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https://www.readbyqxmd.com/read/28684941/-autoimmune-epilepsy-encephalitis-with-autoantibodies-for-epileptologists
#1
Christian G Bien, Martin Holtkamp
Autoimmune encephalitides may account for epilepsies of so far unknown cause. These "autoimmune epilepsies" may respond well to immunotherapy. More than a dozen autoantibodies have been found with this constellation; therefore, broad autoantibody testing of serum-CSF pairs offers the best diagnostic yield. Several particular features raise the suspicion of an autoimmune cause in otherwise unexplained seizure disorders.
May 2017: Epilepsy Currents
https://www.readbyqxmd.com/read/28682567/-pres-posterior-reversible-encephalopathy-syndrome-potential-complication-of-hypertensive-crisis-case-report-and-literature-review
#2
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/28681401/prevalence-and-outcome-of-late-onset-seizures-due-to-autoimmune-etiology-a-prospective-observational-population-based-cohort-study
#3
Felix von Podewils, Marie Suesse, Julia Geithner, Bernadette Gaida, Zhong I Wang, Julia Lange, Alexander Dressel, Matthias Grothe, Christof Kessler, Soenke Langner, Uwe Runge, Christian G Bien
OBJECTIVE: The increasing incidence of new-onset seizures with age is well known. Often, the etiology cannot be clarified. In the present study, patients with unprovoked late-onset seizures and without known neoplasm, who might have had paraneoplastic encephalitis, were investigated for a potentially underlying autoimmunity. METHODS: Sixty-six consecutive patients (36 women; aged ≥55 years) after having at least one seizure or seizures for ≤6 months were prospectively identified over a period of 4...
July 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28680914/antiepileptic-drug-therapy-in-patients-with-autoimmune-epilepsy
#4
Anteneh M Feyissa, A Sebastian López Chiriboga, Jeffrey W Britton
OBJECTIVE: We aimed to report the pattern of usage and efficacy of antiepileptic drugs (AEDs) in patients with autoimmune epilepsy (AE). METHODS: We retrospectively studied the Mayo Clinic's electronic medical record of patients with AE in which seizures were the main presenting feature. Clinical data, including demographics, seizure characteristics, type of AED and immunotherapy used, presence of neural antibody, and treatment outcomes, were reviewed. RESULTS: The medical records of 252 adult patients diagnosed with autoimmune encephalitis and paraneoplastic disorders were reviewed...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28675819/anti-neuronal-autoantibodies-in-both-drug-responsive-and-resistant-focal-seizures-with-unknown-cause
#5
Gokcen Gozubatik-Celik, Cigdem Ozkara, Canan Ulusoy, Aysegul Gunduz, Sakir Delil, Naz Yeni, Erdem Tuzun
BACKGROUND: and Objective Autoimmunity is an emerging field of research in the etiology of different neurological disorders including epilepsy. We aimed to investigate the presence of neuronal autoantibodies in focal epilepsy with unknown cause and their clinical correlates in both drug-responsive and resistant patients. METHOD: Between 2009 and 2010 94 patients were prospectively enrolled, had their antibodies tested and clinically followed." An additional 50 age- and gender-matched controls were also tested for antibodies...
June 17, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28675562/innate-and-adaptive-immunity-in-human-epilepsies
#6
Jan Bauer, Albert J Becker, Wassim Elyaman, Jukka Peltola, Stephan Rüegg, Maarten J Titulaer, James A Varley, Ettore Beghi
Inflammatory mechanisms have been increasingly implicated in the origin of seizures and epilepsy. These mechanisms are involved in the genesis of encephalitides in which seizures are a common complaint. Experimental and clinical evidence suggests different inflammatory responses in the brains of patients with epilepsy depending on the etiology. In general, activation of both innate and adaptive immunity plays a role in refractory forms of epilepsy. Epilepsies in which seizures develop after infiltration of cells of the adaptive immune system in the central nervous system (CNS) include a broad range of epileptic disorders with different (known or unknown) etiologies...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28675561/introduction-to-the-2nd-meeting-on-immunity-and-inflammation-in-epilepsy-iie2016
#7
Annamaria Vezzani, Stephan Rüegg
We report about the main topics presented during this meeting which was devoted to discuss the new emerging evidence for a role of immune- and inflammation-related mechanisms in various autoimmune and nonautoimmune forms of epilepsy. Major emphasis was given to novel immune/inflammatory mechanisms contributing to seizures and neurological comorbidities as well as to the discovery and validation of noninvasive biomarkers of brain inflammation which may be used to monitor epileptogenesis and predict the therapeutic effects of treatments...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28670128/clinical-features-of-limbic-encephalitis-with-lgi1-antibody
#8
Meiling Wang, Xiaoyu Cao, Qingxin Liu, Wenbin Ma, Xiaoqian Guo, Xuewu Liu
OBJECTIVE: The objective of this study was to analyze the clinical manifestation, course, evolution, image manifestation, and treatments of LGI1 limbic encephalitis (LE). PATIENTS AND METHODS: Studies confirmed that LE with the complex antibody of voltage-gated potassium channels is LGI1 LE. Since then, LE cases have been reported. In this study, 10 typical LE cases were searched in PubMed. These cases and one additional case, which we reported herein, were retrospectively analyzed...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28660470/psychosis-secondary-to-an-incidental-teratoma-a-heads-up-for-psychiatrists-and-gynecologists
#9
Jeanette Voice, Jane M Ponterio, Nisha Lakhi
Anti-N-methyl-D-aspartate receptor encephalitis is a potentially fatal form of autoimmune encephalitis that originates secondary to a host immune response to neural tissue within a teratoma. We describe the case of a 17-year old girl who presented with acute onset psychosis, catatonic movements, urinary incontinence, fever, tachycardia, and fluctuating periods of hypotension and hypertension. A CT scan demonstrated an incidental 6 cm ovarian teratoma. The patient fully recovered after ovarian cystectomy, followed by medical management with intravenous immunoglobins and plasmapheresis...
June 28, 2017: Archives of Women's Mental Health
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#10
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628018/autoimmune-limbic-encephalitis-case-reports
#11
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/28615895/study-of-refractory-status-epilepticus-from-a-tertiary-care-center
#12
Sahil Kohli, Suresh Babu Pasangulapati, Sangeetha Yoganathan, Gideon Lyngsyun Rynjah, A T Prabhakar, Sanjith Aaron, Mathew Alexander, Vivek Mathew
OBJECTIVES: To determine the proportion of refractory status epilepticus (RSE) and super-RSE (SRSE) among patients with status epilepticus (SE) and to analyze RSE and non-RSE (NRSE) in terms of etiology and predictors for RSE. MATERIALS AND METHODS: Patients were identified from discharge summaries database with keywords of SE and records of the portable electroencephalogram (EEG) machine from January 2011 to March 2016. RESULTS: Two hundred and eighteen events were included in the study with 114 (52...
April 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28614301/microglia-dependent-synapse-loss-in-type-i-interferon-mediated-lupus
#13
Allison R Bialas, Jessy Presumey, Abhishek Das, Cees E van der Poel, Peter H Lapchak, Luka Mesin, Gabriel Victora, George C Tsokos, Christian Mawrin, Ronald Herbst, Michael C Carroll
Systemic lupus erythematosus (SLE) is an incurable autoimmune disease characterized by autoantibody deposition in tissues such as kidney, skin and lungs. Notably, up to 75% of patients with SLE experience neuropsychiatric symptoms that range from anxiety, depression and cognitive impairment to seizures and, in rare cases, psychosis-collectively this is referred to as central nervous system (CNS) lupus. In some cases, certain autoantibodies, such as anti-NMDAR or anti-phospholipid antibodies, promote CNS lupus...
June 14, 2017: Nature
https://www.readbyqxmd.com/read/28607558/hashimoto-s-encephalopathy-presenting-with-unusual-behavioural-disturbances-in-an-adolescent-girl
#14
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
#15
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
#16
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/28567288/gad-antibody-associated-limbic-encephalitis-in-a-young-woman-with-apeced
#17
Anna Kopczak, Adrian-Minh Schumacher, Sandra Nischwitz, Tania Kümpfel, Günter K Stalla, Matthias K Auer
The autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome is a genetic disorder caused by a mutation in the autoimmune regulator (AIRE) gene. Immune deficiency, hypoparathyroidism and Addison's disease due to autoimmune dysfunction are the major clinical signs of APECED. We report on a 21-year-old female APECED patient with two inactivating mutations in the AIRE gene. She presented with sudden onset of periodic nausea. Adrenal insufficiency was diagnosed by means of the ACTH stimulation test...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28559946/f-18-fdg-pet-findings-for-vogt-koyanagi-harada-disease
#18
Hye Lim Park, Ie Ryung Yoo, Sonya Youngju Park
Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia. Seizures are very rare. We describe a case of (18)F-fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) and software-fused PET-magnetic resonance imaging (MRI) in Vogt-Koyanagi-Harada disease with seizure...
June 2017: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28556688/severe-and-rapidly-progressive-lafora-disease-associated-with-nhlrc1-mutation-a-case-report
#19
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
#20
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
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