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Autoimmune encephalopathy

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https://www.readbyqxmd.com/read/28770118/new-onset-insomnia-in-a-pediatric-patient-a-case-of-anti-nmda-receptor-encephalitis
#1
Tamar N Goldberg, Michael F Cellucci
Anti-NMDAR encephalitis is becoming more widely recognized as a cause of encephalopathy in both adults and children. Certain clinical features such as mood lability, movement disorders, speech dysfunction, seizures, and autonomic instability in a pediatric patient should prompt immediate concern and evaluation for autoimmune encephalitis among providers. We present the case of a pediatric patient with anti-NMDAR encephalitis in which the symptom prompting medical evaluation was insomnia. Insomnia has not previously been emphasized in the literature as a presenting feature of this disease in children and has a broad differential...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28756176/infection-mediated-autoimmune-movement-disorders
#2
Alberto Cucca, Hamzeh A Migdadi, Alessandro Di Rocco
Infectious diseases remain the most common cause of neurological disability in the world. A number of movement disorders can develop in adults and children in response to infections. These can occur in isolation or as part of a broader neurological illness, with movement abnormalities consequent to an encephalopathy or a broader brain dysfunction. While most infection-related movement disorders are direct consequences of an active infectious process affecting cerebral structures implied in the motor network, at times a delayed immune-mediated process in response to a previous infectious is responsible for the neurological dysfunction...
July 21, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28710237/focal-neurological-presentation-in-hashimoto-s-encephalopathy-mimicking-a-vascular-occlusion-of-the-middle-cerebral-artery
#3
Ahmad Alazzeh, Sarah Jaroudi, Matthew Gooch, Alan N Peiris
Hashimoto's encephalopathy is a rare form of autoimmune encephalopathy. It is likely an underdiagnosed entity especially in the setting of focal neurological defects. We report a case of a 49-year-old man who presented with "strange behavior"of a day's duration. Examination was significant only for lethargy, poor attention span and agitation. Routine labs revealed leucocytosis. Head CT scan was unremarkable. Lumbar puncture showed high white blood cells with lymphocytosis and elevated protein level. The patient was empirically treated for meningitis without improvement...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28682567/-pres-posterior-reversible-encephalopathy-syndrome-potential-complication-of-hypertensive-crisis-case-report-and-literature-review
#4
Michele Vergura, Michele Prencipe, Antonio Maria Del Giudice, Rachele Grifa, Filomena Miscio, Anna Maria Pennelli, Teresa Popolizio, Anna Simeone, Mariangela Ferrara, Maurizio Leone, Filippo Aucella
Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome, usually reversible, characterized by vasogenic oedema in cerebral posterior regions in patients with autoimmune diseases, nephropathies, hypertensive crisis, eclampsia and exposure to cytotoxic drugs. The main symptoms are: headache, nausea, vomiting, seizures, visual disturbance and altered consciousness. Complications as cerebral hemorrhage and recurrences are rare. We describe a case of a 65 years old woman, affected by chronic kidney disease, recently exacerbated, diabetes and hypertension in treatment, who showed an heterogeneous clinical presentation with vomiting, headache, blurred vision and impaired consciousness during an episode of acute hypertension...
April 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#5
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28644255/periodic-paralysis-and-encephalopathy-as-initial-manifestations-of-graves-disease-case-report-and-review-of-the-literature
#6
Theocharis Tsironis, Athanasios Tychalas, Dimitrios Kiourtidis, Jannis Kountouras, Georgia Xiromerisiou, Jobst Rudolf, Georgia Deretzi
BACKGROUND: Thyrotoxic periodic paralysis (TPP) is an uncommon complication of Graves' disease, characterized by the triad of acute hypokalemia without total body potassium deficit, episodic muscle paralysis, and thyrotoxicosis. Graves' encephalopathy is an extremely rare form of encephalopathy associated with autoimmune thyroid disease (EAATD), characterized by neuropsychiatric symptoms, increased antithyroid antibodies and cerebrospinal fluid protein concentration, nonspecific electroencephalogram abnormalities, and cortico-responsiveness...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#7
Hussein Algahtani, Bader Shirah, Muhammad Amin, Eyad Altarazi, Hashem Almarzouki
Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28641772/susac-syndrome-a-differential-diagnosis-of-white-matter-lesions
#8
Mariano Marrodan, Jorge Correale, Lucas Alessandro, Mariela Amaya, Maria Eugenia Fracaro, Alejandro Alfredo Köhler, Marcela Fiol
INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28628018/autoimmune-limbic-encephalitis-case-reports
#9
K Kobaidze, A Harrison, Y Burklin, V Patidar, M Riccardi
Limbic encephalitis (LE) is an autoimmune or paraneoplastic disease that affects the medial temporal lobes. The patient will usually present with cognitive impairment, psychiatric changes, and seizures. Autoimmune limbic encephalitis (LE) is a challenging diagnosis as it is not always included in the typical paraneoplastic/autoimmune panels. Anti-GAD antibodies are associated with various disease including type I diabetes mellitus, various autoimmune processes, some neoplastic and infectious diseases. Thus, it is not as specific as some of the antibodies causing LE...
May 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28607558/hashimoto-s-encephalopathy-presenting-with-unusual-behavioural-disturbances-in-an-adolescent-girl
#10
Murugan Selvaraj Karthik, Kulothungan Nandhini, Viswanath Subashini, Ramasamy Balakrishnan
Hashimoto's encephalopathy (HE) is a rare autoimmune disorder with neurological and neuropsychiatric manifestations and elevated titres of anti-thyroid antibodies. Here we are reporting a case of HE in a 19-year-old girl who presented with seizure-like episodes, confusion, and behavioural disturbances with catatonic symptoms such as posturing, echopraxia, echolalia, and ambivalence. Patient did not respond to antipsychotics and anticonvulsants. On further investigation, patient was found to have high serum anti-TPO antibodies of about 1261 U/mL with euthyroid status, which supported a suspicion of HE...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28584608/super-refractory-status-epilepticus-in-hashimoto-s-encephalopathy
#11
Mujahid Al-Busaidi, Jyoti Burad, Asma Al-Belushi, Arun Gujjar
We present a case of a 38-year-old woman who was at eight weeks of gestation and was admitted to Sultan Qaboos University Hospital with refractory status epilepticus (SE). She presented with a two-day history of fever and a depressed level of consciousness that was followed with generalized tonic-clonic seizures. She progressed to refractory SE that required intubation and mechanical ventilation. Autoimmune workup was suggestive of Hashimoto's encephalopathy (HE) as suggested by the high levels of thyroid antibodies...
May 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28572051/late-onset-rasmussen-encephalitis-a-literature-appraisal
#12
REVIEW
Sophie Dupont, Ana Gales, Serge Sammey, Marie Vidailhet, Virginie Lambrecq
Rasmussen Encephalitis (RE) is classically described as a childhood encephalopathy due to a unilateral inflammation of the cerebral cortex with a presumed immune-mediated pathophysiological basis. Unusual variant forms, including adolescent and adult-onset RE have been described but there is still a doubt whether these atypical cases correspond to classical RE patients. To review evidence, a systematic PubMed search was conducted to retrieve papers addressing late onset RE to assess (i) the positivity rate of classical childhood-onset diagnostic criteria for RE in late-onset RE, (ii) the specific clinical and radiological features that could help earlier diagnosis and therapeutic interventions, (iii) the arguments for an autoimmune pathophysiology including (iiia) the association with autoimmune markers or diseases and (iiib) the effects of immunomodulatory or immunosuppressive treatments...
May 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28571184/the-importance-of-liver-fatty-acid-binding-protein-in-diagnosis-of-liver-damage-in-patients-with-acute-hepatitis
#13
Ozlem Ozer Cakir, Aysun Toker, Huseyin Ataseven, Ali Demir, Hakki Polat
INTRODUCTION: Acute hepatitis is acute inflammation of liver elicited by a large number of causes. It sometimes spontaneously recovers, sometimes may progress to chronic hepatitis. Liver- Fatty Acid Binding Protein (L-FABP) is a small protein that is abundant in hepatocytes, and which binds most of the long-chain fatty acids present in the cytosol. AIM: The present study was aimed to investigate the levels of serum and urine L-FABP in acute hepatitis and diagnostic value of serum and urine L-FABP levels in patients with acute hepatitis...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28570330/neurologic-complications-of-transplantation
#14
Amy A Pruitt
PURPOSE OF REVIEW: This article describes the diagnosis and management of neurologic problems during hematopoietic cell and solid organ transplantation using time elapsed since transplantation as a guide to expected complications, including drug toxicities, infections, strokes, autoimmune phenomena, disease recurrence, and secondary neoplasms. RECENT FINDINGS: Growing clinical experience in the neurology of transplantation has led to appreciation of the diverse clinical and radiographic spectrum of calcineurin inhibitor-related posterior reversible encephalopathy syndrome (PRES) and progressive multifocal leukoencephalopathy...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28570327/gastroenterology-and-neurology
#15
Ronald F Pfeiffer
PURPOSE OF REVIEW: Just as gastrointestinal dysfunction may develop in the setting of neurologic disease, neurologic dysfunction may become evident in the setting of gastrointestinal disease. This article describes the range of neurologic features that have been described in three primary gastrointestinal diseases: celiac disease and gluten-related disorders, inflammatory bowel disease, and Whipple disease. Particular emphasis is placed on the controversial and evolving clinical picture of neurologic dysfunction in disorders of gluten sensitivity...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28556688/severe-and-rapidly-progressive-lafora-disease-associated-with-nhlrc1-mutation-a-case-report
#16
Sara Casciato, Stefano Gambardella, Addolorata Mascia, Pier Paolo Quarato, Alfredo D'Aniello, Yana Ackurina, Veronica Albano, Francesco Fornai, Simona Scala, Giancarlo Di Gennaro
Lafora disease (LD), also known as progressive myoclonic epilepsy-2 (EPM2), is a rare, fatal autosomal recessive disorder typically starting during adolescence in otherwise neurologically normal individuals. It is clinically characterized by insidious of progressive neurological features including seizures, action myoclonus, visual hallucination, ataxia and dementia. Mutations in the laforin (EPM2A) gene on chromosome 6q24 or in the malin gene (NHLRC1) on chromosome 6p22 are responsible of LD phenotype. Diagnostic workup includes genetic analysis as well as axillary skin biopsy with evidence of typical periodic acid-Schiff (PAS)-positive polyglucosan inclusion bodies (Lafora bodies) in the apocrine glands and/or in the eccrine duct...
June 12, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28555833/predictive-models-in-the-diagnosis-and-treatment-of-autoimmune-epilepsy
#17
Divyanshu Dubey, Jaysingh Singh, Jeffrey W Britton, Sean J Pittock, Eoin P Flanagan, Vanda A Lennon, Jan-Mendelt Tillema, Elaine Wirrell, Cheolsu Shin, Elson So, Gregory D Cascino, Dean M Wingerchuk, Matthew T Hoerth, Jerry J Shih, Katherine C Nickels, Andrew McKeon
OBJECTIVE: To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy. METHODS: We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28545782/autoimmune-encephalopathy-for-psychiatrists-when-to-suspect-autoimmunity-and-what-to-do-next
#18
REVIEW
Mark Oldham
OBJECTIVE: To provide a critical review of autoimmune encephalopathy-broadly defined as neuropsychiatric features directly related to an autoimmune process-relevant for psychiatric practice. METHODS: We consulted rheumatology textbooks to define the scope of autoimmune conditions and identified recent reviews of rheumatic conditions, autoimmune vasculitis, and autoimmune encephalitis. We integrated these with primary reports to provide a clinically relevant overview of autoimmune encephalopathy...
May 2017: Psychosomatics
https://www.readbyqxmd.com/read/28524218/-autoimmune-pathology-in-neuropaediatrics-what-aspects-have-really-undergone-a-change
#19
V Cantarin-Extremera
Autoimmune neurological diseases are an important group of pathologies in neuropaediatrics. The central nervous system was considered to be an immunologically privileged organ, as it protects itself from the surroundings by means of the blood-brain barrier. In recent years, however, reports have been published of a growing number of disorders produced by antibodies that react against neuronal or glial proteins and give rise to a wide variety of clinical conditions (epilepsy, encephalopathy, movement disorders, etc...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28495438/african-americans-have-a-lower-prevalence-of-portal-vein-thrombosis-at-the-time-of-liver-transplantation
#20
Dmitri Bezinover, Ethan Reeder, Faisal Aziz, Fuat Saner, Patrick McQuillan, Zakiyah Kadry, Thomas Riley, Dmitri Guvakov, Piotr K Janicki
BACKGROUND: Perioperative vascular thrombotic events in patients undergoing liver transplantation (LT) are associated with significant morbidity and mortality. METHODS: In this retrospective UNOS database analysis, we evaluated the prevalence of portal vein thrombosis (PVT) and factors contributing to PVT development in different ethnic groups. RESULTS: Of the 47 953 LT performed between 2002 and 2015, we identified 3642 cases of PVT. African Americans (AA) had a significantly lower prevalence of PVT compared to other ethnic groups (p = 0...
May 8, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
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