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rasmussen encephalitis

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https://www.readbyqxmd.com/read/29118737/a-case-series-of-adult-onset-rasmussen-s-encephalitis-diagnostic-and-therapeutic-challenges
#1
James Francis Castellano, Jenny A Meyer, Fred Alexander Lado
Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29116594/elevated-expression-of-ebv-and-tlrs-in-the-brain-is-associated-with-rasmussen-s-encephalitis
#2
Xin Wang, Yisong Wang, Dong Liu, Peigang Wang, Dongying Fan, Yuguang Guan, Tianfu Li, Guoming Luan, Jing An
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus (EBV) antigens as well as of Toll-like receptor 3 (TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β (TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry (IHC)...
October 2017: Virologica Sinica
https://www.readbyqxmd.com/read/29103423/autoimmune-epilepsies
#3
Anusha K Yeshokumar, Carlos A Pardo
Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, or other focal neurological deficits, or all of these. Traditional neurological diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29096685/clinical-spectrum-of-woolly-hair-indications-for-cerebral-involvement
#4
Piero Pavone, Raffaele Falsaperla, Massimo Barbagallo, Agata Polizzi, Andrea D Praticò, Martino Ruggieri
BACKGROUND: Woolly Hair is an uncommon congenital anomaly of the scalp hair presenting with strongly coiled hair involving a localized area of the scalp or covering the entire side and occurring in non-black people. Isolated or localized wooly hair is usually benign and is not related to other disorders and/or complications. On the contrary, the generalized type may be related to disorders and syndromes affecting heart, cutis, liver and gastrointestinal organs. Among the syndromes presenting with wooly hair, the most known are the Naxos syndrome, the Carvajal-Huerta syndrome, the wooly hair/hypotrichosis, the ectodermal dysplasia-skin fragility, the tricho-hepato-enteric syndrome...
November 2, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29091250/functional-hemispherectomy-for-adult-rasmussen-encephalitis-a-case-report-and-literature-review
#5
Qun Wang, Zhanpeng Zhu, Guangming Wang, Lichao Sun, Jiqing Qiu
BACKGROUND: Rasmussen encephalitis (RE) is a rare and severe brain disorder associated with unilateral hemispheric atrophy, manifesting as severe refractory epilepsy, hemiplegia, defects of motor and speech functions, and cognitive impairment. Treatment of RE, especially in adult patients, is extremely challenging. Herein, we report the case of an adult patient with RE who was treated with a functional hemispherectomy and achieved a favorable prognosis. CASE REPORT: A 29-year-old woman presented with a 24-year history of epileptic seizures...
September 26, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/29054955/trapped-ipsilateral-lateral-ventricle-a-delayed-complication-of-hemispherotomy-for-rasmussen-s-encephalitis
#6
Rajesh Shankar Iyer, Ravi Mohan Rao, Karunakaran Muthukalathi, Praveen Kumar
Hemispherotomy is the currently preferred surgical treatment option for refractory unihemispheric epilepsies. The incidence of hydrocephalus is greatly reduced in this disconnective procedure when compared with the resective procedure of anatomical hemispherectomy. We describe the occurrence of ipsilateral trapped lateral ventricle months after hemispherotomy for Rasmussen's encephalitis. There is enough evidence to suggest that this rare and interesting complication is due to the local inflammatory changes associated with the surgical trauma...
October 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29053073/chronic-subthreshold-cortical-stimulation-for-adult-drug-resistant-focal-epilepsy-safety-feasibility-and-technique
#7
Panagiotis Kerezoudis, Sanjeet S Grewal, Matthew Stead, Brian Nils Lundstrom, Jeffrey W Britton, Cheolsu Shin, Gregory D Cascino, Benjamin H Brinkmann, Gregory A Worrell, Jamie J Van Gompel
OBJECTIVE Epilepsy surgery is effective for lesional epilepsy, but it can be associated with significant morbidity when seizures originate from eloquent cortex that is resected. Here, the objective was to describe chronic subthreshold cortical stimulation and evaluate its early surgical safety profile in adult patients with epilepsy originating from seizure foci in cortex that is not amenable to resection. METHODS Adult patients with focal drug-resistant epilepsy underwent intracranial electroencephalography monitoring for evaluation of resection...
October 20, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28987176/immune-mediated-disorders
#8
Romana Höftberger, Hans Lassmann
Paraneoplastic and autoimmune encephalitis comprise a group of immune-mediated disorders that are associated with different immune effector mechanisms. Classic paraneoplastic neurologic syndromes are triggered by an antitumor immune response. The disease is considered to result from a T-cell response; in addition, patients harbour high titers of autoantibodies against intracellular antigens that are considered as epiphenomenon but are useful diagnostic markers. Neuropathology consists of T-cell-dominated inflammation, marked neuronal loss, and microglial activation with upregulation of HLA-DR...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28987170/neuropathology-of-epilepsy
#9
Eleonora Aronica, Angelika Mühlebner
Epilepsy is one of the most common neurologic disorders, affecting about 50 million people worldwide. The disease is characterized by recurrent seizures, which are due to aberrant neuronal networks resulting in synchronous discharges. The term epilepsy encompasses a large spectrum of syndromes and diseases with different etiopathogenesis. The recent development of imaging and epilepsy surgery techniques is now enabling the identification of structural abnormalities that are part of the epileptic network, and the removal of these lesions may result in control of seizures...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28923528/arterial-spin-labeling-hyperperfusion-in-rasmussen-s-encephalitis-is-it-due-to-focal-brain-inflammation-or-a-postictal-phenomenon
#10
Savith Kumar, Chinmay P Nagesh, Bejoy Thomas, Ashalatha Radhakrishnan, Ramshekhar N Menon, Chandrasekharan Kesavadas
BACKGROUND AND PURPOSE: The study evaluated the utility of arterial spin labeling (ASL) perfusion imaging in Rasmussen's encephalitis (RE). MATERIAL AND METHODS: The hospital electronic database was searched using the search words "encephalitis," "autoimmune encephalitis" and "Rasmussen's encephalitis" for the period of 1 Jan 2015 to 31 Jan 2017. Clinically diagnosed cases of RE for which epilepsy protocol magnetic resonance imaging (MRI) with perfusion imaging (ASL) performed on a 3T scanner were retrieved...
September 18, 2017: Journal of Neuroradiology. Journal de Neuroradiologie
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#11
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28853521/-hemispherotomy-for-treatment-of-refractory-epilepsy-in-children
#12
Jonathan Roth, Sari Nagar, Shlomi Constantini, Itzhak Fried
INTRODUCTION: Hemispherotomies are an extreme treatment for epilepsy. As opposed to focal resections, in hemispherotomies the entire hemisphere is disconnected from the remaining nervous system, including functional regions and fibers. Despite this, hemispherotomy is recommended for certain indications, with good epilepsy and functional outcomes. AIMS: To summarize the experience of hemispherotomy performed at a tertiary national center. METHODS: A retrospective study was conducted including all patients operated on between 2001 and 2014...
August 2017: Harefuah
https://www.readbyqxmd.com/read/28732255/can-head-trauma-trigger-adult-onset-rasmussen-s-encephalitis
#13
Derrick Soh, Dennis J Cordato, Andrew F Bleasel, Peter Brimage, Roy G Beran
Rasmussen's encephalitis (RE) is a rare unilateral inflammatory brain disorder that causes progressive neurocognitive deterioration and refractory epilepsy including epilepsia partialis continua (EPC). We describe a patient with a unique presentation, where right upper limb EPC due to RE began within 2weeks of a concussive left frontal head injury, in a 36-year-old female without other identifiable etiology, no prior neurological deficit nor suggestion of intracranial pathology or infection, and no preceding seizures...
September 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28572051/late-onset-rasmussen-encephalitis-a-literature-appraisal
#14
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...
August 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28534039/alemtuzumab-and-intrathecal-methotrexate-failed-in-the-therapy-of-rasmussen-encephalitis
#15
Zuzana Liba, Petr Sedlacek, Vera Sebronova, Alice Maulisova, Bertil Rydenhag, Josef Zamecnik, Martin Kyncl, Pavel Krsek
No abstract text is available yet for this article.
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28424599/electrophysiological-sequelae-of-hemispherotomy-in-ipsilateral-human-cortex
#16
Ammar H Hawasli, Ravi Chacko, Nicholas P Szrama, David T Bundy, Mrinal Pahwa, Chester K Yarbrough, Brian J Dlouhy, David D Limbrick, Dennis L Barbour, Matthew D Smyth, Eric C Leuthardt
Objectives: Hemispheric disconnection has been used as a treatment of medically refractory epilepsy and evolved from anatomic hemispherectomy to functional hemispherectomies to hemispherotomies. The hemispherotomy procedure involves disconnection of an entire hemisphere with limited tissue resection and is reserved for medically-refractory epilepsy due to diffuse hemispheric disease. Although it is thought to be effective by preventing seizures from spreading to the contralateral hemisphere, the electrophysiological effects of a hemispherotomy on the ipsilateral hemisphere remain poorly defined...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28399506/timing-and-type-of-hemispherectomy-for-rasmussen-s-encephalitis-analysis-of-45-patients
#17
Yuguang Guan, Sichang Chen, Changqing Liu, Xiuyu Du, Yao Zhang, Shuai Chen, Jie Wang, Tianfu Li, Guoming Luan
OBJECTIVE: To describe the surgery outcomes of RE patients in one centerto identify the indication for surgical treatment that results in the most favorable outcome. METHOD: Forty-five RE patients from a single center were retrospectively reviewed. Preoperative evaluations included assessments of clinical manifestations, cognitive status, a physical examination, MRI, positron emission tomography (PET), electroencephalography (EEG), and magnetoencephalography (MEG)...
March 22, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28303446/expression-of-human-cytomegalovirus-components-in-the-brain-tissues-of-patients-with-rasmussen-s-encephalitis
#18
Yao Zhang, Yisong Wang, Sichang Chen, Shuai Chen, Yuguang Guan, Changqing Liu, Tianfu Li, Guoming Luan, Jing An
Rasmussen's encephalitis (RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus (HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters...
April 2017: Virologica Sinica
https://www.readbyqxmd.com/read/28256379/outcome-of-childhood-onset-epilepsy-from-adolescence-to-adulthood-transition-issues
#19
REVIEW
R Nabbout, D M Andrade, N Bahi-Buisson, H Cross, I Desquerre, O Dulac, T Granata, E Hirsch, V Navarro, L Ouss, P L Pearl, D Schmidt, E Thiele, P R Camfield, C S Camfield
This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders)...
April 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28245617/the-diverse-roles-of-microglia-in-the-neurodegenerative-aspects-of-central-nervous-system-cns-autoimmunity
#20
REVIEW
Kaitlyn K Thompson, Stella E Tsirka
Autoimmune diseases of the central nervous system (CNS) involve inflammatory components and result in neurodegenerative processes. Microglia, the resident macrophages of the CNS, are the first responders after insults to the CNS and comprise a major link between the inflammation and neurodegeneration. Here, we will focus on the roles of microglia in two autoimmune diseases: the prevalent condition of multiple sclerosis (MS) and the much rarer Rasmussen's encephalitis (RE). Although there is an abundance of evidence that microglia actively contribute to neuronal damage in pathological states such as MS and RE, there is also evidence of important reparative functions...
February 25, 2017: International Journal of Molecular Sciences
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