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JAMA Neurology

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https://www.readbyqxmd.com/read/28437542/challenges-to-translation-and-the-hippocratic-oath-by-premature-termination-of-spinal-cord-stem-cell-based-trials
#1
Armin Curt, Allan Levi, Jan M Schwab
No abstract text is available yet for this article.
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28437540/characterization-of-gene-expression-phenotype-in-amyotrophic-lateral-sclerosis-monocytes
#2
Weihua Zhao, David R Beers, Kristopher G Hooten, Douglas H Sieglaff, Aijun Zhang, Shanker Kalyana-Sundaram, Christopher M Traini, Wendy S Halsey, Ashley M Hughes, Ganesh M Sathe, George P Livi, Guo-Huang Fan, Stanley H Appel
Importance: Amyotrophic lateral sclerosis (ALS) is a common adult-onset neurodegenerative disease characterized by selective loss of upper and lower motor neurons. Patients with ALS have persistent peripheral and central inflammatory responses including abnormally functioning T cells and activated microglia. However, much less is known about the inflammatory gene profile of circulating innate immune monocytes in these patients. Objective: To characterize the transcriptomics of peripheral monocytes in patients with ALS...
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28437535/clinical-aspects-of-glucose-transporter-type-1-deficiency-information-from-a-global-registry
#3
Jian Hao, Dorothy I Kelly, Jianzhong Su, Juan M Pascual
Importance: Case reports regularly document unique or unusual aspects of glucose transporter type 1 deficiency (G1D). In contrast, population studies from which to draw global inferences are lacking. Twenty-five years after the earliest case reports, this deficiency still particularly affects treatment and prognostic counseling. Objective: To examine the most common features of G1D. Design, Setting, and Participants: In this study, data were collected electronically from 181 patients with G1D through a web-based, worldwide patient registry from December 1, 2013, through December 1, 2016...
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28437521/home-based-trials-in-adolescent-migraine-a-randomized-clinical-trial
#4
Amy A Gelfand, William Qubty, Irene Patniyot, Barbara Grimes, Mark J Pletcher, Peter J Goadsby, Steven R Cummings
No abstract text is available yet for this article.
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28437518/a-middle-aged-man-with-progressive-ophthalmoparesis-ataxia-and-spastic-paraparesis
#5
Nathan H Kung, Gregory P Van Stavern, Robert C Bucelli
A 50-year old man presented for evaluation of progressive gait ataxia with a superimposed spastic paraparesis. During his clinic visit, he was also observed to have slow and limited eye movements. In this article, we discuss the clinical approach to this triad of symptoms and guide the reader to discover the patient's ultimate genetic diagnosis.
April 24, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28418523/neurocognitive-trajectory-of-boys-who-received-a-hematopoietic-stem-cell-transplant-at-an-early-stage-of-childhood-cerebral-adrenoleukodystrophy
#6
Elizabeth I Pierpont, Julie B Eisengart, Ryan Shanley, David Nascene, Gerald V Raymond, Elsa G Shapiro, Rich S Ziegler, Paul J Orchard, Weston P Miller
Importance: Untreated childhood cerebral adrenoleukodystrophy (cALD) is a fatal disease associated with progressive cerebral demyelination and rapid, devastating neurologic decline. The standard of care to enhance long-term survival and stabilize cerebral disease is a hematopoietic stem cell transplant (HSCT). Neurologic outcomes are better when HSCT occurs at an earlier stage of cALD, yet there is limited understanding of the neurocognitive trajectory of patients who undergo HSCT. Objectives: To characterize neurocognitive outcomes of boys with cALD and early-stage cerebral disease who were treated with an allogeneic HSCT and to identify disease- and treatment-related factors associated with long-term functioning...
April 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28418521/evaluation-of-amyloid-protective-factors-and-alzheimer-disease-neurodegeneration-protective-factors-in-elderly-individuals
#7
Prashanthi Vemuri, David S Knopman, Timothy G Lesnick, Scott A Przybelski, Michelle M Mielke, Jonathan Graff-Radford, Melissa E Murray, Rosebud O Roberts, Maria Vassilaki, Val J Lowe, Mary M Machulda, David T Jones, Ronald C Petersen, Clifford R Jack
Importance: While amyloid and neurodegeneration are viewed together as Alzheimer disease pathophysiology (ADP), the factors that influence amyloid and AD-pattern neurodegeneration may be considerably different. Protection from these ADP factors may be important for aging without significant ADP. Objective: To identify the combined and independent protective factors for amyloid and AD-pattern neurodegeneration in a population-based sample and to test the hypothesis that "exceptional agers" with advanced ages do not have significant ADP because they have protective factors for amyloid and neurodegeneration...
April 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28418503/enhanced-epilepsy-surveillance-and-awareness-in-the-age-of-zika
#8
Daniel M Pastula, Marshalyn Yeargin-Allsopp, Rosemarie Kobau
No abstract text is available yet for this article.
April 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28418445/decision-making-in-adrenoleukodystrophy-when-is-a-good-outcome-really-a-good-outcome
#9
Keith Van Haren, Marc Engelen
No abstract text is available yet for this article.
April 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395081/are-more-young-people-having-strokes-a-simple-question-with-an-uncertain-answer
#10
James F Burke, Lesli E Skolarus
No abstract text is available yet for this article.
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395030/clinical-features-molecular-heterogeneity-and-prognostic-implications-in-yars2-related-mitochondrial-myopathy
#11
Ewen W Sommerville, Yi Shiau Ng, Charlotte L Alston, Cristina Dallabona, Micol Gilberti, Langping He, Charlotte Knowles, Sophie L Chin, Andrew M Schaefer, Gavin Falkous, David Murdoch, Cheryl Longman, Marianne de Visser, Laurence A Bindoff, John M Rawles, John C S Dean, Richard K Petty, Maria E Farrugia, Tobias B Haack, Holger Prokisch, Robert McFarland, Douglass M Turnbull, Claudia Donnini, Robert W Taylor, Gráinne S Gorman
Importance: YARS2 mutations have been associated with a clinical triad of myopathy, lactic acidosis, and sideroblastic anemia in predominantly Middle Eastern populations. However, the identification of new patients expands the clinical and molecular spectrum of mitochondrial disorders. Objectives: To review the clinical, molecular, and genetic features of YARS2-related mitochondrial disease and to demonstrate a new Scottish founder variant. Design, Setting, and Participants: An observational case series study was conducted at a national diagnostic center for mitochondrial disease in Newcastle upon Tyne, England, and review of cases published in the literature...
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395018/managing-patients-with-large-ischemic-core-what-is-in-a-match
#12
Thanh G Phan, Henry Ma
No abstract text is available yet for this article.
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395017/prevalence-of-cardiovascular-risk-factors-and-strokes-in-younger-adults
#13
Mary G George, Xin Tong, Barbara A Bowman
Importance: While stroke mortality rates have decreased substantially in the past 2 decades, this trend has been primarily limited to older adults. Increasing trends in stroke incidence and hospitalizations have been noted among younger adults, but there has been concern that this reflected improved diagnosis through an increased use of imaging rather than representing a real increase. Objectives: To determine whether stroke hospitalization rates have continued to increase and to identify the prevalence of associated stroke risk factors among younger adults...
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395015/what-is-in-a-match-reply
#14
Mehdi Bouslama, Leticia Costa Rebello, Raul Gomes Nogueira
No abstract text is available yet for this article.
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28395002/monitored-anesthesia-care-vs-intubation-for-vertebrobasilar-stroke-endovascular-therapy
#15
Ashutosh P Jadhav, Mehdi Bouslama, Amin Aghaebrahim, Leticia C Rebello, Matthew T Starr, Diogo C Haussen, Manasa Ranginani, Matthew K Whalin, Tudor G Jovin, Raul G Nogueira
Importance: No consensus regarding the ideal sedation treatment for stroke endovascular therapy has been reached, and practices remain largely based on local protocols and clinician preferences. Most studies have focused on anterior circulation strokes; therefore, little is known regarding the optimal anesthesia type for vertebrobasilar occlusion strokes. Objective: To compare clinical and angiographic outcomes between monitored anesthesia care (MAC) and general anesthesia (GA) in patients presenting with vertebrobasilar occlusion strokes...
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28384798/multiple-spinal-lesions-in-an-immunocompetent-young-man
#16
Wenhua Zhu, Sushan Luo, Liping Zhu
No abstract text is available yet for this article.
April 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28384785/maternal-body-mass-index-in-early-pregnancy-and-risk-of-epilepsy-in-offspring
#17
Neda Razaz, Kristina Tedroff, Eduardo Villamor, Sven Cnattingius
Importance: There is growing concern about the long-term neurologic effects of prenatal exposure to maternal overweight and obesity. The causes of epilepsy are poorly understood and, in more than 60% of the patients, no definitive cause can be determined. Objectives: To investigate the association between early pregnancy body mass index (BMI) and the risk of childhood epilepsy and examine associations between obesity-related pregnancy and neonatal complications and risks of childhood epilepsy...
April 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28384752/serum-c-reactive-protein-as-a-prognostic-biomarker-in-amyotrophic-lateral-sclerosis
#18
Christian Lunetta, Andrea Lizio, Eleonora Maestri, Valeria Ada Sansone, Gabriele Mora, Robert G Miller, Stanley H Appel, Adriano Chiò
Importance: Various factors have been proposed as possible candidates associated with the prognosis of amyotrophic lateral sclerosis (ALS); however, there is still no consensus on which biomarkers are reliable prognostic factors. C-reactive protein (CRP) is a biomarker of the inflammatory response that shows significant prognostic value for several diseases. Objective: To examine the prognostic significance of CRP in ALS. Design, Setting, and Participants: Patients' serum CRP levels were evaluated from January 1, 2009, to June 30, 2015, in a large cohort of patients with ALS observed by an Italian tertiary multidisciplinary center...
April 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28384731/leptomeningeal-enhancement-for-multiple-sclerosis-another-radiological-biomarker-for-a-relapse
#19
Jagannadha Avasarala
No abstract text is available yet for this article.
April 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28384730/intractable-epilepsy-and-progressive-cognitive-decline-in-a-young-man
#20
Alexander L Cohen, Lyell K Jones, Joseph E Parisi, James P Klaas
A young man with normal neurodevelopment presented with 3 years of medically refractory, progressive epilepsy and myoclonus. Initial examination included neuroimaging, electroencephalography, and biochemical analyses, all of which were unremarkable except for mildly enlarged ventricles. Over the following year, the patient experienced rapid cognitive decline with new-onset recurrent visual hallucinations and progressive lethargy. Results of subsequent electroencephalography and brain imaging were unchanged, and a fluorodeoxyglucose F 18 positron emission tomographic scan was normal...
April 3, 2017: JAMA Neurology
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