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Praveen K Sharma, Afwaan Faizal, Ajay Lucas Rubben Prabhu, Iffath Misbah
Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon neurological condition marked by changes in the skeletal structure, cerebral hemiatrophy, and ventriculomegaly. Manifesting primarily in early life, DDMS presents with seizures, hemiplegia, facial asymmetry, and intellectual disabilities. There are congenital and acquired types of DDMS, with ischemia being the most common cause of the latter. Three cases are presented here to highlight the radiological and clinical characteristics of DDMS. The first case involves a 27-year-old male with generalized seizures and right-sided hemiparesis since childhood, along with developmental delays and facial asymmetry...
February 2024: Curēus
#2
JOURNAL ARTICLE
Alexander Doherty, Kathleen Knudson, Christine Fuller, James L Leach, Anthony C Wang, Neena Marupudi, Rowland H Han, Stuart Tomko, Jeff Ojemann, Matthew D Smyth, Francesco Mangano, Jesse Skoch
PURPOSE: Rasmussen encephalitis (RE) is a very rare chronic neurological disorder of unilateral inflammation of the cerebral cortex. Hemispherotomy provides the best chance at achieving seizure freedom in RE patients, but with significant risks and variable long-term outcomes. The goal of this study is to utilize our multicenter pediatric cohort to characterize if differences in pathology and/or imaging characterization of RE may provide a window into post-operative seizure outcomes, which in turn could guide decision-making for parents and healthcare providers...
March 15, 2024: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
#3
Jaron C Sanchez, Markeeta T Belmar, Jason C Sanchez, Kenny Nguygen
Hemispherectomy is a neurosurgical procedure that is frequently performed in pediatric patients diagnosed with Rasmussen's encephalitis. Postoperative complications include immediate complications such as hydrocephalus and hemorrhage and behavioral complications such as language impairments and contralateral weakness. However, there are limited studies or case reports that address the potential endocrinopathies associated with this and other pediatric epileptic surgeries. This case report describes the endocrinopathies following an anatomical hemispherectomy procedure...
February 2024: Curēus
#4
JOURNAL ARTICLE
Rajesh Verma, Rajarshi Chakraborty
No abstract text is available yet for this article.
January 1, 2024: Indian Journal of Pharmacology
#5
JOURNAL ARTICLE
M Daoud, C Durelle, A Fierain, El Youssef N, F Wendling, G Ruffini, P Benquet, F Bartolomei
Epilepsia partialis continua (EPC) is a rare type of focal motor status epilepticus that causes continuous muscle jerking in a specific part of the body. Experiencing this type of seizure, along with other seizure types, such as focal motor seizures and focal to bilateral tonic-clonic seizures, can result in a disabling situation. Non-invasive brain stimulation methods like transcranial direct current stimulation (tDCS) show promise in reducing seizure frequency (SF) when medications are ineffective. However, research on tDCS for EPC and related seizures is limited...
March 6, 2024: Brain Topography
#6
JOURNAL ARTICLE
Friederike A Arlt, Ramona Miske, Marie-Luise Machule, Peter Broegger Christensen, Swantje Mindorf, Bianca Teegen, Kathrin Borowski, Maria Buthut, Rosa Rößling, Elisa Sanchez-Sendin, Scott van Hoof, César Cordero-Gómez, Isabel Bünger, Helena Radbruch, Andrea Kraft, Ilya Ayzenberg, Jaqueline Klausewitz, Niels Hansen, Charles Timäus, Peter Körtvelyessy, Thomas Postert, Kirsten Baur-Seack, Constanze Rost, Robert Brunkhorst, Kathrin Doppler, Niklas Haigis, Gerhard Hamann, Albrecht Kunze, Alexandra Stützer, Matthias Maschke, Nico Melzer, Felix Rosenow, Kai Siebenbrodt, Christian Stenør, Martin Dichgans, Marios K Georgakis, Rong Fang, Gabor C Petzold, Michael Görtler, Inga Zerr, Silke Wunderlich, Ivan Mihaljevic, Paul Turko, Marianne Schmidt Ettrup, Emilie Buchholz, Helle Foverskov Rasmussen, Mahoor Nasouti, Ivan Talucci, Hans M Maric, Stefan H Heinemann, Matthias Endres, Lars Komorowski, Harald Prüss
BACKGROUND: Autoantibodies against the potassium voltage-gated channel subfamily A member 2 (KCNA2) have been described in a few cases of neuropsychiatric disorders, but their diagnostic and pathophysiological role is currently unknown, imposing challenges to medical practice. DESIGN / METHODS: We retrospectively collected comprehensive clinical and paraclinical data of 35 patients with KCNA2 IgG autoantibodies detected in cell-based and tissue-based assays. Patients' sera and cerebrospinal fluid (CSF) were used for characterization of the antigen, clinical-serological correlations, and determination of IgG subclasses...
February 1, 2024: Brain, Behavior, and Immunity
#7
JOURNAL ARTICLE
Wei Yang, Chong-Yang Tang, Dong-Ying Fan, Yi-Song Wang, Pei-Gang Wang, Jing An, Guo-Ming Luan
Viral encephalitis continues to be a significant public health concern. In our previous study, we discovered a lower expression of antiviral factors, such as IFN-β, STING and IFI16, in the brain tissues of patients with Rasmussen's encephalitis (RE), a rare chronic neurological disorder often occurred in children, characterized by unihemispheric brain atrophy. Furthermore, a higher cumulative viral score of human herpes viruses (HHVs) was also found to have a significantly positively correlated with the unihemispheric atrophy in RE...
January 12, 2024: Virologica Sinica
#8
JOURNAL ARTICLE
Samir Alkabie, Madison T Gray, Jonathan C Lau, Lillian Barra, Jennifer Mandzia, Qi Zhang, Adrian Budhram
A 48-year-old woman was referred with an 18-year history of focal-onset seizures. She also reported years-long slowly progressive right-sided weakness that was corroborated on examination. Repeated brain MRIs over 15 years showed multifocal left hemispheric T2 fluid-attenuated inversion recovery-hyperintense lesions with patchy enhancement and microhemorrhages, no diffusion restriction, and a left cerebellar infarct (Figure 1, A-F). Only 2 nonspecific white matter lesions were seen contralaterally, indicating largely unihemispheric disease...
January 9, 2024: Neurology
#9
Jinkun Han, Xiaotong Fan, Sichang Chen
Rasmussen encephalitis is a rare and unexplained chronic brain hemispheric inflammatory disease. We report a case of epilepsy in which magnetoencephalography showed dipoles localized only in the operculum. Because the patient's clinical presentation and examination findings did not meet the diagnostic criteria for Rasmussen encephalitis, he underwent cortical electroencephalogram (ECoG) record and limited resection surgery. However, the seizures were not relieved after surgery, and imaging findings showed significant features of hemisphere atrophy...
2023: Frontiers in Neurology
#10
JOURNAL ARTICLE
Tobias Bauer, Johannes T Reiter, Selma Enders, Vera C W Keil, Alexander Radbruch, Christoph Helmstaedter, Rainer Surges, Theodor Rüber
Rasmussen's encephalitis is characterized by drug-resistant focal seizures and chronic inflammation of one hemisphere leading to progressive loss of hemispheric volume. In this cohort study, we aimed to investigate subcortical grey matter volumes and asymmetries in Rasmussen's encephalitis longitudinally in clinically relevant subgroups. We retrospectively included all T1 -weighted MRI scans of all people with Rasmussen's encephalitis who were treated at the University Hospital Bonn between 1995 and 2022 ( n = 56, 345 scans, median onset 8 years, 36 female)...
2023: Brain communications
#11
JOURNAL ARTICLE
Noah Trapp, Dominic O Co, Susan Rebsamen, Chris Ikonomidou, Raheel Ahmed, Andrew Knox
BACKGROUND: Bilateral Rasmussen encephalitis is a rare variant of a debilitating, typically unihemispheric disease with limited treatment options. Few cases with bilateral histopathology have been reported, all with poor seizure control following surgery. Here we report a favorable outcome following hemispherotomy in a four-year-old male with biopsy-confirmed bilateral disease. CASE: The patient presented with right hemispheric focal seizures with behavioral arrest and over a year progressed to left lower extremity clonic seizures, epilepsia partialis continua, and loss of ambulation, with transient response to steroids and tacrolimus...
February 2024: Pediatric Neurology
#12
El Harras Yahya, Laasri Khadija, Bahlouli Nourrelhouda, Allali Nazik, Chat Latifa, El Haddad Siham
Relatively rare, Rasmussen encephalitis is a chronic inflammatory disease conditioning a single cerebral hemisphere. It is a progressive pathology with different stages clinically and on MRI. Therefore, imaging has an important role in evoking the diagnosis and also excluding other possible etiologies. We report a case of Rasmussen encephalitis with 2 different stages on MRI and also provide a review of the role of imaging in diagnostic criteria, differential diagnoses, and stages of this pathology.
2023: Global Pediatric Health
#13
Mai Akazawa, Guoshan Yan, Rina Hirai, Hirotoshi Kitagawa
INTRODUCTION: Rasmussen's encephalitis (RE) is a progressive and chronic ailment characterized by drug-resistant epileptic seizures. RE is uncommon, and no documented accounts of its anesthetic management exist. Anesthetic management without causing epileptic seizures is important in RE. Here, we present a case of safe anesthetic management in a pediatric patient with RE. CASE PRESENTATION: A 7-year-old boy who was diagnosed with RE at the age of 6 years was scheduled for supernumerary tooth extraction under general anesthesia...
2023: Case Reports in Neurology
#14
REVIEW
Abhishek Kumar, Harshil Krishnani, Arundhati Pande, Siddhant Jaiswal, Revat J Meshram
Usually affecting one hemisphere of the brain, Rasmussen's encephalitis (RE) is a persistent inflammatory disease of unclear origin. Rasmussen and colleagues presumed a viral etiology of the sickness in their first description. Later, the condition was linked to autoantibodies that were in the blood. Recently, it was shown that the cause of RE was a cytotoxic T-cell reaction to neurons. RE may be identified histopathologically by cortical inflammation, neuronal degeneration, and cerebral hemispheric-specific gliosis...
October 2023: Curēus
#15
JOURNAL ARTICLE
Martin Pilioneta, Hsin-Hung Chen, Emma Losito, Marie Bourgeois, Nicole Chémaly, Monika Eiserman, Lelio Guida, Volodia Dangouloff-Ros, Luca Fumagalli, Anna Kaminska, Nathalie Boddaert, Stéphane Auvin, Rima Nabbout, Christian Sainte-Rose, Thomas Blauwblomme
BACKGROUND AND OBJECTIVES: When seizure onset affects a whole hemisphere, hemispheric disconnections are efficient and safe procedures. However, both lateral peri-insular hemispherotomy and vertical paramedian hemispherotomy approaches report a failure rate around 20%, which can be explained by residual connections giving rise to persistent seizures. In this study, we present the interhemispheric vertical hemispherotomy (IVH), a technical variation of the vertical paramedian hemispherotomy approach, that aims to increase seizure control avoiding residual connections while exposing the corpus callosum...
November 23, 2023: Operative Neurosurgery (Hagerstown, Md.)
#16
JOURNAL ARTICLE
Ursula Thomé, Larissa A Batista, Renata P Rocha, Vera C Terra, Ana Paula A Hamad, Americo C Sakamoto, Antônio C Santos, Marcelo V Santos, Hélio R Machado
BACKGROUND: Rasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients...
January 2024: Pediatric Neurology
#17
JOURNAL ARTICLE
Georgi Galev, Richard A Prayson
INTRODUCTION: Rasmussen's encephalitis (RE) is a rare, predominantly pediatric epilepsy disorder of unknown etiology. It classically affects one of the cerebral hemispheres and histologically shows cortical chronic inflammation, gliosis, and neuronal loss. The etiopathogenesis of RE remains unknown, with genetic, infectious, and autoimmune factors all speculated to play a role. Although the histologic findings in RE are well described, few studies have investigated a large cohort of cases looking for the coexistence of RE with focal cortical dysplasia (FCD)...
February 2024: Annals of Diagnostic Pathology
#18
JOURNAL ARTICLE
Georgia Ramantani, Dorottya Cserpan, Martin Tisdall, Willem M Otte, Georg Dorfmüller, J Helen Cross, Monique van Schooneveld, Pieter van Eijsden, Frauke Nees, Gitta Reuner, Niklaus Krayenbühl, Josef Zentner, Christine Bulteau, Kees Pj Braun
OBJECTIVE: We aimed to evaluate determinants of functional outcome after pediatric hemispherotomy in a large and recent multicenter cohort. METHODS: We retrospectively investigated the functional outcomes of 455 children who underwent hemispherotomy in five epilepsy centers in 2000-2016. We identified determinants of unaided walking, voluntary grasping with the hemiplegic hand, and speaking through Bayesian multivariable regression modeling using missing data imputation...
November 14, 2023: Annals of Neurology
#19
JOURNAL ARTICLE
Nina R Held, Tobias Bauer, Johannes T Reiter, Christian Hoppe, Vera C W Keil, Alexander Radbruch, Christoph Helmstaedter, Rainer Surges, Theodor Rüber
Rasmussen's encephalitis is an immune-mediated brain disorder characterised by progressive unilateral cerebral atrophy, neuroinflammation, drug-resistant seizures and cognitive decline. However, volumetric changes and epileptiform EEG activity were also observed in the contralateral hemisphere, raising questions about the aetiology of contralateral involvement. In this study, we aim to investigate alterations of white matter integrity, structural network topology and network efficiency in Rasmussen's encephalitis using diffusion-tensor imaging...
2023: Brain communications
#20
JOURNAL ARTICLE
Abdullah Arcan, Esra Koçhan Kızılkılıç, Ayşegül Gündüz, Rümeysa Unkun, Annamaria Vezzani, Çiğdem Özkara
BACKGROUND AND OBJECTIVE: Rasmussen's encephalitis (RE) is a rare chronic neurological disorder, characterized by unilateral inflammation of the cerebral cortex, refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline. Interleukin-1 (IL-1) plays an important role in neuroinflammation as a key element in the activation of the inflammatory IL-1β-IL-1 receptor type 1 (IL-1R1) axis. Anakinra, an IL-1 inhibitor, is successfully used in patients with new onset refractory status epilepticus and febrile infection-related epilepsy syndrome...
November 3, 2023: Journal of Neurology
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