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  • The European Physiotherapy Guideline for Parkinson’s Disease: translation for non-English speaking countries
    J. Neurol. (IF 3.956) Pub Date : 2020-08-06
    Josefa M. M. Domingos, Tamine T. C. Capato, Lorena R. S. Almeida, Catarina Godinho, Marlies van Nimwegen, Maarten Nijkrake, Nienke M. de Vries, Bastiaan R. Bloem

    Background The use of the European Physiotherapy Guideline for Parkinson’s Disease is limited in countries where the official language is not English. Objective To provide practical steps on how to translate the European Physiotherapy Guideline for Parkinson’s Disease. Methods We used the translation process of the Portuguese version as an example of how to define the recommended steps. A combination

    更新日期:2020-08-06
  • Association of history of cerebrovascular disease with severity of COVID-19
    J. Neurol. (IF 3.956) Pub Date : 2020-08-06
    Timo Siepmann, Annahita Sedghi, Jessica Barlinn, Katja de With, Lutz Mirow, Martin Wolz, Thomas Gruenewald, Sina Helbig, Percy Schroettner, Simon Winzer, Simone von Bonin, Haidar Moustafa, Lars-Peder Pallesen, Bernhard Rosengarten, Joerg Schubert, Andreas Gueldner, Peter Spieth, Thea Koch, Stefan Bornstein, Heinz Reichmann, Volker Puetz, Kristian Barlinn

    Objective To determine whether a history of cerebrovascular disease (CVD) increases risk of severe coronavirus disease 2019 (COVID-19). Methods In a retrospective multicenter study, we retrieved individual data from in-patients treated March 1 to April 15, 2020 from COVID-19 registries of three hospitals in Saxony, Germany. We also performed a systematic review and meta-analysis following PRISMA recommendations

    更新日期:2020-08-06
  • Mesenchymal stem cell therapy for ischemic stroke: A look into treatment mechanism and therapeutic potential
    J. Neurol. (IF 3.956) Pub Date : 2020-08-06
    Junsheng Li, Qian Zhang, Wen Wang, Fa Lin, Shuo Wang, Jizong Zhao

    Stroke, a global disease with a high disability rate, has limited options for functional rehabilitation and results in an adverse impact on patients’ lives. In recent years, mesenchymal stem cells (MSCs) have become a new focus of treatment owing to their potential for neuroregeneration. MSCs have demonstrated therapeutic efficacy capable of cell migration, angiogenesis, immunomodulation, neuroprotection

    更新日期:2020-08-06
  • The capacity to consent to treatment in amyotrophic lateral sclerosis: a preliminary report
    J. Neurol. (IF 3.956) Pub Date : 2020-08-06
    Rossella Spataro, Vincenzo La Bella

    Background Facing the relentless worsening of their condition, ALS patients are required to make decisions on treatments and end-of-life care. A cognitive impairment showed to be a negative prognostic factor in ALS patients, perhaps affecting the ability to make informed decisions. Notwithstanding its crucial role, the capacity to consent to treatment (CCT) has never been evaluated in these patients

    更新日期:2020-08-06
  • The impact of the initial severity on later outcome: retrospective analysis of a large cohort of botulinum toxin naïve patients with idiopathic cervical dystonia
    J. Neurol. (IF 3.956) Pub Date : 2020-08-05
    Harald Hefter, Sara Samadzazeh, Dietmar Rosenthal

    Background The aim of study was to demonstrate that the first three injections of botulinum neurotoxin type A (BoNT/A) appear to be less effective in botulinum toxin naïve patients with idiopathic cervical dystonia (CD) with mild symptoms and low severity scores (TSUI-scores) at onset of BoNT/A-therapy compared to patients with full-blown CD and high initial TSUI-scores. Methods In 337 patients with

    更新日期:2020-08-06
  • Changes in the EEG spectral power during dual-task walking with aging and Parkinson’s disease: initial findings using Event-Related Spectral Perturbation analysis
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Daniel Possti, Firas Fahoum, Ronen Sosnik, Nir Giladi, Jeffrey M. Hausdorff, Anat Mirelman, Inbal Maidan

    Background The ability to maintain adequate motor-cognitive performance under increasing task demands depends on the regulation and coordination of neural resources. Studies have shown that such resources diminish with aging and disease. EEG spectral analysis is a method that has the potential to provide insight into neural alterations affecting motor-cognitive performance. The aim of this study was

    更新日期:2020-08-05
  • Gluten neuropathy: electrophysiological progression and HLA associations
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Panagiotis Zis, Ptolemaios Sarrigiannis, Artemios Artemiadis, David S. Sanders, Marios Hadjivassiliou

    Objective Gluten neuropathy (GN) is the term used to describe peripheral neuropathy that occurs in patients with gluten sensitivity (GS) or coeliac disease (CD) in the absence of other risk factors. We aimed to describe the neurophysiological progression rate of GN across time and look into the potential role of genetic susceptibility in its development. Methods This is a cohort study of 45 patients

    更新日期:2020-08-05
  • Diagnosis of depression in multiple sclerosis is predicted by frontal–parietal white matter tract disruption
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Kira Ashton, Tom A. Fuchs, Devon Oship, Robert Zivadinov, Dejan Jakimovski, Niels Bergsland, Deepa P. Ramasamy, Caila Vaughn, Bianca Weinstock-Guttman, Ralph H. B. Benedict, Michael G. Dwyer

    Background Persons with multiple sclerosis (PwMS) are at an elevated risk of depression. Decreased Conscientiousness may affect patient outcomes in PwMS. Low Conscientiousness has a strong correlation with depression. Previous work has also reported that white matter (WM) tract disruption in frontal–parietal networks explains reduced Conscientiousness in PwMS. Objective We hypothesized that Consci

    更新日期:2020-08-05
  • Early discrimination of cognitive motor dissociation from disorders of consciousness: pitfalls and clues
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Alessandro Pincherle, Frederic Rossi, Jane Jöhr, Vincent Dunet, Philippe Ryvlin, Mauro Oddo, Nicolas Schiff, Karin Diserens

    Bedside assessment of consciousness and awareness after a severe brain injury might be hampered by confounding clinical factors (i.e., pitfalls) interfering with the production of behavioral or motor responses to external stimuli. Despite the use of validated clinical scales, a high misdiagnosis rate is indeed observed. We retrospectively analyzed a cohort of 49 patients with severe brain injury admitted

    更新日期:2020-08-04
  • Neurological comorbidity and severity of COVID-19
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Alberto Romagnolo, Roberta Balestrino, Gabriele Imbalzano, Giovannino Ciccone, Franco Riccardini, Carlo Alberto Artusi, Marco Bozzali, Bruno Ferrero, Elisa Montalenti, Elisa Montanaro, Mario Giorgio Rizzone, Giovanna Vaula, Maurizio Zibetti, Leonardo Lopiano

    Objective Neurological symptoms of COVID-19 patients have been recently described. However, no comprehensive data have been reported on pre-existing neurological comorbidities and COVID-19. This study aims at evaluating the prevalence of neurological comorbidities, and their association with COVID-19 severity. Methods We evaluated all consecutive patients admitted to the Emergency Room (ER) of our

    更新日期:2020-08-04
  • Nerve ultrasound in hereditary transthyretin amyloidosis: red flags and possible progression biomarkers
    J. Neurol. (IF 3.956) Pub Date : 2020-08-04
    Alessandro Salvalaggio, Daniele Coraci, Mario Cacciavillani, Laura Obici, Anna Mazzeo, Marco Luigetti, Francesca Pastorelli, Marina Grandis, Tiziana Cavallaro, Giulia Bisogni, Alessandro Lozza, Chiara Gemelli, Luca Gentile, Mario Ermani, Gian Maria Fabrizi, Rosaria Plasmati, Marta Campagnolo, Francesca Castellani, Roberto Gasparotti, Carlo Martinoli, Luca Padua, Chiara Briani

    Background Diagnostic delay of hereditary transthyretin amyloidosis (ATTRv, v for variant) prevents timely treatment and, therefore, concurs to the mortality of the disease. The aim of the present study was to explore with nerve ultrasound (US) possible red flags for early diagnosis in ATTRv patients with carpal tunnel syndrome (CTS) and/or polyneuropathy and in pre-symptomatic carriers. Methods Patients

    更新日期:2020-08-04
  • Impaired naming performance in temporal lobe epilepsy: language fMRI responses are modulated by disease characteristics
    J. Neurol. (IF 3.956) Pub Date : 2020-08-03
    Karin Trimmel, Lorenzo Caciagli, Fenglai Xiao, Louis A. van Graan, Matthias J. Koepp, Pamela J. Thompson, John S. Duncan

    Objective To investigate alterations of language networks and their relation to impaired naming performance in temporal lobe epilepsy (TLE) using functional MRI. Methods Seventy-two adult TLE patients (41 left) and 36 controls were studied with overt auditory and picture naming fMRI tasks to assess temporal lobe language areas, and a covert verbal fluency task to probe frontal lobe language regions

    更新日期:2020-08-03
  • Role of aquaporins in hydrocephalus: what do we know and where do we stand? A systematic review
    J. Neurol. (IF 3.956) Pub Date : 2020-08-03
    Camilla de Laurentis, Paola Cristaldi, Andrea Arighi, Clarissa Cavandoli, Andrea Trezza, Erik P. Sganzerla, Carlo G. Giussani, Andrea Di Cristofori

    Introduction Glymphatic fluid circulation may be considered the lymphatic system of the brain and the main role of such system seems to be played by aquaporins (AQPs), a family of proteins which regulates water exchange, in particular AQP4 and 1. Alterations of glymphatic fluid circulation through AQPs variations are now emerging as central elements in the pathophysiology of different brain conditions

    更新日期:2020-08-03
  • Development and validation of a classification algorithm to diagnose and differentiate spontaneous episodic vertigo syndromes: results from the DizzyReg patient registry
    J. Neurol. (IF 3.956) Pub Date : 2020-07-13
    Groezinger Michael, Huppert Doreen, Strobl Ralf, Grill Eva

    Background Spontaneous episodic vertigo syndromes, namely vestibular migraine (VM) and Menière’s disease (MD), are difficult to differentiate, even for an experienced clinician. In the presence of complex diagnostic information, automated systems can support human decision making. Recent developments in machine learning might facilitate bedside diagnosis of VM and MD. Methods Data of this study originate

    更新日期:2020-07-13
  • Significant reduction of physical activity in patients with neuromuscular disease during COVID-19 pandemic: the long-term consequences of quarantine
    J. Neurol. (IF 3.956) Pub Date : 2020-07-13
    Vincenzo Di Stefano, Giuseppe Battaglia, Valerio Giustino, Andrea Gagliardo, Michele D’Aleo, Ottavio Giannini, Antonio Palma, Filippo Brighina

    Background Quarantine was the measure taken by governments to control the rapid spread of COVID-19. This restriction resulted in a sudden change in people’s lifestyle, leading to an increase in sedentary behavior and a related decrease in the practice of physical activity (PA). However, in neuromuscular diseases patients need to perform regular PA to counteract the negative consequences of the disease

    更新日期:2020-07-13
  • Behr syndrome and hypertrophic cardiomyopathy in a family with a novel UCHL1 deletion
    J. Neurol. (IF 3.956) Pub Date : 2020-07-12
    Grace McMacken, Hanns Lochmüller, Boglarka Bansagi, Angela Pyle, Angela Lochmüller, Patrick F. Chinnery, Steve Laurie, Sergi Beltran, Leslie Matalonga, Rita Horvath

    Background Behr syndrome is a clinically distinct, but genetically heterogeneous disorder characterized by optic atrophy, progressive spastic paraparesis, and motor neuropathy often associated with ataxia. The molecular diagnosis is based on gene panel testing or whole-exome/genome sequencing. Methods Here, we report the clinical presentation of two siblings with a novel genetic form of Behr syndrome

    更新日期:2020-07-13
  • Telling friend from foe in emergency vertigo and dizziness: does season and daytime of presentation help in the differential diagnosis?
    J. Neurol. (IF 3.956) Pub Date : 2020-07-11
    Klaus Jahn, Antoanela Kreuzpointner, Thomas Pfefferkorn, Andreas Zwergal, Thomas Brandt, Andreas Margraf

    Distinguishing between serious (e.g., stroke) and benign (e.g., benign paroxysmal positional vertigo, BPPV) disorders remains challenging in emergency consultations for vertigo and dizziness (VD). A number of clues from patient history and clinical examination, including several diagnostic index tests have been reported recently. The objective of the present study was to analyze frequency and distribution

    更新日期:2020-07-13
  • The DizzyQuest: to have or not to have… a vertigo attack?
    J. Neurol. (IF 3.956) Pub Date : 2020-07-11
    L. E. G. H. de Joode, E. C. Martin, J. J. A. Stultiens, C. Leue, P. Delespaul, F. Peeters, A. Erdkamp, S. van de Weijer, H. Blom, T. Bruintjes, A. Zwergal, E. Grill, N. Guinand, A. Perez-Fornos, M. R. van de Berg, J. Widdershoven, H. Kingma, R. van de Berg

    Background The DizzyQuest, an app-based vestibular diary, provides the opportunity to capture the number and nature of vertigo attacks in daily life. To accomplish this, the DizzyQuest provides different strategies: event sampling using an attack questionnaire, and time sampling using an evening questionnaire. Objective of this study was to investigate whether the number and nature of reported vertigo

    更新日期:2020-07-13
  • Repetitive transcranial magnetic stimulation as an alternative therapy for stroke with spasticity: a systematic review and meta-analysis
    J. Neurol. (IF 3.956) Pub Date : 2020-07-11
    Peng Xu, Yan Huang, Jie Wang, Xu An, Tianyou Zhang, Yuechun Li, Jun Zhang, Baojun Wang

    Repetitive transcranial magnetic stimulation (rTMS) and intermittent theta-burst stimulation (iTBS) can be used to manage post-stroke spasticity, but a meta-analysis of the recent randomized-controlled trials (RCTs) is lacking. Our aim is to perform a meta-analysis of the RCTs that investigated the efficacy of rTMS in patients with post-stroke spasticity. PubMed, Embase, and Cochrane Library databases

    更新日期:2020-07-13
  • Impact of COVID-19 lockdown on sleep quality in university students and administration staff
    J. Neurol. (IF 3.956) Pub Date : 2020-07-11
    Sara Marelli, Alessandra Castelnuovo, Antonella Somma, Vincenza Castronovo, Samantha Mombelli, Daniela Bottoni, Caterina Leitner, Andrea Fossati, Luigi Ferini-Strambi

    Introduction In Italy, lockdown due to COVID-19 health emergency started on March 10 and partially ended on May 3rd, 2020. There was a significant increase of psychological distress and symptoms of mental illness, and worsening of quality of sleep in the general population. Methods Participants completed an anonymous web-based survey that include questionnaires about sleep and anxiety and depression

    更新日期:2020-07-13
  • Correction to: Diagnostic accuracy of a smartphone bedside test to assess the fixation suppression of the vestibulo‑ocular reflex: when nothing else matters
    J. Neurol. (IF 3.956) Pub Date : 2020-07-10
    Florin Gandor, Manfred Tesch, Hannelore Neuhauser, Doreen Gruber, Hans-Jochen Heinze, Georg Ebersbach, Thomas Lempert

    The article Diagnostic accuracy of a smartphone bedside test to assess the fixation suppression of the vestibulo‑ocular reflex: when nothing else matters, written by Florin Gandor, Manfred Tesch, Hannelore Neuhauser, Doreen Gruber, Hans‑Jochen Heinze, Georg Ebersbach and Thomas Lempert, was originally published electronically on the publisher’s internet portal on 01 June 2020 without open access

    更新日期:2020-07-10
  • Risk incidence of fractures and injuries: a multicenter video-EEG study of 626 generalized convulsive seizures
    J. Neurol. (IF 3.956) Pub Date : 2020-07-10
    Katharina Frey, Johann Philipp Zöllner, Susanne Knake, Yulia Oganian, Lara Kay, Katharina Mahr, Fee Keil, Laurent M. Willems, Katja Menzler, Sebastian Bauer, Susanne Schubert-Bast, Felix Rosenow, Adam Strzelczyk

    Objective To evaluate the incidence and risk factors of generalized convulsive seizure (GCS)-related fractures and injuries during video-EEG monitoring. Methods We analyzed all GCSs in patients undergoing video-EEG-monitoring between 2007 and 2019 at epilepsy centers in Frankfurt and Marburg in relation to injuries, falls and accidents associated with GCSs. Data were gathered using video material,

    更新日期:2020-07-10
  • Early functional MRI changes in a prodromal semantic variant of primary progressive aphasia: a longitudinal case report
    J. Neurol. (IF 3.956) Pub Date : 2020-07-10
    Elisa Canu, Valentina Bessi, Davide Calderaro, David Simoni, Veronica Castelnovo, Michela Leocadi, Sonia Padiglioni, Salvatore Mazzeo, Camilla Cividini, Benedetta Nacmias, Sandro Sorbi, Massimo Filippi, Federica Agosta

    Objective To assess longitudinal patterns of brain functional MRI (fMRI) activity in a case of prodromal semantic variant of a primary progressive aphasia (svPPA). Methods Clinical, cognitive and neuroimaging data (T1-weighted and task-based fMRI during silent naming [SN] and object knowledge [OK]) were obtained at baseline, month 8 and month 16 from a 49-year-old lady presenting with anomias and evolving

    更新日期:2020-07-10
  • Deep brain stimulation reduces (nocturnal) dyskinetic exacerbations in patients with ADCY5 mutation: a case series
    J. Neurol. (IF 3.956) Pub Date : 2020-07-09
    Ana Luísa de Almeida Marcelino, Tina Mainka, Patricia Krause, Werner Poewe, Christos Ganos, Andrea A. Kühn

    Mutations in the ADCY5 gene can cause a complex hyperkinetic movement disorder. Episodic exacerbations of dyskinesia are a particularly disturbing symptom as they occur predominantly during night and interrupt sleep. We present the clinical short- and long-term effects of pallidal deep brain stimulation (DBS) in three patients with a confirmed pathogenic ADCY5 mutation. Patients were implanted with

    更新日期:2020-07-09
  • Urinary neopterin, a new marker of the neuroinflammatory status in amyotrophic lateral sclerosis
    J. Neurol. (IF 3.956) Pub Date : 2020-07-08
    Christian Lunetta, Andrea Lizio, Francesca Gerardi, Claudia Tarlarini, Massimo Filippi, Nilo Riva, Lucio Tremolizzo, Susanna Diamanti, Cinzia Carla Dellanoce, Lorena Mosca, Valeria Ada Sansone, Jonica Campolo

    Objective To comprehensively assess whether neopterin in urine could be a candidate biomarker for determining the neuroinflammatory status in ALS. Methods We performed an observational, cross-sectional study in 81 pALS, 68 age- and sex-comparable healthy controls (HC), 14 patients affected by MS and 24 OND patients. ALS patients underwent a neurological evaluation to assess the global functional status

    更新日期:2020-07-08
  • Yield of the PMP22 deletion analysis in patients with compression neuropathies
    J. Neurol. (IF 3.956) Pub Date : 2020-07-08
    Vukan Ivanovic, Marija Brankovic, Bogdan Bjelica, Aleksandra Kacar, Radoje Tubic, Milena Jankovic, Ana Marjanovic, Ivana Novakovic, Vidosava Rakocevic-Stojanovic, Stojan Peric

    Introduction Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare neuromuscular disorder, mostly caused by PMP22 deletion. Aim To determine a yield of the genetic analysis of PMP22 gene deletion in patients with compression neuropathies. Method We included 112 patients with clinical suspicion of HNPP diagnosis. Nerve conduction studies (NCS) were performed for motor and sensory

    更新日期:2020-07-08
  • Clinical and radiological differences between patients with probable cerebral amyloid angiopathy and mixed cerebral microbleeds
    J. Neurol. (IF 3.956) Pub Date : 2020-07-08
    Ulf R. Jensen-Kondering, Caroline Weiler, Patrick Langguth, Naomi Larsen, Charlotte Flüh, Gregor Kuhlenbäumer, Olav Jansen, Nils G. Margraf

    Background The key imaging features of cerebral amyloid angiopathy (CAA) are lobar, cortical, or cortico-subcortical microbleeds, macrohaemorrhages and cortical superficial siderosis (cSS). In contrast, hypertensive angiopathy is characterized by (micro) haemorrhages in the basal ganglia, thalami, periventricular white matter or the brain stem. Another distinct form of haemorrhagic microangiopathy

    更新日期:2020-07-08
  • Disease progression but not physical state per se determines mental wellbeing in ALS
    J. Neurol. (IF 3.956) Pub Date : 2020-07-08
    Cynthia R. Vázquez Medrano, Helena E. A. Aho-Özhan, Ulrike Weiland, Ingo Uttner, Albert C. Ludolph, Dorothée Lulé

    Background Studies provide inconclusive results on the question whether loss of mental wellbeing is actually associated with decline in physical function in amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine predictors of mental wellbeing in ALS. Methods In total, n = 330 ALS patients were interviewed on parameters of mental wellbeing to evaluate the patients’ capacity

    更新日期:2020-07-08
  • Neurological update: neuroimaging in dementia
    J. Neurol. (IF 3.956) Pub Date : 2020-07-07
    Timothy Rittman

    Neuroimaging for dementia has made remarkable progress in recent years, shedding light on diagnostic subtypes of dementia, predicting prognosis and monitoring pathology. This review covers some updates in the understanding of dementia using structural imaging, positron emission tomography (PET), structural and functional connectivity, and using big data and artificial intelligence. Progress with neuroimaging

    更新日期:2020-07-07
  • Impact of COVID-19 on U.S. and Canadian neurologists’ therapeutic approach to multiple sclerosis: a survey of knowledge, attitudes, and practices
    J. Neurol. (IF 3.956) Pub Date : 2020-07-07
    Farrah J. Mateen, Shawheen Rezaei, Nicholas Alakel, Brittany Gazdag, Aditya Ravi Kumar, Andre Vogel

    Objective To report the understanding and decision-making of neuroimmunologists and their treatment of patients with multiple sclerosis (MS) during the early stages of the SARS-CoV-2 (COVID-19) outbreak. Methods A survey instrument was designed and distributed online to neurologists in April 2020. Results There were 250 respondents (response rate 21.8%). 243 saw > = 10 MS patients in the prior 6 months

    更新日期:2020-07-07
  • Targeted next-generation sequencing study in familial ALS-FTD Portuguese patients negative for C9orf72 HRE
    J. Neurol. (IF 3.956) Pub Date : 2020-07-07
    Marta Gromicho, Ana Margarida Coutinho, Ana Catarina Pronto-Laborinho, Rita Raposeiro, Joana Tavares, Diana Antunes, Mamede de Carvalho

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with clinical and etiological heterogeneity and a complex genetic contribution. Clinical, neuropathological, and genetic evidence revealed that ALS and frontotemporal dementia (FTD) are in part of a single disease continuum. Genetic causes have been identified in sporadic (SALS) and familial patients (FALS) and the recurrent genetic

    更新日期:2020-07-07
  • Treatment of MOG antibody associated disorders: results of an international survey
    J. Neurol. (IF 3.956) Pub Date : 2020-07-04
    D. H. Whittam, V. Karthikeayan, E. Gibbons, R. Kneen, S. Chandratre, O. Ciccarelli, Y. Hacohen, J. de Seze, K. Deiva, R. Q. Hintzen, B. Wildemann, S. Jarius, I. Kleiter, K. Rostasy, P. Huppke, B. Hemmer, F. Paul, O. Aktas, A. K. Pröbstel, G. Arrambide, M. Tintore, M. P. Amato, M. Nosadini, M. M. Mancardi, M. Capobianco, Z. Illes, A. Siva, A. Altintas, G. Akman-Demir, L. Pandit, M. Apiwattankul, J.

    Introduction While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to be developed. Objective To survey the current global clinical practice of clinicians treating MOGAD. Method Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February–April

    更新日期:2020-07-05
  • Cell-based assays for the detection of MOG antibodies: a comparative study
    J. Neurol. (IF 3.956) Pub Date : 2020-07-04
    Matteo Gastaldi, Silvia Scaranzin, Sven Jarius, Brigitte Wildeman, Elisabetta Zardini, Giulia Mallucci, Eleonora Rigoni, Elisa Vegezzi, Thomas Foiadelli, Salvatore Savasta, Paola Banfi, Maurizio Versino, Luana Benedetti, Giovanni Novi, Margherita Maria Mancardi, Thea Giacomini, Pietro Annovazzi, Damiano Baroncini, Diana Ferraro, Vito Lampasona, Markus Reindl, Patrick Waters, Diego Franciotta

    Background The detection of antibodies to myelin oligodendrocyte glycoprotein (MOG) is fundamental for the identification of MOG antibody-associated disorders (MOGAD), and the differential diagnosis of acquired demyelinating syndromes of the CNS, among which multiple sclerosis (MS). We compared the diagnostic performance of four cell-based assays (CBAs) for their detection. Methods Consecutive sera

    更新日期:2020-07-05
  • Bi-allelic variants in PNPLA6 possibly associated with Parkinsonian features in addition to spastic paraplegia phenotype
    J. Neurol. (IF 3.956) Pub Date : 2020-07-04
    Kuntal Sen, Melesilika Finau, Pritha Ghosh

    Variants in the PNPLA6 gene are known to cause 4 distinct phenotypes. One known phenotype is Hereditary Spastic Paraplegia type 39 (HSP 39), a rare neurodegenerative condition characterized by variable onset of lower limb spasticity, weakness and ataxia. Little is known about complications of HSP 39 in adulthood. Here, we report a family of three siblings who presented with bilateral lower limb spasticity

    更新日期:2020-07-05
  • Neurogenic calf amyotrophy with CK elevation by entrapment radiculopathy; clinical, radiological, and pathological analyses of 18 cases
    J. Neurol. (IF 3.956) Pub Date : 2020-07-03
    Makoto Shibata, Hiroo Kasahara, Kouki Makioka, Masaki Ikeda, Kazuaki Nagashima, Yukio Fujita, Yoshio Ikeda

    Objective To characterize the clinical, radiological, and pathological manifestations of 18 cases showing neurogenic calf amyotrophy with creatine kinase (CK) elevation by entrapment radiculopathy (NCACKEER). Methods We retrospectively reviewed and evaluated the medical records of patients who complained of weakness or atrophy of the calf muscles in our department between 2004 and 2019. We identified

    更新日期:2020-07-03
  • Reduced accuracy of MRI deep grey matter segmentation in multiple sclerosis: an evaluation of four automated methods against manual reference segmentations in a multi-center cohort
    J. Neurol. (IF 3.956) Pub Date : 2020-07-03
    Alexandra de Sitter, Tom Verhoeven, Jessica Burggraaff, Yaou Liu, Jorge Simoes, Serena Ruggieri, Miklos Palotai, Iman Brouwer, Adriaan Versteeg, Viktor Wottschel, Stefan Ropele, Mara A. Rocca, Claudio Gasperini, Antonio Gallo, Marios C. Yiannakas, Alex Rovira, Christian Enzinger, Massimo Filippi, Nicola De Stefano, Ludwig Kappos, Jette L. Frederiksen, Bernard M. J. Uitdehaag, Frederik Barkhof, Charles

    Background Deep grey matter (DGM) atrophy in multiple sclerosis (MS) and its relation to cognitive and clinical decline requires accurate measurements. MS pathology may deteriorate the performance of automated segmentation methods. Accuracy of DGM segmentation methods is compared between MS and controls, and the relation of performance with lesions and atrophy is studied. Methods On images of 21 MS

    更新日期:2020-07-03
  • Discontinuation of disease-modifying treatments for multiple sclerosis in patients aged over 50 with disease Inactivity
    J. Neurol. (IF 3.956) Pub Date : 2020-07-02
    Anne-Laure Kaminsky, Abdou Yacoubou Omorou, Marc Soudant, Sophie Pittion-Vouyovitch, Maud Michaud, René Anxionnat, Francis Guillemin, Marc Debouverie, Guillaume Mathey

    Background Treatments may become redundant in older patients with multiple sclerosis (MS). Our aim was to explore whether stopping treatments might be possible in patients aged over 50 with disease inactivity. Methods Patients over 50 were included from the population-based MS Lorraine registry if they had a relapsing–remitting course at onset and had experienced no relapse for ≥ 3 years. Patients

    更新日期:2020-07-02
  • Cognitive impairment in benign multiple sclerosis: a multiparametric structural and functional MRI study
    J. Neurol. (IF 3.956) Pub Date : 2020-07-02
    Gianna C. Riccitelli, Elisabetta Pagani, Alessandro Meani, Paola Valsasina, Paolo Preziosa, Massimo Filippi, Maria A. Rocca

    Introduction The substrates of cognitive impairment in benign MS (BMS) still need to be identified. We investigated whether cognitive impairment in BMS patients is associated with specific patterns of brain structural and functional abnormalities. Methods Thirty-seven BMS patients (EDSS score ≤ 3.0 and disease duration ≥ 15 years) and 50 healthy controls (HC) were studied. In BMS patients, a cognitive

    更新日期:2020-07-02
  • Long-term efficacy of immunoglobulins in small fiber neuropathy related to Sjögren’s syndrome
    J. Neurol. (IF 3.956) Pub Date : 2020-07-01
    Taylor Pindi Sala, Mona Villedieu, Laura Damian, Jean-Charles Crave, Vivien Pautot, Ljudmila Stojanovich, Jan Willem Cohen Tervaert, Patrick Cherin, Cristina Belizna

    The most common peripheral nervous system manifestations in Sjogren’s syndrome are small fiber sensory neuropathies (SFPN) and axonal sensorimotor polyneuropathies. Currently, treatment in small fiber neuropathy is mainly symptomatic and based on anti-depressors and anti-epileptics. The benefit of treatment with polyvalent immunoglobulins for SFPN has been reported in small series of patients, although

    更新日期:2020-07-01
  • Apomorphine formulation may influence subcutaneous complications from continuous subcutaneous apomorphine infusion in Parkinson’s disease
    J. Neurol. (IF 3.956) Pub Date : 2020-07-01
    Peter Hagell, Arja Höglund, Carina Hellqvist, Eva-Lena Johansson, Berit Löwed, Anne-Christine Sjöström, Carina Karlberg, Margareth Lundgren, Nil Dizdar, Anders Johansson, Thomas Willows, Johan Rådberg, Filip Bergquist

    Continuous subcutaneous (s.c.) apomorphine infusion is an effective therapy for Parkinson’s disease (PD), but a limitation is the formation of troublesome s.c. nodules. Various chemically non-identical apomorphine formulations are available. Anecdotal experiences have suggested that shifting from one of these (Apo-Go PumpFill®; apoGPF) to another (Apomorphine PharmSwed®; apoPS) may influence the occurrence

    更新日期:2020-07-01
  • High diagnostic value of plasma Niemann-Pick type C biomarkers in adults with selected neurological and/or psychiatric disorders
    J. Neurol. (IF 3.956) Pub Date : 2020-06-26
    Daniele Mandia, Marion Plaze, Isabelle Le Ber, Claire Ewenczyk, Alexandre Morin, Guilhem Carle, Angèle Consoli, Adrian Degardin, Ali Amad, Caroline Moreau, Mathieu Anheim, Christine Tranchant, Nicolas Mélé, Carole Roue-Jagot, Julien Lagarde, Marie Sarazin, Lorraine Hamelin, Pierre Ellul, Cécile Pagan, Magali Pettazzoni, Soumeya Bekri, Serge Belliard, Cyril Goizet, David Wallon, Foudil Lamari, Yann

    Late-onset Niemann-Pick type C (NP-C) is a rare, underdiagnosed lysosomal disease with neurological manifestations. A specific treatment, miglustat, can stabilize the disease if given early. Recently, three plasma screening biomarkers (PSBs) were developed [cholestane3β,5α,6βtriol (C-triol), 7-ketocholesterol (7-KC), and lysosphingomyelin-509 (LSM-509)], allowing a simpler and quite robust screening

    更新日期:2020-06-26
  • Relationship between stroke etiology and collateral status in anterior circulation large vessel occlusion.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-25
    Eva Hassler,Markus Kneihsl,Hannes Deutschmann,Nicole Hinteregger,Marton Magyar,Ulrike Wießpeiner,Melanie Haidegger,Simon Fandler-Höfler,Sebastian Eppinger,Kurt Niederkorn,Christian Enzinger,Franz Fazekas,Thomas Gattringer

    Background and purpose Clinical outcome after mechanical thrombectomy (MT) for large vessel occlusion (LVO) stroke is influenced by the intracerebral collateral status. We tested the hypothesis that patients with preexisting ipsilateral extracranial carotid artery stenosis (CAS) would have a better collateral status compared to non-CAS patients. Additionally, we evaluated MT-related adverse events

    更新日期:2020-06-25
  • What is the efficacy of gentamicin on the incidence of vertigo attacks and hearing in patients with Meniere's disease compared with steroids? A meta-analysis.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-25
    Minlin Jiang,Zimu Zhang,Chuanliang Zhao

    Objective To compare the efficacy of gentamicin and steroids in patients with Meniere's disease (MD). Methods The study protocol was structured in accordance with the Population, Intervention, Comparison and Outcome (PICO) framework. We used “Endnote” (Thomson ResearchSoft; Stanford, Connecticut, the United States) to retrieve database documents. A systematic literature search of Web of Science and

    更新日期:2020-06-25
  • Reversible Encephalopathy Syndrome (PRES) in a COVID-19 patient
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Lucia Princiotta Cariddi, Payam Tabaee Damavandi, Federico Carimati, Paola Banfi, Alessandro Clemenzi, Margherita Marelli, Andrea Giorgianni, Gabriele Vinacci, Marco Mauri, Maurizio Versino

    Recently WHO has declared novel coronavirus disease 2019 (COVID-19) outbreak a pandemic. Acute respiratory syndrome seems to be the most common manifestation of COVID-19. Besides pneumonia, it has been demonstrated that SARS-CoV-2 infection affects multiple organs, including brain tissues, causing different neurological manifestations, especially acute cerebrovascular disease (ischemic and hemorrhagic

    更新日期:2020-06-24
  • Long-term efficacy and safety of alemtuzumab in patients with RRMS: 12-year follow-up of CAMMS223.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Brian Steingo,Yaser Al Malik,Ann D Bass,Regina Berkovich,Matthew Carraro,Óscar Fernández,Carolina Ionete,Luca Massacesi,Sven G Meuth,Dimos D Mitsikostas,Gabriel Pardo,Renata Faria Simm,Anthony Traboulsee,Zia Choudhry,Nadia Daizadeh,D Alastair S Compston,

    Background In the phase 2 CAMMS223 trial (NCT00050778), alemtuzumab significantly improved clinical and MRI outcomes versus subcutaneous interferon beta-1a over 3 years in treatment-naive patients with relapsing–remitting MS. Here, we assess efficacy and safety of alemtuzumab over 12 years in CAMMS223 patients who enrolled in the CAMMS03409 extension (NCT00930553), with available follow-up through

    更新日期:2020-06-24
  • Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP).
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Luis Querol,M Crabtree,M Herepath,E Priedane,I Viejo Viejo,S Agush,P Sommerer

    Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present study provides a comprehensive literature review of the burden of illness of CIDP. Methods Systematic literature search of PubMed, Embase, and key conferences in May 2019. Search terms identified studies on the epidemiology,

    更新日期:2020-06-24
  • Olfactory testing does not predict β-amyloid, MRI measures of neurodegeneration or vascular pathology in the British 1946 birth cohort
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Sarah M. Buchanan, Thomas D. Parker, Christopher A. Lane, Ashvini Keshavan, Sarah E. Keuss, Kirsty Lu, Sarah-Naomi James, Heidi Murray-Smith, Andrew Wong, Jennifer Nicholas, David M. Cash, Ian B. Malone, William Coath, David L. Thomas, Carole Sudre, Nick C. Fox, Marcus Richards, Jonathan M. Schott

    Objective To explore the value of olfactory identification deficits as a predictor of cerebral β-amyloid status and other markers of brain health in cognitively normal adults aged ~ 70 years. Methods Cross-sectional observational cohort study. 389 largely healthy and cognitively normal older adults were recruited from the MRC National Survey of Health and Development (1946 British Birth cohort) and

    更新日期:2020-06-24
  • Early cognitive and emotional outcome after stroke is independent of discharge destination.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Jos P L Slenders,Daan P J Verberne,Johanna M A Visser-Meily,Renske M Van den Berg-Vos,Vincent I H Kwa,Caroline M van Heugten

    Background and purpose Cognitive and emotional problems occur frequently after stroke. Patients with minor stroke are more likely to be discharged home. This paper compares early cognitive and emotional outcomes in patients discharged home after stroke versus patients discharged to inpatient rehabilitation, and examines the effect of cognitive and emotional outcomes on long-term participation. Methods

    更新日期:2020-06-24
  • Use of diffusion-weighted imaging to distinguish seizure-related change from limbic encephalitis.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Adrian Budhram,Jeffrey W Britton,Greta B Liebo,Divyanshu Dubey,Anastasia Zekeridou,Eoin P Flanagan,Andrew McKeon,Sean J Pittock,Sherri A Braksick,Nicholas L Zalewski

    Objective To determine whether diffusion-weighted imaging (DWI) can help differentiate peri-ictal signal abnormality from limbic encephalitis (LE) among patients with medial temporal lobe T2-hyperintensity. Methods We retrospectively identified patients with peri-ictal medial temporal lobe T2-hyperintensity using a Mayo Clinic database, and reviewed their DWI to look for unique diffusion restriction

    更新日期:2020-06-24
  • Balance and coordination training for patients with genetic degenerative ataxia: a systematic review.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-24
    Miao He,Hai-Nan Zhang,Zhen-Chu Tang,Shu-Guang Gao

    Background and purpose The group of genetic degenerative ataxias shares the same feature of gradual deterioration in balance and coordination. However, no cure is yet available for this group of disorders, while rehabilitation remains a cornerstone in the current therapy. This review aims to present a summary of the current knowledge of balance and coordination training in patients with inherited degenerative

    更新日期:2020-06-24
  • "Myo-neuropathy" is commonly associated with mitochondrial tRNALysine mutation.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-23
    Kunqian Ji,Bing Zhao,Yan Lin,Wei Wang,Fuchen Liu,Wei Li,Yuying Zhao,Chuanzhu Yan

    The mitochondrial tRNALys (mt-tRNALys) mutation is initially associated with myoclonic epilepsy and ragged-red fibers (MERRF). The clinical, laboratory, morphologic and molecular findings from 22 mt-tRNALys mutation carriers from local database in East China were analyzed retrospectively. We identified 13 symptomatic and 9 asymptomatic individuals with a known pathogenic mitochondrial tRNALys mutation

    更新日期:2020-06-23
  • Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-23
    Jochem Helleman,Esther T Kruitwagen-van Reenen,J Bakers,Willeke J Kruithof,Annerieke C van Groenestijn,Rineke J H Jaspers Focks,Arthur de Grund,Leonard H van den Berg,Johanna M A Visser-Meily,Anita Beelen

    Background Poor monitoring of respiratory function may lead to late initiation of non-invasive ventilation (NIV) in patients with motor neuron diseases (MND). Monitoring could be improved by remotely assessing hypoventilation symptoms between clinic visits. We aimed to determine which patient-reported hypoventilation symptoms are best for screening reduced respiratory function in patients with MND

    更新日期:2020-06-23
  • Features of intracranial hemorrhage in cerebral venous thrombosis.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-22
    K Afifi,G Bellanger,P J Buyck,S M Zuurbier,C G Esperon,M A Barboza,P Costa,I Escudero,D Renard,R Lemmens,N Hinteregger,F Fazekas,J Jimenez Conde,E Giralt-Steinhauer,S Hiltunen,A Arauz,A Pezzini,J Montaner,J Putaala,C Weimar,Marc Schlamann,T Gattringer,T Tatlisumak,J M Coutinho,P Demaerel,V Thijs

    Background Cerebral venous thrombosis (CVT) is associated with intracranial hemorrhage. Aim To identify clinical and imaging features of CVT-associated intracranial hemorrhage. We hypothesized that higher clot burden would be associated with a higher risk of intracranial hemorrhage. Methods We performed a retrospective analysis of an international, multicenter cohort of patients with confirmed cerebral

    更新日期:2020-06-23
  • Late-onset post-lesional paroxysmal hypothermia: a case series and literature review.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-22
    Perrine Devic,Laure Peter-Derex,Aurélie Richard-Mornas,Sylvie Ernesto,Pascale Nesme,François Mauguière,Syvain Rheims

    Background Paroxysmal hypothermia (PH) is a rare condition characterized by recurrent episodes of spontaneous hypothermia, bradycardia, disorders of consciousness and, in some cases, hyperhidrosis. When associated with a detectable hypothalamic lesion, PH episodes usually occur shortly after the brain insult. Methods We performed a retrospective study to identify patients who had demonstrated at least

    更新日期:2020-06-23
  • First case of SARS-COV-2 sequencing in cerebrospinal fluid of a patient with suspected demyelinating disease.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-20
    Renan Barros Domingues,Maria Cássia Mendes-Correa,Fernando Brunale Vilela de Moura Leite,Ester Cerdeira Sabino,Diego Zanotti Salarini,Ingra Claro,Daniel Wagner Santos,Jaqueline Goes de Jesus,Noely Evangelista Ferreira,Camila Malta Romano,Carlos Augusto Senne Soares

    The association between coronaviruses and central nervous system (CNS) demyelinating lesions has been previously shown. However, no case has been described of an association between the novel coronavirus (SARS-COV-2) and CNS demyelinating disease so far. SARS-COV-2 was previously detected in cerebrospinal fluid (CSF) sample of a patient with encephalitis. However, the virus identity was not confirmed

    更新日期:2020-06-23
  • Midlife alcohol consumption and longitudinal brain atrophy: the PREVENT-Dementia study.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-20
    Michael J Firbank,John T O'Brien,Karen Ritchie,Katie Wells,Guy Williams,Li Su,Craig W Ritchie

    Background and aims Consensus is lacking on whether light to moderate consumption of alcohol compared to abstinence is neuroprotective. In this study, we investigated the relationship between self-reported alcohol use and brain volume change over 2 years in middle-aged subjects. Methods A sample of 162 subjects (aged 40–59 at baseline) from the PREVENT-Dementia programme underwent MRI scans on two

    更新日期:2020-06-23
  • Clinico-radiological dissociation of disease activity in MS patients: frequency and clinical relevance.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-20
    Nina L van Faals,Iris Dekker,Lisanne J Balk,Bastiaan Moraal,Frederik Barkhof,Bernard M J Uitdehaag,Joep Killestein,Mike P Wattjes

    Objective To investigate the prevalence and clinical relevance regarding disability progression in multiple sclerosis patients with a dissociation in clinical and radiological disease expression. Methods We prospectively selected patients with early relapsing–remitting multiple sclerosis (MS) or a clinically isolated syndrome (CIS) from the Amsterdam MS cohort. Patients underwent clinical examination

    更新日期:2020-06-23
  • Guillain-Barré syndrome in the COVID-19 era: another occasional cluster?
    J. Neurol. (IF 3.956) Pub Date : 2020-06-23
    Laurent Tatu,Sandra Nono,Simone Grácio,Serdar Koçer

    更新日期:2020-06-23
  • Neurological involvement of coronavirus disease 2019: a systematic review.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-19
    Malik Ghannam,Qasem Alshaer,Mustafa Al-Chalabi,Lara Zakarna,Jetter Robertson,Georgios Manousakis

    Background In December 2019, unexplained cases of pneumonia emerged in Wuhan, China, which were found to be secondary to the novel coronavirus SARS-CoV-2. On March 11, 2020, the WHO declared the Coronavirus Disease 2019 (COVID-2019) outbreak, a pandemic. Objective To clarify the neurological complications of SARS-CoV-2 infection including the potential mechanisms and therapeutic options. Methods We

    更新日期:2020-06-19
  • Investigation of poststroke epilepsy (INPOSE) study: a multicenter prospective study for prediction of poststroke epilepsy.
    J. Neurol. (IF 3.956) Pub Date : 2020-06-19
    Shuichi Yamada,Ichiro Nakagawa,Kentaro Tamura,Fumihiko Nishimura,Yasushi Motoyama,Young-Soo Park,Hiroyuki Nakase

    Background and purpose Poststroke epilepsy is a common problem in clinical practice. This study aimed to determine the predictors of poststroke epilepsy and to identify the risk factors. Methods A total of 436 patients were enrolled for this study. Baseline characteristics were recorded. Patients were followed up for 3 years. According to the occurrence of late seizures (i.e., seizure occurring 8 days

    更新日期:2020-06-19
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