R Bialas, Jessy Presumey, Abhishek Das, Cees E van der Poel, Peter H Lapchak, Luka Mesin, Gabriel Victora, George C Tsokos, Christian Mawrin, Ronald Herbst, Michael C Carroll
Systemic lupus erythematosus (SLE) is an incurable autoimmune disease characterized by autoantibody deposition in tissues such as kidney, skin and lungs. Notably, up to 75% of patients with SLE experience neuropsychiatric symptoms that range from anxiety, depression and cognitive impairment to seizures and, in rare cases, psychosis-collectively this is referred to as central nervous system (CNS) lupus. In some cases, certain autoantibodies, such as anti-NMDAR or anti-phospholipid antibodies, promote CNS lupus...
June 14, 2017: Nature
https://www.readbyqxmd.com/read/28607558/hashimoto-s-encephalopathy-presenting-with-unusual-behavioural-disturbances-in-an-adolescent-girl
#12
Murugan Selvaraj Karthik, Kulothungan Nandhini, Viswanath Subashini, Ramasamy Balakrishnan
Hashimoto's encephalopathy (HE) is a rare autoimmune disorder with neurological and neuropsychiatric manifestations and elevated titres of anti-thyroid antibodies. Here we are reporting a case of HE in a 19-year-old girl who presented with seizure-like episodes, confusion, and behavioural disturbances with catatonic symptoms such as posturing, echopraxia, echolalia, and ambivalence. Patient did not respond to antipsychotics and anticonvulsants. On further investigation, patient was found to have high serum anti-TPO antibodies of about 1261 U/mL with euthyroid status, which supported a suspicion of HE...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28604632/experimental-autoimmune-encephalomyelitis-eae-induced-elevated-expression-of-the-e1-isoform-of-methyl-cpg-binding-protein-2-mecp2e1-implications-in-multiple-sclerosis-ms-induced-neurological-disability-and-associated-myelin-damage
#13
Tina Khorshid Ahmad, Ting Zhou, Khaled AlTaweel, Claudia Cortes, Ryan Lillico, Ted Martin Lakowski, Kiana Gozda, Michael Peter Namaka
Multiple sclerosis (MS) is a chronic neurological disease characterized by the destruction of central nervous system (CNS) myelin. At present, there is no cure for MS due to the inability to repair damaged myelin. Although the neurotrophin brain derived neurotrophic factor (BDNF) has a beneficial role in myelin repair, these effects may be hampered by the over-expression of a transcriptional repressor isoform of methyl CpG binding protein 2 (MeCP2) called MeCP2E1. We hypothesize that following experimental autoimmune encephalomyelitis (EAE)-induced myelin damage, the immune system induction of the pathogenic MeCP2E1 isoform hampers the myelin repair process by repressing BDNF expression...
June 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28603493/role-of-atypical-chemokine-receptors-in-microglial-activation-and-polarization
#14
REVIEW
Valentina Salvi, Francesca Sozio, Silvano Sozzani, Annalisa Del Prete
Inflammatory reactions occurring in the central nervous system (CNS), known as neuroinflammation, are key components of the pathogenic mechanisms underlying several neurological diseases. The chemokine system plays a crucial role in the recruitment and activation of immune and non-immune cells in the brain, as well as in the regulation of microglia phenotype and function. Chemokines belong to a heterogeneous family of chemotactic agonists that signal through the interaction with G protein-coupled receptors (GPCRs)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28601293/from-dizziness-to-severe-ataxia-and-dysarthria-new-cases-of-anti-ca-arhgap26-autoantibody-associated-cerebellar-ataxia-suggest-a-broad-clinical-spectrum
#15
Ulrike Wallwitz, Sebastian Brock, Antje Schunck, Brigitte Wildemann, Sven Jarius, Frank Hoffmann
In 2010, a novel anti-neuronal autoantibody, termed anti-Ca, was described in a patient with subacute cerebellar ataxia, and Rho GTPase-activating protein 26 (ARHGAP26) was identified as the target antigen. Recently, three additional cases of anti-Ca-positive cerebellar ataxia have been published. In addition to ataxia, cognitive decline and depression have been observed in some patients. Here, we report two new cases of anti-Ca-associated autoimmune cerebellar ataxia. Patient 1 presented with dizziness and acute yet mild limb and gait ataxia...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28601291/absence-of-autoreactive-cd4-t-cells-targeting-hla-dqa1-01-02-dqb1-06-02-restricted-hypocretin-orexin-epitopes-in-narcolepsy-type-1-when-detected-by-elispot
#16
Birgitte Rahbek Kornum, Kristoffer Sølvsten Burgdorf, Anja Holm, Henrik Ullum, Poul Jennum, Stine Knudsen
Narcolepsy type 1, a neurological sleep disorder strongly associated with Human Leukocyte Antigen (HLA-)DQB1*06:02, is caused by the loss of hypothalamic neurons producing the wake-promoting neuropeptide hypocretin (hcrt, also known as orexin). This loss is believed to be caused by an autoimmune reaction. To test whether hcrt itself could be a possible target in the autoimmune attack, CD4(+) T-cell reactivity towards six different 15-mer peptides from prepro-hypocretin with high predicted affinity to the DQA1*01:02/DQB1*06:02 MHC class II dimer was tested using EliSpot in a cohort of 22 narcolepsy patients with low CSF hcrt levels, and 23 DQB1*06:02 positive healthy controls...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28600040/anti-n-methyl-d-aspartate-receptor-nmdar-encephalitis-during-pregnancy-clinical-analysis-of-reported-cases
#17
Yan-Chao Shi, Xiu-Ju Chen, Hong-Mei Zhang, Zhen Wang, Da-Yong Du
OBJECTIVE: To analyze the clinical features of 13 pregnant patients with anti-N-Methyl-d-Aspartate receptor (NMDAR) encephalitis. MATERIALS AND METHODS: Retrospective review of thirteen reported cases was conducted for anti-NMDAR encephalitis patients during pregnancy. The clinical data were collected from papers published in PubMed prior to 16 February 2016. Statistical analysis of the data was performed, which encompasses the patients' age, past medical history, onset of symptoms, concomitant with ovarian teratomas, immunotherapy, outcomes of mothers and newborns...
June 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28590049/chromatographic-analysis-of-tryptophan-metabolites
#18
REVIEW
Ilona Sadok, Andrzej Gamian, Magdalena Maria Staniszewska
The kynurenine pathway generates multiple tryptophan metabolites called collectively kynurenines and leads to formation of the enzyme co-factor NAD. The first step in this pathway is tryptophan degradation, initiated by the rate-limiting enzymes indoleamine 2,3-dioxygenase or tryptophan 2,3-dioxygenase, depending on the tissue. The balanced kynurenine metabolism, which has been a subject of multiple studies in last decades, plays an important role in several physiological and pathological conditions such as infections, autoimmunity, neurological disorders, cancer, cataracts, as well as pregnancy...
June 7, 2017: Journal of Separation Science
https://www.readbyqxmd.com/read/28589388/-efficacy-and-safety-analysis-of-off-label-treatment-with-biologics-in-autoinflammatory-diseases-experiences-from-a%C3%A2-german-registry%C3%A2-graid2
#19
F Proft, M Fleck, C Fiehn, H Schulze-Koops, M Witt, T Dörner, J C Henes
OBJECTIVE: To evaluate the safety and efficacy of therapy with biologics in patients with autoinflammatory diseases (AIF) or macrophage activating syndrome (MAS) in a real-life setting in Germany. METHODS: The German Register of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy and treated with initial off-label biologics between August 2006 and December 2013...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28588791/synchronous-adie-s-syndrome-and-type-1-antineuronal-nuclear-antibody-anti-hu-related-paraneoplastic-neurological-syndromes-as-predictors-of-complete-response-in-limited-stage-small-cell-lung-cancer-a-case-report
#20
Katherina Bernadette Sreter, Blazenka Barisic, Marija Barisic Kutija, Suzana Kukulj, Miroslav Samarzija
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies...
June 2017: Molecular and Clinical Oncology
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