Journal of Neurology

Joachim Havla, Marlene Moser, Clara Sztatecsny, Amelie S Lotz-Havla, Esther M Maier, Baccara Hizli, Regina Schinner, Tania Kümpfel, Michael Strupp, Tatiana Bremova-Ertl, Susanne A Schneider
OBJECTIVE: Niemann-Pick disease type C1 (NPC1) is a rare autosomal-recessive lysosomal storage disorder presenting with a broad clinical spectrum ranging from a severe infantile-onset neurovisceral disorder to late-onset neurodegenerative disease. Optical coherence tomography (OCT) is established to detect retinal degeneration in vivo. We examined NPC1-patients (NPC1-P), clinically asymptomatic NPC1-mutation carriers (NPC1-MC), and healthy controls (HC) to (1) identify retinal degeneration in NPC1-disease and (2) to investigate possible subclinical retinal degeneration in NPC1-MC...
March 28, 2020: Journal of Neurology
Alessandra Tesei, Maria Nobile, Paola Colombo, Federica Civati, Sandra Gandossini, Elisa Mani, Massimo Molteni, Nereo Bresolin, Grazia D'Angelo
BACKGROUND: Living with a progressive disease as muscular dystrophy (MD) can be challenging for the patient and the entire family from both emotional and practical point of view. We aimed to extend our previously published data about mental health in patients with MDs, also investigating coping profiles of both themselves and their parents. Furthermore, we wanted to verify whether psychological adaptation of patients can be predicted by coping strategies, taking also into account physical impairment, cognitive level and socioeconomic status...
March 28, 2020: Journal of Neurology
Bastien Joubert, Aude Belbezier, Julie Haesebaert, Sylvain Rheims, François Ducray, Géraldine Picard, Véronique Rogemond, Dimitri Psimaras, Giulia Berzero, Virginie Desestret, Jérôme Honnorat
OBJECTIVE: To assess the long-term outcomes of patients with temporal lobe epilepsy and CSF anti-glutamate decarboxylase antibodies (GAD65-Abs). METHODS: We retrospectively analyzed the clinical records of 35 patients with temporal lobe epilepsy and CSF GAD65-Abs, collected from January 1993 to December 2016 and assessed cognitive impairment and seizure activity at last visit. Cognitive impairment was considered significant if impacting on daily life activities...
March 28, 2020: Journal of Neurology
Heiko Gaßner, Dennis Jensen, Franz Marxreiter, Anja Kletsch, Stefan Bohlen, Robin Schubert, Lisa M Muratori, Bjoern Eskofier, Jochen Klucken, Jürgen Winkler, Ralf Reilmann, Zacharias Kohl
The original version of this article unfortunately contained a mistake. The name of one author is not presented correctly in the author group.
March 27, 2020: Journal of Neurology
Yoshihisa Masakado, Masahiro Abo, Kunitsugu Kondo, Satoru Saeki, Eiichi Saitoh, Andrzej Dekundy, Angelika Hanschmann, Ryuji Kaji
BACKGROUND: Upper-limb spasticity frequently occurs after stroke and there is a clinical need for more effective therapies. The Phase III J-PURE study assessed the efficacy and safety of incobotulinumtoxinA up to 400 U for post-stroke upper-limb spasticity in Japan. METHODS: In the 12-week main period (MP) of this double-blind, placebo-controlled study, Japanese subjects with upper-limb spasticity received one injection cycle of incobotulinumtoxinA 400 U, 250 U, or matching placebo...
March 26, 2020: Journal of Neurology
J M Poos, L C Jiskoot, S M J Leijdesdorff, H Seelaar, J L Panman, E L van der Ende, M O Mol, L H H Meeter, Y A L Pijnenburg, L Donker Kaat, F J de Jong, J C van Swieten, J M Papma, E van den Berg
The original version of this article unfortunately contained a mistake.
March 26, 2020: Journal of Neurology
Antonio Carotenuto, Heather Wilson, Beniamino Giordano, Silvia P Caminiti, Zachary Chappell, Steven C R Williams, Alexander Hammers, Eli Silber, Peter Brex, Marios Politis
There is mounting evidence regarding the role of impairment in neuromodulatory networks for neurodegenerative diseases, such as Parkinson's and Alzheimer's disease. However, the role of neuromodulatory networks in multiple sclerosis (MS) has not been assessed. We applied resting-state functional connectivity and graph theory to investigate the changes in the functional connectivity within neuromodulatory networks including the serotonergic, noradrenergic, cholinergic, and dopaminergic systems in MS. Twenty-nine MS patients and twenty-four age- and gender-matched healthy controls performed clinical and cognitive assessments including the expanded disability status score, symbol digit modalities test, and Hamilton Depression rating scale...
March 26, 2020: Journal of Neurology
Jeffrey Shi Kai Chan, Dawnie Ho Hei Lau
No abstract text is available yet for this article.
March 23, 2020: Journal of Neurology
Svenja Stoll, Kosmas Macha, Armin Marsch, Stefan T Gerner, Gabriela Siedler, Kilian Fröhlich, Bastian Volbers, Erwin F Strasser, Stefan Schwab, Bernd Kallmünzer
BACKGROUND: Oral Factor Xa inhibitors for the prevention of stroke in atrial fibrillation require dose adjustment based on certain clinical criteria, but the off-label use of the reduced doses is common. METHODS: Data from an observational registry including patients admitted with acute cerebral ischemia while taking oral Factor Xa inhibitors for atrial fibrillation between April 2016 and December 2018 were investigated. The dose regimen of the Xa inhibitor was classified as "appropriate", "underdosed" and "overdosed" in conformity with the European Medicines Agency labelling...
March 23, 2020: Journal of Neurology
Julian Conrad, Matthias Ertl, Meret H Oltmanns, Peter Zu Eulenburg
BACKGROUND AND AIM: The extent of penumbra tissue and outcome in stroke patients depend on the collateral cranial vasculature. To provide optimal individualized care for stroke patients in the emergency room setting we investigated the predictive capability of a stringent evaluation of the collateral vessels in ischemic stroke on clinical outcome and infarct size. METHODS: We retrospectively studied uniform clinical and radiological data of 686 consecutive patients admitted to the emergency department with suspected acute ischemic stroke...
March 23, 2020: Journal of Neurology
Jean-Yves Hogrel, Valérie Decostre, Isabelle Ledoux, Marie de Antonio, Erik H Niks, Imelda de Groot, Volker Straub, Francesco Muntoni, Valeria Ricotti, Thomas Voit, Andreea Seferian, Teresa Gidaro, Laurent Servais
OBJECTIVE: The main aim was to explore the changes in hand-grip strength in patients with Duchenne muscular dystrophy (DMD) aged 5-29 years. Secondary aims were to test the effect of mutation, ambulatory status and glucocorticoid use on grip strength and its changes over time and to compute the number of subjects needed for a clinical trial to stabilize grip strength. METHODS: The analysis was performed on data collected during five international natural history studies on a cohort of DMD patients...
March 21, 2020: Journal of Neurology
Angelos Sharobeam, Brett Jones, Dianne Walton-Sonda, Christian J Lueck
BACKGROUND/AIMS: This review examined factors that delay thrombolysis and what management strategies are currently employed to minimise this delay, with the aim of suggesting future directions to overcome bottlenecks in treatment delivery. METHODS: A systematic review was performed according to PRISMA guidelines. The search strategy included a combination of synonyms and controlled vocabularies from Medical Subject Headings (MeSH) and EmTree covering brain ischemia, cerebrovascular accident, fibrinolytic therapy and Alteplase...
March 21, 2020: Journal of Neurology
Chloe K Nobuhara, W Michael Bullock, Thomas Bunning, Brian Colin, Mary Cooter, Michael J Devinney, Michael N Ferrandino, Jeffrey Gadsden, Grant Garrigues, Ashraf S Habib, Eugene Moretti, Judd Moul, Brian Ohlendorf, Aaron Sandler, Randall Scheri, Bharat Sharma, Jake P Thomas, Christopher Young, Joseph P Mathew, Miles Berger
OBJECTIVE: Lumbar punctures (LPs) are important for obtaining CSF in neurology studies but are associated with adverse events and feared by many patients. We determined adverse event rates and pain scores in patients prospectively enrolled in two cohort studies who underwent LPs using a standardized protocol and 25 g needle. METHODS: Eight hundred and nine LPs performed in 262 patients age ≥ 60 years in the MADCO-PC and INTUIT studies were analyzed. Medical records were monitored for LP-related adverse events, and patients were queried about subjective complaints...
March 20, 2020: Journal of Neurology
Aurore Thibaut, Yelena G Bodien, Steven Laureys, Joseph T Giacino
Following electronic publication of the above-referenced manuscript, we discovered that one of the three criteria we proposed to establish command-following in the MCS+ syndrome was inadvertently omitted in some parts of the manuscript.
March 19, 2020: Journal of Neurology
Gabriela S Gilmour, Glenn Nielsen, Tiago Teodoro, Mahinda Yogarajah, Jan Adriaan Coebergh, Michael D Dilley, Davide Martino, Mark J Edwards
Functional neurological disorder (FND) is a common cause of persistent and disabling neurological symptoms. These symptoms are varied and include abnormal control of movement, episodes of altered awareness resembling epileptic seizures and abnormal sensation and are often comorbid with chronic pain, fatigue and cognitive symptoms. There is increasing evidence for the role of neurologists in both the assessment and management of FND. The aim of this review is to discuss strategies for the management of FND by focusing on the diagnostic discussion and general principles, as well as specific treatment strategies for various FND symptoms, highlighting the role of the neurologist and proposing a structure for an interdisciplinary FND service...
March 19, 2020: Journal of Neurology
Michael Strupp, Ludwig Kraus, Franz Schautzer, Dan Rujescu
The authors have retracted this article.
March 18, 2020: Journal of Neurology
John Vissing, Saiju Jacob, Kenji P Fujita, Fanny O'Brien, James F Howard
BACKGROUND: The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), and its open-label extension. METHODS: Attainment of 'minimal symptom expression' was evaluated using patient-reported outcome measures of gMG symptoms [MG activities of daily living scale (MG-ADL), 15-item MG quality of life questionnaire (MG-QOL15)] at the completion of REGAIN and during the open-label extension...
March 18, 2020: Journal of Neurology
Karol Wiśniewski, Joanna Wojtkiewicz
No abstract text is available yet for this article.
March 16, 2020: Journal of Neurology
Huan Cai, Biyang Cai, Zhonghua Liu, Wenjun Wu, Dihong Chen, Liang Fang, Liyi Chen, Wen Sun, Jialin Liang, Hao Zhang
BACKGROUND AND PURPOSE: Considerable studies have reported inconsistent relationships between ischemic stroke and a large number of factors. These uncertainties may reflect the susceptibility to confounding in observational studies. We aimed to assess genetic correlations and causal relationships between ischemic stroke and diverse phenotypes. METHODS: Summary-level data for ischemic stroke (34,217 cases and 406,111 controls) from the MEGASTROKE consortium were used as the outcome...
March 14, 2020: Journal of Neurology
Clara Alexandra Stafford, Bobby Stojanoski, Conor J Wild, Danielle Brewer-Deluce, Timothy D Wilson, Adrian M Owen
OBJECTIVE: We investigated the long-term cognitive effects of concussion in 19,261 members of the general population and a cohort of varsity American football players with a history of frequent head impacts, using tests that are known to be sensitive to small changes in performance. METHODS: We asked 19,261 participants to complete a demographic questionnaire and 12 cognitive tests measuring aspects of executive function, including inhibitory control. We compared the performance of those reporting a history of concussion (post-concussion) to those reporting no history of concussion (non-concussed) on the cognitive battery and four non-cognitive variables...
March 14, 2020: Journal of Neurology
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