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https://www.readbyqxmd.com/read/29662677/auto-antibodies-against-p-q-and-n-type-voltage-dependent-calcium-channels-mimicking-frontotemporal-dementia
#1
Kyan Younes, Lauren A Lepow, Cynthia Estrada, Paul E Schulz
The behavioral variant of frontotemporal dementia is usually a sporadic and progressive neurodegenerative disorder. Here, we report the subacute onset of a frontotemporal dementia phenotype with a treatable etiology. The patient has a history of rheumatoid arthritis, episcleritis, and thyroid eye disease on immunosuppressive therapy. He experienced a rapid personality change, including inappropriate behavior, which suggested frontotemporal dementia. Results of imaging and neuropsychological testing also suggested frontotemporal dementia...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29643688/simultaneous-18f-fdg-pet-mri-in-autoimmune-limbic-encephalitis
#2
Sangeeta Taneja, Vinit Suri, Aashim Ahuja, Amarnath Jena
Limbic encephalitis is an autoimmune disorder characterized by inflammation of the brain with rapidly progressing dementia which requires definitive neurological evaluation. We describe both clinical as well as imaging findings in a case of limbic encephalitis using positron emission tomography/magnetic resonance imaging.
April 2018: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/29549218/clinical-and-pathogenic-significance-of-igg-iga-and-igm-antibodies-against-the-nmda-receptor
#3
Makoto Hara, Eugenia Martinez-Hernandez, Helena Ariño, Thais Armangué, Marianna Spatola, Mar Petit-Pedrol, Albert Saiz, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To determine the frequency and clinical relevance of immunoglobulin (Ig)G, IgA, and IgM N -methyl-d-aspartate receptor (NMDAR) antibodies in several diseases, and whether the IgG antibodies occur in disorders other than anti-NMDAR encephalitis. METHODS: Evaluation of IgG, IgA, and IgM NMDAR antibodies in serum of 300 patients with anti-NMDAR encephalitis, stroke, dementia, schizophrenia, or seronegative autoimmune encephalitis. Antibodies and their effect on cultured neurons were examined with cell-based assays and brain and live neuronal immunostaining...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29538413/functional-autoantibodies-in-patients-with-different-forms-of-dementia
#4
Gerd Wallukat, Harald Prüss, Johannes Müller, Ingolf Schimke
Dementia in general and Alzheimer's disease in particular is increasingly seen in association with autoimmunity being causatively or supportively involved in the pathogenesis. Besides classic autoantibodies (AABs) present in dementia patients, there is the new autoantibody class called functional autoantibodies, which is directed against G-protein coupled receptors (GPCRs; GPCR-AABs) and are seen as pathogenic players. However, less is known about dementia patients' burden with functional autoantibodies. We present here for the first time a study analyzing the prevalence of GPCR-AABs in patients with different dementia forms such as unclassified, Lewy body, vascular and Alzheimer's dementia...
2018: PloS One
https://www.readbyqxmd.com/read/29525731/atypical-presentation-of-probable-creutzfeldt-jakob-disease-associated-with-anti-zic4-antibody-literature-review-of-neuronal-antibodies-in-creutzfeldt-jakob-disease
#5
Richard Salazar
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. CASE REPORT: A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia...
May 2018: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29499794/rosacea-comorbidities
#6
REVIEW
Nora Vera, Nupur U Patel, Lucia Seminario-Vidal
Rosacea is a chronic inflammatory cutaneous disorder with an unclear pathogenesis. It has been associated with multiple comorbidities, including cardiovascular diseases, malignancies, depression, migraines, dementia, Parkinson disease, gastrointestinal disorders, and autoimmune conditions. The extent, clinical significance, and implications of these associations remain a topic of discussion. Further evaluation of these comorbidities may offer valuable insight for future screening practices and treatment recommendations...
April 2018: Dermatologic Clinics
https://www.readbyqxmd.com/read/29425965/the-small-heat-shock-proteins-especially-hspb4-and-hspb5-are-promising-protectants-in-neurodegenerative-diseases
#7
REVIEW
Zhihui Zhu, Georg Reiser
Small heat shock proteins (sHsps) are a group of proteins with molecular mass between 12 and 43 kDa. Currently, 11 members of this family have been classified, namely HspB1 to HspB11. HspB1, HspB2, HspB5, HspB6, HspB7, and HspB8, which are expressed in brain have been observed to be related to the pathology of neurodegenerative diseases, including Parkinson's, Alzheimer's, Alexander's disease, multiple sclerosis, and human immunodeficiency virus-associated dementia. Specifically, sHsps interact with misfolding and damaging protein aggregates, like Glial fibrillary acidic protein in AxD, β-amyloid peptides aggregates in Alzheimer's disease, Superoxide dismutase 1 in Amyotrophic lateral sclerosis and cytosine-adenine-guanine/polyglutamine (CAG/PolyQ) in Huntington's disease, Spinocerebellar ataxia type 3, Spinal-bulbar muscular atrophy, to reduce the toxicity or increase the clearance of these protein aggregates...
February 6, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29422904/emerging-role-of-immunity-in-cerebral-small-vessel-disease
#8
REVIEW
Ying Fu, Yaping Yan
Cerebral small vessel disease (CSVD) is one of the main causes of vascular dementia in older individuals. Apart from risk containment, efforts to prevent or treat CSVD are ineffective due to the unknown pathogenesis of the disease. CSVD, a subtype of stroke, is characterized by recurrent strokes and neurodegeneration. Blood-brain barrier (BBB) impairment, chronic inflammatory responses, and leukocyte infiltration are classical pathological features of CSVD. Understanding how BBB disruption instigates inflammatory and degenerative processes may be informative for CSVD therapy...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29386827/mortality-and-comorbidity-profiles-of-patients-with-bullous-pemphigoid-in-korea
#9
Hyeon Woo Jeon, Sook Jung Yun, Seung-Chul Lee, Young Ho Won, Jee-Bum Lee
Background: Bullous pemphigoid (BP) is a common autoimmune-mediated blistering skin disease that is significantly associated with mortality and morbidity. However, few studies regarding the mortality and comorbidity profiles of BP have been reported in Korea. Objective: To evaluate and compare the mortality, comorbidity profiles, and risk factors between patients with BP who visited our clinic and an age-matched general population of Korea. Methods: We retrospectively evaluated 103 patients diagnosed with BP between 2006 and 2013 at Chonnam National University Hospital in Gwangju, Korea...
February 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29357796/autoimmunity-and-frontotemporal-dementia
#10
Antonella Alberici, Viviana Cristillo, Stefano Gazzina, Alberto Benussi, Alessandro Padovani, Barbara Borroni
Frontotemporal Dementia (FTD) is a neurodegenerative disorder which asymmetrically affects the frontotemporal lobes; it is characterized by behavioural abnormalities, language impairment, and deficits of executive functions. Behavioural variant FTD (bvFTD) and Primary Progressive Aphasias (PPAs) represent the most common phenotypes. The identification of mutations responsible for autosomal dominant inherited disorder, namely Microtubule Associated Protein Tau (MAPT), Granulin (GRN) and chromosome 9 open reading frame 72 (C9orf72) mutations, contributed to elucidate the molecular pathways involved in brain depositions of either Tau or TAR DNA-binding protein 43 (TDP43) inclusions...
January 18, 2018: Current Alzheimer Research
https://www.readbyqxmd.com/read/29344342/frontotemporal-dementia-latest-evidence-and-clinical-implications
#11
REVIEW
Juan Joseph Young, Mallika Lavakumar, Deena Tampi, Silpa Balachandran, Rajesh R Tampi
Background: Frontotemporal dementia (FTD) describes a cluster of neurocognitive syndromes that present with impairment of executive functioning, changes in behavior, and a decrease in language proficiency. FTD is the second most common form of dementia in those younger than 65 years and is expected to increase in prevalence as the population ages. This goal in our review is to describe advances in the understanding of neurobiological pathology, classification, assessment, and treatment of FTD syndromes...
January 2018: Therapeutic Advances in Psychopharmacology
https://www.readbyqxmd.com/read/29310343/cerebrospinal-fluid-real-time-quaking-induced-conversion-test-for-sporadic-creutzfeldt-jakob-disease-in-an-18-year-old-woman-a-case-report
#12
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29304089/autoimmune-rheumatic-diseases-increase-dementia-risk-in-middle-aged-patients-a-nationwide-cohort-study
#13
Tzu-Min Lin, Wei-Sheng Chen, Jau-Jiuan Sheu, Yi-Hsuan Chen, Jin-Hua Chen, Chi-Ching Chang
OBJECTIVE: Dementia is a common neurological disease that substantially affects public health. A previous study revealed that dementia occurs when the body's immune system attacks the cells of the brain, indicating that dementia may be similar to autoimmune rheumatic diseases (ARDs). In the current retrospective cohort study, we focused on middle-aged ARD patients (45 years or older) to investigate the association between ARDs in middle-aged people and dementia by using a nationwide population-based database in Taiwan...
2018: PloS One
https://www.readbyqxmd.com/read/29244265/herpes-zoster-and-dementia-a-nationwide-population-based-cohort-study
#14
Vincent Chin-Hung Chen, Shu-I Wu, Kuo-You Huang, Yao-Hsu Yang, Ting-Yu Kuo, Hsin-Yi Liang, Kuan-Lun Huang, Michael Gossop
OBJECTIVE: Some infectious diseases have been found to be associated with cognitive impairment and dementia. However, the relationship between herpes zoster and dementia has received little attention. This study aimed to investigate this association as well as associations of antiviral treatments for herpes zoster and incident dementia using a large national sample. METHODS: Cases were identified from the Taiwan National Health Insurance Research Database with a new diagnosis of herpes zoster (ICD-9-CM code: 053) between 1997 and 2013...
January 2018: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/29198043/the-diagnosis-of-dementias-a-practical-tool-not-to-miss-rare-causes
#15
Camilla Ferrari, Benedetta Nacmias, Sandro Sorbi
Dementia represents one of the most diffuse disorders of our Era. Alzheimer's disease is the principle cause of dementia worldwide. Metabolic, infectious, autoimmune, inflammatory, and genetic dementias represent a not negligible number of disorders, with increasing numbers in younger subjects. Due to the heterogeneity of patients and disorders, the diagnosis of dementia is challenging. In the present article, we propose a practical diagnostic approach following the two-step investigation procedure. The first step includes basic blood tests and brain neuroimaging, performed on all patients...
December 2, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29193473/autoimmune-glial-fibrillary-acidic-protein-astrocytopathy-in-chinese-patients-a-retrospective-study
#16
Y Long, J Liang, H Xu, Q Huang, J Yang, C Gao, W Qiu, S Lin, X Chen
BACKGROUND AND PURPOSE: The aim was to describe the clinical, radiological and pathological features of an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. METHODS: Data from 19 patients with positive GFAP-immunoglobulin G in cerebrospinal fluid (CSF) were retrospectively analyzed. RESULTS: The main disease manifestations included myelitis (68.4%), headache (63.2%), abnormal vision (63.2%), fever (52.6%), ataxia (36.8%), psychosis (31...
March 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29175155/neuroanatomical-distribution-of-dek-protein-in-corticolimbic-circuits-associated-with-learning-and-memory-in-adult-male-and-female-mice
#17
Valentina Ghisays, Elizabeth T Nguyen, Joshua Streicher, Nicholas A Pease, Maureen Fitzgerald, Christina M Estrada, Ana Franco-Villanueva, Lisa Privette Vinnedge, Matia B Solomon
DEK, a chromatin-remodeling gene expressed in most human tissues, is known for its role in cancer biology and autoimmune diseases. DEK depletion in vitro reduces cellular proliferation, induces DNA damage subsequently leading to apoptosis, and down-regulates canonical Wnt/β-catenin signaling, a molecular pathway essential for learning and memory. Despite a recognized role in cancer (non-neuronal) cells, DEK expression and function is not well characterized in the central nervous system. We conducted a gene ontology analysis (ToppGene), using a cancer database to identify genes associated with DEK deficiency, which pinpointed several genes associated with cognitive-related diseases (i...
February 10, 2018: Neuroscience
https://www.readbyqxmd.com/read/29154389/latent-autoimmune-diabetes-and-limbic-encephalitis-with-antibodies-against-glutamic-acid-decarboxylase
#18
Stoyan Popkirov, Seena Sebastian, Fatme S Ismail, Jörg Wellmer
No abstract text is available yet for this article.
April 2018: Journal of Diabetes
https://www.readbyqxmd.com/read/29145189/rem-sleep-behavior-disorder
#19
Claudio L Bassetti, Panagiotis Bargiotas
Rapid eye movement sleep behavior disorder (RBD) is a brain disorder, characterized by the dream enactment during rapid eye movement (REM) sleep due to a lack of physiologic muscle atonia and increased muscle twitching. Schenk was the first to describe this disorder in 1986; however, few authors reported in the 1970-1980s loss of physiological muscle atonia combined with dream enactment in the course of brainstem disorders and as a consequence of alcoholism and antidepressant treatment. RBD affects less than 1% of the adult population, but can be found in up to 25-50% of neurodegenerative disorders including Parkinson's disease, multisystem atrophy, and dementia with Lewy body...
2018: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/29114974/high-proportions-of-dementia-among-sle-patients-a-big-data-analysis
#20
Omer Gendelman, Shmuel Tiosano, Yehuda Shoenfeld, Doron Comaneshter, Howard Amital, Arnon D Cohen, Daniela Amital
OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting a wide range of systems including the peripheral and central nervous system. Cognitive impairment leading to dementia is one of the harmful central nervous system afflictions of SLE. The aim of this study was to investigate the association of SLE with dementia. METHODS: A cross-sectional study was conducted using Clalit Health Care database, the largest health maintenance organization in Israel with more than 4...
November 8, 2017: International Journal of Geriatric Psychiatry
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