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Autoimmune encephalopathy

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https://www.readbyqxmd.com/read/27919115/febrile-infection-related-epilepsy-syndrome-clinical-review-and-hypotheses-of-epileptogenesis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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...
October 12, 2016: 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
#11
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
#12
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
https://www.readbyqxmd.com/read/27697033/recombinant-antibody-fragments-for-neurodegenerative-diseases
#13
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
https://www.readbyqxmd.com/read/27688922/delirious-mania-associated-with-autoimmune-gastrothyroidal-syndrome-of-a-mid-life-female-the-role-of-hashimoto-encephalopathy-and-a-3-year-follow-up-including-serum-autoantibody-levels
#14
Udo Bonnet, Claudia Selle, Ralf Kuhlmann
We report the case study of a 57-year-old Caucasian female with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), commonly termed Hashimoto encephalopathy (HE). This presentation includes one of the longest lasting follow-up studies of HE considering the neuropsychiatric symptoms (here delirium, mania, and EEG-slowing) and their relation to serum autoantibody levels. Antithyroid-peroxidase autoantibodies, the hallmark of autoimmune thyroiditis, were found in the serum and also in the cerebrospinal fluid...
2016: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/27672526/autoimmune-schizophrenia-psychiatric-manifestations-of-hashimoto-s-encephalitis
#15
Ali S Haider, Maryam Alam, Ebun Adetutu, Richa Thakur, Caleb Gottlich, Danielle L DeBacker, Lianne Marks
Hashimoto's encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity...
July 5, 2016: Curēus
https://www.readbyqxmd.com/read/27668357/efficacy-and-safety-of-rituximab-therapy-in-neuromyelitis-optica-spectrum-disorders-a-systematic-review-and-meta-analysis
#16
Valentina Damato, Amelia Evoli, Raffaele Iorio
Importance: Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune astrocytopathies characterized by predominant involvement of the optic nerves and spinal cord. In most patients, an IgG autoantibody binding to astrocytic aquaporin 4, the principal water channel of the central nervous system, is detected. Rituximab, a chimeric monoclonal antibody specific for the CD20 B-lymphocyte surface antigen, has been increasingly adopted as a first-line off-label treatment for patients with NMOSDs...
September 26, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27667488/-clinical-features-and-treatment-of-hashimoto-encephalopathy
#17
Yoshimitsu Maki, Hiroshi Takashima
Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27660052/c14orf132-gene-is-possibly-related-to-extremely-low-birth-weight
#18
Airi Tiirats, Triin Viltrop, Margit Nõukas, Ene Reimann, Andres Salumets, Sulev Kõks
BACKGROUND: Despite extensive research the genetic component of extremely low birth weight (ELBW) in newborns has remained obscure. RESULTS: The aim of the case study was to identify candidate gene(s) causing ELBW in newborns and hypotrophy in infants. A family of four was studied: mother, father and two ELBW-phenotype children. Studies were made of the medical conditions of the second child at birth and post-partum - peculiar phenotype, micro-anomalies, recurrent infections, suspicion of autoimmune hepatitis, multifactorial encephalopathy and suspected metabolic and chromosomal abnormalities...
2016: BMC Genetics
https://www.readbyqxmd.com/read/27652003/long-term-poor-rapport-lack-of-spontaneity-and-passive-social-withdrawal-related-to-acute-post-infectious-encephalitis-a-case-report
#19
Atsurou Yamada, Nobuhiro Miyachi, Toshiyasu Miura, Masako Suzuki, Norio Watanabe, Tatsuo Akechi
INTRODUCTION: Post-infectious encephalitis/encephalopathy is a neurological syndrome that sometimes develops following common viral or bacterial infections. The most common form is acute disseminated encephalomyelitis (ADEM). ADEM is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalitis. Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is another type of severe autoimmune disorder, characterized by seizures, movement disorders and psychiatric symptoms...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27639840/steroid-responsive-encephalopathy-associated-with-autoimmune-thyroiditis-sreat-characteristics-treatment-and-outcome-in-251-cases-from-the-literature
#20
Charlotte Laurent, Jean Capron, Bluenn Quillerou, Guy Thomas, Sonia Alamowitch, Olivier Fain, Arsène Mekinian
BACKGROUND: Steroid-responsive encephalopathy and associated autoimmune thyroiditis (SREAT) is characterized by encephalopathy and the presence of antithyroid antibodies. We describe the clinical presentation, outcome and treatments for SREAT by a systematic review of the literature. METHODS: MEDLINE via PubMed, Web of Science and the Cochrane Library were searched for articles published until 2015. Inclusion criteria were unexplained encephalopathy with antithyroid antibodies...
September 15, 2016: Autoimmunity Reviews
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