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Johanna Junker, Valerie Brandt, Brian D Berman, Marie Vidailhet, Emmanuel Roze, Anne Weissbach, Cynthia Comella, Irene A Malaty, Joseph Jankovic, Mark S LeDoux, Alfredo Berardelli, Richard Barbano, Stephen G Reich, Joel S Perlmutter, H A Jinnah, Norbert Brüggemann
OBJECTIVE: To determine predictors of alcohol responsiveness in a large cohort of patients with dystonia. METHODS: A total of 2,159 participants with dystonia were prospectively enrolled in the cross-sectional Dystonia Coalition multicenter study. Patients with secondary, combined, or confirmed genetic dystonia (total n = 164) or unknown alcohol responsiveness (n = 737) were excluded. Patients answered a standardized questionnaire and were clinically examined using a standardized video protocol and the Burke-Fahn-Marsden Dystonia Rating Scale...
October 19, 2018: Neurology
Jane Maguire, John Attia
No abstract text is available yet for this article.
October 19, 2018: Neurology
Colin Groot, Carole H Sudre, Frederik Barkhof, Charlotte E Teunissen, Bart N M van Berckel, Sang Won Seo, Sébastien Ourselin, Philip Scheltens, M Jorge Cardoso, Wiesje M van der Flier, Rik Ossenkoppele
OBJECTIVE: To examine the clinical phenotype, gray matter atrophy patterns, and small vessel disease in patients who developed prodromal or probable Alzheimer disease dementia, despite carrying the protective APOE ε2 allele. METHODS: We included 36 β-amyloid-positive (by CSF or PET) APOE ε2 carriers (all ε2/ε3) with mild cognitive impairment or dementia due to Alzheimer disease who were matched for age and diagnosis (ratio 1:2) to APOE ε3 homozygotes and APOE ε4 carriers (70% ε3/ε4 and 30% ε4/ε4)...
October 19, 2018: Neurology
Aravind Ganesh, Ramon Luengo-Fernandez, Rose M Wharton, Peter M Rothwell
OBJECTIVE: To compare how 3 common representations (ordinal vs dichotomized as 0-1/2-6 or 0-2/3-6) of the modified Rankin Scale (mRS)-a commonly used trial outcome measure-relate to long-term outcomes, and quantify trial ineligibility rates based on premorbid mRS. METHODS: In consecutive patients with ischemic stroke in a population-based, prospective, cohort study (Oxford Vascular Study; 2002-2014), we related 3-month mRS to 1-year and 5-year disability and death (logistic regressions), and health/social care costs (generalized linear model), adjusted for age/sex, and compared goodness-of-fit values (C statistic, mean absolute error)...
October 19, 2018: Neurology
Kun-Hsing Yu, Oren Miron, Nathan Palmer, Dario Lemos, Kathe Fox, S C Kou, Mustafa Sahin, Isaac S Kohane
No abstract text is available yet for this article.
October 17, 2018: Neurology
Margot G A Van Cauwenberge, Sven Dekeyzer, Omid Nikoubashman, Manuel Dafotakis, Martin Wiesmann
OBJECTIVE: To study the diagnostic value of volume perfusion CT (VPCT) in patients with transient focal neurologic deficits following and during epileptic seizures, that mimic symptoms of stroke. METHODS: A retrospective case-control study was performed on 159 patients who presented with a seizure and received an emergency VPCT within the first 3.5 hours of admission, after being misjudged to have an acute stroke. The reference test was a clinical-based, EEG-supported diagnostic algorithm for seizure...
October 17, 2018: Neurology
Kristen M Krysko, Jennifer Graves, Mary Rensel, Bianca Weinstock-Guttman, Gregory Aaen, Leslie Benson, Tanuja Chitnis, Mark Gorman, Manu Goyal, Lauren Krupp, Timothy Lotze, Soe Mar, Moses Rodriguez, John Rose, Michael Waltz, T Charles Casper, Emmanuelle Waubant
OBJECTIVE: To characterize the use and safety of newer disease-modifying therapies (DMTs) in children with multiple sclerosis (MS) and clinically isolated syndrome (CIS) treated under 18 years of age. METHODS: This is a cohort study including children with MS or CIS followed at 12 outpatient practices participating in the US Network of Pediatric MS Centers. DMT use, including duration, dose, and side effects, was analyzed. Newer DMTs were defined as agents receiving Food and Drug Administration approval or with increased use in adult MS after 2005...
October 17, 2018: Neurology
Brandon J Lew, Timothy J McDermott, Alex I Wiesman, Jennifer O'Neill, Mackenzie S Mills, Kevin R Robertson, Howard S Fox, Susan Swindells, Tony W Wilson
OBJECTIVE: To identify the neural markers of attention dysfunction in patients with HIV-associated neurocognitive disorder (HAND). METHODS: Sixty participants, including 40 HIV-infected adults (half with HAND) and 20 demographically matched controls performed a visual selective attention task while undergoing high-density magnetoencephalography. Neuronal activity related to selective attention processing was quantified and compared across the 3 groups, and correlated with neuropsychological measures of attention and executive function...
October 17, 2018: Neurology
Ilse Baert, Tori Smedal, Alon Kalron, Kamila Rasova, Adnan Heric-Mansrud, Rainer Ehling, Iratxe Elorriaga Minguez, Una Nedeljkovic, Andrea Tacchino, Peter Hellinckx, Greet Adriaenssens, Gosia Stachowiak, Klaus Gusowski, Davide Cattaneo, Sophie Borgers, Jeffrey Hebert, Ulrik Dalgas, Peter Feys
OBJECTIVE: To determine responsiveness of functional mobility measures, and provide reference values for clinically meaningful improvements, according to disability level, in persons with multiple sclerosis (pwMS) in response to physical rehabilitation. METHODS: Thirteen mobility measures (clinician- and patient-reported) were assessed before and after rehabilitation in 191 pwMS from 17 international centers (European and United States). Combined anchor- and distribution-based methods were used...
October 17, 2018: Neurology
Olivier Godefroy, Hugo Yaïche, Hervé Taillia, Flavie Bompaire, Claudine Nédélec-Ciceri, Camille Bonnin, Jérôme Varvat, Françoise Vincent-Grangette, Momar Diouf, Jean-Louis Mas, Sandrine Canaple, Chantal Lamy, Audrey Arnoux, Claire Leclercq, Sophie Tasseel-Ponche, Martine Roussel, Mélanie Barbay
OBJECTIVE: To validate the ability of a specifically developed cognitive risk score to identify patients at risk of poststroke neurocognitive disorders (NCDs) who are eligible for a comprehensive cognitive assessment. METHODS: After assessing 404 patients (infarct 91.3%) in the Groupe de Réflexion pour l'Evaluation Cognitive VASCulaire (GRECogVASC) cross-sectional study with the National Institute of Neurological Disorders and Stroke-Canadian Stroke Network battery 6 months after stroke, we used multivariable logistic regression and bootstrap analyses to determine factors associated with NCDs...
October 17, 2018: Neurology
Elisabeth B Marsh, Franz Fazekas
No abstract text is available yet for this article.
October 17, 2018: Neurology
Vera Zietemann, Marios K Georgakis, Thibaut Dondaine, Claudia Müller, Anne-Marie Mendyk, Anna Kopczak, Hilde Hénon, Stéphanie Bombois, Frank Arne Wollenweber, Régis Bordet, Martin Dichgans
OBJECTIVE: To examine whether the Montreal Cognitive Assessment (MoCA) administered within 7 days after stroke predicts long-term cognitive impairment, functional impairment, and mortality. METHODS: MoCA was administered to 274 patients from 2 prospective hospital-based cohort studies in Germany (n = 125) and France (n = 149). Cognitive and functional outcomes were assessed at 6, 12, and 36 months after stroke by comprehensive neuropsychological testing, the Clinical Dementia Rating (CDR) scale, the modified Rankin Scale (mRS), and Instrumental Activities of Daily Living (IADL) and analyzed with generalized estimating equations...
October 17, 2018: Neurology
Teresa Coelho, Mónica Inês, Isabel Conceição, Marta Soares, Mamede de Carvalho, João Costa
OBJECTIVE: To assess the natural history and treatment effect on survival among patients with transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) stage 1 Val30Met. METHODS: Multi-institutional, hospital-based study of patients with TTR-FAP Val30Met prospectively followed up until December 2016, grouped into untreated (n = 1,771), liver transplant (LTx)-treated (n = 957), or tafamidis-treated (n = 432) cohorts. Standardized mortality ratios, Kaplan-Meier, and Cox methods were used to estimate excess mortality, survival, and adjusted hazard ratios (HRs) for all-cause mortality...
October 17, 2018: Neurology
Karoliina Aarnio, Jorge Rodríguez-Pardo, Bob Siegerink, Juliane Hardt, Jenna Broman, Lauri Tulkki, Elena Haapaniemi, Markku Kaste, Turgut Tatlisumak, Jukka Putaala
OBJECTIVE: We aimed to investigate the proportion of young patients not returning to work (NRTW) at 1 year after ischemic stroke (IS) and during follow-up, and clinical factors associated with NRTW. METHODS: Patients from the Helsinki Young Stroke Registry with an IS occurring in the years 1994-2007, who were at paid employment within 1 year before IS, and with NIH Stroke Scale score ≤15 points at hospital discharge, were included. Data on periods of payment came from the Finnish Centre for Pensions, and death data from Statistics Finland...
October 12, 2018: Neurology
Sebastian F Ameriso
No abstract text is available yet for this article.
October 12, 2018: Neurology
Thomas Trieu, Seyed Ahmad Sajjadi, Claudia H Kawas, Peter T Nelson, María M Corrada
OBJECTIVE: To examine the risk factors and comorbidities of hippocampal sclerosis (HS) in the oldest-old. METHODS: A total of 134 participants with dementia from The 90+ Study with longitudinal evaluations and autopsy were included in this investigation. Participants were divided into 2 groups, one with and one without HS pathology, and differences in clinical and pathologic characteristics were compared. RESULTS: Persons with HS tended to have a longer duration of dementia compared to participants without HS (mean 4...
October 12, 2018: Neurology
Yanping Sun, Udaya Seneviratne, Piero Perucca, Zhibin Chen, Meng Kee Tan, Terence J O'Brien, Wendyl D'Souza, Patrick Kwan
OBJECTIVE: To identify clinical and EEG biomarkers of drug resistance in adults with idiopathic generalized epilepsy. METHODS: We conducted a case-control study consisting of a discovery cohort and a replication cohort independently assessed at 2 different centers. In each center, patients with the idiopathic generalized epilepsy phenotype and generalized spike-wave discharges on EEG were classified as drug-resistant or drug-responsive. EEG changes were classified into predefined patterns and compared between the 2 groups in the discovery cohort...
October 12, 2018: Neurology
David Michelson, Emma Ciafaloni, Stephen Ashwal, Elliot Lewis, Pushpa Narayanaswami, Maryam Oskoui, Melissa J Armstrong
OBJECTIVE: To identify the level of evidence for use of nusinersen to treat spinal muscular atrophy (SMA) and review clinical considerations regarding use. METHODS: The author panel systematically reviewed nusinersen clinical trials for patients with SMA and assigned level of evidence statements based on the American Academy of Neurology's 2017 therapeutic classification of evidence scheme. Safety information, regulatory decisions, and clinical context were also reviewed...
October 12, 2018: Neurology
Michelle E Farrell, Xi Chen, Melissa M Rundle, Micaela Y Chan, Gagan S Wig, Denise C Park
OBJECTIVE: To assess whether global or regional changes in amyloid burden over 4 years predict early declines in episodic memory in initially amyloid-negative adults. METHODS: One hundred twenty-six initially amyloid-negative, cognitively normal participants (age 30-89 years) were included from the Dallas Lifespan Brain Study who completed florbetapir PET and a cognitive battery at baseline and 4-year follow-up. Standardized uptake value ratio (SUVR) change was computed across 8 bilateral regions of interest...
October 10, 2018: Neurology
Carine W Maurer, Kathrin LaFaver, Gaurang S Limachia, Geanna Capitan, Rezvan Ameli, Stephen Sinclair, Steven A Epstein, Mark Hallett, Silvina G Horovitz
OBJECTIVE: To explore alterations in gray matter volume in patients with functional movement disorders. METHODS: We obtained T1-weighted MRI on 48 patients with clinically definite functional movement disorders, a subset of functional neurologic symptom disorder characterized by abnormal involuntary movements, and on 55 age- and sex-matched healthy controls. We compared between-group differences in gray matter volume using voxel-based morphometry across the whole brain...
October 10, 2018: Neurology
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