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https://www.readbyqxmd.com/read/28628018/autoimmune-limbic-encephalitis-case-reports
#1
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/28597112/serial-magnetic-resonance-study-in-super-refractory-status-epilepticus-progressive-involvement-of-striatum-and-pallidus-is-a-possible-predictive-marker-of-negative-outcome
#2
Alessandra Ferrari, Paolo Renzetti, Carlo Serrati, Roberto Fancellu
Super refractory status epilepticus (SRSE) is a life-threatening condition in which seizures do not respond to third-line anticonvulsant drug therapy. SRSE is associated with high mortality. How often SRSE occurs, what are the risk factors leading to this condition, and what is the effect on clinical outcome of failure to control seizures are poorly defined. Several studies have evaluated magnetic resonance imaging (MRI) findings in status epilepticus (SE), confirming that SE may directly cause selective neuronal necrosis due to excitotoxic mechanisms, as described in clinical case reports and experimental models...
June 8, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28584608/super-refractory-status-epilepticus-in-hashimoto-s-encephalopathy
#3
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/28551039/clinical-features-in-very-early-onset-demyelinating-disease-with-anti-mog-antibody
#4
Masahiro Nishiyama, Hiroaki Nagase, Masaaki Matsumoto, Kazumi Tomioka, Hiroyuki Awano, Tsukasa Tanaka, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Yoshinobu Oyazato, Keisuke Saeki, Kazuhiro Shiraishi, Satoshi Takada, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima, Kazumoto Iijima
BACKGROUND: The clinical features of patients with very early-onset acquired demyelinating syndrome (ADS) with the anti-myelin oligodendrocyte glycoprotein (MOG) antibody are unknown. We investigated the clinical characteristics and described detailed treatment of weekly intramuscular interferon β-1a (IFNβ-1a) in children aged <4years with ADS and the anti-MOG antibody. METHODS: We conducted a retrospective chart review of patients with anti-MOG positivity who were diagnosed as having multiple sclerosis (MS) at <4years of age...
May 24, 2017: Brain & Development
https://www.readbyqxmd.com/read/28551036/a-patient-with-muenke-syndrome-manifesting-migrating-neonatal-seizures
#5
Yukimune Okubo, Taro Kitamura, Mai Anzai, Wakaba Endo, Takehiko Inui, Yusuke Takezawa, Sato Suzuki-Muromoto, Takuya Miyabayashi, Noriko Togashi, Hiroshi Oba, Hirotomo Saitsu, Naomichi Matsumoto, Kazuhiro Haginoya
We report a patient with Muenke syndrome who had repetitive apneic spell followed by focal status epilepticus in the early infancy. Ictal EEG showed focal spikes bursts originated from the left hemisphere and sifted to the right hemisphere, during which he had migrating tonic seizures from right side of the body to the left side of the body. Brain MRI showed abnormal development of bilateral hippocampus, which was characterized as abnormal folding of hippocampal gyri. However, the long-term seizure prognosis was favorable...
May 24, 2017: Brain & Development
https://www.readbyqxmd.com/read/28547540/investigation-of-anti-neuronal-antibodies-in-status-epilepticus-of-unknown-etiology-a-prospective-study
#6
Murat Mert Atmaca, Erdem Tuzun, Ece Erdag, Nerses Bebek, Betul Baykan, Candan Gurses
There have been recent reports of antibody-mediated status epilepticus. The objective of our study was to investigate the prevalence of neuronal autoantibodies in patients with status epilepticus (SE) with unresolved etiology. The presence of neuronal autoantibodies was investigated prospectively in adult patients with SE who presented to our clinic between February 2012 and December 2013 with unresolved etiology. Clinical and electrophysiologic features of seropositive patients were recorded. Also, seronegative and seropositive patient groups were compared in terms of demographic and clinical features, treatment responses, and outcomes...
May 25, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28448686/plasma-cytokines-associated-with-febrile-status-epilepticus-in-children-a-potential-biomarker-for-acute-hippocampal-injury
#7
William B Gallentine, Shlomo Shinnar, Dale C Hesdorffer, Leon Epstein, Douglas R Nordli, Darrell V Lewis, L Matthew Frank, Syndi Seinfeld, Ruth C Shinnar, Karen Cornett, Binyi Liu, Solomon L Moshé, Shumei Sun
OBJECTIVE: Our aim was to explore the association between plasma cytokines and febrile status epilepticus (FSE) in children, as well as their potential as biomarkers of acute hippocampal injury. METHODS: Analysis was performed on residual samples of children with FSE (n = 33) as part of the Consequences of Prolonged Febrile Seizures in Childhood study (FEBSTAT) and compared to children with fever (n = 17). Magnetic resonance imaging (MRI) was obtained as part of FEBSTAT within 72 h of FSE...
June 2017: Epilepsia
https://www.readbyqxmd.com/read/28444141/imaging-blood-brain-barrier-dysfunction-as-a-biomarker-for-epileptogenesis
#8
Guy Bar-Klein, Svetlana Lublinsky, Lyn Kamintsky, Iris Noyman, Ronel Veksler, Hotjensa Dalipaj, Vladimir V Senatorov, Evyatar Swissa, Dror Rosenbach, Netta Elazary, Dan Z Milikovsky, Nadav Milk, Michael Kassirer, Yossi Rosman, Yonatan Serlin, Arik Eisenkraft, Yoash Chassidim, Yisrael Parmet, Daniela Kaufer, Alon Friedman
A biomarker that will enable the identification of patients at high-risk for developing post-injury epilepsy is critically required. Microvascular pathology and related blood-brain barrier dysfunction and neuroinflammation were shown to be associated with epileptogenesis after injury. Here we used prospective, longitudinal magnetic resonance imaging to quantitatively follow blood-brain barrier pathology in rats following status epilepticus, late electrocorticography to identify epileptic animals and post-mortem immunohistochemistry to confirm blood-brain barrier dysfunction and neuroinflammation...
June 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28441824/-clinical-and-neuroimaging-features-of-acute-encephalopathy-after-status-epilepticus-in-dravet-syndrome
#9
X J Tian, Y H Zhang, A J Liu, X L Yang, Q Zeng, Z X Yang, J T Ye, X Y Liu, Y W Jiang, X R Wu
Objective: To investigate the clinical and neuroimaging characteristics of acute encephalopathy (AE) after status epilepticus (SE) of patients with Dravet syndrome (DS). Method: The clinical data of DS patients who had AE (coma ≥24 h) after SE were retrospectively collected from February 2005 to August 2016 in Peking University First Hospital and SCN1A gene tests were performed.The clinical and neuroimaging features were summarized. Result: Twenty-two patients (9 males and 13 females) with AE were collected among 412 DS patients during follow-up...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28440867/the-epileptology-of-koolen-de-vries-syndrome-electro-clinico-radiologic-findings-in-31-patients
#10
Kenneth A Myers, Simone A Mandelstam, Georgia Ramantani, Elisabeth J Rushing, Bert B de Vries, David A Koolen, Ingrid E Scheffer
OBJECTIVE: This study was designed to describe the spectrum of epilepsy phenotypes in Koolen-de Vries syndrome (KdVS), a genetic syndrome involving dysmorphic features, intellectual disability, hypotonia, and congenital malformations, that occurs secondary to 17q21.31 microdeletions and heterozygous mutations in KANSL1. METHODS: We were invited to attend a large gathering of individuals with KdVS and their families. While there, we recruited individuals with KdVS and seizures, and performed thorough phenotyping...
June 2017: Epilepsia
https://www.readbyqxmd.com/read/28436816/-almost-lost-at-sea-an-unusual-cause-of-cortical-blindness
#11
Jeffrey Walden, Edward Van Williamson
A middle-aged man with a history of type 2 diabetes mellitus, hypertension, hyperlipidemia, prior cerebral vascular accident, and remote history of generalized seizure disorder presented with worsening right-sided visual deficits and focal seizures. On examination the patient had dense homonymous hemianopsia on the right side. He was initially diagnosed with a stroke and underwent further investigation for potential causes. However, upon further review, magnetic resonance imaging (MRI) findings and repeat examination were more consistent with seizure-related effects on cortical brain matter as the cause of his visual disturbances rather than stroke...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28424403/-non-convulsive-status-epilepticus-caused-by-hyponatremia-in-an-elderly-woman-a-case-report
#12
Yoshinori Kurauchi, Masahiro Yasaka, Keisuke Tokunaga, Masaki Saito, Shun Shimohama, Yasushi Okada
We report the case of a 67-year-old woman with non-convulsive status epilepticus (NCSE) due to hyponatremia. She had a history of psychogenic polydipsia but not epilepsy. She was admitted to our hospital with dysbulia. On admission, she was confused and disoriented (Glasgow Coma Scale: 6, E1V1M4). Magnetic resonance imaging of the brain showed no abnormalities. Laboratory test showed hyponatremia (Na<sup>+</sup> level: 115 mEq/L). The electroencephalography (EEG) showed a generalized slow wave of 5 Hz during recording...
April 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28423465/febrile-status-epilepticus-due-to-respiratory-syncytial-virus-infection
#13
Kazuhiro Uda, Katsuhiko Kitazawa
BACKGROUND: Febrile status epilepticus can have neurological sequelae. The type of sequelae, however, may depend on the etiology, including infections by viral agents such as the influenza virus. Respiratory syncytial virus (RSV) infection in childhood suggests a similar role for this pathogen. The aim of this study was to characterize febrile status epilepticus associated with RSV infection, and to determine whether this type of infection is a risk factor for neurological sequelae with febrile status epilepticus...
April 19, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28413125/transient-dysautonomia-in-an-acute-phase-of-encephalopathy-with-biphasic-seizures-and-late-reduced-diffusion
#14
Yuko Ichimiya, Noriyuki Kaku, Yasunari Sakai, Fumiya Yamashita, Wakato Matsuoka, Mamoru Muraoka, Satoshi Akamine, Soichi Mizuguchi, Michiko Torio, Yoshitomo Motomura, Yuichiro Hirata, Yoshito Ishizaki, Masafumi Sanefuji, Hiroyuki Torisu, Hidetoshi Takada, Yoshihiko Maehara, Shouichi Ohga
Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection...
April 13, 2017: Brain & Development
https://www.readbyqxmd.com/read/28396650/new-onset-refractory-status-epilepticus-with-claustrum-damage-definition-of-the-clinical-and-neuroimaging-features
#15
Stefano Meletti, Giada Giovannini, Giuseppe d'Orsi, Lisa Toran, Giulia Monti, Rahul Guha, Andreas Kiryttopoulos, Maria Grazia Pascarella, Tommaso Martino, Haris Alexopoulos, Martha Spilioti, Jana Slonkova
New-onset refractory status epilepticus (NORSE) is a rare but challenging condition occurring in a previously healthy patient, often with no identifiable cause. We describe the electro-clinical features and outcomes in a group of patients with NORSE who all demonstrated a typical magnetic resonance imaging (MRI) sign characterized by bilateral lesions of the claustrum. The group includes 31 patients (12 personal and 19 previously published cases; 17 females; mean age of 25 years). Fever preceded status epilepticus (SE) in 28 patients, by a mean of 6 days...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28357173/status-epilepticus-and-blindness-in-a-patient-with-carfilzomib-associated-posterior-reversible-encephalopathy-syndrome
#16
Salam Kadhem, Rawaa Ebrahem, Scott Cooper, Emily Manlove, Ricky Lee
Posterior reversible encephalopathy syndrome (PRES) is a neurological condition characterized by headaches, visual disturbances, and seizures. A magnetic resonance imaging (MRI) scan of an affected brain typically shows symmetrical white matter edema in the posterior cerebral hemispheres. The onset of PRES can constitute a medical emergency, especially when accompanied by status epilepticus. If promptly recognized and treated, the clinical syndrome and associated radiological findings are usually resolved in a matter of weeks or months...
February 19, 2017: Curēus
https://www.readbyqxmd.com/read/28343008/electroclinical-features-of-epilepsy-in-patients-with-invdup-15
#17
REVIEW
Alberto Verrotti, Fiammetta Sertorio, Sara Matricardi, Pietro Ferrara, Pasquale Striano
PURPOSE: InvDup(15) syndrome is one of the most common chromosomal abnormalities associated with epilepsy. Here we review the seizure types described in InvDup(15) patients and the main electroclinical, therapeutic, and prognostic aspects of the syndrome. METHODS: A literature search of PubMed, MEDLINE, and EMBASE was performed to identify papers examining InvDup(15) syndrome and epilepsy. RESULTS: About 65% of the InvDup(15) patients described in the literature had multiple seizure types with a predominance (40...
April 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28330972/fatal-cerebral-edema-with-status-epilepticus-in-children-with-dravet-syndrome-report-of-5-cases
#18
Kenneth A Myers, Jacinta M McMahon, Simone A Mandelstam, Mark T Mackay, Renate M Kalnins, Richard J Leventer, Ingrid E Scheffer
Dravet syndrome (DS) is a well-recognized developmental and epileptic encephalopathy associated with SCN1A mutations and 15% mortality by 20 years. Although over half of cases succumb to sudden unexpected death in epilepsy, the cause of death in the remainder is poorly defined. We describe the clinical, radiologic, and pathologic characteristics of a cohort of children with DS and SCN1A mutations who developed fatal cerebral edema causing mass effect after fever-associated status epilepticus. Cases were identified from a review of children with DS enrolled in the Epilepsy Genetics Research Program at The University of Melbourne, Austin Health, who died after fever-associated status epilepticus...
April 2017: Pediatrics
https://www.readbyqxmd.com/read/28329842/from-the-cover-magneticresonance-imaging-reveals-progressive-brain-injury-in-rats-acutely-intoxicated-with-diisopropylfluorophosphate
#19
Brad A Hobson, Sílvia Sisó, Douglas J Rowland, Danielle J Harvey, Donald A Bruun, Joel R Garbow, Pamela J Lein
Acute intoxication with organophosphates (OPs) can trigger seizures that progress to status epilepticus, and survivors often exhibit chronic neuropathology, cognitive impairment, affective disorders, and/or electroencephalographic abnormalities. Understanding how acute injury transitions to persistent neurological sequelae is critical to developing medical countermeasures for mitigating damage following OP-induced seizures. Here, we used in vivo magnetic resonance imaging (MRI) to monitor the spatiotemporal patterns of neuropathology for 1 month after acute intoxication with diisopropylfluorophosphate (DFP)...
June 1, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28320152/electrographic-patterns-in-patients-with-posterior-reversible-encephalopathy-syndrome-and-seizures
#20
Carlos Kamiya-Matsuoka, Sudhakar Tummala
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic encephalopathic state associated with reversible cerebral vasogenic edema. Seizures are a common clinical presentation in PRES, however its electroencephalographic and radiologic pattern correlation is limited in this subset of patients. The aim of this study is to analyze the origin of electrographic dysfunction according to the radiologic pattern in patients with PRES and seizures. METHODS: We retrospectively identified 46 cancer patients who developed PRES and seizures at The University of Texas MD Anderson Cancer Center between January 2006 and June 2012...
April 15, 2017: Journal of the Neurological Sciences
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