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Autoimmune encephalopathy

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https://www.readbyqxmd.com/read/28292360/acute-on-chronic-liver-failure-causes-clinical-characteristics-and-predictors-of-mortality
#1
Abbas Ali Tasneem, Nasir Hassan Luck
OBJECTIVE: To determine the causes, characteristics and predictors of mortality in patients with acute-on-chronic liver failure (ACLF). STUDY DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, from July 2014 to June 2016. METHODOLOGY: All patients with acute-on-chronic liver disease (ACLD) with ages > 12 were included...
January 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28289809/imaging-characteristics-associated-with-clinical-outcomes-in-posterior-reversible-encephalopathy-syndrome
#2
Andrew D Schweitzer, Neal S Parikh, Gulce Askin, Ajay Nemade, John Lyo, Sasan Karimi, Anna Knobel, Babak B Navi, Robert J Young, Ajay Gupta
PURPOSE: Posterior reversible encephalopathy syndrome (PRES) is a disorder of cerebrovascular autoregulation that can result in brain edema, hemorrhage, and infarction. We sought to investigate whether certain imaging characteristics in PRES are associated with clinically significant patient outcomes. METHODS: We retrospectively reviewed all cases of PRES occurring between 2008 and 2014 at two major academic medical centers. Demographic, clinical, and radiographic data were collected...
March 13, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28287071/auditory-seizures-in-autoimmune-epilepsy-a-case-with-anti-thyroid-antibodies
#3
Claudia Varrasi, Domizia Vecchio, Luca Magistrelli, Gionata Strigaro, Laura Tassi, Roberto Cantello
In its classic presentation, Hashimoto's encephalopathy is an acute-subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti-thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non-convulsive status epilepticus...
March 8, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28283100/antecedent-anti-nmda-receptor-encephalitis-in-two-patients-with-multiple-sclerosis
#4
A Baheerathan, W J Brownlee, D T Chard, K Shields, R Gregory, S A Trip
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD). In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28282627/anti-nmdar-encephalitis-followed-by-seropositive-neuromyelitis-optica-spectrum-disorder-a-case-report-and-literature-review
#5
REVIEW
Ye Ran, Lu Wang, Fangfang Zhang, Ran Ao, Zhao Dong, Shengyuan Yu
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory central nervous system syndrome, and encephalitis associated with anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is an autoimmune encephalopathy. A patient with both diseases, separately or simultaneously, is rare as a clinical phenomenon, but cannot be ignored. We report the clinical characteristics and imaging features of a special case with anti-NMDAR encephalitis followed by NMOSD. We subsequently reviewed the English language literature about demyelinating disorders with anti-NMDAR encephalitis...
February 27, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28272206/limbic-encephalitis-associated-with-anti-nh2-terminal-of-%C3%AE-enolase-antibodies-a-clinical-subtype-of-hashimoto-encephalopathy
#6
Toru Kishitani, Akiko Matsunaga, Masamichi Ikawa, Kouji Hayashi, Osamu Yamamura, Tadanori Hamano, Osamu Watanabe, Keiko Tanaka, Yasunari Nakamoto, Makoto Yoneda
Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagnosed with HE and respond to immunotherapy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28272079/natural-history-and-treatment-outcomes-of-severe-autoimmune-hepatitis
#7
Nikhil Sonthalia, Pravin M Rathi, Samit S Jain, Ravindra G Surude, Ashok R Mohite, Sunil V Pawar, Qais Contractor
GOALS: The aim of this study was to analyze the natural history and treatment outcomes of autoimmune hepatitis (AIH) variants presenting with severe-AIH. BACKGROUND: Severe acute presentation is an uncommon manifestation of AIH, and it remains poorly characterized. MATERIALS AND METHODS: We included 101 patients with AIH from January 2011 to December 2015. Patients were classified as seropositive-AIH and seronegative-AIH. Patients with acute liver failure, acute-on-chronic liver failure, and severe acute hepatitis were defined as severe-AIH patients...
March 7, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28260460/sirolimus-induced-combined-posterior-reversible-encephalopathy-syndrome-and-lymphocytic-pneumonitis-in-a-renal-transplant-recipient-case-report-and-review-of-the-literature
#8
Osama Gheith, Magdalena Cerna, Medhat A Halim, Narayanam Nampoory, Torki Al-Otaibi, Prasad Nair, Tarek Said, Hassanein Abo Atteya, Kenneth Katchy
The mammalian target of rapamycin inhibitor sirolimus was introduced into clinical transplant practice in 1999. Dose-related myelosuppression and hyper lipidemia are the most common adverse effects. Pulmonary toxicity has been reported since 2004 and can cause interstitial pneumonitis, organizing pneumonia, and alveolar hemorrhage. Moreover, it can occasionally induce posterior reversible encephalopathy syndrome, as documented in scarce reports. To our knowledge; this is the 1st report of combined posterior reversible encephalopathy syndrome and lymphocytic pneumonitis to be induced by sirolimus...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28238523/posterior-reversible-encephalopathy-syndrome-pres-with-sub-arachnoid-haemorrhage-after-bevacizumab-and-5-fu
#9
Jennifer Massey
PRES is a neurological syndrome characterised by reversible subcortical vasogenic brain oedema in patients with acute neurological signs and symptoms. It occurs in the context of fluctuations in blood pressure, renal failure, autoimmune disorders, eclamptic syndromes and with use of cytotoxic drugs. We present the case of a 60year old female with advanced bowel cancer who was admitted with seizures and sub-arachnoid haemorrhage, with radiographic features of PRES, shortly after receiving bevacizumab (Avastin), a VEGF-inhibitor...
February 23, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28203468/nonconvulsive-status-epilepticus-resembling-clinical-absence-with-atypical-eeg-pattern
#10
Channaiah Srikanth Mysore, Najib Murr, Rana Zabad, John Bertoni
Objective. We are reporting two cases: a patient with steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and another patient with secondary progressive multiple sclerosis (SPMS), both presenting with altered mental status (AMS) and later diagnosed with nonconvulsive atypical absence status epilepticus (AS), with atypical EEG changes. Methods. A report of two cases. Results. A patient with history of SREAT and the other with SPMS had multiple admissions due to AMS. For both, EEG revealed the presence of a high voltage generalized sharply contoured theta activity...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28202821/-pres-posterior-reversible-encephalopathy-syndrome
#11
Kouichirou Okamoto, Kunio Motohashi, Hidemoto Fujiwara, Tomohiko Ishihara, Itaru Ninomiya, Osamu Onodera, Yukihiko Fujii
Posterior reversible encephalopathy syndrome (PRES) is suggested in patients with acute neurological symptoms in the appropriate clinical context, including acute hypertension, blood pressure fluctuations, renal failure, blood transfusion, immunosuppression, autoimmune disorders, and eclampsia. PRES is a clinical syndrome, and refers to a disorder with reversible subcortical vasogenic brain edema caused by endothelial dysfunction, predominantly involving the bilateral parieto-occipital regions. Although the clinical course and prognosis are favorable in most cases, intracranial hemorrhage and/or restricted diffusion similar to acute infarction could be seen in some lesions on brain magnetic resonance imaging (MRI)...
February 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28190431/posterior-reversible-encephalopathy-in-the-intensive-care-unit
#12
M Toledano, J E Fugate
Posterior reversible encephalopathy syndrome (PRES) is increasingly diagnosed in the emergency department, and medical and surgical intensive care units. PRES is characterized by acute onset of neurologic symptoms in the setting of blood pressure fluctuations, eclampsia, autoimmune disease, transplantation, renal failure, or exposure to immunosuppressive or cytotoxic drugs, triggers known to admit patients to the intensive care unit (ICU). Although the exact pathophysiology remains unknown, there is growing consensus that PRES results from endothelial dysfunction...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28183838/autoimmune-encephalitis-pathophysiology-and-imaging-review-of-an-overlooked-diagnosis
#13
REVIEW
B P Kelley, S C Patel, H L Marin, J J Corrigan, P D Mitsias, B Griffith
Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteristic findings within limbic structures suggestive of autoimmune encephalitis can be a key step in alerting clinicians to the potential diagnosis and ensuring a prompt and appropriate clinical work-up...
February 9, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28162871/a-young-female-with-urinary-retention-a-case-report-of-hashimoto-s-encephalopathy
#14
Benjamin L Cooper, Shane E Appel, Hussam M Ammar
Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis in which patients experience cognitive impairment and various neurologic symptoms. We present a case of a young female that presented to the emergency department with urinary retention, and was ultimately diagnosed with HE. Examination was significant for direction-changing and vertical nystagmus (direction-changing nystagmus describes a phenomenon where the fast beat changes with the direction of gaze), hyperreflexia, clonus, and Babinski and Hoffman's reflexes (all upper motor neuron (UMN) signs)...
January 26, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28144796/clinical-predictors-and-differential-diagnosis-of-posterior-reversible-encephalopathy-syndrome
#15
Laetitia Della Faille, S Fieuws, W Van Paesschen
The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914-925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or other focal neurological signs that appear in the clinical setting of risk factors such as hypertension/blood pressure fluctuations, chemotherapy, renal failure, autoimmune disorders, or eclampsia, in whom imaging of the brain was performed to exclude PRES...
January 31, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28127211/posterior-reversible-encephalopathy-syndrome-in-alcoholic-hepatitis-hepatic-encephalopathy-a-common-theme
#16
Elizabeth S John, Ramy Sedhom, Ishita Dalal, Ranita Sharma
Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiologic diagnosis that has become more widely recognized and reported over the past few decades. As such, there are a number of known risk factors that contribute to the development of this syndrome, including volatile blood pressures, renal failure, cytotoxic drugs, autoimmune disorders, pre-eclampsia, and eclampsia. This report documents the first reported case of PRES in a patient with severe alcoholic hepatitis with hepatic encephalopathy and delves into a molecular pathophysiology of the syndrome...
January 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28101487/immunoglobulin-g4-related-thyroid-diseases
#17
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
#18
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
#19
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
#20
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
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