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Autoimmune encephalopathy

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https://www.readbyqxmd.com/read/29325172/pres-review-of-histological-features
#1
Nicholas Willard, Justin M Honce, B K Kleinschmidt-DeMasters
Posterior reversible encephalopathy syndrome was described in 1996 as a clinical-neuroimaging entity characterized by parieto-occipital watershed-region edema without overt infarction. It has been linked to hypertension, eclampsia, immunosuppressive therapies, infections, and autoimmune disorders. The condition usually has an acute onset, presents with seizures, and ameliorates within days. There have been few neuropathological studies, but in some cases, tissue damage may be more permanent.
February 1, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29303953/voltage-gated-potassium-channel-antibody-autoimmune-encephalopathy-presenting-with-isolated-psychosis-in-an-adolescent
#2
David A Kahn
Antibody-mediated encephalopathies associated with serum or cerebrospinal fluid antibodies directed against neuronal structures may present with a multitude of neuropsychiatric syndromes. Although some of the antibody-driven conditions are now well recognized in adults (eg, N-methyl-D-aspartate receptor antibody encephalitis), the spectrum of neuropsychiatric manifestations in the pediatric population is less clear. Psychosis, confusion, catatonia, and additional behavioral changes, along with seizures, encephalopathy, and movement disorders, may be initial manifestations or concurrent features in all age groups...
November 2017: Journal of Psychiatric Practice
https://www.readbyqxmd.com/read/29303952/voltage-gated-potassium-channel-antibody-autoimmune-encephalopathy-presenting-with-isolated-psychosis-in-an-adolescent
#3
Natalie C Pon, Kimberly M Houck, Eyal Muscal, Sindhu A Idicula
Antibody-mediated encephalopathies associated with serum or cerebrospinal fluid antibodies directed against neuronal structures may present with a multitude of neuropsychiatric syndromes. Although some of the antibody-driven conditions are now well recognized in adults (eg, N-methyl-D-aspartate receptor antibody encephalitis), the spectrum of neuropsychiatric manifestations in the pediatric population is less clear. Psychosis, confusion, catatonia, and additional behavioral changes, along with seizures, encephalopathy, and movement disorders, may be initial manifestations or concurrent features in all age groups...
November 2017: Journal of Psychiatric Practice
https://www.readbyqxmd.com/read/29282342/-motor-symptoms-of-autoimmune-encephalopathies
#4
Yuichi Tashiro, Hiroshi Takashima
Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. At least 20 types of autoimmune encephalopathies have been discovered, with the most common type being Hashimoto encephalopathy. In clinical situations, we often observe that patients with autoimmune encephalopathy are misdiagnosed because they exhibit signs similar to those observed in functional psychogenic movement, conversion, or somatoform disorders. We clinically examined over 100 patients with autoimmune encephalopathy...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29277332/cytokine-chemokine-elevation-during-the-transition-phase-from-hsv-encephalitis-to-autoimmune-anti-nmda-receptor-encephalitis
#5
Takanori Omae, Yoshiaki Saito, Hirokazu Tsuchie, Koyo Ohno, Yoshihiro Maegaki, Hiroshi Sakuma
A 3-year-old girl suffered from anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis after resolution of herpes simplex virus encephalitis (HSE). Methylprednisolone pulse and immunoglobulin therapies showed little effect, but the patient completely recovered after six courses of monthly cyclophosphamide pulse therapy and successive maintenance on mycophenolate mofetil for one year. Anti-NMDA receptor antibody in the cerebrospinal fluid (CSF) was minimally detected during the prodromal febrile period and then was seen to be markedly elevated at the onset of second encephalopathy phase...
December 22, 2017: Brain & Development
https://www.readbyqxmd.com/read/29258968/refractory-chronic-epilepsy-associated-with-neuronal-auto-antibodies-could-perisylvian-semiology-be-a-clue
#6
Lisa Gillinder, Linda Tjoa, Basil Mantzioris, Stefan Blum, Sasha Dionisio
We report a case series of 10 patients with chronic medically refractory antibody-positive autoimmune epilepsy and assess their common clinical features. Immune-mediated seizures are most commonly reported in the context of encephalitis or encephalopathy, with few reports focusing on lone, chronic epilepsy in the outpatient setting. Our aim was to define the potential diagnostic clues that might be present in these cases, leading to consideration of an autoimmune cause of the epilepsy. We performed a retrospective review of all patients presenting to the outpatient department of our unit who underwent autoimmune screening...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29240027/congenital-hemophilia-a-presenting-with-hashimoto-s-encephalopathy-and-myocarditis-the-first-reported-case
#7
Ayse B Ergul, Yasemin Altuner Torun, Umut Altug, Fatma T Mutlu, Serkan F Celik, Ahmet S Guven
Here, we report a case of hemophilia A with myocarditis, encephalopathy, and spontaneous intramedullary hemorrhage. A 14-month-old male infant presented with loss of consciousness, generalized tonic-clonic convulsions, and cardiac failure. The neurological examination was normal. Myocarditis was diagnosed. After administration of fresh frozen plasma, the aPTT did not return to normal. The factor VIII (FVIII) level was 10.2% the normal level, and the patient was diagnosed with hemophilia A. The cerebrospinal fluid (CSF) evaluation was unremarkable, with the exception of elevated CSF protein levels...
December 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29234548/steroid-responsive-encephalopathy-associated-with-autoimmune-thyroiditis-presenting-with-fever-and-confusion
#8
Chiranthi Kongala Liyanage, Tilak Manthi Janake Munasinghe, Adsareswary Paramanantham
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) is a diagnostic conundrum as it may present with a myriad of nonspecific clinical features and laboratory and neuroimaging investigations are not diagnostic. We report a case of a 65-year-old female who presented with an acute febrile illness associated with headache and confusion, tangential thoughts, and loose association. Based on neutrophil leukocytosis in the full blood count and elevated inflammatory markers, she was commenced on empirical intravenous antibiotics suspecting meningoencephalitis...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/29203057/febrile-infection-related-epilepsy-syndrome-fires-with-super-refractory-status-epilepticus-revealing-autoimmune-encephalitis-due-to-gabaar-antibodies
#9
D Caputo, R Iorio, F Vigevano, L Fusco
BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. CASE STUDY: We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus...
November 24, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29103423/autoimmune-epilepsies
#10
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/29066789/identification-of-14-dehydroergosterol-as-a-novel-anti-inflammatory-compound-inducing-tolerogenic-dendritic-cells
#11
Yasuhisa Ano, Kumiko Ikado, Kazutoshi Shindo, Hideki Koizumi, Daisuke Fujiwara
Tolerogenic dendritic cells (DCs) have the ability to induce regulatory T cells and play an important role in preventing chronic inflammatory and autoimmune diseases. We have identified a novel compound, 14-dehydroergosterol, from Koji, a Japanese traditional food material fermented with fungi. 14-dehydroergosterol is an ergosterol analogue with a conjugated double bond, but the activity of 14-dehydroergosterol is much higher than that of ergosterol. 14-dehydroergosterol induces the conversion of murine bone marrow (BM)-derived DCs and differentiated DCs into tolerogenic DCs, in which the production of IL-12 is suppressed and that of IL-10 is increased...
October 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29044307/immune-reconstitution-inflammatory-syndrome-as-a-cause-of-autoimmune-hepatitis-and-acute-liver-failure
#12
Edison Moraes Rodrigues, Rogério Fernandes, Ruth Susin, Bárbara Fior
Acute liver failure is a rare syndrome with high mortality and is often diagnosed late. Intensivist physicians play fundamental roles in the diagnostic suspicion and the management of the multiple-organic dysfunctions characteristic of this entity. Immune reconstitution inflammatory syndrome is an entity that is characterized by the paradoxical worsening of the patient's previous condition, after the initiation of antiretrovirals, triggered against either pathogens present in the host or autoantigens. Autoimmune hepatitis has recently been described as one of these autoimmune manifestations...
July 2017: Revista Brasileira de Terapia Intensiva
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
#13
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/29042526/-differential-diagnosis-of-immune-mediated-encephalopathies-neurological-symptoms-of-diffuse-brain-damage-a-new-concept
#14
Yoshimitsu Maki, Hiroshi Takashima
In recent years, incidence of autoimmune encephalopathies has increased. The diagnosis of the severe form of autoimmune encephalopathy is not difficult; however, milder forms can be misdiagnosed as general encephalopathies. We often treat Hashimoto's encephalopathy, which has diverse clinical symptoms and is often misdiagnosed as a psychosomatic disease. We have found that the neurological findings and symptoms of patients with Hashimoto's encephalopathy are similar to those of psychogenic diseases, such as giveway weakness and atypical sensory disorder...
October 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28936074/syndromes-of-rapidly-progressive-cognitive-decline-our-experience
#15
Sadanandavalli Retnaswami Chandra, Lakshminarayanapuram Gopal Viswanathan, Anupama Ramakanth Pai, Rahul Wahatule, Suvarna Alladi
BACKGROUND: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. AIMS: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center...
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28926420/seronegative-paraneoplastic-limbic-encephalitis-associated-with-thymoma
#16
Jaime Toro, David Cuellar-Giraldo, Alejandra Duque, Karla Minota, Jorge Patiño, Manuel García
Paraneoplastic limbic encephalitis is an autoimmune syndrome characterized by the acute or subacute onset of encephalopathy, memory loss, confusion, temporal lobe seizures, and behavioral and mood changes. Although most patients with paraneoplastic limbic encephalitis have antineuronal antibodies, advances in the field now permit the diagnosis without autoantibody test results. In this case illustrating the new diagnostic criteria, we report a 70-year-old woman who was brought to the emergency room after the acute onset of cognitive impairment, altered mental status, and choreoathetoid movements...
September 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28918510/acquired-hepatocerebral-degeneration-ahd-a-peculiar-neurological-impairment-in-advanced-chronic-liver-disease
#17
A Pigoni, F Iuculano, C Saetti, L Airaghi, L Burdick, S Spreafico, M Curioni, R Lombardi, L Valenti, A L Fracanzani, S Fargion
We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyramidal signs, arisen in a period of few days. Brain Magnetic Resonance Imaging (MRI) showed, in T1-weighted images, diffuse bilateral hyper intensities in basal ganglia and biemispheric brain and cerebellar cortices, resembling paramagnetic deposits...
September 16, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#18
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28910681/the-imaging-spectrum-of-posterior-reversible-encephalopathy-syndrome-a-pictorial-review
#19
REVIEW
Emily Brady, Neal S Parikh, Babak B Navi, Ajay Gupta, Andrew D Schweitzer
Posterior reversible encephalopathy syndrome (PRES) is characterized by the acute onset of neurologic symptoms (headache, altered mental status, visual changes, seizures) with accompanying vasogenic edema on brain imaging. Risk factors for PRES include infection, uremia, malignancy, autoimmune disorders, the peripartum state and hypertension. PRES is classically described as being posterior (i.e. parieto-occipital) but radiologic variants are increasingly recognized. This pictorial review demonstrates the heterogeneity of the different radiologic presentations of PRES in reference to lesion distribution, hemorrhage, diffusion restriction, contrast enhancement, and other associated findings...
August 30, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#20
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
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