Journal of Neurology OnlineFirst articles

26.05.2024 | Original Communication

Clinical characteristics of cerebral amyloid angiopathy and risk factors of cerebral amyloid angiopathy related intracerebral hemorrhage

Cerebral amyloid angiopathy (CAA) is a common cause of spontaneous intracerebral hemorrhage (ICH) due to excessive deposition of amyloid β (Aβ) mainly in the cortex and leptomeningeal vessels and subsequent destruction of vascular structure. Its …

verfasst von:: Juanjuan Wu, Ziyue Liu, Ming Yao, Yicheng Zhu, Bin Peng, Jun Ni

26.05.2024 | Letter to the Editors

Senna intoxication as a cause of extrapontine myelinolysis mimicking rapidly progressive dementia: a case description

verfasst von:: Alma Ghirelli, Davide Gusmeo Curti, Sonia Francesca Calloni, Maurizio Barbera, Rosalinda Cardamone, Anna Bellini, Andrea Panzacchi, Arturo Chiti, Andrea Falini, Federica Agosta, Massimo Filippi, Francesca Caso

26.05.2024 | Original Communication

Quantification of saccadic fatigability and diagnostic efficacy for myasthenia gravis

Myasthenia Gravis (MG) is an autoimmune disorder characterized by impaired neuromuscular transmission, resulting in fatigable, fluctuating muscle weakness [ 1 ]. It represents the most prevalent disorder of neuromuscular transmission, primarily …

verfasst von:: Juhee Chae, Thanh Tin Nguyen, Sun-Young Oh

25.05.2024 | Short Commentary

Incidence and predictors of postural abnormalities in Parkinson’s disease: a PPMI cohort study

Axial postural abnormalities (PA) are part of the frequent and invalidating axial motor symptoms associated with Parkinson's disease (PD) [ 1 ]. PA are typically defined as an abnormal spine flexion occurring when sitting or standing, worsened …

verfasst von:: Margherita Fabbri, Corrado Campisi, Claudia Ledda, Domiziana Rinaldi, Kazuto Tsukita, Alberto Romagnolo, Gabriele Imbalzano, Maurizio Zibetti, Mario Giorgio Rizzone, Francesco Ernesto Pontieri, Leonardo Lopiano, Carlo Alberto Artusi

Open Access 23.05.2024 | Original Communication

The ocrelizumab wearing-off phenomenon is associated with reduced immunomodulatory response and increased neuroaxonal damage in multiple sclerosis

Ocrelizumab is a humanized anti-CD20 monoclonal antibody that is approved for the treatment of relapsing and primary progressive multiple sclerosis (MS), and acts primarily through the depletion of B cells [ 1 ]. Ocrelizumab is administrated …

verfasst von:: Isabel Monteiro, Valerio Nicolella, Mariano Fiorenza, Federica Novarella, Antonio Carotenuto, Roberta Lanzillo, Lucia Mauriello, Giulia Scalia, Giuseppe Castaldo, Daniela Terracciano, Vincenzo Brescia Morra, Marcello Moccia

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Open Access 23.05.2024 | Review

Efficacy and safety of intravenous tenecteplase compared to alteplase before mechanical thrombectomy in acute ischemic stroke: a meta-analysis

Mechanical thrombectomy (MT) has emerged as the treatment of choice for acute ischemic stroke (AIS) with large vessel occlusion, with its effectiveness confirmed in multiple randomized trials [ 1 ]. Prior to MT, intravenous thrombolysis is widely …

verfasst von:: Nihong Wu, Thorsten R. Doeppner, Dirk M. Hermann, Janine Gronewold

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Open Access 23.05.2024 | Review

The efficacy and safety of ketamine in the treatment of super-refractory status epilepticus: a systematic review

Status epilepticus (SE) is characterized by continuous seizures with incomplete recovery of consciousness between episodes or seizures lasting for more than 30 min without self-termination. Refractory status epilepticus (RSE) is defined as …

verfasst von:: Mingyuan Yan, Tianye Sun, Jinmin Liu, Qing Chang

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22.05.2024 | Short Commentary

Clinical and serological insights into paraneoplastic brachial amyotrophic diplegia

Brachial amyotrophic diplegia (BAD; also known as flail arm syndrome) is a rare phenotypic presentation, often presenting with asymmetric onset of proximal upper limb weakness with bilateral progression [ 1 ]. Few cases with distal predominant …

verfasst von:: Haidara Kherbek, Christopher Y. Itoh, Catherine Daley, Scott D. Eggers, Shannon Hinson, Pallab Sarker, Nathan P. Staff, Sean J. Pittock, Divyanshu Dubey

Open Access 22.05.2024 | Original Communication

How much of the variance in functional outcome related to intracerebral hemorrhage volume is already apparent in neurological status at admission?

Hematoma volume is a major pathophysiological hallmark of acute primary intracerebral hemorrhages (ICH) and a significant predictor of ICH patients’ outcome [ 4 , 22 ]. However, while preliminary results of the ongoing ENRICH trial (NCT02880878) …

verfasst von:: Vincent Geest, Janja Pretnar Oblak, Katarina Šurlan Popović, Jawed Nawabi, Sarah Elsayed, Constanze Friedrich, Maik Böhmer, Burak Akkurt, Peter Sporns, Andrea Morotti, Frieder Schlunk, Paul Steffen, Gabriel Broocks, Lukas Meyer, Uta Hanning, Götz Thomalla, Susanne Gellissen, Jens Fiehler, Senta Frol, Helge Kniep

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22.05.2024 | Review

Exploring therapeutic interventions for functional neurological disorders: a comprehensive scoping review

Functional Neurological Disorders (FNDs) are characterized by the symptoms experienced by the individuals but also by how they express personal experiences and concerns related to the clinical condition. Access to care programs for functional …

verfasst von:: Francesca Sireci, Federica Ragucci, Chiara Menozzi, Maria Paola Cabboi, Livio Picchetto, Maria Chiara Bassi, Luca Ghirotto, Francesco Cavallieri, Cristina Pedroni, Franco Valzania

Open Access 22.05.2024 | Letter to the Editors

Dystonia and mitochondrial disease: the movement disorder connection revisited in 900 genetically diagnosed patients

Dystonia is characterized by excessive muscle contractions leading to abnormal posturing or movements, typically worsening with voluntary action and overflow muscle activation [ 1 ]. Dystonia can arise at any age and affect virtually any muscle [ 1 …]. The disease often co-occurs in combination with other movement disorders or additional neurological signs, resulting in a wide range of clinical outcomes [

verfasst von:: Elisabetta Indelicato, Lea D. Schlieben, Sarah L. Stenton, Sylvia Boesch, Matej Skorvanek, Jan Necpal, Robert Jech, Juliane Winkelmann, Holger Prokisch, Michael Zech

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21.05.2024 | Review

Moving towards a new era for the treatment of neuromyelitis optica spectrum disorders

Neuromyelitis optica spectrum disorders (NMOSD) include a rare group of autoimmune conditions that primarily affect the central nervous system. They are characterized by inflammation and damage to the optic nerves, brain and spinal cord, leading …

verfasst von:: Paolo Preziosa, Maria Pia Amato, Luca Battistini, Marco Capobianco, Diego Centonze, Eleonora Cocco, Antonella Conte, Claudio Gasperini, Matteo Gastaldi, Carla Tortorella, Massimo Filippi

Open Access 21.05.2024 | Review

Neurological symptoms in adults with Gaucher disease: a systematic review

Gaucher disease (GD), one of the most common lysosomal storage diseases, results from biallelic variants in the GBA1 gene located on chromosome 1 (1q21), leading to a defective glucocerebrosidase protein (GCase). This lysosomal enzyme plays a …

verfasst von:: Gabriele Imbalzano, Claudia Ledda, Alberto Romagnolo, Anna Covolo, Leonardo Lopiano, Carlo Alberto Artusi

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21.05.2024 | Original Communication

Anti-neurofascin-155 antibody mediated a distinct phenotype of chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents a clinically heterogeneous group of disabling disorders [ 1 ]. Autoantibodies targeting membrane proteins located at or near the node of Ranvier have been identified in …

verfasst von:: Lijie Zhang, Yuanyuan Zhang, Runyun Li, Jiting Zhu, Aiyu Lin, Yaping Yan, Zaiqiang Zhang, Ning Wang, Guorong Xu, Ying Fu

20.05.2024 | Original Communication

Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome

Gangliosides, including GQ1b, are glycosphingolipids with a structure in which a hydrophobic ceramide and a hydrophilic sugar chain portion are bound, and sialic acid is bound to the sugar chain portion [ 1 , 2 ]. Gangliosides are abundant in the …

verfasst von:: Martin Coly, David Adams, Shahram Attarian, Françoise Bouhour, Jean-Philippe Camdessanché, Guillaume Carey, Cécile Cauquil, Jean-Baptiste Chanson, Pascale Chrétien, Alain Créange, Emilien Delmont, Guillaume Fargeot, Simon Frachet, Thierry Gendre, Thierry Kuntzer, Céline Labeyrie, Thierry Maisonobe, Maud Michaud, Maximilien Moulin, Guillaume Nicolas, Jean-Baptiste Noury, Yann Péréon, Angela Puma, Guilhem Sole, Frédéric Taithe, Céline Tard, Marie Théaudin, Serge Timsit, Laura Venditti, Andoni Echaniz-Laguna

Open Access 20.05.2024 | Original Communication

Success rates of intensive aphasia therapy: real-world data from 448 patients between 2003 and 2020

Aphasia is an acquired language disorder commonly occurring after ischemic or hemorrhagic stroke in the dominant hemisphere. It affects about 40% of stroke survivors [ 1 ] and persists to 1 year or more after stroke in up to 30% [ 2 ]. Aphasia …

verfasst von:: Dorothea Peitz, Beate Schumann-Werner, Katja Hussmann, João Pinho, Hong Chen, Ferdinand Binkofski, Walter Huber, Klaus Willmes, Stefan Heim, Jörg B. Schulz, Bruno Fimm, Cornelius J. Werner

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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

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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

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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

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Die Highlights vom Kongress des American College of Cardiology 2024

Springer Medizin