دسترسی یکساله به بیش از ۵۰۰ ژورنال روز جهان موجود در سامانه
-
http://medilib.ir
- ﻣﺪﺕ ﺯﻣﺎﻥ : 365 ﺭﻭﺯ
قیمت : 3,800,000 تومان- قیمت ویژه : 1,900,000تومان
Annals of Neurology: Volume 90, Number 6, December 2021
doi : 10.1002/ana.26269
Volume 90, Issue 6 p. C1-C1
A diffusion tensor imaging MRI scan showing fractional anisotropy of the prefrontal cortex of a 7 week old pig, used to study the effect of cardiopulmonary bypass on developing brains. Increased cortical fractional anisotropy after severe injury due to cardiopulmonary bypass served as a measure of the impact on cortical maturation. See paper by Dhari and colleagues in this issue (pp. 913-926).
Time to Publication of Clinical Trials Funded by the National Institute of Neurological Disorders and Stroke
Jeremy Brown MD,Paul G. Wakim PhD,Carlos Faraco PhD,Cristina Saugar-Lanchas PhD,Codrin Lungu MD,Carolina Mendoza-Puccini MD,Ellen Rosenberg MhA,Clinton Wright MD
doi : 10.1002/ana.26246
Volume 90, Issue 6 p. 861-864
Advances in Treatment of Progressive Multifocal Leukoencephalopathy
Raphaël Bernard-Valnet MD, PhD,Igor J. Koralnik MD,Renaud Du Pasquier MD
doi : 10.1002/ana.26198
Volume 90, Issue 6 p. 865-873
Progressive multifocal encephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS) caused by JC virus (JCV), which occurs in immunocompromised individuals. Management of PML relies on restoration of immunity within the CNS. However, when this restoration cannot be readily achieved, PML has a grim prognosis. Innovative strategies have shown promise in promoting anti-JCV immune responses, and include T-cell adoptive transfer or immune checkpoint inhibitor therapies. Conversely, management of immune reconstitution inflammatory syndrome, particularly in iatrogenic PML, remains a major challenge. In this paper, we review recent development in the treatment of PML. ANN NEUROL 2021;90:865–873
Profile of Autism Spectrum Disorder in Tuberous Sclerosis Complex: Results from a Longitudinal, Prospective, Multisite Study
Jamie K. Capal,Marian E. Williams PhD,Deborah A. Pearson PhD,Robin Kissinger PhD,Paul S. Horn PhD,Donna Murray PhD, CCC-SLP,Kristn Currans PsyD,Bridget Kent MA, CCC-SLP,Martina Bebin MD,Hope Northrup,Joyce Y. Wu,Mustafa Sahin,Darcy A. Krueger MD,TACERN Study Group
doi : 10.1002/ana.26249
Volume 90, Issue 6 p. 874-886
Tuberous sclerosis complex (TSC) is highly associated with autism spectrum disorder (ASD). Objectives of the study were to characterize autistic features in young children with TSC.
Sorbitol Is a Severity Biomarker for PMM2-CDG with Therapeutic Implications
Anna N. Ligezka MSc,Silvia Radenkovic PhD,Mayank Saraswat PhD,Kishore Garapati MBBS,Wasantha Ranatunga PhD,Wirginia Krzysciak PhD,Hitoshi Yanaihara MD, PhD,Graeme Preston PhD,William Brucker MD, PhD,Renee M. McGovern BA,Joel M. Reid PhD,David Cassiman MD, PhD,Karthik Muthusamy MD,Christin Johnsen MD,Saadet Mercimek-Andrews MD, PhD,Austin Larson MD,Christina Lam MD,Andrew C. Edmondson MD, PhD,Bart Ghesquière PhD,Peter Witters MD,Kimiyo Raymond MD,Devin Oglesbee MD,Akhilesh Pandey MD, PhD,Ethan O. Perlstein PhD,Tamas Kozicz MD, PhD,Eva Morava MD, PhD
doi : 10.1002/ana.26245
Volume 90, Issue 6 p. 887-900
Epalrestat, an aldose reductase inhibitor increases phosphomannomutase (PMM) enzyme activity in a PMM2-congenital disorders of glycosylation (CDG) worm model. Epalrestat also decreases sorbitol level in diabetic neuropathy. We evaluated the genetic, biochemical, and clinical characteristics, including the Nijmegen Progression CDG Rating Scale (NPCRS), urine polyol levels and fibroblast glycoproteomics in patients with PMM2-CDG.
Cardiac Troponin and Recurrent Major Vascular Events after Minor Stroke or Transient Ischemic Attack
Simon Hellwig MD, MSc,Thomas Ihl MD,Ramanan Ganeshan MD,Inga Laumeier MD,Michael Ahmadi MD,Maureen Steinicke MD,Joachim E. Weber MD,Matthias Endres MD,Heinrich J. Audebert MD,Jan F. Scheitz MD
doi : 10.1002/ana.26225
Volume 90, Issue 6 p. 901-912
This study was undertaken to investigate whether high-sensitivity cardiac troponin T (hs-cTnT) is associated with major adverse cardiovascular events (MACE) in patients with minor stroke or transient ischemic attack (TIA), and whether this association differs after risk stratification based on the Age, Blood Pressure, Clinical Features, Duration of Symptoms, Diabetes (ABCD2) score.
Impact of Cardiopulmonary Bypass on Neurogenesis and Cortical Maturation
Zaenab Dhari MD,Camille Leonetti PhD,Stephen Lin MEngr,Arianna Prince BS,James Howick MD, MS,David Zurakowski MS, PhD,Paul C. Wang PhD,Richard A. Jonas MD,Nobuyuki Ishibashi MD
doi : 10.1002/ana.26235
Volume 90, Issue 6 p. 913-926
Neurodevelopmental delays and frontal lobe cortical dysmaturation are widespread among children with congenital heart disease (CHD). The subventricular zone (SVZ) is the largest pool of neural stem/progenitor cells in the postnatal brain. Our aim is to determine the effects of cardiopulmonary bypass (CPB) on neurogenesis and cortical maturation in piglets whose SVZ development is similar to human infants.
Comparative Effectiveness of Stereotactic Electroencephalography Versus Subdural Grids in Epilepsy Surgery
Lara Jehi MD,Marcia Morita-Sherman MD,Thomas E. Love PhD,Fabrice Bartolomei MD,William Bingaman MD,Kees Braun MD,Robyn M. Busch PhD,John Duncan FRCP,Walter J. Hader MD,Guoming Luan MD,John D. Rolston MD, PhD,Stephan Schuele MD,Laura Tassi MD,Sumeet Vadera MD,Shehryar Sheikh MD, MPH,Imad Najm MD,Amir Arain MD,Justin Bingaman BS,Beate Diehl MD,Jane de Tisi BA,Matea Rados MD,Pieter Van Eijsden MD,Sandra Wahby MSc,Xiongfei Wang MD,Samuel Wiebe MD
doi : 10.1002/ana.26238
Volume 90, Issue 6 p. 927-939
The aim was to compare the outcomes of subdural electrode (SDE) implantations versus stereotactic electroencephalography (SEEG), the 2 predominant methods of intracranial electroencephalography (iEEG) performed in difficult-to-localize drug-resistant focal epilepsy.
Hydroxychloroquine for Primary Progressive Multiple Sclerosis
Marcus W. Koch MD, PhD,Sharanjit Kaur MPH,Kayla Sage BHSc,Janet Kim MPH,Myriam Levesque-Roy MD,Graziela Cerchiaro PhD,Voon Wee Yong PhD,Gary R. Cutter PhD,Luanne M. Metz MD
doi : 10.1002/ana.26239
Volume 90, Issue 6 p. 940-948
Primary progressive multiple sclerosis (PPMS) does not respond well to immunomodulatory or immunosuppressive treatment. Chronic activation of microglia has been implicated in the pathophysiology of PPMS. The antimalarial drug hydroxychloroquine (HCQ) reduces the activity of human microglia and has neuroprotective effects in vitro.
Long-Term Cognitive Outcome in Anti–N-Methyl-D-Aspartate Receptor Encephalitis
Josephine Heine MSc,Ute A. Kopp PhD,Johanna Klag,Christoph J. Ploner MD,Harald Prüss MD,Carsten Finke MD
doi : 10.1002/ana.26241
Volume 90, Issue 6 p. 949-961
Cognitive dysfunction is a core symptom of anti–N-methyl-D-aspartate receptor (NMDAR) encephalitis, but detailed studies on prevalence, characteristics of cognitive deficits, and the potential for recovery are missing. Here, we performed a prospective longitudinal study to assess cognitive long-term outcome and identify clinical predictors.
End-Truncated LAMB1 Causes a Hippocampal Memory Defect and a Leukoencephalopathy
Chaker Aloui PhD,Dominique Hervé MD,Gaelle Marenne PhD,Florian Savenier MD,Kilan Le Guennec PhD,Francoise Bergametti PhD,Edgard Verdura PhD,Thomas E. Ludwig PhD,Jessica Lebenberg PhD,Waliyde Jabeur MD,Hélène Morel PharmD,Thibault Coste PharmD,Geneviève Demarquay MD,Panagiotis Bachoumas MD,Julien Cogez MD,Guillaume Mathey MD,Emilien Bernard MD,FREX consortium,Hugues Chabriat MD, PhD,Emmanuelle Génin PhD,Elisabeth Tournier-Lasserve MD
doi : 10.1002/ana.26242
Volume 90, Issue 6 p. 962-975
The majority of patients with a familial cerebral small vessel disease (CSVD) referred for molecular screening do not show pathogenic variants in known genes. In this study, we aimed to identify novel CSVD causal genes.
Complement Activation Is a Prominent Feature of MOGAD
Christian W. Keller MD, PhD,Joseph A. Lopez BSc,Eva-Maria Wendel MD,Sudarshini Ramanathan MD, PhD,Catharina C. Gross PhD,Luisa Klotz MD,Markus Reindl PhD,Russell C. Dale MD,Heinz Wiendl MD,Kevin Rostásy MD,Fabienne Brilot PhD,Jan D. Lünemann MD
doi : 10.1002/ana.26226
Volume 90, Issue 6 p. 976-982
Myelin oligodendrocyte glycoprotein (MOG)-antibody (Ab)–associated diseases (MOGADs) account for a substantial proportion of pediatric and adult patients who present with acquired demyelinating disorders. Its pathogenesis and optimal therapy are incompletely understood. We profiled systemic complement activation in adult and pediatric patients with MOGAD compared with patients with relapse-onset multiple sclerosis, patients with neuromyelitis optica spectrum disorder, and pediatric control and adult healthy donors. Proteins indicative of systemic classical and alternative complement activation were substantially increased in patients with MOGAD compared to control groups. Elevated levels were detected in both adult and pediatric cases and across all clinical syndromes. Complement inhibition should be explored for its therapeutic merit in patients with MOGAD. ANN NEUROL 2021;90:976–982
Comparing Seizure-Related Death and Suicide in Younger Adults with Epilepsy
Marius Kløvgaard MD,Thomas H. Lynge MD, PhD,Ioannis Tsiropoulos MD, PhD,Peter V. Uldall MD, PhD,Jytte Banner MD, PhD,Bo G. Winkel MD, PhD,Philippe Ryvlin MD,Jacob Tfelt-Hansen MD, PhD, DMSc,Anne Sabers MD, DMSc
doi : 10.1002/ana.26227
Volume 90, Issue 6 p. 983-987
Younger adults with epilepsy have an increased mortality. Some deaths are seizure-related, for example, sudden unexpected death in epilepsy (SUDEP), whereas others, for example, suicide, have multiple causes, including adverse effects of the treatment on mood. In this retrospective population-based study of all Danish persons with epilepsy aged 18 to 49?years during 2007 to 2009 we evaluated the risk of death from seizures and suicide. SUDEP comprised 82.7% of all seizure-related death. Younger adults with epilepsy had an 8.3-fold increased risk of death from seizure-related causes compared with suicide. This underpins the importance of effective seizure control in preventing premature death. ANN NEUROL 2021;90:983–987
Fusiform Gyrus Phospho-Tau is Associated with Failure of Proper Name Retrieval in Aging
Victoria R. Tennant BA,Theresa M. Harrison PhD,Jenna N. Adams PhD,Renaud La Joie PhD,Joseph R. Winer PhD,William J. Jagust MD
doi : 10.1002/ana.26237
Volume 90, Issue 6 p. 988-993
Difficulty retrieving proper names is common in older adults, coinciding with the accumulation of aggregated proteins in mid-life. We investigated the ability of healthy older adults to retrieve the names of famous faces in relation to positron emission tomography measurements of amyloid-? plaques and tau neurofibrillary tangles. More tau in the left fusiform and parahippocampal gyrus was related to reduced proper name retrieval performance and this effect was potentiated by amyloid-?. These findings provide an explanation for a common complaint of older adults and link proper name retrieval to neural systems involved in face perception, memory, and naming. ANN NEUROL 2021;90:988–993
LTA4H Prevalence and Mortality in Adult Zambians with Tuberculous Meningitis
Omar K. Siddiqi MD, MPH,Musie Ghebremichael PhD,Kalo Musukuma MSc,Xin Dang PhD,Eugene Mubanga MSc,Gretchen L. Birbeck MD, MPH,Shawn Love MD,Clayton Buback MD,Barry Kosloff BS,Helen Ayles MBBS, PhD,Masharip Atadzhanov MD, PhD,Igor J. Koralnik MD
doi : 10.1002/ana.26240
Volume 90, Issue 6 p. 994-998
We conducted a prospective cohort study to determine the prevalence of leukotriene A4 hydrolase (LTA4H) polymorphisms in Zambian adults with tuberculous meningitis (TBM) and its association with mortality. We completed genotype testing on 101 definite cases of TBM and 119 consecutive non-TBM controls. The distribution of genotypes among TBM patients was as follows: C/C (0.83), C/T (0.14), T/T (0.03). There was no significant difference in genotype distribution between TBM and non-TBM patients. We found no relationship between LTA4H polymorphism and survival. Prospective studies are needed to determine the benefit of adjuvant steroids in TBM based upon population LTA4H genotype. ANN NEUROL 2021;90:994–998
Unilateral Primary Angiitis of the Central Nervous System
Brendan Santyr MD,Shervin Pejhan MD,Qi Zhang MBBS, PhD,Adrian Budhram MD
doi : 10.1002/ana.26234
Volume 90, Issue 6 p. 999-1000
“Disappearing Infarct” Is Late-Onset MELAS
Timothy M. Landis MD,William B. Hannah MD,William J. Powers MD
doi : 10.1002/ana.26236
Volume 90, Issue 6 p. 1001-1002
Thinking beyond the Aducanumab Controversy
Khalid Iqbal PhD
doi : 10.1002/ana.26252
Volume 90, Issue 6 p. 1003-1004
Reply to “Thinking beyond Aducanumab Controversy”
Ronald C. Petersen PhD, MD
doi : 10.1002/ana.26253
Volume 90, Issue 6 p. 1004-1004
Letter Concerning Medicare Should Not Cover Aducanumab as a Treatment for Alzheimer's Disease by Moghavem, Henderson, and Greicius
Alfred Sandrock MD, PhD
doi : 10.1002/ana.26254
Volume 90, Issue 6 p. 1004-1006
Reply to “Letter Concerning Moghavem, Henderson, and Greicius Article”
Nuriel Moghavem MD,Victor W. Henderson MD, MS,Michael D. Greicius MD, MPH
doi : 10.1002/ana.26255
Volume 90, Issue 6 p. 1006-1007
Correction to “Hartung H-P. Neuromyelitis Optica Spectrum Disorder: Therapeutic Innovations and Complex Decision-Making.”
Hans-Peter Hartung
doi : 10.1002/ana.26243
Volume 90, Issue 6 p. 1008-1008
