[Series] Diagnosis, pathophysiology, and management of cluster headache
Cluster headache is a trigeminal autonomic cephalalgia characterised by extremely painful, strictly unilateral, short-lasting headache attacks accompanied by ipsilateral autonomic symptoms or the sense of restlessness and agitation, or both. The severity of the disorder has major effects on the patient's quality of life and, in some cases, might lead to suicidal ideation. Cluster headache is now thought to involve a synchronised abnormal activity in the hypothalamus, the trigeminovascular system, and the autonomic nervous system. (Source: Lancet Neurology)
Source: Lancet Neurology - November 23, 2017 Category: Neurology Authors: Jan Hoffmann, Arne May Tags: Series Source Type: research

[In Context] Prescribing sleep for better health
It was a dark, autumnal, Monday evening, and for many of the audience at the Royal Institution of Great Britain (London, UK), the benefits of any extra sleep over the weekend probably felt all but over after the first day back at work. Rather than opting for an early night, however, we gathered to listen to a public lecture by Matthew Walker (Professor of Neuroscience and Psychology, University of California, Berkeley, CA, USA), entitled Why we sleep. As Director of the Center for Human Sleep Science, Walker's research aim is to uncover the effects of sleep in health and disease, but he is also active in science communicat...
Source: Lancet Neurology - November 17, 2017 Category: Neurology Authors: Cheryl Lai Tags: In Context Source Type: research

[In Context] The fantastic legacy of Terry Pratchett
Terry Pratchett, the author who died in 2015, was perhaps best known for his seminal Discworld novels: fantasies set on a flat disc of a world, held aloft by gargantuan elephants, on the back of a giant turtle swimming through space. Silly? Perhaps, but also utterly, utterly brilliant. (Source: Lancet Neurology)
Source: Lancet Neurology - November 16, 2017 Category: Neurology Authors: Andrew Bianchi Tags: In Context Source Type: research

[Rapid Review] Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications
The disease course of amyotrophic lateral sclerosis (ALS) is rapid and, because its pathophysiology is unclear, few effective treatments are available. Genetic research aims to understand the underlying mechanisms of ALS and identify potential therapeutic targets. The first gene associated with ALS was SOD1, identified in 1993 and, by early 2014, more than 20 genes had been identified as causative of, or highly associated with, ALS. These genetic discoveries have identified key disease pathways that are therapeutically testable and could potentially lead to the development of better treatments for people with ALS. (Source: Lancet Neurology)
Source: Lancet Neurology - November 16, 2017 Category: Neurology Authors: Ruth Chia, Adriano Chi ò, Bryan J Traynor Tags: Rapid Review Source Type: research

[In Context] The longest sleep
Why should I fear death? If I am, death is not. If death is, I am not. Why should I fear that which cannot exist when I do?Epicurus (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: Clare Caldwell, Matthew Kiernan Tags: In Context Source Type: research

[In Context] Sonja Scholz
Sonja Scholz is a neurologist –scientist specialised in neurodegenerative diseases. She obtained her medical doctorate from the Medical University Innsbruck, Austria and her PhD in neurogenomics from University College London, UK. She trained as a postdoctoral fellow in neurogenetics before completing an internship and neurolo gy residency training at Johns Hopkins University, USA. In 2015, she joined the National Institute of Neurological Disorders and Stroke (Bethesda, USA) as an Assistant Clinical Investigator to study neurodegenerative diseases using modern genomic technologies. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Tags: In Context Source Type: research

[Correspondence] Portrayal of progressive supranuclear palsy in the 16th century
A stolid face stares outwards from an oak panel, painted five centuries ago by Cornelis Anthonisz (figure). Skin creases in his neck suggest that the man in this portrait was middle-aged or older. The portrait's most intriguing element, however, is what the artist has captured in his patron's face. Unlike an expressive mien that Dutch artists of this period were so skilled at portraying, this man's face seems oddly lacking in emotion. My impression is that this portrait speaks across the centuries to convey something wrong with the health of the sitter. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: Peter LeWitt Tags: Correspondence Source Type: research

[Correspondence] Chelated or dechelated gadolinium deposition – Authors' reply
We thank Alexander Radbruch and colleagues for their comments regarding our and more recent publications. They highlight an experiment comparing gadolinium retention in the rat brain after high doses of linear or macrocyclic gadolinium-based contrast agents (GBCAs),1 demonstrating retention of tiny amounts of soluble gadolinium in brain tissue for all agents, but higher concentrations of the insoluble fraction for linear than for macrocyclic agents. Of note, another paper2 by the same group reported that brain tissue of rats with proven gadolinium deposition showed no histopathological changes with either linear or macrocy...
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: Vikas Gulani, Fernando Calamante, Frank G Shellock, Emanuel Kanal, Scott B Reeder, International Society for Magnetic Resonance in Medicine Tags: Correspondence Source Type: research

[Correspondence] Chelated or dechelated gadolinium deposition
Although Vikas Gulani and colleagues1 cite most of the recent literature on gadolinium deposition in their Personal View, we believe that pharmacokinetic evidence obtained from recently published studies2 –4 in rats should have also been discussed. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: Alexander Radbruch, Donna R Roberts, Olivier Clement, Alex Rovira, Carlo Cosimo Quattrocchi Tags: Correspondence Source Type: research

[Corrections] Corrections
Kathrin Reetz, Imis Dogan, Ralf-Dieter Hilgers, et al, for the EFACTS Study Group. Progression characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS): a 2 year cohort study. Lancet Neurol 2016; 15: 1346 –54—This Article has been updated so that the members of the EFACTS Study Group are now indexed as authors. This correction has been made to the online version as of Nov 14, 2017. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Tags: Corrections Source Type: research

[Corrections] Corrections
Wild E J, Tabrizi S J. Therapies targeting DNA and RNA in Huntington's disease. Lancet Neurol 2017; 16: 837 –47—In table 1, the final row and footnote have been updated to reflect that CHDI Foundation's small-molecule huntingtin-lowering programme is currently at the screening stage and that the mechanisms of action, route of delivery, and advantages and disadvantages of the small molecules being inve stigated remain to be determined. This correction has been made to the online version as of Nov 14, 2017. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Tags: Corrections Source Type: research

[Corrections] Corrections
Howard J F Jr, Utsugisawa K, Benatar M, et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study. Lancet Neurol 2017; 16: 976 –86—In this Article, (published online Oct 20), the p value for one of the datapoints in figure 2 should be 0·0007. Additionally, in the sentence ‘Half of all patients reported their worst MGFA classification since diagnosis as class IV or V’ on page 6, the citation at the end of the senten ce should be ‘appendix’. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Tags: Corrections Source Type: research

[Editorial] Alzheimer's disease: evolution of research diagnostic criteria
The diagnosis of Alzheimer's disease in research settings has evolved rapidly over the past decade, with both the International Working Group (IWG) and the US National Institute on Aging and Alzheimer's Association (NIA-AA) contributing to endeavours to integrate biomarkers into research diagnostic criteria. These efforts have been important to distinguish the range of disease stages, from asymptomatic to severe dementia. The NIA-AA work group is now proposing a draft research framework that builds upon the 2011 NIA-AA and 2014 IWG criteria to include biomarkers more comprehensively than previously. (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: The Lancet Neurology Tags: Editorial Source Type: research

[Comment] Balancing ethics and care in disorders of consciousness
Neuromodulatory interventions that rely on the premise that stimulation activates or promotes brain circuit signals are being applied to a wide range of therapeutic targets in neurological and psychiatric disorders. The numbers of patients with whom these interventions are being tested, the range of approaches, and the variety of methods are all on the rise. Paralleling these trends are the increasing numbers of countries doing clinical trials, and the coverage of them in the press.1 (Source: Lancet Neurology)
Source: Lancet Neurology - November 15, 2017 Category: Neurology Authors: Laura Y Cabrera, Judy Illes Tags: Comment Source Type: research

[Articles] Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial
This study, which is to our knowledge, the largest trial of CIDP to date and the first to study two administrations of immunoglobulins and two doses, showed that both doses of SCIg IgPro20 were efficacious and well tolerated, suggesting that SCIg can be used as a maintenance treatment for CIDP. (Source: Lancet Neurology)
Source: Lancet Neurology - November 6, 2017 Category: Neurology Authors: Ivo N van Schaik, Vera Bril, Nan van Geloven, Hans-Peter Hartung, Richard A Lewis, Gen Sobue, John-Philip Lawo, Michaela Praus, Orell Mielke, Billie L Durn, David R Cornblath, Ingemar S J Merkies, PATH study group Tags: Articles Source Type: research