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Journal of Neurology

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https://www.readbyqxmd.com/read/29785524/a-9-year-follow-up-study-of-quantitative-muscle-strength-changes-in-myotonic-dystrophy-type-1
#1
Cynthia Gagnon, Émilie Petitclerc, Marie Kierkegaard, Jean Mathieu, Élise Duchesne, Luc J Hébert
Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder presenting with major muscle impairments. However, few studies have addressed muscle strength progression using quantitative methods. The aims of this study were to document muscle strength changes in eight muscle groups among adults with DM1 over a 9-year period, and to compare this progression between phenotypes (adult and late-onset) and sex. Patients with a genetic diagnosis of DM1 with the late-onset or the adult phenotype were recruited at baseline through the clinical registry of the Saguenay Neuromuscular Clinic...
May 21, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29785523/efficacy-and-safety-of-rituximab-in-relapsing-and-progressive-multiple-sclerosis-a-hospital-based-study
#2
Carmen Alcalá, F Gascón, F Pérez-Miralles, S Gil-Perotín, A Navarré, I Boscá, F Coret, B Casanova
INTRODUCTION: Rituximab is considered as a potential therapeutic option in relapsing-remitting (RRMS) and progressive forms (PMS) of multiple sclerosis (MS). OBJECTIVE: To investigate the effectiveness and safety of rituximab in MS. PATIENTS AND METHODS: Observational study of effectiveness (clinical and radiological) and safety of rituximab in RRMS and PMS. RESULTS: A total of 90 rituximab-treated patients were collected: 31 RRMS and 59 PMS All patients had an active disease despite standard treatment...
May 21, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29785522/non-invasive-vagus-nerve-stimulation-significantly-improves-quality-of-life-in-patients-with-persistent-postural-perceptual-dizziness
#3
Ozan E Eren, Filipp Filippopulos, Kristina Sönmez, Ken Möhwald, Andreas Straube, Florian Schöberl
Persistent postural-perceptual dizziness (PPPD) is one of the most common causes of chronic vestibular disorders, with a substantial portion of the affected patients showing no significant improvement to standard therapies (i.e., pharmacotherapy, behavioral psychotherapy). Patients with PPPD have been shown to have a significant comorbidity with anxiety disorders and depression. Further, these patients show an activation of the autonomic nervous system resulting in symptoms such as nausea, increase of heart rate, and sweating...
May 21, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29777362/the-silver-effect-of-admission-glucose-level-on-excellent-outcome-in-thrombolysed-stroke-patients
#4
Charlotte Rosso, Flore Baronnet, Belen Diaz, Raphael Le Bouc, Giulia Frasca Polara, Eric Jr Moulton, Sandrine Deltour, Anne Leger, Sophie Crozier, Yves Samson
Higher admission glucose levels (AGL) are associated with less favorable outcome in thrombolysis. But, could AGL's impact on outcome vary by onset-to-treatment (OTT) time? Is hyperglycemia associated with a shorter therapeutic time window for excellent outcome for thrombolysed stroke patients? We assessed predictive values of AGL, baseline NIHSS, age, and OTT time quartiles on excellent outcome (3-month modified Rankin score of 0-1) in 773 patients treated by rt-Pa. We added the AGL × OTT time quartile interaction in the model and separately analyzed the predictive values of AGL, age, and NIHSS for each OTT time quartile if the interaction was significant...
May 18, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29777361/tremor-induced-by-calcineurin-inhibitor-immunosuppression-a-single-centre-observational-study-in-kidney-transplanted-patients
#5
R Erro, Ruggero Bacchin, F Magrinelli, P Tomei, C Geroin, G Squintani, A Lupo, G Zaza, M Tinazzi
INTRODUCTION: Tremor is the most frequent and disabling neurological side effect under Calcineurin inhibitor-induced immunosuppression, but no studies have defined its phenomenology, severity, distribution, the impact on quality of life, as well as of other neurological symptoms associated. METHODS: 126 consecutive kidney-transplanted patients, under treatment with Cyclosporin A, Tacrolimus and non-Calcineurin inhibitors, within therapeutic range, were enrolled...
May 18, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29777360/crossed-aphasia-following-cerebral-infarction-in-a-right-handed-patient-with-atypical-cerebral-language-dominance
#6
Xiaoping Tan, Yang Guo, Saihong Dun, Hongzan Sun
OBJECTIVE: Crossed aphasia (CA), usually referred to as an acquired language disturbance, is caused by a lesion in the cerebral hemisphere ipsilateral to the dominant hand, and the exact mechanism is not clear. The development of handedness is influenced by education and training and the impact of habitualization, while language is more susceptible to the impact of speech habits, and it is not absolutely accurate to judge cerebral language dominance by the degree of hand preference. METHODS: We describe a case of CA after right hemispheric stroke in a right-handed patient with atypical language dominance and attempt to analyze the mechanism of CA based on functional imaging methods, including arterial spin labeling (ASL) and positron emission tomography/magnetic resonance imaging (PET-MRI)...
May 18, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29767354/proprioceptive-muscle-tendon-stimulation-reduces-symptoms-in-primary-orthostatic-tremor
#7
M Wuehr, C Schlick, K Möhwald, R Schniepp
INTRODUCTION: Primary orthostatic tremor (OT) is characterized by high-frequency lower limb muscle contractions and a disabling sense of unsteadiness while standing. To date, therapeutic options for OT are limited. Here, we examined the effects of proprioceptive leg muscle stimulation via muscle tendon vibration (MTV) on tremor and balance control in patients with primary OT. METHODS: Tremor in nine patients with primary OT was examined during four conditions: standing (1), standing with MTV on the bilateral soleus muscles (2), lying (3), and lying with MTV (4)...
May 16, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29767353/influences-of-temporal-lobe-epilepsy-and-temporal-lobe-resection-on-olfaction
#8
Richard L Doty, Isabelle Tourbier, Jessica K Neff, Jonathan Silas, Bruce Turetsky, Paul Moberg, Taehoon Kim, John Pluta, Jaqueline French, Ashwini D Sharan, Michael J Sperling, Natasha Mirza, Anthony Risser, Gordon Baltuch, John A Detre
Although temporal lobe epilepsy (TLE) and resection (TLR) impact olfactory eloquent brain structures, their influences on olfaction remain enigmatic. We sought to more definitively assess the influences of TLE and TLR on olfaction using three well-validated olfactory tests and measuring  the tests' associations with the volume of numerous temporal lobe brain structures. The University of Pennsylvania Smell Identification Test and an odor detection threshold test were administered to 71 TLE patients and 71 age- and sex-matched controls; 69 TLE patients and controls received an odor discrimination/memory test...
May 16, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29767352/multifocal-motor-neuropathy-following-treatment-with-adalimumab-for-ulcerative-colitis
#9
LETTER
Laura Fionda, Fiammetta Vanoli, Antonella Di Pasquale, Luca Leonardi, Stefania Morino, Giovanni Antonini
No abstract text is available yet for this article.
May 16, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29766271/obstructive-sleep-apnea-and-cerebral-white-matter-change-a-systematic-review-and-meta-analysis
#10
Bo-Lin Ho, Ping-Tao Tseng, Chiou-Lian Lai, Meng-Ni Wu, Ming-Ju Tsai, Cheng-Fang Hsieh, Tien-Yu Chen, Chung-Yao Hsu
Obstructive sleep apnea (OSA) can cause sleep fragmentation and intermittent hypoxemia, which are linked to oxidative stress. White matter changes (WMCs) representing cerebrovascular burden and are at risk factor for oxidative ischemic injury. The current study explores the mutual relationships between OSA and WMCs. We performed a systematic review of electronic databases for clinical studies investigating OSA and WMCs. Random-effects models were used for pooled estimates calculation. A total of 22 studies were included in the meta-analysis...
May 15, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29761297/severe-neurologic-complications-of-immune-checkpoint-inhibitors-a-single-center-review
#11
Sarah Mancone, Thomas Lycan, Tamjeed Ahmed, Umit Topaloglu, Andrew Dothard, William J Petty, Roy E Strowd
BACKGROUND: Immune checkpoint inhibitors (ICIs) are a promising class of anticancer drugs associated with immune-related adverse events (IRAEs). In registration studies of selected cancer populations, neurologic IRAEs were rare. Post-marketing experience describing their prevalence in clinical practice continues to be reported. METHODS: A retrospective cohort of patients treated at our institution with ICIs from 2005 to 2017 was identified. Patients with new neurologic ICD codes documented during or after ICI treatment were enrolled...
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29761296/neuroimaging-in-lewy-body-dementia
#12
REVIEW
Tayyabah Yousaf, George Dervenoulas, Polytimi-Eleni Valkimadi, Marios Politis
Lewy body dementia (DLB) is a common form of cognitive impairment, accounting for 30% of dementia cases in ages over 65 years. Early diagnosis of DLB has been challenging; particularly in the context of differentiation with Parkinson's disease dementia and other forms of dementias, such as Alzheimer's disease and rapidly progressive dementias. Current practice involves the use of [123 I]FP-CIT-SPECT, [18 F]FDG PET and [123 I]MIBG molecular imaging to support diagnostic procedures. Structural imaging techniques have an essential role for excluding structural causes, which could lead to a DLB-like phenotype, as well as aiding differential diagnosis through illustrating disease-specific patterns of atrophy...
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29761295/a-startling-acoustic-stimulus-facilitates-voluntary-lower-extremity-movements-and-automatic-postural-responses-in-people-with-chronic-stroke
#13
Milou J M Coppens, Jolanda M B Roelofs, Nicole A J Donkers, Jorik Nonnekes, Alexander C H Geurts, Vivian Weerdesteyn
A startling acoustic stimulus (SAS) involuntary releases prepared movements at accelerated latencies, known as the StartReact effect. Previous work has demonstrated intact StartReact in paretic upper extremity movements in people after stroke, suggesting preserved motor preparation. The question remains whether motor preparation of lower extremity movements is also unaffected after stroke. Here, we investigated StartReact effects on ballistic lower extremity movements and on automatic postural responses (APRs) following perturbations to standing balance...
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29761294/monoclonal-antibodies-for-migraine-an-update
#14
Daniel Castle, Neil P Robertson
No abstract text is available yet for this article.
May 14, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29737427/serum-neurofilament-light-is-increased-in-multiple-system-atrophy-of-cerebellar-type-and-in-repeat-expansion-spinocerebellar-ataxias-a-pilot-study
#15
Carlo Wilke, Friedemann Bender, Stefanie N Hayer, Kathrin Brockmann, Ludger Schöls, Jens Kuhle, Matthis Synofzik
Blood biomarkers in degenerative ataxias are still largely missing. Here, we aimed to provide piloting proof-of-concept that serum Neurofilament light (NfL) could offer a promising peripheral blood biomarker in degenerative ataxias. Specifically, as a marker of neuronal damage, NfL might (1) help to differentiate multiple system atrophy of cerebellar type (MSA-C) from sporadic adult-onset ataxia (SAOA), and (2) show increases in repeat-expansion spinocerebellar ataxias (SCAs) which might be amenable to treatment in the future...
May 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29737426/elevated-factor-viii-increases-the-risk-of-cerebral-venous-thrombosis-a-case-control-study
#16
Loes Vecht, Susanna M Zuurbier, Joost C M Meijers, Jonathan M Coutinho
BACKGROUND: Elevated factor VIII (FVIII) is a risk factor for leg-vein thrombosis and pulmonary embolism. We assessed whether elevated FVIII is also a risk factor for cerebral venous thrombosis (CVT). METHODS: We performed a matched case-control study. We assessed patients with CVT, as cases, admitted between July 2006 and December 2016. The controls were healthy hospital-staff employees matched for age (within 5 years) and sex. FVIII activity was measured at least 3 months after CVT diagnosis...
May 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29737425/haemorrhagic-stroke-related-to-the-use-of-4-fluoroamphetamine
#17
C H W Wijers, M C Visser, R T H van Litsenburg, R J M Niesink, R B Willemse, Esther A Croes
INTRODUCTION: The use of the new psychoactive substance 4-fluoroamphetamine (4-FA) and the number of 4-FA-related intoxications substantially increased in The Netherlands in recent years. We describe two patients with severe 4-FA-related complications and the characteristics of a large sample of 4-FA-intoxicated patients. METHODS: Information on patients with 4-FA-related intoxications between January 2009 and June 2017 was available from the Monitor Drug-related Incidents...
May 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29728768/therapeutic-decisions-in-als-patients-cross-cultural-differences-and-clinical-implications
#18
Peter M Andersen, Magdalena Kuzma-Kozakiewicz, Jürgen Keller, Helena E A Aho-Oezhan, Katarzyna Ciecwierska, Natalia Szejko, Cynthia Vázquez, Sarah Böhm, Gisela Badura-Lotter, Thomas Meyer, Susanne Petri, Katharina Linse, Andreas Hermann, Olof Semb, Erica Stenberg, Simona Nackberg, Johannes Dorst, Ingo Uttner, Ann-Cristin Häggström, Albert C Ludolph, Dorothée Lulé
OBJECTIVE: Quantitative analysis of decision-making on therapeutic options in different sociocultural context in amyotrophic lateral sclerosis (ALS). METHODS: ALS patients (n = 244) were consecutively recruited in Germany (n = 83), Poland (n = 83), and Sweden (n = 78) in a prospective cross-cultural study ( www.NEEDSinALS.com ). They were interviewed on preferences for therapeutic techniques including invasive (IV) and non-invasive ventilation (NIV), as well as percutaneous endoscopic gastrostomy (PEG) and on hypothetical termination of these using quantitative questions...
May 4, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29725843/vestibular-rehabilitation-therapy-and-nintendo-wii-balance-board-training-both-improve-postural-control-in-bilateral-vestibulopathy
#19
LETTER
Klaus Jahn, Ann Kathrin Saul, Matthias Elstner, Katrin Sapa, Silvy Kellerer
No abstract text is available yet for this article.
May 3, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29725842/motor-neuron-disease-of-paraneoplastic-origin-a-rare-but-treatable-condition
#20
Nicolas Mélé, Giulia Berzero, Thierry Maisonobe, François Salachas, Guillaume Nicolas, Nicolas Weiss, Guillemette Beaudonnet, Francois Ducray, Dimitri Psimaras, Timothée Lenglet
Paraneoplastic motor neuron disorders (MND) are rare conditions; their exact clinical and electrophysiological phenotype have not been exhaustively described yet. The purpose of this study is to depict the main characteristics of paraneoplastic MND to highlight the features that may allow its diagnosis. Based on the description of eight original cases, and on the revision of 21 patients identified from a systematic review of the literature, the main features of paraneoplastic MND can be summarized as follows: (1) subacute; (2) lower motor neuron syndrome, associated or not with upper motor neuron involvement; (3) predominant asymmetric upper limb involvement; (4) presence of other non-motor neurological manifestations, including sensory neuronopathy; (5) signs of inflammation in the cerebrospinal fluid (CSF); (6) neurological improvement or stabilization after immunotherapy and tumor treatment...
May 3, 2018: Journal of Neurology
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