Explanipedia

MRI cortical thickness in paediatric auto-immune encephalitis and acute disseminated encephalomyelitis Open

Charly Billaud, Daniel Griffiths-King, Sukhvir Wright, Elaine Foley, Amanda Wood · 2025

Children with autoimmune encephalitis may be subject to local cortical thinning in the long term.

Paramagnetic Rim Lesions in Pediatric Multiple Sclerosis and Their Association With Brain Tissue Atrophy Open

Riccardo Nistri, Ermelinda De Meo, Nee Na Kim, Valeria Pozzilli, Philip Goebl , et al. · 2025

PRLs are common in pediatric MS and are linked to greater lesion burden and gray matter atrophy. These findings suggest that PRLs are promising imaging biomarkers of more severe brain tissue damage although their ability to predict future …

Entropy of the resting state cortex in epilepsy Open

Kirandeep Kaur, Jack O'Brien-Cairney, Gaurav Singh, Manoj A. Upadhya, Aphrodite Chakraborty , et al. · 2025

Background Epilepsy has long been conceptualised as a disorder in which aberrant brain dynamics extend beyond the epileptogenic zone. Evidence demonstrates that loss of entropy is a generic feature of pathological dynamics in the brain, in…

Patient-derived monoclonal LGI1 autoantibodies elicit seizures, behavioral changes and brain MRI abnormalities in rodent models Open

Manoj A. Upadhya, Alexander Stumpf, Jack O'Brien-Cairney, César Cordero Gómez, Jan Henje Döring , et al. · 2025

Pediatric MOG-Ab–Associated Encephalitis Open

Nee Na Kim, Dimitrios Champsas, Michael Eyre, Omar Abdel‐Mannan, Vanessa Wan Mun Lee , et al. · 2024

MOG-Ab should be tested in all patients with suspected encephalitis even in the context of initially normal brain MRI. Although exclusion of infections should be part of the diagnostic process of any child with encephalitis, in immunocompe…

Impact of autoantibodies against myelin oligodendrocyte glycoprotein in paediatric acquired demyelinating disease: Intellectual functioning and academic performance Open

Daniel Griffiths-King, Charly Billaud, Lydiah Makusha, Ling Lynette Looi, Evangeline Wassmer , et al. · 2024

Paediatric acquired demyelinating syndromes (pADS) attack white matter pathways in the brain during an important period of development. Affected children can experience poor functional outcomes, including deficits in specific cognitive dom…

Human cerebrospinal fluid monoclonal CASPR2 autoantibodies induce changes in electrophysiology, functional MRI, and behavior in rodent models Open

Scott van Hoof, Jakob Kreye, César Cordero-Gómez, Julius Hoffmann, S. Momsen Reincke , et al. · 2024

Anti-contactin associated protein receptor 2 (CASPR2) encephalitis is a severe autoimmune encephalitis with a variable clinical phenotype including behavioral abnormalities, cognitive decline, epileptic seizures, peripheral nerve hyperexci…

Myo-inositol and total NAA in the hippocampus are linked to CSF tau pathology in cognitively normal older adults Open

JA Noche, Casey R. Vanderlip, Sukhvir Wright, Luis Sordo, Elizabeth Head , et al. · 2024

INTRODUCTION Understanding relationships between in vivo neurometabolic changes and Alzheimer’s disease (AD) pathology in the hippocampus, a region vulnerable to early changes in AD, will support early diagnosis. METHODS Two studies using …

NORSE/FIRES: how can we advance our understanding of this devastating condition? Open

Dimitrios Champsas, Xushuo Zhang, Richard Rosch, Evangelia Ioannidou, Kimberly Gilmour , et al. · 2024

Introduction New onset refractory status epilepticus (NORSE) is a rare and devastating condition characterised by the sudden onset of refractory status epilepticus (RSE) without an identifiable acute or active structural, toxic, or metabol…

Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes Open

Evangeline Wassmer, Charly Billaud, Michael Absoud, Omar Abdel‐Mannan, Christina Benetou , et al. · 2024

The risk of relapse in non-MS ADS depends on initial diagnosis, and MOG-Ab positivity. Long-term difficulties are observed regardless of relapses and are determined by presenting phenotype.

Peripherally-derived LGI1-reactive monoclonal antibodies cause epileptic seizures in vivo Open

Manoj A. Upadhya, Toni Kirmann, Max A Wilson, Christian M. Simon, Divya Dhangar , et al. · 2024

One striking clinical hallmark in patients with autoantibodies to leucine-rich glioma inactivated 1 (LGI1) is the very frequent focal seizure semiologies, including faciobrachial dystonic seizures (FBDS), in addition to the amnesia. Polycl…

Linking brain networks to cognition with magnetoencephalography in paediatric autoimmune encephalitis Open

Charly Billaud, Amanda Wood, Daniel Griffiths-King, Klaus Kessler, Evangeline Wassmer , et al. · 2024

Paediatric autoimmune encephalitis (e.g., acute disseminated encephalomyelitis, N-methyl-D-aspartate receptor antibody encephalitis) is an inflammatory brain disease that causes cognitive deficits, psychiatric symptoms, seizures, MRI, and …

Examining cognition and brain networks using magnetoencephalography in paediatric autoimmune encephalitis and acute disseminated encephalomyelitis: a preliminary study Open

Charly Hugo Alexandre Billaud, Amanda Wood, Daniel Griffiths-King, Klaus Kessler, Evangeline Wassmer , et al. · 2024

Paediatric autoimmune encephalitis, including acute disseminated encephalomyelitis, are inflammatory brain diseases presenting with cognitive deficits, psychiatric symptoms, seizures, MRI and EEG abnormalities. Despite improvements in dise…

Peripherally derived LGI1-reactive monoclonal antibodies cause epileptic seizuresin vivo Open

Manoj A. Upadhya, Toni Kirmann, Max A Wilson, Christian M. Simon, Divya Dhangar , et al. · 2023

One striking clinical hallmark in patients with autoantibodies to leucine-rich glioma inactivated 1 (LGI1) is the very frequent focal seizure semiologies, including faciobrachial dystonic seizures (FBDS), in addition to the amnesia. Polycl…

Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study Open

Saraswathy Sabanathan, Omar Abdel‐Mannan, Kshitij Mankad, Ata Siddiqui, Krishna B. Das , et al. · 2022

Objectives To describe the clinical presentation, investigations, management, and disease course in pediatric autoimmune limbic encephalitis (LE). Methods In this retrospective observational study, from the UK Childhood Neuroinflammatory D…

Encephalitis patient-derived monoclonal GABAA receptor antibodies cause epileptic seizures. Open

Jakob Kreye, Sukhvir Wright, Adriana van Casteren, Laura Stöffler, Marie-Luise Machule , et al. · 2021

Autoantibodies targeting the GABAA receptor (GABAAR) hallmark an autoimmune encephalitis presenting with frequent seizures and psychomotor abnormalities. Their pathogenic role is still not well-defined, given the common overlap with furthe…

Incidence of paediatric multiple sclerosis and other acquired demyelinating syndromes: 10‐year follow‐up surveillance study Open

Omar Abdel‐Mannan, Michael Absoud, Christina Benetou, Helga Hickson, Cheryl Hemingway , et al. · 2021

Aim To describe a 10‐year follow‐up of children (<16y) with acquired demyelinating syndromes (ADS) from a UK‐wide prospective surveillance study. Method Diagnoses were retrieved from the patients' records via the patients' paediatric or ad…

Multimodal electrophysiological analyses reveal that reduced synaptic excitatory neurotransmission underlies seizures in a model of NMDAR antibody-mediated encephalitis Open

Sukhvir Wright, Richard Rosch, Max A Wilson, Manoj A. Upadhya, Divya Dhangar , et al. · 2021

Pathogenic antibodies to AQP4: Neuromyelitis optica spectrum disorder (NMOSD) Open

Sukhvir Wright, Evangeline Wassmer, Angela Vincent · 2021

Use of Disease-Modifying Therapies in Pediatric Relapsing-Remitting Multiple Sclerosis in the United Kingdom Open

Omar Abdel‐Mannan, Celeste Manchoon, Thomas Rossor, Justine-Clair Southin, Carmen Tur , et al. · 2021

OBJECTIVES: To compare the real-world effectiveness of newer disease-modifying therapies (DMTs) vs injectables in children with relapsing-remitting multiple sclerosis (RRMS). METHODS: In this retrospective, multicenter study, from the UK C…

Encephalitis patient derived monoclonal GABAAreceptor antibodies cause catatonia and epileptic seizures Open

Jakob Kreye, Sukhvir Wright, Adriana van Casteren, Marie-Luise Machule, S. Momsen Reincke , et al. · 2021

Autoantibodies targeting the GABA A receptor (GABA A R) hallmark an autoimmune encephalitis presenting with frequent seizures and psychomotor abnormalities. Their pathogenic role is still not well-defined, given the common overlap with fur…

Use of disease-modifying therapies in pediatric relapsing remitting multiple sclerosis in the UK Open

Omar Abdel‐Mannan, Celeste Manchoon, Thomas Rossor, JC Southin, CT Gomez , et al. · 2021

In vitrocharacterisation and neurosteroid treatment of an N-Methyl-D-Aspartate receptor antibody-mediated seizure model Open

Sukhvir Wright, Richard Rosch, Max A Wilson, Manoj A. Upadhya, Divya Dhangar , et al. · 2020

Seizures are a prominent feature in N-Methyl-D-Aspartate receptor antibody (NMDAR-Ab) encephalitis, a distinct neuro-immunological disorder in which specific human autoantibodies bind and crosslink the surface of NMDAR proteins thereby cau…

Abolishing spontaneous epileptiform activity in human brain tissue through AMPA receptor inhibition Open

Sukhvir Wright, Max A Wilson, Richard Walsh, William B. Lo, Nilesh Mundil , et al. · 2020

Objective The amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid receptor (AMPAR) is increasingly recognized as a therapeutic target in drug‐refractory pediatric epilepsy. Perampanel (PER) is a non‐competitive AMPAR antagonist, and pre‐cli…

In vivo Mechanisms of Antibody-Mediated Neurological Disorders: Animal Models and Potential Implications Open

Maria Pia Giannoccaro, Sukhvir Wright, Angela Vincent · 2020

Over the last two decades, the discovery of antibodies directed against neuronal surface antigens (NSA-Abs) in patients with different forms of encephalitis has provided a basis for immunotherapies in previously undefined disorders. Nevert…

Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies Open

Snehal Surana, Ratna Kumar, Matthew Pitt, Patricia Hafner, Ailsa McLellan , et al. · 2019

Acquired neuromyotonia is a form of peripheral nerve hyperexcitability. In adults, pathogenic antibodies that target the extracellular domains of leucine‐rich glioma‐inactivated protein 1 ( LGI 1) and contactin‐associated protein‐like 2 ( …

NMDA-receptor antibodies alter cortical microcircuit dynamics Open

Richard Rosch, Sukhvir Wright, Gerald Cooray, Margarita Papadopoulou, Sushma Goyal , et al. · 2018

NMDA-receptor antibodies (NMDAR-Abs) cause an autoimmune encephalitis with a diverse range of EEG abnormalities. NMDAR-Abs are believed to disrupt receptor function, but how blocking this excitatory synaptic receptor can lead to paroxysmal…

Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease Open

Yael Hacohen, Yu Yi M. Wong, Christian Lechner, Maciej Juryńczyk, Sukhvir Wright , et al. · 2018

Although commonly used to treat patients with multiple sclerosis, DMDs were not associated with clinical improvement in children with MOG-Ab-associated disease, whereas azathioprine, mycophenolate mofetil, rituximab, and particularly intra…

NMDA-receptor antibodies alter cortical microcircuit dynamics Open

Richard Rosch, Sukhvir Wright, Gerald Cooray, Μarianna Papadopoulou, Samta Goyal , et al. · 2017

NMDA-receptor antibodies (NMDAR-Ab) cause an autoimmune encephalitis with a diverse range of electroencephalographic (EEG) abnormalities. NMDAR-Ab are believed to disrupt receptor function, but how blocking this excitatory neurotransmitter…

N‐methyl‐d‐aspartate (NMDA) receptor antibodies encephalitis mimicking an autistic regression Open

Yael Hacohen, Sukhvir Wright, Jonathan Gadian, Angela Vincent, Ming Lim , et al. · 2016

Expressive dysphasia and mutism are common clinical features in children and adults with N ‐methyl‐ d ‐aspartate receptor antibodies ( NMDAR ‐Ab) encephalitis, and are likely to result from NMDAR hypofunction. A prodromal loss of social an…

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