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Autoimmune encephalopathy

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https://www.readbyqxmd.com/read/28101487/immunoglobulin-g4-related-thyroid-diseases
#1
REVIEW
Dulani Kottahachchi, Duncan J Topliss
Immunoglobulin G4-related disease (IgG4-RD) is a new disease category involving many organ systems, including the endocrine system in general and the thyroid in particular. Since an initial association was made between hypothyroidism and autoimmune (IgG4-related) pancreatitis, more forms of IgG4-related thyroid disease (IgG4-RTD) have been recognized. Four subcategories of IgG4-RTD have so far been identified: Riedel thyroiditis (RT), fibrosing variant of Hashimoto thyroiditis (FVHT), IgG4-related Hashimoto thyroiditis, and Graves disease with elevated IgG4 levels...
December 2016: European Thyroid Journal
https://www.readbyqxmd.com/read/28094021/neuropsychological-characteristics-of-encephalopathy-in-susac-s-syndrome-case-report
#2
Magdalena Roessler-Górecka, Tadeusz Mendel, Justyna Wiśniowska, Joanna Seniów
Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning...
January 10, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28092152/issues-in-nutrition-dietary-considerations-in-select-chronic-conditions
#3
Margaret E Thompson, Mary Barth Noel
Chronic diseases that affect the gastrointestinal tract also tend to affect nutrition. The incidence of chronic liver disease is increasing. As the prevalence of obesity rises, so do the incidences of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis. Patients with chronic liver disease usually have some degree of malnutrition. In the absence of encephalopathy, patients with chronic liver disease should consume more protein than that in the average diet. There is some controversy about whether diet plays a role in the development of inflammatory bowel disease...
January 2017: FP Essentials
https://www.readbyqxmd.com/read/28086912/wwl70-attenuates-pge2-production-derived-from-2-arachidonoylglycerol-in-microglia-by-abhd6-independent-mechanism
#4
Mikiei Tanaka, Sean Moran, Jie Wen, Kwame Affram, Tinghua Chen, Aviva J Symes, Yumin Zhang
BACKGROUND: α/β-Hydrolase domain 6 (ABHD6) is one of the major enzymes for endocannabinoid 2-arachidonoylglycerol (2-AG) hydrolysis in microglia cells. Our recent studies have shown that a selective ABHD6 inhibitor WWL70 has anti-inflammatory and neuroprotective effects in animal models of traumatic brain injury and multiple sclerosis. However, the role of ABHD6 in the neuroinflammatory response and the mechanisms by which WWL70 suppresses inflammation has not yet been elucidated in reactive microglia...
January 10, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28054130/posterior-reversible-encephalopathy-syndrome
#5
REVIEW
Marlene Fischer, Erich Schmutzhard
The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a majority of patients the clinical presentation includes elevated arterial blood pressure up to hypertensive emergencies. Neuroimaging, in particular magnetic resonance imaging, frequently shows a distinctive parieto-occipital pattern with a symmetric distribution of changes reflecting vasogenic edema...
January 4, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28009770/fatal-morvan-syndrome-associated-with-myasthenia-gravis
#6
Madhu Nagappa, Anita Mahadevan, Sanjib Sinha, Parayil S Bindu, Pavagada S Mathuranath, Cheminikara Bineesh, Rose D Bharath, Arun B Taly
INTRODUCTION: Morvan syndrome is a rare and complex autoimmune disorder affecting multiple sites of neuraxis. CASE REPORT: We present fulminant Morvan syndrome, developing on a background of chronic myasthenia gravis. A 54-year-old gentleman presented with fluctuating ophthalmoplegia and proximal muscles weakness of 7 years duration that remitted with pyridostigmine and prednisolone. He developed insomnia of 2 months duration, worsening of myasthenic symptoms and respiratory distress, dysautonomia, encephalopathy, and peripheral nerve hyperexcitability...
January 2017: Neurologist
https://www.readbyqxmd.com/read/28009765/autoimmune-encephalitides-a-broadening-field-of-treatable-conditions
#7
Bernadette Kalman
BACKGROUND: Neurology has been continuously transforming by the refinement of molecular diagnostics and the development of disease-modifying treatments. The discovery of new antibody markers has elucidated the pathogenesis, provided the means of diagnostics, and offered cure or treatment for several immune-mediated neurological and neuropsychiatric disorders. The identification of pathogenic and marker autoantibodies has also facilitated defining the associated phenotypic spectra and the overlap among the phenotypes linked to individual immune markers...
January 2017: Neurologist
https://www.readbyqxmd.com/read/27993886/acquired-dysfibrinogenemia-caused-by-autoantibody-inhibiting-fibrin-polymerization-in-a-patient-with-melas-syndrome-and-bleeding-tendency
#8
Nuri Lee, Ji-Eun Kim, Hyun Ju Yoo, JaYoon Gu, Hyori Kim, Junho Chung, Youngil Koh, Hyun Kyung Kim
We present a case of acquired dysfibrinogenemia caused by an autoantibody that inhibited fibrin polymerization in a patient previously diagnosed with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes). The patient showed prolonged PT, aPTT, and thrombin time. There was no factor deficiency but fibrinogen antigen and activity were decreased. ELISA for detection of fibrinogen antibodies were performed and IgG purified from the patient's plasma bound to fibrinogen more strongly than did control IgG, indicating the presence of a fibrinogen-specific antibody...
December 2016: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/27919115/febrile-infection-related-epilepsy-syndrome-clinical-review-and-hypotheses-of-epileptogenesis
#9
Andreas van Baalen, Annamaria Vezzani, Martin Häusler, Gerhard Kluger
Febrile infection-related epilepsy syndrome (FIRES, AERRPS, or DESC) is one of the most severe, mostly irreversible, and presumably immune-mediated epileptic encephalopathies affecting healthy children. Refractory status epilepticus or a cluster of seizures start a few days after the onset of an acute febrile illness; however, encephalitis cannot be proved. Sequelae of FIRES are drug-resistant epilepsy and neuropsychological impairments occurring without latency. Clinical knowledge is limited because FIRES is sporadic and extremely rare...
December 5, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27917700/posterior-reversible-encephalopathy-syndrome-following-elevated-mean-arterial-pressures-for-cervical-spinal-cord-injury
#10
Jeffrey H Zimering, Addisu Mesfin
BACKGROUND: Increasing the mean arterial pressure (MAP) is an accepted treatment modality to minimize the risk for irreversible neurologic damage secondary to spinal cord ischemia. Posterior reversible encephalopathy syndrome (PRES) is a rare complication occurring after transplantation surgery, in persons having an autoimmune disorder or after abrupt increases in blood pressure of various etiologies. STUDY DESIGN: Case report. METHODS: Retrospective evaluation of medical records...
December 5, 2016: Journal of Spinal Cord Medicine
https://www.readbyqxmd.com/read/27854095/cerebral-venous-sinus-thrombosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-with-atypical-antibodies-a-case-report
#11
Tsung-Han Ho, Yu-Wei Hsu, Chih-Wei Wang, Jiunn-Tay Lee, Chi-Hsin Ting, Fu-Chi Yang
BACKGROUND: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare. CASE REPORT: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait...
June 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/27852413/-clinical-analysis-of-acute-disseminated-encephalomyelitis-in-44-cases
#12
X N Zhong, B J Zhang, Y G Wang, Y L Huang, Y Q Shu, Z Z Lu, X Q Hu, W Qiu
Objective: To investigate the clinical features in 44 patients with acute disseminated encephalomyelitis (ADEM). Methods: Consecutive ADEM patients admitted to Neurology Department of the Third Affiliated Hospital of Sun yat-sen University during August 2009 to July 2014 were enrolled.Clinical and laboratory data of the patients were reviewed and analyzed. Results: Forty-four patients with ADEM based on the 2012 criteria were recruited, including 23 male and 21 female; 9 children, 11 teenagers and 24 adults...
October 25, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27843471/brain-on-fires-super-refractory-seizure-in-a-7-yr-old-boy
#13
Alireza Tavasoli, Behdad Gharib, Houman Alizadeh, Hossein Farshadmoghaddam, Sara Memarian, Mahmoodreza Ashrafi, Meisam Sharifzade
We present a 7 yr old boy afflicted with super-refractory seizure that responded poorly to antiepileptic drugs and sustained a long course of hospitalization and complications of high doses of medications as well as longstanding stay in hospital. The differential diagnoses were, fever-induced refractory epileptic encephalopathy (FIRES), and infectious and autoimmune encephalitis. However, work-ups had not revealed any evidence of any specific diagnosis, so we assumed that he was afflicted by viral infectious encephalitis as he had, fever, vomiting, and prodromal symptoms of infectious (most probably viral) disease prior to onset of the seizure attacks...
2016: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/27832476/neurological-and-neuropsychiatric-adverse-effects-of-dermatologic-medications
#14
REVIEW
Melinda Liu, Yuan Yu M Huang, Sylvia Hsu, Joseph S Kass
Severe, recalcitrant dermatologic conditions often require systemic treatment. Although efficacious, these medications have been associated with wide-ranging adverse reactions. Some are reversible, predictable, and either dose-dependent or treatment length-dependent, while others are unpredictable, irreversible, and potentially fatal. This review examines the neuropsychiatric adverse effects associated with US FDA-approved medications for treatment of the following dermatologic pathologies that typically require systemic therapy: autoimmune dermatoses, acne, psoriasis, and melanoma...
November 10, 2016: CNS Drugs
https://www.readbyqxmd.com/read/27761227/the-chronic-encephalopathy-of-parry-romberg-syndrome-and-en-coupe-de-sabre-with-a-31-year-history-in-a-west-indian-woman-clinical-immunologic-and-neuroimaging-abnormalities
#15
Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...
September 30, 2016: Neurology International
https://www.readbyqxmd.com/read/27749626/acute-liver-failure-outcome-and-value-of-pediatric-end-stage-liver-disease-score-in-pediatric-cases
#16
Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27747458/acute-on-chronic-liver-failure
#17
REVIEW
Shiv Kumar Sarin, Ashok Choudhury
Acute-on-chronic liver failure (ACLF) is a distinct entity that differs from acute liver failure and decompensated cirrhosis in timing, presence of treatable acute precipitant, and course of disease, with a potential for self-recovery. The core concept is acute deterioration of existing liver function in a patient of chronic liver disease with or without cirrhosis in response to an acute insult. The insult should be a hepatic one and presentation in the form of liver failure (jaundice, encephalopathy, coagulopathy, ascites) with or without extrahepatic organ failure in a defined time frame...
December 2016: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/27743761/hashimoto-s-encephalopathy-mimicking-creutzfeldt-jakob-disease
#18
Angela C Gauthier, Joachim M Baehring
Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved...
January 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27734702/-psycho-immuno-endocrinology-of-the-thyroid-gland
#19
Ivan Šterzl, Karolína Absolonová, Petr Matucha
Historically endocrinologists and psychiatrists are aware that disturbances in thyroid disease in beginning or even in clinically intensified states of thyrotoxicosis or hypothyroidism exhibit pathological mental manifestations, masking or potentiating the underlying disease. Immune system disorders cause thyroid organ-specific autoimmune process. This autoimmune thyroid disease binds with a number of disorders in both endocrine or non-endocrine organs. This appears in vascular, neurological, skin, connective tissue, gastrointestinal tract and mental pathology...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27726296/posterior-reversible-encephalopathy-syndrome-mimicking-brain-metastases-in-a-patient-with-metastatic-transitional-cell-carcinoma
#20
Kelsey Wong, Margaret Lee, Ian D Davis, Phillip Parente, Joseph Mckendrick, Carmel Pezaro
Posterior reversible encephalopathy syndrome (PRES) has been described in the context of uncontrolled hypertension, eclampsia, renal disease and autoimmune conditions, or in patients treated with chemotherapy or immunosuppressive agents. In contrast, we report the occurrence of PRES in a patient with untreated metastatic transitional cell carcinoma. The case emphasizes important diagnostic challenges associated with atypical presentations without "typical" risk factors and the limitations of common diagnostic imaging modalities...
October 11, 2016: Asia-Pacific Journal of Clinical Oncology
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