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Journal of Neurology OnlineFirst articles

Open Access 18.05.2024 | Original Communication

Validation of a risk score to differentiate autoimmune and viral encephalitis: a Nationwide Cohort Study in Denmark

Encephalitis is a rare but severe clinical condition with a substantial mortality and a high risk of neurological sequelae [ 1 – 3 ]. Early treatment initiation is paramount for optimized patient outcomes, but determining the aetiology is often …

verfasst von:
Lasse Fjordside, Mette Scheller Nissen, Anna Maria Florescu, Merete Storgaard, Lykke Larsen, Lothar Wiese, Hans Rudolf von Lüttichau, Micha Phill Grønholm Jepsen, Birgitte Rønde Hansen, Christian Østergaard Andersen, Jacob Bodilsen, Henrik Nielsen, Morten Blaabjerg, Anne-Mette Lebech, Helene Mens

Open Access 18.05.2024 | Review

Overcoming traps and pitfalls leading to misinterpretation of normal EEG variants and variation of the background activity

Normal EEG variants, especially the epileptiform variants, can be challenging to interpret because they often have sharp contours and may be confused with “epileptic” interictal activities. However, they can be recognized by the fact that “most …

verfasst von:
Philippe Gélisse, Selim R. Benbadis, Arielle Crespel, William O. Tatum

17.05.2024 | Original Communication

Expanding the genetic and phenotypic relevance of CLCN4 variants in neurodevelopmental condition: 13 new patients

Recently, we and others identified CLCN4 variants as causal factors for complex neurodevelopmental disorders (NDDs) [ 1 – 6 ]. The phenotypic spectrum is highly variable, with developmental delay, various degrees of intellectual disability (ID) as …

verfasst von:
Hailan He, Xinyi Li, G. A. Guzman, Stefanie Bungert-Plümke, Arne Franzen, XueQin Lin, Hongmin Zhu, Guilan Peng, Hongwei Zhang, Yonglin Yu, Suzhen Sun, Zhongqin Huang, Qiongxiang Zhai, Zheng Chen, Jing Peng, Raul E. Guzman

Open Access 17.05.2024 | Journal club

Diagnostic criteria for MOGAD

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare antibody-mediated inflammatory demyelinating disorder of the central nervous system with a broad clinical spectrum. Distinct from multiple sclerosis (MS) and …

verfasst von:
Sophie Voase, Neil P. Robertson

17.05.2024 | Original Communication

Neuroimaging assessment of facility-bound severely-affected MS reveals the critical role of cortical gray matter pathology: results from the CASA–MS case-controlled study

Multiple sclerosis (MS) is a chronic, inflammatory, demyelinating disease of the central nervous system (CNS) that mainly affects people between the ages of 20 and 50 years [ 23 ]. People with MS (pwMS) experience an unpredictable disease course …

verfasst von:
Robert Zivadinov, Dejan Jakimovski, Alex Burnham, Jens Kuhle, Zachary Weinstock, Taylor R. Wicks, Murali Ramanathan, Tommaso Sciortino, Mark Ostrem, Christopher Suchan, Michael G. Dwyer, Jessica Reilly, Niels Bergsland, Ferdinand Schweser, Cheryl Kennedy, David Young-Hong, Svetlana Eckert, David Hojnacki, Ralph H. B. Benedict, Bianca Weinstock-Guttman

Open Access 17.05.2024 | Original Communication

Profiles of motivational impairment and their relationship to functional decline in frontotemporal dementia

Motivational disturbances are pervasive in frontotemporal dementia (FTD) and impact negatively on everyday functioning. Despite mounting evidence of anhedonia in FTD, it remains unclear how such changes fit within the broader motivational symptom …

verfasst von:
Siobhán R. Shaw, Kristina S. Horne, Olivier Piguet, Rebekah M. Ahmed, Alexis E. Whitton, Muireann Irish

16.05.2024 | Review

Incidence of intracranial bleeding in mild traumatic brain injury patients taking oral anticoagulants: a systematic review and meta-analysis

Traumatic brain injury (TBI) is one of the leading causes of disability and death worldwide and poses a considerable burden to the healthcare system and society [ 1 ]. As the population ages, the use of anticoagulants for common comorbidities, such …

verfasst von:
Armin Karamian, Ali Seifi, Amin Karamian, Brandon Lucke-Wold

Open Access 16.05.2024 | Original Communication

Predictors of futile recanalization in nonagenarians treated with mechanical thrombectomy: a multi-center observational study

Mechanical thrombectomy (MT) currently represents the standard of care of acute ischemic stroke due to large vessel occlusion (LVO) regardless of the age of the patient [ 1 ]. A recent analysis of data from the Multicenter Randomized Clinical Trial …

verfasst von:
Lucio D’Anna, Giovanni Merlino, Michele Romoli, Liqun Zhang, Caterina Del Regno, Mohammed Aggour, Viva Levee, Matteo Foschi, Massimo Sponza, Francesco Toraldo, Razan Algazlan, Maria Ruggiero, Marco Longoni, Kyriakos Lobotesis, Samir Abu-Rumeileh, Daniele Bagatto, Nina Mansoor, Gian Luigi Gigli, Mariarosaria Valente, Soma Banerjee

Open Access 15.05.2024 | Original Communication

Postural motion perception during vestibular stimulation depends on the motion perception threshold in persistent postural-perceptual dizziness

Patients with persistent postural-perceptual dizziness (PPPD) perceive postural instability larger than the observed sway. It is unknown whether the concept of postural misperception prevails during vestibular stimulation and whether it may …

verfasst von:
Christoph Helmchen, Smila-Karlotta Blüm, Renana Storm, Janina Krause, Andreas Sprenger

Open Access 14.05.2024 | Original Communication

Regional hippocampal atrophy reflects memory impairment in patients with early relapsing remitting multiple sclerosis

Multiple sclerosis (MS) is a chronic immune-mediated inflammatory disease of the central nervous system, characterized by the involvement of white matter (WM) and gray matter (GM) since the early stages [ 1 ]. The hippocampus, a complex structure …

verfasst von:
Rosa Cortese, Marco Battaglini, Maria Laura Stromillo, Ludovico Luchetti, Matteo Leoncini, Giordano Gentile, Daniele Gasparini, Domenico Plantone, Manuela Altieri, Alessandro D’Ambrosio, Antonio Gallo, Costanza Giannì, Claudia Piervincenzi, Patrizia Pantano, Elisabetta Pagani, Paola Valsasina, Paolo Preziosa, Nicolo’ Tedone, Maria Assunta Rocca, Massimo Filippi, Nicola De Stefano, INNI Network

Open Access 14.05.2024 | Short Commentary

Optical coherence tomography angiography suggests different retinal pathologies in multiple sclerosis and Sjögren’s syndrome

Multiple sclerosis (MS) is a chronic autoimmune disorder characterized by inflammation, demyelination, and axonal loss in the central nervous system (CNS). Ocular manifestations, particularly optic neuritis (ON), are commonly observed in …

verfasst von:
Elisabeth Wolf, Rebecca Wicklein, Lilian Aly, Christoph Schmaderer, Ali Maisam Afzali, Christian Mardin, Thomas Korn, Bernhard Hemmer, Benedikt Hofauer, Benjamin Knier

Open Access 11.05.2024 | Original Communication

JEWELFISH: 24-month results from an open-label study in non-treatment-naïve patients with SMA receiving treatment with risdiplam

Spinal muscular atrophy (SMA) is a genetic disorder that affects motor neurons, resulting in muscle weakness [ 1 ]. It is caused by mutations in the survival of motor neuron 1 ( SMN1 ) gene that lead to low levels of functional SMN protein [ 2 ].

verfasst von:
Claudia A. Chiriboga, Claudio Bruno, Tina Duong, Dirk Fischer, Eugenio Mercuri, Janbernd Kirschner, Anna Kostera-Pruszczyk, Birgit Jaber, Ksenija Gorni, Heidemarie Kletzl, Imogen Carruthers, Carmen Martin, Renata S. Scalco, Paulo Fontoura, Francesco Muntoni, the JEWELFISH Study Group

11.05.2024 | Editorial

Pioneers in neurology: a silver anniversary perspective

verfasst von:
Andrew J. Larner, Jan van Gijn, Lazaros C. Triarhou

11.05.2024 | Original Communication

The influence of depression and anxiety on cognition in people with multiple sclerosis: a cross-sectional analysis

There are conflicting findings about the relationships between depression, anxiety, and cognitive dysfunction in people with multiple sclerosis (MS), and a paucity of research has examined the cumulative influence on cognition of depression plus …

verfasst von:
David E. Freedman, Jiwon Oh, Alex Kiss, Juliana Puopolo, Margaret Wishart, Cecilia Meza, Anthony Feinstein

Open Access 10.05.2024 | Original Communication

Perampanel as monotherapy or first adjunctive therapy in pediatric and adult patients with epilepsy: the first United States-based phase IV open-label ELEVATE study

ELEVATE (Study 410; NCT03288129) is the first prospective, multicenter, open-label, Phase IV study of perampanel as monotherapy or first adjunctive therapy in patients aged ≥ 4 years with focal-onset seizures or generalized tonic–clonic seizures …

verfasst von:
Vineet Punia, Pavel Klein, Temenuzhka Mihaylova, Victor Biton, Omar Samad, Leock Y. Ngo, Dinesh Kumar, Manoj Malhotra

10.05.2024 | Original Communication

Stride width and postural stability in frontal gait disorders and Parkinson’s disease

Older adults, as well as those with certain neurological disorders, may compensate for poor neural control of postural stability by widening their base of foot support while walking. However, the extent to which this wide-based gait improves …

verfasst von:
Carolin Curtze, Vrutangkumar V. Shah, Alexa M. Stefanko, Marian L. Dale, John G. Nutt, Martina Mancini, Fay B. Horak

Open Access 10.05.2024 | Original Communication

Long-term clinical outcomes in patients with CIS treated with interferon beta-1b: results from the 15-year follow up of the BENEFIT trial

Multiple sclerosis (MS) treatment intervention with immunomodulating therapy at early disease stage improves short term clinical outcomes. The objective of this study is to describe the long-term outcomes and healthcare utilization of patients …

verfasst von:
Ludwig Kappos, Gilles Edan, Mark S. Freedman, Hans-Peter Hartung, Xavier Montalbán, Frederik Barkhof, Ralf Koelbach, David G. MacManus, Eva-Maria Wicklein, for the BENEFIT and BENEFIT 15 Study Groups

10.05.2024 | Letter to the Editors

Diverse faces of GNAO1: mild forms in epilepsy and autism

Patient 1, a 14-year-old Spanish male, was born at full term. He displayed a mild motor delay, achieving the ability to sit independently at 9–10 months of age and walk unassisted at 15 months. He exhibited impaired social interaction, literal …

verfasst von:
William Grant Ludlam, Luca Soliani, Jana Domínguez-Carral, Duccio Maria Cordelli, Valentina Marchiani, Nerea Gorría-Redondo, Sergio Aguilera-Albesa, Kirill A. Martemyanov, Juan Darío Ortigoza-Escobar

Open Access 10.05.2024 | Original Communication

Prevalence of vestibular disease in France: analysis of prescription data from a national health insurance database

Vestibular problems are frequent reasons for primary care consultations. However, there is considerable uncertainty about the prevalence and cost of vestibular disorders. Despite ambiguous effectiveness data, the histamine analogue betahistine is …

verfasst von:
Eva Grill, Vincent Darrouzet, Ulrich Mansmann, Christian Chabbert

09.05.2024 | Pioneers in Neurology

Charles Bland Radcliffe (1822–1889)

verfasst von:
Andrew J. Larner, Lazaros C. Triarhou