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To predict or not to predict: Multiple sclerosis and B-cell subset-specific genetic risk scores
Radu Tanasescu, Andrew Chan
doi : 10.1111/ene.15609
Chronic inflammatory demyelinating polyneuropathy with positive anti-myelin associated glycoprotein antibodies: Back to clinical basics
Yusuf A. Rajabally
doi : 10.1111/ene.15642
Chronic inflammatory demyelinating polyneuropathy with anti-myelin associated glycoprotein antibodies represents a small subgroup of patients for whom management may not be straightforward. A recent study offers insight into this issue, with results suggesting priority should be given to the clinical phenotype.
Severe acute respiratory syndrome coronavirus 2 vaccines and neurological diseases: Cause or coincidence?
Peter Berlit
doi : 10.1111/ene.15618
Prioritization process for European Academy of Neurology clinical practice guidelines
Katina Aleksovska, Claudio L. A. Bassetti, Thomas Berger, Vanessa Carvalho, Joao Costa, Günther Deuschl, Kristian S. Frederiksen, Joke Jaarsma, Teia Kobulashvili, Maurizio Leone, Lucia Pavlakova, Michele Romoli, Luca Vignatelli
doi : 10.1111/ene.15608
The development of high-quality clinical practice guidelines (CPGs) takes substantial time, effort, and resources. During the past years, the European Academy of Neurology (EAN) guideline production was significantly increased, so the need to develop clear, transparent, and methodologically solid criteria for prioritizing guideline topics became apparent.
The many faces of globular glial tauopathy: A clinical and imaging study
Marina Buciuc, Shunsuke Koga, Nha Trang Thu Pham, Joseph R. Duffy, David S. Knopman, Farwa Ali, Bradley F. Boeve, Jon Graff-Radford, Hugo Botha, Val J. Lowe, Aivi Nguyen, Ross R. Reichard, Dennis W. Dickson, Ronald C. Petersen, Jennifer L. Whitwell, Keith A. Josephs
doi : 10.1111/ene.15603
Globular glial tauopathy (GGT) has been associated with frontotemporal dementia syndromes; little is known about the clinical and imaging characteristics of GGT and how they differ from other non-globular glial 4-repeat tauopathies (N4GT) such as progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD).
The spectrum of functional tic-like behaviours: Data from an international registry
Davide Martino, Tammy Hedderly, Tara Murphy, Kirsten R. Müller-Vahl, Russell C. Dale, Donald L. Gilbert, Renata Rizzo, Andreas Hartmann, Peter Nagy, Mathieu Anheim, Tamsin Owen, Osman Malik, Morvwen Duncan, Isobel Heyman, Holan Liang, Andrew McWilliams, Shauna O'Dwyer, Carolin Fremer, Natalia Szejko, Velda X. Han, Kasia Kozlowska, Tamara M. Pringsheim
doi : 10.1111/ene.15611
Between 2019 and 2022, there was a marked rise in adolescents/young adults seeking urgent help for functional tic-like behaviours (FTLBs). Given the global scale of this phenomenon, we aimed to pool cases from different institutions in an international registry to better characterize this spectrum and facilitate future longitudinal observation.
Dual-phase 18F-FP-CIT positron emission tomography and cardiac 123I-MIBG scintigraphy of Parkinson's disease patients with GBA mutations: evidence of the body-first type?
Min Seung Kim, Don Gueu Park, Young-Sil An, Jung Han Yoon
doi : 10.1111/ene.15615
Parkinson's disease (PD) with glucocerebrosidase (GBA) gene mutation (GBA-PD) is known to show more rapid clinical progression than sporadic PD without GBA mutation (sPD). This study was performed to delineate the specific patterns of cortical hypoperfusion, dopamine transporter uptake and cardiac meta-iodobenzylguanidine (MIBG) uptake of GBA-PD in comparison to sPD.
Ischaemic stroke in South Asians: The BRAINS study
Taylor Aurelius, Ankita Maheshwari, Gie Ken-Dror, Sapna D. Sharma, Sageet Amlani, Gunaratnam Gunathilagan, David L. Cohen, Chakravarthi Rajkumar, Stuart Maguire, Sissi Ispoglou, Ibrahim Balogun, Anthea Parry, Lakshmanan Sekaran, Hafiz Syed, Enas Lawrence, Ravneeta Singh, Ahamad Hassan, Chris Wharton, Khalid Javaid, Neetish Goorah, Peter Carr, Eman Abdus Sami, Shri Ram Sharma, Padmavathy N. Sylaja, Kameshwar Prasad, Pankaj Sharma, the BRAINS collaborators
doi : 10.1111/ene.15605
Studies on stroke in South Asian populations are sparse. The aim of this study was to compare differences in age of onset of ischaemic stroke in South Asian patients living in the United Kingdom and South Asian patients living in India versus White British stroke patients.
Direct current stimulation increases blood flow and permeability of cortical microvasculature in vivo
Anne-Kathrin Gellner, Sibylle Frase, Janine Reis, Brita Fritsch
doi : 10.1111/ene.15616
Transcranial direct current stimulation (DCS) structurally and functionally modulates neuronal networks and microglia dynamics. Neurovascular coupling adapts regional cerebral blood flow to neuronal activity and metabolic demands.
The location of intraparenchymal bleeding determines functional outcome after spontaneous subarachnoid hemorrhage
Anna Lindner1 | Stefan Kunst1 | Bogdan-Andrei Ianosi1,2 | Verena Rass1 | Alois Josef Schiefecker1 | Mario Kofler1 | Victoria Limmert1 | Astrid E. Grams 3 | Bettina Pfausler1 | Ronny Beer1 | Claudius Thomé 4 | Raimund Helbok
doi : 10.1111/ene.15621
Non-traumatic subarachnoid hemorrhage (SAH) is a devastat- ing disease associated with high morbidity and mortality. A higher blood burden and the presence of intraparenchymal extension of the bleeding (intracerebral hemorrhage [ICH]) are well known predictors of poor outcome.
Etiologic and prognostic value of external carotid artery thrombus detection during endovascular therapy for anterior circulation proximal occlusions
Thomas Courret, Thomas Tourdias, Jean Papaxanthos, Julien Labreuche, Florent Gariel, Jean-Sebastien Liegey, Stephane Olindo, Pauline Renou, Jerome Berge, Xavier Barreau, Sharmila Sagnier, Patrice Menegon, Ludovic Lucas, Pierre Briau, Mathilde Poli, Sabrina Debruxelles, François Rouanet, Vincent Dousset, Igor Sibon, Gaultier Marnat
doi : 10.1111/ene.15623
An early understanding of stroke mechanism may improve treatment and outcome in patients presenting with large vessel occlusion stroke (LVOS) treated with mechanical thrombectomy (MT). We aimed to investigate whether spontaneous external carotid artery (ECA) embolism detection during MT is associated with stroke etiology and clinical outcome.
Small intracranial aneurysms of the anterior circulation: A negligible risk?
Thiemo Florin Dinger, Jonas Peschke, Mehdi Chihi, Meltem Gümüs, Maryam Said, Alejandro Nicolas Santos, Jan Rodemerk, Anna Michel, Marvin Darkwah Oppong, Yan Li, Cornelius Deuschl, Karsten Henning Wrede, Philipp René Dammann, Benedikt Frank, Christoph Kleinschnitz, Michael Forsting, Ulrich Sure, Ramazan Jabbarli
doi : 10.1111/ene.15625
According to the International Study of Unruptured Intracranial Aneurysms, small (<7 mm) unruptured intracranial aneurysms (IAs) of the anterior circulation (aC) carry a neglectable 5-year rupture risk.
Factors associated with the severity of COVID-19 outcomes in people with neuromuscular diseases: Data from the International Neuromuscular COVID-19 Registry
Chiara Pizzamiglio, Robert D. S. Pitceathly, Michael P. Lunn, Stefen Brady, Fabiola De Marchi, Lucia Galan, Jeannine M. Heckmann, Alejandro Horga, Maria J. Molnar, Acary S. B. Oliveira, Wladimir B. V. R. Pinto, Guido Primiano, Ernestina Santos, Benedikt Schoser, Serenella Servidei, Paulo V. Sgobbi Souza, Vishnu Venugopalan, Michael G. Hanna, Mazen M. Dimachkie, Pedro M. Machado, for the Neuromuscular Diseases and COVID-19 Study Group
doi : 10.1111/ene.15613
Clinical outcome information on patients with neuromuscular diseases (NMDs) who have been infected with SARS-CoV-2 is limited. The aim of this study was to determine factors associated with the severity of COVID-19 outcomes in people with NMDs.
Comparative features and outcomes of major neurological complications of COVID-19
Ettore Beghi, Elena Moro, Eugenia Irene Davidescu, Bogdan Ovidiu Popescu, Oxana Grosu, Franco Valzania, Maria Sofia Cotelli, Gordana Kiteva-Trenchevska, Maria Zakharova, Tibor Kovács, Carmel Armon, Waldemar Brola, Clarissa Lin Yasuda, LuÃÂs F. Maia, Arijana Lovrencic-Huzjan, Mafalda Maria Laracho de Seabra, Rafael Avalos-Pavon, Anne Hege Aamodt, Sara Meoni, Victoria Gryb, Serefnur Ozturk, Omer Karadas, Ingomar Krehan, Maurizio A. Leone, Maria Lolich, Elisa Bianchi, Verena Rass, Raimund Helbok, Claudio L. A. Bassetti, the ENERGY Study Group
doi : 10.1111/ene.15617
The aim of this study was to assess the neurological complications of SARS-CoV-2 infection and compare phenotypes and outcomes in infected patients with and without selected neurological manifestations.
Multiple sclerosis risk variants influence the peripheral B-cell compartment early in life in the general population
Casper L. de Mol, Marvin M. van Luijn, Karim L. Kreft, Kirsten I. M. Looman, Menno C. van Zelm, Tonya White, Henriette A. Moll, Joost Smolders, Rinze F. Neuteboom
doi : 10.1111/ene.15582
Multiple sclerosis (MS) is associated with abnormal B-cell function, and MS genetic risk alleles affect multiple genes that are expressed in B cells. However, how these genetic variants impact the B-cell compartment in early childhood is unclear.
Baseline retinal nerve fiber layer thickness as a predictor of multiple sclerosis progression: New insights from the FREEDOMS II study
Liang Wang, Hongmei Tan, Jian Yu, Jingzi ZhangBao, Wenjuan Huang, Xuechun Chang, Lei Zhou, Chuanzhen Lu, Yiqin Xiao, Jiahong Lu, Chongbo Zhao, Min Wang, Xue Wu, Mengyun Wu, Qiang Dong, Kok Yew Ngew, Chao Quan
doi : 10.1111/ene.15612
The aim was to evaluate the potential of retinal nerve fiber layer thickness (RNFLT) measured with optical coherence tomography in predicting disease progression in relapsing–remitting multiple sclerosis (RRMS).
Early spinal cord pseudoatrophy in interferon-beta-treated multiple sclerosis
Britta Matusche, Ludmila Litvin, Ruth Schneider, Barbara Bellenberg, Mark Mühlau, Viola Pongratz, Achim Berthele, Sergiu Groppa, Muthuraman Muthuraman, Frauke Zipp, Friedemann Paul, Heinz Wiendl, Sven G. Meuth, Philipp Sämann, Frank Weber, Ralf A. Linker, Tania Kümpfel, Ralf Gold, Carsten Lukas
doi : 10.1111/ene.15620
Brain pseudoatrophy has been shown to play a pivotal role in the interpretation of brain atrophy measures during the first year of disease-modifying therapy in multiple sclerosis. Whether pseudoatrophy also affects the spinal cord remains unclear.
Neurological autoimmune diseases following vaccinations against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2): A follow-up study
Sofia Doubrovinskaia, Christoph M. Mooshage, Corinna Seliger, Hanns-Martin Lorenz, Simon Nagel, Pascal Lehnert, Jan Purrucker, Brigitte Wildemann, Martin Bendszus, Wolfgang Wick, Silvia Schönenberger, Leon D. Kaulen
doi : 10.1111/ene.15602
Population-based studies suggest severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines may trigger neurological autoimmunity including immune-mediated thrombotic thrombocytopenia. Long-term characterization of cases is warranted to facilitate patient care and inform vaccine-hesitant individuals.
Clinical characteristics, treatments, outcome, and prognostic factors of severe autoimmune encephalitis in the intensive care unit: Standard treatment and the value of additional plasma cell–depleting escalation therapies for treatment-refractory patients
Lisa Schwarz, Nilufar Akbari, Harald Prüss, Andreas Meisel, Franziska Scheibe
doi : 10.1111/ene.15585
To investigate severe autoimmune encephalitis (AE) in the intensive care unit (ICU) with regard to standard treatment in responsive patients and additional escalation therapies for treatment-refractory cases.
Immune-mediated diseases involving central and peripheral nervous systems
Aurelie Leboyan, Florence Esselin, Anne-Laure Bascou, Claire Duflos, Ioana Ion, Mahmoud Charif, Giovanni Castelnovo, Clarisse Carra-Dalliere, Xavier Ayrignac, Philippe Kerschen, Mohamed Chbicheb, Ludovic Nguyen, Alexandre T. J. Maria, Philippe Guilpain, Mathilde Carriere, Nicolas Menjot de Champfleur, Thierry Vincent, Alexandre Jentzer, Pierre Labauge, Jérôme J. Devaux, Guillaume Taieb
doi : 10.1111/ene.15628
In addition to combined central and peripheral demyelination, other immune diseases could involve both the central nervous system (CNS) and peripheral nervous system (PNS).
A diagnostic score for anti-myelin-associated-glycoprotein neuropathy or chronic inflammatory demyelinating polyradiculoneuropathy in patients with anti-myelin-associated-glycoprotein antibody
Pietro E. Doneddu, Marta Ruiz, Elisa Bianchi, Giuseppe Liberatore, Fiore Manganelli, Dario Cocito, Giuseppe Cosentino, Luana Benedetti, Girola A. Marfia, Massimiliano Filosto, Chiara Briani, Claudia Giannotta, Eduardo Nobile-Orazio
doi : 10.1111/ene.15296
A diagnostic score was developed to discriminate anti-myelin-associated-glycoprotein (MAG) neuropathy from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and applied it to patients with atypical anti-MAG neuropathy.
Mutations in MYO9B are associated with Charcot–Marie–Tooth disease type 2 neuropathies and isolated optic atrophy
Silvia Cipriani, Marta Guerrero-Valero, Stefano Tozza, Edward Zhao, Veith Vollmer, Danique Beijer, Matt Danzi, Cristina Rivellini, Dejan Lazarevic, Giovanni Battista Pipitone, Bianca Rose Grosz, Costanza Lamperti, Stefania Bianchi Marzoli, Paola Carrera, Marcella Devoto, Chiara Pisciotta, Davide Pareyson, Marina Kennerson, Stefano C. Previtali, Stephan Zuchner, Steven S. Scherer, Fiore Manganelli, Martin Bähler, Alessandra Bolino
doi : 10.1111/ene.15601
Charcot–Marie–Tooth disease (CMT) is a heterogeneous group of disorders caused by mutations in at least 100 genes. However, approximately 60% of cases with axonal neuropathies (CMT2) still remain without a genetic diagnosis. We aimed at identifying novel disease genes responsible for CMT2.
Subclinical peripheral neuropathy is common in neuronal intranuclear inclusion disease with dominant encephalopathy
Daojun Hong, Hui Wang, Min Zhu, Yun Peng, Pengcheng Huang, Yilei Zheng, Meng Yu, Lingchao Meng, Fan Li, Jiaxi Yu, Meihong Zhou, Jianwen Deng, Zhaoxia Wang, Yun Yuan
doi : 10.1111/ene.15606
Neuronal intranuclear inclusion disease (NIID) is associated with CGG repeat expansion in the NOTCH2NLC gene. Although pure or dominant peripheral neuropathy has been described as a subtype of NIID in a few patients, most NIID patients predominantly show involvements of the central nervous system (CNS). It is necessary to further explore whether these patients have subclinical peripheral neuropathy.
High-dose oral glutamine supplementation reduces elevated glutamate levels in cerebrospinal fluid in patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome
MarÃÂa Paz Guerrero-Molina, Montserrat Morales-Conejo, Aitor Delmiro, MarÃÂa Morán, Cristina DomÃÂnguez-González, Elena Arranz-Canales, Ana Ramos-González, JoaquÃÂn Arenas, Miguel A. MartÃÂn, Jesús González de la Aleja
doi : 10.1111/ene.15626
Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a genetically heterogeneous disorder caused by mitochondrial DNA mutations.
Scale for the assessment and rating of ataxia: Age-dependent performance of healthy adults
Marcus Grobe-Einsler, Alina Schmidt, Tamara Schaprian, Ina R. Vogt, Thomas Klockgether
doi : 10.1111/ene.15596
The Scale for Assessment and Rating of Ataxia (SARA) is a widely used clinical scale. The objective was to study the age dependence of SARA in healthy adults and to define age-specific cut-off values to differentiate healthy from ataxic individuals.
Comparison between PFN1 and SOD1 mutations in amyotrophic lateral sclerosis
Philippe Corcia, Pascal Lejeune, Patrick Vourc'h, Stephane Beltran, Anne-Sophie Piegay, Helene Blasco, Vincent Meininger
doi : 10.1111/ene.15583
The objective of this study was to characterize the prototypical phenotype of patients with amyotrophic lateral sclerosis (ALS) associated with PFN1 mutations in profilin 1 (PFN1) and to determine clinical indications to test for mutations in this gene.
The first European Academy of Neurology (EAN) Science School: shaping the next generation of translational neurologists
Alan Cronemberger Andrade, David Raphael Schreier, Lucas Barea-Moya, Patricia Ribeiro Faustino
doi : 10.1111/ene.15614
Comment on “Association between depression and motoric cognitive risk syndrome among community-dwelling older adults in China: A 4-year prospective cohort study�
Mehmet Ilkin Naharci, Ilker Tasci
doi : 10.1111/ene.15595
Mass “psychogenic transient loss of consciousness� after COVID-19 vaccination
Diogo Reis Carneiro, Rui Araújo
doi : 10.1111/ene.15639
Hindsight for ultrasound imaging/measurement of the tibial nerve
Vincenzo Ricci, Orhan Güvener, Levent Özçakar
doi : 10.1111/ene.15622
Hindsight for ultrasound imaging/measurement of the tibial nerve (answer)
Roshan Dhanapalaratnam, Tushar Issar, Arun V. Krishnan
doi : 10.1111/ene.15619
