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Long-term consequences of subarachnoid haemorrhage: Is it time for new strategies?
Alessandro Pezzini
doi : 10.1111/ene.15580
The borderland of multiple sclerosis and functional neurological disorder: A call for clinical research and vigilance
Selma Aybek, Andrew Chan
doi : 10.1111/ene.15568
Functional neurological disorders (FNDs) have attracted much attention from the neurological medical community over the last decades as new developments in neurosciences have reduced stigma around these by showing brain network dysfunctions.
Diagnosis and treatment of progressive multiple sclerosis: A position paper
Carlo Pozzilli, Maura Pugliatti, Patrick Vermersch, Nikolaos Grigoriadis, Mona Alkhawajah, Laura Airas, Celia Oreja-Guevara
doi : 10.1111/ene.15593
Multiple sclerosis (MS) is an unpredictable disease characterised by a highly variable disease onset and clinical course. Three main clinical phenotypes have been described.
Stratifying sudden death risk in adults with drug-resistant focal epilepsy: The SUDEP-CARE score
Chris Serrand, Sylvain Rheims, Marie Faucanié, Arielle Crespel, Vera Dinkelacker, William Szurhaj, Arnaud Biraben, Fabrice Bartolomei, Nathalie de Grissac, Elizabeth Landré, Marie Denuelle, Laurent Vercueil, Cécile Marchal, Louis Maillard, Philippe Derambure, Sophie Dupont, Vincent Navarro, Thibault Mura, Audrey Jaussent, Valérie Macioce, Philippe Ryvlin, Marie-Christine Picot
doi : 10.1111/ene.15566
A clinical risk score for sudden unexpected death in epilepsy (SUDEP) in patients with drug-resistant focal epilepsy could help improve prevention.
Magnetic resonance imaging Locus Coeruleus abnormality in amnestic Mild Cognitive Impairment is associated with future progression to dementia
Alessandro Galgani, Francesco Lombardo, Nicola Martini, Andrea Vergallo, Luca Bastiani, Harald Hampel, Hana Hlavata, Filippo Baldacci, Gloria Tognoni, Daniele De Marchi, Irene Ghicopulos, Sara De Cori, Francesca Biagioni, Carla Letizia Busceti, Roberto Ceravolo, Ubaldo Bonuccelli, Dante Chiappino, Gabriele Siciliano, Francesco Fornai, Nicola Pavese, Filippo Sean Giorgi
doi : 10.1111/ene.15556
Human neuropathological studies indicate that the pontine nucleus Locus Coeruleus (LC) undergoes significant and early degeneration in Alzheimer's disease. This line of evidence alongside experimental data suggests that the LC functional/structural decay may represent a critical factor for Alzheimer's disease pathophysiological and clinical progression
Mild behavioral impairment as a potential marker of predementia risk states in motor neuron diseases
Pilar M. Ferraro, Ester Gervino, Emiliano De Maria, Giuseppe Meo, Marta Ponzano, Matteo Pardini, Alessio Signori, Angelo Schenone, Luca Roccatagliata, Claudia Caponnetto
doi : 10.1111/ene.15570
Mild behavioral impairment (MBI) has been increasingly regarded as the neurobehavioral axis of predementia risk states, but a specific investigation of its detection as a potential marker of prodromal dementia in motor neuron diseases (MNDs) is still lacking.
Lower hypothalamic volume with lower body mass index is associated with shorter survival in patients with amyotrophic lateral sclerosis
Jeryn Chang, Thomas B. Shaw, Cory J. Holdom, Pamela A. McCombe, Robert D. Henderson, Jurgen Fripp, Markus Barth, Christine C. Guo, Shyuan T. Ngo, Frederik J. Steyn, For the Alzheimer's Disease Neuroimaging Initiative
doi : 10.1111/ene.15589
Weight loss in patients with amyotrophic lateral sclerosis (ALS) is associated with faster disease progression and shorter survival. Decreased hypothalamic volume is proposed to contribute to weight loss due to loss of appetite and/or hypermetabolism.
Effect of RNS60 in amyotrophic lateral sclerosis: a phase II multicentre, randomized, double-blind, placebo-controlled trial
Ettore Beghi, Elisabetta Pupillo, Elisa Bianchi, Valentina Bonetto, Silvia Luotti, Laura Pasetto, Caterina Bendotti, Massimo Tortarolo, Francesca Sironi, Laura Camporeale, Alexander V. Sherman, Sabrina Paganoni, Ada Scognamiglio, Fabiola De Marchi, Paolo Bongioanni, Renata Del Carratore, Claudia Caponnetto, Luca Diamanti, Daniele Martinelli, Andrea Calvo, Massimiliano Filosto, Alessandro Padovani, Stefano Cotti Piccinelli, Claudia Ricci, Stefania Dalla Giacoma, Nicoletta De Angelis, Maurizio Inghilleri, Rossella Spataro, Vincenzo La Bella, Giancarlo Logroscino, Christian Lunetta, Claudia Tarlarini, Jessica Mandrioli, Ilaria Martinelli, Cecilia Simonini, Elisabetta Zucchi, Maria Rosaria Monsurrò, Dario Ricciardi, Francesca Trojsi, Nilo Riva, Massimo Filippi, Isabella Laura Simone, Gianni Sorarù, Cristina Spera, Lucia Florio, Sonia Messina, Massimo Russo, Gabriele Siciliano, Amelia Conte, Maria Valeria Saddi, Nicola Carboni, Letizia Mazzini, the RNS60-ALS Study Group
doi : 10.1111/ene.15573
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with limited treatment options. RNS60 is an immunomodulatory and neuroprotective investigational product that has shown efficacy in animal models of ALS and other neurodegenerative diseases. Its administration has been safe and well tolerated in ALS subjects in previous early phase trials.
Mutation-specific metabolic profiles in presymptomatic amyotrophic lateral sclerosis
Kailin Xia, Simon Witzel, Christina Witzel, Veronika Klose, Dongsheng Fan, Albert C. Ludolph, Johannes Dorst
doi : 10.1111/ene.15584
Growing evidence shows that ALS patients feature a disturbed energy metabolism. However, these features have rarely been investigated in the presymptomatic stage.
How resistant are levodopa-resistant axial symptoms? Response of freezing, posture, and voice to increasing levodopa intestinal infusion rates in Parkinson disease
Gabriele Imbalzano, Domiziana Rinaldi, Giovanna Calandra-Buonaura, Manuela Contin, Federica Amato, Giulia Giannini, Luisa Sambati, Claudia Ledda, Alberto Romagnolo, Gabriella Olmo, Pietro Cortelli, Maurizio Zibetti, Leonardo Lopiano, Carlo Alberto Artusi
doi : 10.1111/ene.15558
Treatment of freezing of gait (FoG) and other Parkinson disease (PD) axial symptoms is challenging. Systematic assessments of axial symptoms at progressively increasing levodopa doses are lacking. We sought to analyze the resistance to high levodopa doses of FoG, posture, speech, and altered gait features presenting in daily-ON therapeutic condition.
Association of cholesterol level with dopamine loss and motor deficits in Parkinson disease: A cross-sectional study
Seong Ho Jeong, Hye Sun Lee, Seok Jong Chung, Han Soo Yoo, Jin Ho Jung, Kyoungwon Baik, Jong Sam Baik, Young H. Sohn, Phil Hyu Lee
doi : 10.1111/ene.15592
Cholesterol is vital in neuronal function; however, the influence of cholesterol levels on parkinsonism is unclear. This study investigated the relationship between baseline total cholesterol (TC) levels, dopamine loss, and motor symptoms in drug-naïve Parkinson disease (PD).
Genetic variation in NFE2L2 is associated with outcome following aneurysmal subarachnoid haemorrhage
Ben Gaastra, Poppy Duncan, Mark K. Bakker, Isabel C. Hostettler, Varinder S. Alg, Henry Houlden, Ynte M. Ruigrok, Ian Galea, Will Tapper, David Werring, Diederik Bulters
doi : 10.1111/ene.15571
Nuclear factor erythroid 2-related factor 2 (NRF2; encoded by the NFE2L2 gene) has been implicated in outcome following aneurysmal subarachnoid haemorrhage (aSAH) through its activity as a regulator of inflammation, oxidative injury and blood breakdown product clearance. The aim of this study was to identify whether genetic variation in NFE2L2 is associated with clinical outcome following aSAH.
Clinical presentation and neural correlates of stroke-associated spatial delusions
Pedro N. Alves, Ana C. Fonseca, Teresa Pinho-e-Melo, Isabel P. Martins
doi : 10.1111/ene.15557
Incongruent beliefs about self-localization in space markedly disturb patients' behavior. Spatial delusions, or reduplicative paramnesias, are characterized by a firm conviction of place reduplication, transformation, or mislocation. Evidence suggests they are frequent after right hemisphere lesions, but comprehensive information about their clinical features is lacking.
Incidence and association of seizures in stroke patients following endovascular treatment: A systematic review and meta-analysis
Fangzhou Liu, Deng Chen, Yaoqi Fu, Haijiao Wang, Ling Liu
doi : 10.1111/ene.15564
Post-stroke seizures (PSSs) are some of the most common complications of stroke and are associated with poor outcomes in patients. Endovascular treatment (EVT) is the standard of care for patients with acute ischaemic stroke related large-vessel occlusion. However, whether EVT increases the risk of PSSs remains controversial; the association between PSSs and EVT is poorly understood.
Central nervous system cavernous malformations: cross-sectional study assessing rebleeding risk after a second haemorrhage
Alejandro N. Santos, Laurèl Rauschenbach, Hanah Hadice Gull, Angelina Olbrich, Kirstin Lahl, Marvin Darkwah Oppong, Thiemo F. Dinger, Christoph Rieß, Bixia Chen, Annika Lenkeit, Börge Schmidt, Yan Li, Ramazan Jabbarli, Karsten H. Wrede, Ulrich Sure, Philipp Dammann
doi : 10.1111/ene.15574
The purpose of this study was to investigate the 5-year risk of a third bleeding event in cavernous malformations (CMs) of the central nervous system.
Impact of edema formation on functional outcome in pediatric stroke patients
Peter B. Sporns, Thilo Rusche, Sarah Lee, Uta Hanning, Lukas Meyer, Tobias Faizy, Jens Fiehler, Marios Psychogios, Andre Kemmling, Gabriel Broocks
doi : 10.1111/ene.15576
Quantitative lesion net water uptake (NWU) has been described as an imaging biomarker reflecting vasogenic edema as an early indicator of infarct progression. We hypothesized that edema formation measured by NWU is higher in children compared to adults but despite this functional outcome may be better in children.
The effect of head-down tilt in experimental acute ischemic stroke
Zi-Ai Zhao, Nan-Nan Zhang, Yu Cui, Hui-Sheng Chen
doi : 10.1111/ene.15597
Collateral therapeutics exert a promising protective effect on the outcome of acute ischemic stroke. Cerebral blood flow (CBF) may be modulated by different head positioning. The current study aimed to determine the effect of head-down tilt (HDT) on stroke in a rodent model.
Pregnancy outcomes after early fetal exposure to injectable first-line treatments, dimethyl fumarate, or natalizumab in Danish women with multiple sclerosis
Johanna Balslev Andersen, Finn Sellebjerg, Melinda Magyari
doi : 10.1111/ene.15559
Data on pregnancy outcomes following fetal exposure to disease-modifying drugs (DMDs) in women with multiple sclerosis (MS) are sparse although growing.
Correlation between patient-reported manual ability and three objective measures of upper limb function in people with multiple sclerosis
Claudio Solaro, Rachele Di Giovanni, Erica Grange, Giampaolo Brichetto, Margit Mueller, Andrea Tacchino, Rita Bertoni, Francesco Patti, Angelo Pappalardo, Luca Prosperini, Letizia Castelli, Rosalba Rosato, Davide Cattaneo, Davide Marengo
doi : 10.1111/ene.15560
Upper limb (UL) function is often affected in people with multiple sclerosis (PwMS) and is typically assessed through objective measures, including the Nine Hole Peg Test (9-HPT), Box and Block Test (BBT), and Hand Grip Strength (HGS).
Reduction in grey matter atrophy in patients with relapsing multiple sclerosis following treatment with cladribine tablets
Rosa Cortese, Marco Battaglini, Maria Pia Sormani, Ludovico Luchetti, Giordano Gentile, Maira Inderyas, Nektaria Alexandri, Nicola De Stefano
doi : 10.1111/ene.15579
Measures of atrophy in the whole brain can be used to reliably assess treatment effect in clinical trials of patients with multiple sclerosis (MS). Trials assessing the effect of treatment on grey matter (GM) and white matter (WM) atrophy are very informative, but hindered by technical limitations.
Serum neurofilament-light and glial fibrillary acidic protein levels in hydroxychloroquine-treated primary progressive multiple sclerosis
Carlos Camara-Lemarroy, Claudia Silva, Jit Gohill, V. Wee Yong, Marcus Koch
doi : 10.1111/ene.15588
In a recent trial, hydroxychloroquine (HCQ) treatment reduced the expected rate of disability worsening at 18 months in primary progressive multiple sclerosis (PPMS). Neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) are emerging biomarkers in multiple sclerosis.
Batoclimab as an add-on therapy in neuromyelitis optica spectrum disorder patients with acute attacks
Yuge Wang, Xiaonan Zhong, Honghao Wang, Yu Peng, Fudong Shi, Dongmei Jia, Huan Yang, Qiuming Zeng, Chao Quan, Jingzi ZhangBao, Michael Lee, Jun Qi, Xiaoxiang Chen, Wei Qiu, Batoclimab NMOSD Study Group
doi : 10.1111/ene.15561
Neuromyelitis optica spectrum disorder (NMOSD) is a severe neurological inflammatory disease mainly caused by pathogenic aquaporin-4 antibodies (AQP4-IgG). The safety and efficacy of the neonatal Fc receptor antagonist batoclimab addition to conventional intravenous methylprednisolone pulse (IVMP) therapy in patients with NMOSD acute attacks was assessed
Electroencephalography functional connectivity�A biomarker for painful polyneuropathy
Leah Shafran Topaz, Alex Frid, Yelena Granovsky, Rabab Zubidat, Shoshana Crystal, Chen Buxbaum, Noam Bosak, Rafi Hadad, Erel Domany, Tayir Alon, Lian Meir Yalon, Merav Shor, Mogher Khamaisi, Irit Hochberg, Nataliya Yarovinsky, Zeev Volkovich, David L. Bennett, David Yarnitsky
doi : 10.1111/ene.15575
Advanced analysis of electroencephalography (EEG) data has become an essential tool in brain research. Based solely on resting state EEG signals, a data-driven, predictive and explanatory approach is presented to discriminate painful from non-painful diabetic polyneuropathy (DPN) patients.
Shedding light on motor premanifest myotonic dystrophy type 1: A molecular, muscular and central nervous system follow-up study
Joana Garmendia, Garazi Labayru, Miren Zulaica, Jorge Villanúa, Adolfo López de Munain, Andone Sistiaga
doi : 10.1111/ene.15604
Myotonic dystrophy type 1 (DM1) is a hereditary and multisystemic disease that is characterized by heterogeneous manifestations. Although muscular impairment is central to DM1, a premanifest DM1 form has been proposed for those characterized by the absence of muscle signs in precursory phases. Nevertheless, subtle signs and/or symptoms related to other systems, such as the central nervous system (CNS), may emerge and progress gradually. This study aimed to validate the premanifest DM1 concept and to characterize and track affected individuals from a CNS centred perspective.
Maintenance of response and predictive factors of 1-year GalcanezumAb treatment in real-life migraine patients in Italy: The multicenter prospective cohort GARLIT study
Fabrizio Vernieri, Nicoletta Brunelli, Marilena Marcosano, Cinzia Aurilia, Gabriella Egeo, Carlo Lovati, Valentina Favoni, Armando Perrotta, Ilaria Maestrini, Renata Rao, Luigi d'Onofrio, Cinzia Finocchi, Marco Aguggia, Francesco Bono, Angelo Ranieri, Maria Albanese, Vittorio Di Piero, Sabina Cevoli, Claudia Altamura, Piero Barbanti, for the GARLIT Study Group
doi : 10.1111/ene.15563
To evaluate the 1-year effectiveness and tolerability of galcanezumab in real life and the prognostic indicators of persistent response.
Cerebrospinal fluid biomarkers of superficial siderosis in patients with spontaneous intracranial hypotension
Levin Häni, Christian Fung, Christopher Marvin Jesse, Christof Schild, Eike Immo Piechowiak, Tomas Dobrocky, Andreas Raabe, Jürgen Beck
doi : 10.1111/ene.15591
Spontaneous intracranial hypotension (SIH) is an important etiology of infratentorial superficial siderosis (iSS) of the central nervous system. However, the prevalence of iSS amongst patients with SIH is unknown and the imaging findings of iSS might represent a late stage of disease. The aim was to identify cerebrospinal fluid (CSF) biomarkers of iSS in patients with SIH.
Telemedicine for neurological diseases: A systematic review and meta-analysis
Beatriz León-Salas, Yadira González-Hernández, Diego Infante-Ventura, Aythami de Armas-Castellano, Javier GarcÃÂa-GarcÃÂa, Miguel GarcÃÂa-Hernández, Montserrat Carmona-RodrÃÂguez, Javier Olazarán, José Luis Dobato, Leticia RodrÃÂguez-RodrÃÂguez, MarÃÂa M. Trujillo-MartÃÂn
doi : 10.1111/ene.15599
The aim was to systematically review the effectiveness and safety of telemedicine combined with usual care (in-person visits) compared to usual care for the therapeutic management and follow-up assessment of neurological diseases.
Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis
Andreas Liampas, Konstantinos Parperis, Maria Faidra Erotocritou, Antonios Nteveros, Marianna Papadopoulou, Christos Moschovos, Mohammed Akil, Stefano Coaccioli, Georgios M. Hadjigeorgiou, Marios Hadjivassiliou, Panagiotis Zis
doi : 10.1111/ene.15555
Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement.
Role of autoantibody levels as biomarkers in the management of patients with myasthenia gravis: A systematic review and expert appraisal
Andreas Meisel, Fulvio Baggi, Anthony Behin, Amelia Evoli, Anna Kostera-Pruszczyk, Renato Mantegazza, Raul Juntas Morales, Anna Rostedt Punga, Sabrina Sacconi, Michael Schroeter, Jan Verschuuren, Louise Crathorne, Kris Holmes, Maria-Isabel Leite
doi : 10.1111/ene.15565
Although myasthenia gravis (MG) is recognized as an immunoglobulin G autoantibody-mediated disease, the relationship between autoantibody levels and disease activity in MG is unclear. We sought to evaluate this landscape through systematically assessing the evidence, testing the impact of predefined variables on any relationship, and augmenting with expert opinion.
Reply to the Letter to the Editor in response to “Integrated evaluation of a panel of neurochemical biomarkers to optimize diagnosis and prognosis in amyotrophic lateral sclerosis�
Nilo Riva, Angelo Quattrini
doi : 10.1111/ene.15586
“Integrated evaluation of a panel of neurochemical biomarkers to optimize diagnosis and prognosis in amyotrophic lateral sclerosis�
Ali Aamir, Hussain Haider Shah, Tehreem Mansure
doi : 10.1111/ene.15590
Hearing impairment and development of parkinsonism and possible rapid eye movement sleep behaviour disorder: A CLSA prospective population-based study
Chun W. Yao, Aliya Szpindel, Amélie Pelletier, Ronald B. Postuma
doi : 10.1111/ene.15567
Letter to the editor re “Minor stroke in large vessel occlusion: A matched analysis of patients from the German Stroke Registry�Endovascular Treatment and patients from the Safe Implementation of Treatments in Stroke�International Stroke Thrombolysis Register�
Ajay Malhotra, Mihir Khunte, Xiao Wu, Mahla Radmard
doi : 10.1111/ene.15598
Response to the letter to the editor regarding “Minor stroke in large vessel occlusion: A matched analysis of patients from the German Stroke Registry–Endovascular Treatment (GSR-ET) and patients from the Safe Implementation of Treatments in Stroke–International Stroke Thrombolysis Register (SITS-ISTR)�
Katharina Feil, Marius Matusevicius, Niaz Ahmed, Lars Kellert
doi : 10.1111/ene.15600
European Psychiatric Association–European Academy of Neurology statement on post-COVID syndrome
Claudio L. A. Bassetti, Raimund Helbok, Kristina Adorjan, Peter Falkai
doi : 10.1111/ene.15572
Secondary hypokalemic periodic paralysis in Crohn disease: Two case reports
Francisco de Assis Aquino Gondim, Antônio Miguel Furtado Leitão, José Marcelino Aragão Fernandes, Davi Farias de Araújo, Marcellus Henrique Loiola Ponte Souza, Lúcia Libanez Bessa Campelo Braga
doi : 10.1111/ene.15610
