Journal of Neurology OnlineFirst articles

28.05.2024 | Original Communication

Parkinson’s disease with hyposmia and dysautonomia: does it represent a distinct subtype?

Parkinson’s disease (PD) is clinically characterized by cardinal motor symptoms of bradykinesia, rigidity, resting tremor, and various nonmotor symptoms. The primary pathological substrate of PD is α-synuclein which forms Lewy bodies and Lewy …

verfasst von:: So Hoon Yoon, Dae Hyuk You, Han Kyu Na, Sungwoo Kang, Kyoungwon Baik, Mincheol Park, Chul Hyoung Lyoo, Young H. Sohn, Phil Hyu Lee

Open Access 28.05.2024 | Original Communication

Progression independent of relapse activity in relapsing multiple sclerosis: impact and relationship with secondary progression

Disability accrual in patients with multiple sclerosis (pwMS) can derive from two main mechanisms, relapse-associated worsening (RAW) on the one hand, and progression independent from relapse activity (PIRA) on the other [ 1 , 2 ]. RAW has been …

verfasst von:: Emilio Portaccio, Matteo Betti, Ermelinda De Meo, Ilaria Addazio, Luisa Pastò, Lorenzo Razzolini, Rocco Totaro, Daniele Spitaleri, Alessandra Lugaresi, Eleonora Cocco, Marco Onofrj, Franco Di Palma, Francesco Patti, Davide Maimone, Paola Valentino, Valentina Torri Clerici, Alessandra Protti, Diana Ferraro, Giacomo Lus, Giorgia Teresa Maniscalco, Vincenzo Brescia Morra, Giuseppe Salemi, Franco Granella, Ilaria Pesci, Roberto Bergamaschi, Umberto Aguglia, Marika Vianello, Marta Simone, Vito Lepore, Pietro Iaffaldano, Giancarlo Comi, Massimo Filippi, Maria Trojano, Maria Pia Amato, the Italian Multiple Sclerosis Register

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Open Access 28.05.2024 | Original Communication

Exploratory study to evaluate the acceptability of a wearable accelerometer to assess motor progression in motor neuron disease

Motor neuron disease (MND) is a rapidly progressive condition traditionally assessed using a questionnaire to evaluate physical function, the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). Its use can be associated with …

verfasst von:: Emily Beswick, Alexander Christides, Alexander Symonds, Micheaela Johnson, Thomas Fawcett, Judith Newton, Dawn Lyle, Christine Weaver, Siddharthan Chandran, Suvankar Pal

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Open Access 28.05.2024 | Review

Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive degeneration of both upper and lower motor neurons. A defining histopathological feature in approximately 97% of all ALS cases is the …

verfasst von:: Bogdan Bjelica, Maj-Britt Bartels, Jasper Hesebeck-Brinckmann, Susanne Petri

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27.05.2024 | Review

Loss of the “swallow tail sign” on susceptibility-weighted imaging in the diagnosis of dementia with Lewy bodies: a systematic review and meta-analysis

Dementia with Lewy bodies (DLB) is one of the most common types of dementia, second only to Alzheimer’s disease (AD) [ 1 , 2 ]. Clinical characteristics of DLB include fluctuating cognitive impairment, visual hallucinations, confusion, rapid-eye …

verfasst von:: Vasilis-Spyridon Tseriotis, Theodoros Mavridis, Kyriaki Eleftheriadou, Georgios Konstantis, David-Dimitris Chlorogiannis, Pavlos Pavlidis, Chryssa Pourzitaki, Marianthi Arnaoutoglou, Konitsiotis Spyridon

27.05.2024 | Correction

Correction to: RFC1‑related disorder presenting recurrent syncope

verfasst von:: Yoko Tsuboyama, Akiko Takahashi, Sawako Furukawa, Asem Almansour, Masashi Hamada, Akatsuki Kubota, Jun Shimizu, Makoto Kinoshita, Chisato Fujimoto, Jun Mitsui, Takashi Matsukawa, Hiroya Naruse, Hiroyuki Ishiura, Shoji Tsuji, Tatsushi Toda

27.05.2024 | Original Communication

Prognostic factors underlying the development of drug-resistant epilepsy in patients with autoimmune encephalitis: a retrospective cohort study

Autoimmune encephalitis (AE) is being increasingly recognized as one of the major causes of acute symptomatic seizures [ 1 ]. According to previous studies, approximately 70% of patients with AE have seizures, and most of them respond well to …

verfasst von:: Wenyao Huang, Heyu Zhang, Xi Li, Jinming Zhang, Jingjing Chen, Ziyi Chen, Guanzhong Ni

Open Access 27.05.2024 | Neurological Update

Update on recent advances in amyotrophic lateral sclerosis

In the last few years, our understanding of disease molecular mechanisms underpinning ALS has advanced greatly, allowing the first steps in translating into clinical practice novel research findings, including gene therapy approaches. Similarly …

verfasst von:: Nilo Riva, Teuta Domi, Laura Pozzi, Christian Lunetta, Paride Schito, Edoardo Gioele Spinelli, Sara Cabras, Enrico Matteoni, Monica Consonni, Eleonora Dalla Bella, Federica Agosta, Massimo Filippi, Andrea Calvo, Angelo Quattrini

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Open Access 27.05.2024 | Original Communication

Recurrent cerebrovascular events after recent small subcortical infarction

Cerebral small vessel disease (SVD) is an important cause of ischaemic stroke as well as intracerebral haemorrhage, and is also associated with the development of vascular dementia, gait disturbance and depression [ 1 , 2 ].

verfasst von:: Melanie Haidegger, Nina Klock, Markus Kneihsl, Simon Fandler-Höfler, Sebastian Eppinger, Kathrin Eller, Stephan Seiler, Christian Enzinger, Thomas Gattringer

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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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