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Journal of Neurology

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https://www.readbyqxmd.com/read/28516332/prevalence-and-mortality-of-patients-with-multiple-sclerosis-in-france-in-2012-a-study-based-on-french-health-insurance-data
#1
Stéphanie Foulon, Géric Maura, Marie Dalichampt, François Alla, Marc Debouverie, Thibault Moreau, Alain Weill
Data on the prevalence of multiple sclerosis (MS) in France are scarce. National and regional updated estimates are needed to better plan health policies. In this nationwide study, we provided estimates of the prevalence of MS in France in 2012 and mortality rate in 2013. MS cases were identified in the French national health insurance database (SNIIRAM-PMSI) using reimbursement data for disease-modifying treatment, long-term disease status for MS, disability pension for MS, and hospitalisation for MS (MS ICD-10 code: G35)...
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28516331/burden-of-herpes-simplex-virus-encephalitis-in-the-united-states
#2
S Modi, Abhimanyu Mahajan, D Dharaiya, P Varelas, P Mitsias
Herpes simplex virus encephalitis (HSVE) is a disease of public health concern, but its burden on the healthcare of United States has not been adequately assessed recently. We aimed to define the incidence, complications and outcomes of HSVE in the recent decade by analyzing data from a nationally representative database. Healthcare Cost and Utilization Project databases were utilized to identify patients with primary discharge diagnosis of HSVE. Annual hospitalization rate was estimated and several preselected inpatient complications were identified...
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28516330/why-antisense-could-make-sense-for-neurodegeneration
#3
T H Massey, N P Robertson
No abstract text is available yet for this article.
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28516329/screening-for-lipoprotein-receptor-related-protein-4-agrin-and-titin-antibodies-and-exploring-the-autoimmune-spectrum-in-myasthenia-gravis
#4
Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S Tzartos, Lin Mei, Jens Reimann, Philip Van Damme, Michael H Rivner, Alain Vigneron, Joachim Weis, Jörg B Schulz, Socrates J Tzartos, Kristl G Claeys
In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin...
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28516328/chronic-traumatic-encephalopathy-identifying-those-at-risk-and-understanding-pathogenesis
#5
M D Willis, N P Robertson
No abstract text is available yet for this article.
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28516327/long-term-outcomes-of-epilepsy-surgery-in-tuberous-sclerosis-complex
#6
Shuli Liang, Juncheng Zhang, Zhixian Yang, Shaohui Zhang, Zhiqiang Cui, Jianfei Cui, Jiwu Zhang, Na Liu, Ping Ding
Approximately 50% of patients with tuberous sclerosis complex (TSC) present intractable epilepsy, and surgery is an option for those patients. Hereby, we analyze long-term seizure control and neuropsychological outcomes of epilepsy surgery in patients with TSC. Clinical data were retrospectively collected from 66 patients with TSC and epilepsy followed up over 5 years, 51 of whom underwent epilepsy surgery between 2001 and 2011. Reductions in the number of seizures were analyzed at 1-year (1FU), 5-year (5FU), and 10-year (10FU) follow-ups visits after the operation...
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28503706/an-eye-tracker-controlled-cognitive-battery-overcoming-verbal-motor-limitations-in-als
#7
Barbara Poletti, Laura Carelli, Federica Solca, Annalisa Lafronza, Elisa Pedroli, Andrea Faini, Nicola Ticozzi, Andrea Ciammola, Paolo Meriggi, Pietro Cipresso, Dorothée Lulé, Albert C Ludolph, Giuseppe Riva, Vincenzo Silani
We assessed language, attention, executive, and social cognition abilities in a sample of patients with Amyotrophic Lateral Sclerosis (ALS) by means of a recently developed cognitive battery based on oculomotor control with eye-tracking (ET) technology. Twenty-one ALS patients and 21 age- and education-matched healthy subjects underwent the ET-based cognitive assessment, together with the standard cognitive screening tools [Frontal Assessment Battery (FAB); Montreal Cognitive Assessment (MoCA); and Digit Sequencing Task]...
May 13, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28503705/mri-in-neutral-lipid-storage-disease-nlsd
#8
Matteo Garibaldi, Giorgio Tasca, Jordi Diaz-Manera, Pierfancesco Ottaviani, Francesco Laschena, Donatella Pantoli, Simonetta Gerevini, Chiara Fiorillo, Lorenzo Maggi, Elisabetta Tasca, Adele D'Amico, Olimpia Musumeci, Antonio Toscano, Claudio Bruno, Roberto Massa, Corrado Angelini, Enrico Bertini, Giovanni Antonini, Elena Maria Pennisi
Neutral lipid storage disease (NLSD) is a rare inherited disorder of lipid metabolism resulting in lipid droplets accumulation in different tissues. Skeletal muscle could be affected in both two different form of disease: NLSD with myopathy (NLSD-M) and NLSD with ichthyosis (NLSD-I). We present the muscle imaging data of 12 patients from the Italian Network for NLSD: ten patients presenting NLSD-M and two patients with NLSD-I. In NLSD-M gluteus minimus, semimembranosus, soleus and gastrocnemius medialis in the lower limbs and infraspinatus in the upper limbs were the most affected muscles...
May 13, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28503704/use-of-eeg-in-critically-ill-children-and-neonates-in-the-united-states-of-america
#9
Marina Gaínza-Lein, Iván Sánchez Fernández, Tobias Loddenkemper
The objective of the study was to estimate the proportion of patients who receive an electroencephalogram (EEG) among five common indications for EEG monitoring in the intensive care unit: traumatic brain injury (TBI), extracorporeal membrane oxygenation (ECMO), cardiac arrest, cardiac surgery and hypoxic-ischemic encephalopathy (HIE). We performed a retrospective cross-sectional descriptive study utilizing the Kids' Inpatient Database (KID) for the years 2010-2012. The KID is the largest pediatric inpatient database in the USA and it is based on discharge reports created by hospitals for billing purposes...
May 13, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28503703/abnormal-vestibular-evoked-myogenic-potentials-as-an-isolated-finding-of-probable-transient-labyrinthine-ischemia
#10
LETTER
Sun-Uk Lee, Hyo-Jung Kim, Jeong-Yoon Choi, Ji-Soo Kim
No abstract text is available yet for this article.
May 13, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28492970/oral-and-intravenous-steroids-for-multiple-sclerosis-relapse-a-systematic-review-and-meta-analysis
#11
REVIEW
Simona Lattanzi, Claudia Cagnetti, Maura Danni, Leandro Provinciali, Mauro Silvestrini
Glucocorticoids are the standard of care for multiple sclerosis (MS) relapses, but the most desirable route of administration is still matter of debate. The aim of the study was to compare the efficacy and safety of oral versus intravenous steroids for treatment of acute relapses in patients with MS. Randomized or quasi-randomized, parallel group trials with direct comparison between oral and intravenous steroid treatment in MS patients with acute relapse were identified through a systematic literature search...
May 10, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28484839/plasma-exchanges-for-severe-acute-neurological-deterioration-in-patients-with-igm-anti-myelin-associated-glycoprotein-anti-mag-neuropathy
#12
M Baron, P Lozeron, S Harel, D Bengoufa, M Vignon, B Asli, M Malphettes, N Parquet, A Brignier, J P Fermand, N Kubis, Bertrand Arnulf
Monoclonal IgM anti-myelin-associated glycoprotein (MAG) antibody-related peripheral neuropathy (anti-MAG neuropathy) is predominantly a demyelinating sensory neuropathy with ataxia and distal paresthesia. The clinical course of anti-MAG neuropathy is usually slowly progressive making difficult the identification of clear criteria to start a specific treatment. Although no consensus treatment is yet available, a rituximab-based regimen targeting the B-cell clone producing the monoclonal IgM may be proposed, alone or in combination with alkylating agents or purine analogs...
May 8, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28478596/deciphering-the-causes-of-sporadic-late-onset-cerebellar-ataxias-a-prospective-study-with-implications-for-diagnostic-work
#13
O Gebus, S Montaut, B Monga, T Wirth, C Cheraud, C Alves Do Rego, I Zinchenko, G Carré, M Hamdaoui, G Hautecloque, L Nguyen-Them, B Lannes, J B Chanson, O Lagha-Boukbiza, M C Fleury, D Devys, G Nicolas, G Rudolf, M Bereau, M Mallaret, M Renaud, C Acquaviva, M Koenig, M Koob, S Kremer, I J Namer, C Cazeneuve, A Echaniz-Laguna, C Tranchant, Mathieu Anheim
The management of sporadic late-onset cerebellar ataxias represents a very heterogeneous group of patients and remains a challenge for neurologist in clinical practice. We aimed at describing the different causes of sporadic late-onset cerebellar ataxias that were diagnosed following standardized, exhaustive investigations and the population characteristics according to the aetiologies as well as at evaluating the relevance of these investigations. All patients consecutively referred to our centre due to sporadic, progressive cerebellar ataxia occurring after 40 years of age were included in the prospective, observational study...
May 6, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28470593/clinical-assessment-of-dysphagia-in-neurodegeneration-cadn-development-validity-and-reliability-of-a-bedside-tool-for-dysphagia-assessment
#14
Adam P Vogel, Natalie Rommel, Carina Sauer, Marius Horger, Patrick Krumm, Marc Himmelbach, Matthis Synofzik
Screening assessments for dysphagia are essential in neurodegenerative disease. Yet there are no purpose-built tools to quantify swallowing deficits at bedside or in clinical trials. A quantifiable, brief, easy to administer assessment that measures the impact of dysphagia and predicts the presence or absence of aspiration is needed. The Clinical Assessment of Dysphagia in Neurodegeneration (CADN) was designed by a multidisciplinary team (neurology, neuropsychology, speech pathology) validated against strict methodological criteria in two neurodegenerative diseases, Parkinson's disease (PD) and degenerative ataxia (DA)...
May 3, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28470592/japanese-encephalitis-can-trigger-anti-n-methyl-d-aspartate-receptor-encephalitis
#15
Jiannan Ma, Ting Zhang, Li Jiang
Japanese encephalitis (JE) is usually a monophasic disease; however, in rare cases, patients with JE may have an early relapse after a partial recovery, giving rise to a biphasic pattern for the disease. In this study, we report three pediatric cases in which post-JE relapse was characterized by movement disorder and/or behavioral problems, and was related to anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G (IgG). Serum and cerebrospinal fluid were examined for anti-NMDAR IgG in three patients who had confirmed JE and then developed relapsing symptoms which were similar to those of anti-NMDAR encephalitis...
May 3, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28470591/high-intensity-interval-training-in-facioscapulohumeral-muscular-dystrophy-type-1-a-randomized-clinical-trial
#16
Grete Andersen, Karen Heje, Astrid Emile Buch, John Vissing
Increasing evidence suggests that high-intensity training (HIT) is a time-efficient exercise strategy to improve fitness. HIT has never been explored in neuromuscular diseases, likely because it may seem counterintuitive. A single session of high-intensity exercise has been studied without signs of muscle damage in facioscapulohumeral muscular dystrophy type 1 (FSHD1). We aimed to determine whether HIT is safe and effective in FSHD1 in a randomized, controlled parallel study. Untrained adults with genetically verified FSHD1 (n = 13) able to perform cycle-ergometer exercise were randomized to 8 weeks of supervised HIT (n = 6) (3 × 10-min cycle-ergometer-HIT/week) or 8 weeks of usual care (n = 7)...
May 3, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28455667/adult-onset-ataxia-or-developmental-disorder-with-seizures-two-sides-of-missense-changes-in-cacna1a
#17
LETTER
Alexander Balck, Henrike Hanssen, Yorck Hellenbroich, Katja Lohmann, Alexander Münchau
No abstract text is available yet for this article.
April 28, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28455666/revisiting-progressive-stroke-incidence-predictors-pathophysiology-and-management-of-unexplained-early-neurological-deterioration-following-acute-ischemic-stroke
#18
Pierre Seners, Jean-Claude Baron
Early neurological deterioration (END) following acute ischemic stroke is a serious clinical event strongly associated with poor outcome. Regarding specifically END occurring within 24 h following stroke onset, apart from straightforward causes such as symptomatic intracranial haemorrhage and malignant edema, the cause of END remains unclear in more than a half of cases. In the latter situation, patients are often referred to as 'progressive stroke', a default clinical category that does not imply underlying mechanisms, precluding informed management...
April 28, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28455665/cross-coupling-vestibular-stimulation-motion-sickness-and-the-vestibulo-sympathetic-reflex
#19
Fausto Romano, Nicoletta Caramia, Dominik Straumann, Eugene Nalivaiko, Giovanni Bertolini
Motion sickness is associated with a variety of autonomic symptoms, presumably due to proximity or functional interconnectivity between the autonomic centers in the brainstem and the vestibular system. A direct influence of the vestibular system on cardiovascular variables, defined as the vestibulo-sympathetic reflex, has been reported previously. Our aim was to investigate the sudomotor components of the autonomic responses associated with motion sickness during passive cross-coupling stimulation ("roll while rotating")...
April 28, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28444447/william-g-niederland-1904-1993
#20
Frank W Stahnisch
No abstract text is available yet for this article.
April 25, 2017: Journal of Neurology
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