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Autoimmune dementia

Chan Hong Jeon
Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death...
November 2016: Journal of Korean Medical Science
Karen Manoutcharian, Roxanna Perez-Garmendia, Goar Gevorkian
Recombinant antibody fragments are promising alternatives to full-length immunoglobulins and offer important advantages compared with conventional monoclonal antibodies: extreme specificity, higher affinity, superior stability and solubility, reduced immunogenicity as well as easy and inexpensive large-scale production. Different antibody formats such as single-chain fragment variable (scFv), single-domain antibody fragments (VHHs or sdAbs), bispecific antibodies (bsAbs), intrabodies and nanobodies, are currently being studied in pre-clinical models of cancer as well as infectious and autoimmune diseases and many of them are being tested as therapeutics in clinical trials...
September 30, 2016: Current Neuropharmacology
Maryam Daneshpazhooh, Javad Khorassani, Kamran Balighi, Narges Ghandi, Hamidreza Mahmoudi, Hamidreza Tohidinik, Shahin Hamzelou, Cheyda Chams-Davatchi
INTRODUCTION: Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis. OBJECTIVES: Our aim was to examine the association of bullous pemphigoid and neurological diseases in Iranian bullous pemphigoid patients...
September 26, 2016: Indian Journal of Dermatology, Venereology and Leprology
Jenny Linnoila, Sean J Pittock
Autoimmune neurology is a rapidly evolving new subspecialty driven by the discovery of novel neural- (neuronal- or glial-) specific autoantibodies and their target antigens. The neurologic manifestations affecting the central nervous system include encephalitis, dementia, epilepsy, and movement and sleep disorders. Laboratory testing is now available for most of these neural-specific autoantibodies, which serve as diagnostic markers, in some instances directing the physician toward specific cancer types (e...
August 2016: Seminars in Neurology
Michal Schwartz, Aleksandra Deczkowska
Neuroinflammation is common to various diseases of the central nervous system (CNS), but its imprecise definition has led to many misconceptions in research and clinical approaches. It is now recognized that neuroinflammation in chronic neurodegenerative conditions, including Alzheimer's disease (AD) and age-related dementia, is distinct from the inflammation that accompanies relapsing-remitting multiple sclerosis (RRMS), and its experimental animal model, experimental autoimmune encephalomyelitis (EAE). Here, we discuss the discrete features of inflammation in different CNS pathologies, given the current understanding of the CNS-immune crosstalk; the roles of the immune cells that are involved, their phenotypes, and their location and route of entry to the CNS...
October 2016: Trends in Immunology
Y C Lai, Y W Yew, W C Lambert
Bullous pemphigoid (BP) is a chronic, autoimmune vesiculobullous disease that frequently occurs in the elderly population. Previous epidemiological studies have suggested an association between BP and neurological diseases; some studies, however, showed conflicting results. This study aimed to investigate if patients with BP have significantly higher risks for neurological disorders, compared to controls. A comprehensive search was performed using MEDLINE, EMBASE and Cochrane library databases. Case-control and cohort studies that assessed the relationship between BP and neurological diseases were included...
September 7, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
A Röh, P Falkai, A Hasan
BACKGROUND: Many patients with psychiatric and organic disorders may present primary or secondary psychotic symptoms. Based on the ICD 10 classification, this article describes the possible underlying diseases and their subdivision. The main focus will be differential-diagnostic evaluations of psychotic syndromes. RESULTS: In the clinical setting, the differential diagnosis of the paranoid schizophrenia and other psychotic disorders into three main disease groups is important: 1...
August 2016: Fortschritte der Neurologie-Psychiatrie
Kristian P Doyle, Marion S Buckwalter
Post-stroke cognitive decline and dementia pose a significant public health problem, with 30% of stroke survivors suffering from dementia. The reason for this high prevalence is not well understood. Pathogenic B cell responses to the damaged CNS are one possible contributing factor. B-lymphocytes and antibodies are present in and around the stroke core of some human subjects who die with stroke and dementia, and mice that develop delayed cognitive dysfunction after stroke have clusters of B-lymphocytes in the stroke lesion, and antibody infiltration in the stroked hemisphere...
August 13, 2016: Brain, Behavior, and Immunity
Tiffany Field
In this review, massage therapy has been shown to have beneficial effects on varying conditions including prenatal depression, preterm infants, full-term infants, autism, skin conditions, pain syndromes including arthritis and fibromyalgia, hypertension, autoimmune conditions including asthma and multiple sclerosis, immune conditions including HIV and breast cancer and aging problems including Parkinson's and dementia. Although many of the studies have involved comparisons between massage therapy and standard treatment control groups, several have compared different forms of massage (e...
August 2016: Complementary Therapies in Clinical Practice
G Westman, M Studahl, C Ahlm, B M Eriksson, B Persson, J Rönnelid, S Schliamser, E Aurelius
OBJECTIVES: To investigate the prevalence and temporal development of N-methyl-d-aspartate receptor (NMDAR) autoantibodies in relation to neurocognitive performance in patients with herpes simplex encephalitis (HSE). METHODS: This prospective observational study enrolled a total of 49 HSE patients within a randomized controlled trial of valacyclovir. Cerebrospinal fluid and serum samples were drawn in the initial stage of disease, after 2 to 3 weeks and after 3 months...
August 3, 2016: Clinical Microbiology and Infection
Paroni Giulia, Lauriola Michele, Fontana Andrea, D'Onofrio Grazia, Ciccone Filomena, Paris Francesco, Cascavilla Leandro, Urbano Maria, Gravina Carolina, Copetti Massimiliano, Greco Antonio
Cortical atrophy, neuronal loss, beta-amyloid deposition, neuritic plaques, and neurofibrillary tangles are neuropathological key features in the Alzheimer's disease (AD). Antibodies against beta-amyloid, neurotransmitters, microvascular endothelium components and microglial cells have been detected in AD serum suggesting that AD could be another autoimmune disease and provides a link between vascular pathology, endothelium dysfunction and neuronal cells death. Aim of the present study was to evaluate the association between autoantibody profile and cognitive impairment in geriatric patients, accounting for ApoE genotype as a potential confounding factor...
August 2016: Aging and Disease
Alireza Atri
This chapter reviews clinical applications and imaging findings useful in medical practice relating to neurodegenerative cognitive/dementing disorders. The preponderance of evidence and consensus guidelines support an essential role of multitiered neuroimaging in the evaluation and management of neurodegenerative cognitive/dementia syndrome that range in severity from mild impairments to frank dementia. Additionally, imaging features are incorporated in updated clinical and research diagnostic criteria for most dementias, including Alzheimer's disease (AD), Dementia with Lewy bodies (DLB), Frontotemporal Lobar Degenerations/Frontotemporal Dementia (FTD), and Vascular Cognitive Impairment (VCI)...
2016: Handbook of Clinical Neurology
Rachael Gardner, Rajesh Rangaswamy, Yen-Yi Peng
With the increased availability of laboratory tests, glutamic acid decarboxylase (GAD) antibody-positive limbic encephalitis has become an emerging diagnosis. The myriad symptoms of limbic encephalitis make the diagnosis challenging. Symptoms range from seizures, memory loss, dementia, confusion, to psychosis. We present a case of a 21-year-old female with GAD65 antibody-positive limbic encephalitis. The case is unique because the clinical course suggests that non-convulsive seizures are the major cause of this patient's clinical manifestations...
August 2016: Journal of Clinical Medicine Research
Aaron Burberry, Naoki Suzuki, Jin-Yuan Wang, Rob Moccia, Daniel A Mordes, Morag H Stewart, Satomi Suzuki-Uematsu, Sulagna Ghosh, Ajay Singh, Florian T Merkle, Kathryn Koszka, Quan-Zhen Li, Leonard Zon, Derrick J Rossi, Jennifer J Trowbridge, Luigi D Notarangelo, Kevin Eggan
C9ORF72 mutations are found in a significant fraction of patients suffering from amyotrophic lateral sclerosis and frontotemporal dementia, yet the function of the C9ORF72 gene product remains poorly understood. We show that mice harboring loss-of-function mutations in the ortholog of C9ORF72 develop splenomegaly, neutrophilia, thrombocytopenia, increased expression of inflammatory cytokines, and severe autoimmunity, ultimately leading to a high mortality rate. Transplantation of mutant mouse bone marrow into wild-type recipients was sufficient to recapitulate the phenotypes observed in the mutant animals, including autoimmunity and premature mortality...
July 13, 2016: Science Translational Medicine
Boaz Kim, Patrick Yoo, Tom Sutherland, Alison Boyd, Christiane Stehmann, Catriona McLean, Steven Collins
OBJECTIVE: To report a rare case of leucine-rich, glioma inactivated 1 (LGI1) antibody-mediated autoimmune encephalopathy clinically overlapping with pathologically confirmed sporadic Creutzfeldt-Jakob disease (CJD). METHODS: The patient was investigated with repeated brain MRI, EEG, CSF examination, whole-body fluorodeoxy-glucose positron emission tomography, genetic analysis of the prion protein gene (PRNP), and extensive serologic screening for paraneoplastic and autoimmune encephalopathy markers...
August 2016: Neurology® Neuroimmunology & Neuroinflammation
Z Ren, D Y Hsu, J Brieva, N B Silverberg, S M Langan, J I Silverberg
BACKGROUND: Bullous pemphigoid (BP) is associated with significant disability and comorbid health disorders that may lead to or result from hospitalization. However, little is known about the inpatient burden and comorbidities of BP. METHODS: We analyzed data from the 2002-2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the US (n=72,108,077 adults). RESULTS: The prevalences of hospitalization for BP increased from 25...
June 25, 2016: British Journal of Dermatology
Hsing-Chuan Tsai, May H Han
Sphingosine-1-phosphate (S1P) and S1P receptors (S1PR) are ubiquitously expressed. S1P-S1PR signaling has been well characterized in immune trafficking and activation in innate and adaptive immune systems. However, the full extent of its involvement in the pathogenesis of autoimmune diseases is not well understood. FTY720 (fingolimod), a non-selective S1PR modulator, significantly decreased annualized relapse rates in relapsing-remitting multiple sclerosis (MS). FTY720, which primarily targets S1P receptor 1 as a functional antagonist, arrests lymphocyte egress from secondary lymphoid tissues and reduces neuroinflammation in the central nervous system (CNS)...
July 2016: Drugs
Keigo Koda, Yasunori Enomoto, Minako Omae, Daisuke Akahori, Takefumi Abe, Hirotsugu Hasegawa, Takashi Matsui, Koshi Yokomura, Takafumi Suda
An 86-year-old woman with severe dementia had been treated with oral prednisolone at 2 mg/day for autoimmune bullous dermatosis for several years. One year ago, she referred to our hospital due to an ulcerative skin lesion over the right tibial tuberosity. The lesion was treated by an iodine-containing ointment, but did not heal. Subsequently, a new skin lesion appeared in the right popliteal fossa. One month ago, the patient had increased sputum production that was accompanied by fever, anorexia, and dyspnea; consequently, she visited our department...
February 2016: Kekkaku: [Tuberculosis]
Yu Chen, Xiao-Wei Xing, Jia-Tang Zhang, Ruo-Xi Wang, Wei Zhao, Qing-Che Tan, Ruo-Zhuo Liu, Xiang-Qing Wang, Xu-Sheng Huang, Sheng-Yuan Yu
Autoimmune encephalitis associated with anti-voltage-gated potassium channel antibodies are most likely to be misdiagnosed as sporadic Creutzfeldt-Jakob disease (sCJD). Our goal was to delineate patients who were initially suspected to have CJD but were later found to have AE. We performed a retrospective clinical review of cases of individuals and made a comparison between groups of patients diagnosed with sCJD and AE. Patients who had rapidly progressing dementia and focal neurological impairment, such as aphasia, gait disturbance, visual disturbance, and depression, at onset were diagnosed with sCJD, whereas epilepsy, hyponatremia and dysautonomia were strong hints for AE...
June 15, 2016: Journal of Neuroimmunology
Brin Freund, Manoj Maddali, Thomas E Lloyd
INTRODUCTION: Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying to differentiate from amyotrophic lateral sclerosis with frontotemporal dementia. METHODS: A 76-year-old man with a history of untreated prostate adenocarcinoma was evaluated for subacute cognitive decline, diffuse muscle cramps, and hyponatremia...
June 2016: Journal of Clinical Neuromuscular Disease
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