annals of neurology

BACKGROUND: Classical galactosaemia (OMIM 230400) is a rare disorder of carbohydrate metabolism caused by deficiency of the galactose-1-phosphate uridyltransferase enzyme. The pathophysiology of the long-term complications, mainly cognitive, neurological and female fertility problems, remains poorly understood. Current clinical methods of biochemical monitoring lack precision and individualisation with an identified need for improved biomarkers for this condition. METHODS: We report the development and detailed validation of an automated ultra-performance liquid chromatography (UPLC) N-glycan analytical method of high peak resolution applied to galactose incorporation into human serum IgG...

No abstract text is available yet for this article.

Hugues Duffau is a Professor and Chairman of the Neurosurgery Department in the Montpellier University Medical Center and Head of the INSERM 1051 Team "plasticity of the central nervous system, human stem cells and glial tumors" at the Institute for Neurosciences of Montpellier (France). He is an expert in the awake cognitive neurosurgery of slow-growing brain tumors, such as low-grade gliomas, a routine which he has developed since 20 years. His fundamental approach is centered on the concepts of the brain connectomics and neuroplasticity, breaking with the traditional localizationist view of cerebral processing...

Media reporting of science has consequences for public debates on the ethics of research. Accordingly, it is crucial to understand how the sciences of the brain and the mind are covered in the media, and how coverage is received and negotiated. The authors report here their sociological findings from a case study of media coverage and associated reader comments of an article ('Does bilingualism influence cognitive aging?') from Annals of Neurology. The media attention attracted by the article was high for cognitive science; further, as associates/members of the Centre where it was produced, the authors of the research reported here had rare insight into how the scientists responsible for the Annals of Neurology article interacted with the media...

OBJECTIVE: To determine the nature and frequency of distorted presentation or "spin" (ie, specific reporting strategies which highlight that the experimental treatment is beneficial, despite a statistically nonsignificant difference for the primary outcome, or distract the reader from statistically nonsignificant results) in published reports of randomized controlled trials (RCTs) with statistically nonsignificant results for primary outcomes in surgical journals. BACKGROUND: Multiple reports have suggested that interpretation of RCT results in medical journals can be distorted by authors of published reports...

We request to retract our Letter to the Editor entitled "DNAJC6 variants in Parkinson's disease and amyotrophic lateral sclerosis". We inappropriately used discoveries from PDGene and ALSGene public databases and claimed them as our own. We also = improperly used their figure without attribution. We understand that this is unacceptable, and deeply apologize to Drs. Lill and Bertram as well as to the readers and editors of the Annals of Neurology for this action. This article is protected by copyright. All rights reserved...

Tau PET study from a 70 year-old individual with a diagnosis of Mild Cognitive Impairment due to Alzheimer's disease, on the basis of a history of progressive memory impairment and elevated brain amyloid. Coronal PET image with the tau tracer 18F T807 (also known as AV1451; superimposed on MRI) shows high binding in red, localized in temporal neocortex, most prominently in inferior temporal gyrus bilaterally, with extension into middle and superior temporal gyri. The pattern of 18F T807 cortical binding is consistent with neurofibrillary tangle stage III/IV of Braak and Braak...

ON THE COVER Notch3 aggregation is one of the important hallmarks of CADASIL. Confocal microscopy image here shows Notch3 extra cellular domain aggregates (magenta) in the proximity of PDGFRβ-positive pericytes (red) on lectin-positive capillaries (green) in a 12-month-old transgenic CADASIL mouse brain section. For more information, see the article by Ghosh et al. (pages 887-900).

OBJECTIVE: To evaluate the disparity in authorship based on gender and nationality of institutional affiliation among journals from developed and developing countries. MATERIALS AND METHODS: Original articles from two neuroscience journals, with a 5 year impact factor >15 (Neuron and Nature Neuroscience) and from two neurology journals from a developing country (Neurology India and Annals of Indian Academy of Neurology) were categorized by gender and institutional affiliation of first and senior authors...

BACKGROUND: Zinc-induced copper deficiency is a condition whose diagnosis is often delayed allowing severe and usually irreversible neurology symptoms to develop. Plasma copper concentrations are usually low and plasma zinc concentrations high. The aim of this study was to measure the predictive value of this combination of results as a means of facilitating its early diagnosis. METHODS: Low plasma copper (≤6 µmol/L) and high plasma zinc results (>18 µmol/L) were retrieved from the laboratory database from 2000 to 2014...

One of the greatest differences I encountered in moving from being Editor-in-Chief of a basic science journal to the same position at Annals of Neurology was the much greater importance of meticulous review of the statistical treatment of data. In basic science, the conditions of an experiment can be set up by the investigator so that relatively simple statistical treatments can often be used with clear-cut results. Comparing the treatment and outcomes of human studies is much messier. Humans have a history before the study started; they have lives that often cause them to deviate from the protocol; and it is much harder to measure the outcomes because our methods have to be so much less invasive than they can be in animal studies...

We examined the use of clinical trials registries in published systematic reviews and meta-analyses from clinical neurology. A review of publications between January 1, 2008 and December 31, 2014 from five neuroscience journals (Annals of Neurology, Brain, Lancet Neurology, Neurology, and The Neuroscientist) was performed to identify eligible systematic reviews. The systematic reviews comprising the final sample were independently appraised to determine if clinical trials registries had been included as part of the search process...

A middle-aged woman with neutropenia and ataxia was found to have raised plasma zinc and profoundly low plasma copper concentrations. When found that she had been prescribed 135 mg zinc/day for seven years, a diagnosis of zinc-induced copper deficiency was made. After the zinc prescription was stopped, her copper and zinc concentrations and neutropenia normalized but she only had partial improvement in neurological status. The diagnosis of zinc-induced copper deficiency can be facilitated by the laboratory through measurement of plasma zinc concentration in patients with a low plasma copper concentration...

BACKGROUND: Vascular endothelial growth factor A stimulates angiogenesis, but is also pro-inflammatory and plays an important role in the development of neurological disease. This study aimed to investigate whether vascular endothelial growth factor A mRNA expression could be used as a marker for the prediction of susceptibility to multiple sclerosis and relate vascular endothelial growth factor to the clinical phases of multiple sclerosis. METHODS: This was a cross-sectional study, consisting of a total of 60 subjects with multiple sclerosis and 20 healthy controls...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

BACKGROUND: Low back pain with or without radiculopathy is an important cause of disability and economic expenditure. However, many patients are not meeting optimal pain control through existing treatments. Recent studies have linked nerve growth factor (NGF) and the pathophysiology of persistent pain. Anti-NGF could be an alternative drug treatment for low back pain. OBJECTIVE: Systematically review the efficacy and safety of anti-NGF in the treatment of low back pain...

INTRODUCTION: A. R. Luria was the first author to hypothesize that executive dysfunction can lead to specific deficits in arithmetic problem solving, showing that patients' performance depends on the structure of the tasks. Cummings (1995. Anatomic and behavioral aspects of frontal-subcortical circuits. Annals of the New York Academy of Sciences, 15, 1-13) proposed the term "environmental dependency" to define such behavioral disorders triggered by the characteristics of the test and pointed out also the role of executive impairments...

Pulp and paper wood feedstocks have been previously implicated as a source of chemicals with the ability to interact with or disrupt key neuroendocrine endpoints important in the control of reproduction. We tested nine Canadian conifers commonly used in pulp and paper production as well as 16 phytochemicals that have been observed in various pulp and paper mill effluent streams for their ability to interact in vitro with the enzymes monoamine oxidase (MAO), glutamic acid decarboxylase (GAD), and GABA-transaminase (GABA-T), and bind to the benzodiazepine-binding site of the GABA(A) receptor (GABA(A)-BZD)...

From November 2nd - 4th 2012, the 4th NEUROWIND e.V. meeting was held in Motzen, Brandenburg, Germany. Again more than 60 participants, predominantly at the doctoral student or postdoc level, gathered to share their latest findings in the fields of neurovascular research, neurodegeneration and neuroinflammation. Like in the previous years, the symposium provided an excellent platform for scientific exchange and the presentation of innovative projects in the stimulating surroundings of the Brandenburg outback...