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Cumulative Distribution of Amyotrophic Lateral Sclerosis Functional Rating Scale- Revised (ALSFRS-R) Scores in a Phase 3 Study of Edaravone (MCI-186) for the Treatment of Amyotrophic Lateral Sclerosis (ALS): A Posthoc Assessment (P5.110)
Conclusions:Demonstration of median shift and proportion of patients with no decline in ALSFRS-R, and those whose change in ALSFRS-R was limited to ≤2 points during 24 weeks of treatment, may appear to provide supporting evidence for the efficacy of edaravone over placebo.Study Supported by: MTPC was responsible for funding and conduct of the study. MTDA was responsible for executing this analysis.Disclosure: Dr. Takei has received personal compensation for activities with Mitsubishi Tanabe as an employee. Dr. Palumbo has received personal compensation for activities with Mitsubishi Tanabe as an employee. Dr. Palumbo ho...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Takei, K., Palumbo, J. M., Tsuda, K., Liu, S. Tags: Neuromuscular and Clinical Neurophysiology (EMG) Poster Discussion Session Source Type: research

Tale of the Good and the Bad Cdk5: Remodeling of the Actin Cytoskeleton in the Brain
AbstractCdk5 kinase, a cyclin-dependent kinase family member, is a key regulator of cytoskeletal remodeling in the brain. Cdk5 is essential for brain development during embryogenesis. After birth, it is essential for numerous neuronal processes such as learning and memory formation, drug addiction, pain signaling, and long-term behavior changes, all of which rely on rapid alterations in the cytoskeleton. Cdk5 activity is deregulated in various brain disorders including Alzheimer ’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, and ischemic stroke, resulting in profound remodeling of the neuronal cytoskel...
Source: Molecular Neurobiology - May 13, 2017 Category: Neurology Source Type: research

Macrophage migration inhibitory factor: A multifaceted cytokine implicated in multiple neurological diseases.
Abstract Macrophage migration inhibitory factor (MIF) is a conserved cytokine found as a homotrimer protein. It is found in a wide spectrum of cell types in the body including neuronal and non-neuronal cells. MIF is implicated in several biological processes; chemo-attraction, cytokine activity, and receptor binding, among other functions. More recently, a chaperone-like activity has been added to its repertoire. In this review, we focus on the implication of MIF in the central nervous system and peripheries, its role in neurological disorders, and the mechanisms by which MIF is regulated. Numerous studies have as...
Source: Experimental Neurology - July 2, 2017 Category: Neurology Authors: Leyton-Jaimes MF, Kahn J, Israelson A Tags: Exp Neurol Source Type: research

Degeneration of serotonin neurons triggers spasticity in amyotrophic lateral sclerosis
This article is protected by copyright. All rights reserved.
Source: Annals of Neurology - August 30, 2017 Category: Neurology Authors: Hajer El Oussini, Jelena Scekic ‐Zahirovic, Pauline Vercruysse, Christine Marques, Sylvie Dirrig‐Grosch, Stéphane Dieterlé, Gina Picchiarelli, Jérôme Sinniger, Caroline Rouaux, Luc Dupuis Tags: Research Article Source Type: research

Neurological diseases and risk of suicide attempt: a case –control study
ConclusionNine out of ten chronic neurological diseases were associated with an increased risk of suicide attempt. These data must be considered for clinicians treating this vulnerable group of patients.
Source: Journal of Neurology - March 21, 2018 Category: Neurology Source Type: research

The Primitive Palmomental Reflex in Amyotrophic Lateral Sclerosis
Conclusion: We demonstrate a higher frequency of the PMR in ALS patients compared to NC or HC. Its expression increases with age, being higher in bulbar-onset patients. Given that the reflex circuit is located in the brain stem, its release due to inhibition might be associated to the presence of a cortico-bulbar tract dysfunction in ALS.Eur Neurol 2018;79:187 –191
Source: European Neurology - March 23, 2018 Category: Neurology Source Type: research

Raloxifene, a promising estrogen replacement, limits TDP-25 cell death by enhancing autophagy and suppressing apoptosis.
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset neurodegenerative disease, and at present, therapies for ALS are limited. Estrogen is a potential therapeutic agent for ALS but has undesirable effects that might increase the risk of breast and uterine cancers or stroke. Raloxifene (Ral) has estrogenic properties but does not exhibit these adverse effects. However, the mechanism of Ral in ALS has not been studied. We thus investigated the effects of Ral in an NSC34 model of ALS that stably expresses the 25-kDa C-terminal fragment of TDP-43 (i.e., TDP-25 cells) and found that GPR30 (G protein-coup...
Source: Brain Research Bulletin - May 25, 2018 Category: Neurology Authors: Zhou F, Dong H, Liu Y, Yan L, Sun C, Hao P, Liu Y, Zhai J, Liu Y Tags: Brain Res Bull Source Type: research

Comparison of Premortem Magnetic Resonance Imaging and Postmortem Autopsy Findings of a Cortical Microinfarct
An 85-year-old woman diagnosed with amyotrophic lateral sclerosis died of pneumonia and was autopsied. Magnetic resonance imaging (MRI) performed 16days before death revealed an intracortical high-intensity lesion in her right temporal cortex on three-dimensional (3D)-double inversion recovery (DIR) and 3D-fluid-attenuated inversion recovery (FLAIR) images. Histopathological examination indicated a cortical microinfarct (CMI) juxtaposed to cerebral amyloid angiopathy. Recently, in vivo detection of CMIs using 3D-DIR and 3D-FLAIR on 3-tesla MRI has been reported, and postmortem MRI study confirmed the presence of CMIs.
Source: Journal of Stroke and Cerebrovascular Diseases - July 2, 2018 Category: Neurology Authors: Hidehiro Ishikawa, Yuichiro Ii, Atsushi Niwa, Akihiro Shindo, Ai Ito, Keita Matsuura, Ryogen Sasaki, Kenichiro Uno, Masayuki Maeda, Hidekazu Tomimoto Source Type: research

Yawning in neurology: a review
ABSTRACT Yawning is a stereotyped physiological behavior that can represent a sign or symptom of several conditions, such as stroke, parakinesia brachialis oscitans, parkinsonism, Parkinson ’ s disease and epilepsy. More rarely, it can occur in patients with intracranial hypertension, brain tumor, multiple sclerosis, migraine, Chiari malformation type I, and amyotrophic lateral sclerosis. Drug-induced yawning is an uncommon clinical condition and yawning in patients with autism or schizophrenia is very rare. The aim of this review is to describe in detail the occurrence of the phenomenon in such conditions, and its ’ p...
Source: Arquivos de Neuro-Psiquiatria - July 26, 2018 Category: Neurology Source Type: research

Targeting pericytes for therapeutic approaches to neurological disorders
AbstractMany  central nervous system diseases currently lack effective treatment and are often associated with defects in microvascular function, including a failure to match the energy supplied by the blood to the energy used on neuronal computation, or a breakdown of the blood–brain barrier. Pericytes, an u nder-studied cell type located on capillaries, are of crucial importance in regulating diverse microvascular functions, such as angiogenesis, the blood–brain barrier, capillary blood flow and the movement of immune cells into the brain. They also form part of the “glial” scar isolating damaged parts of the CN...
Source: Acta Neuropathologica - August 10, 2018 Category: Neurology Source Type: research

Stem cell therapy for neurological disorders: A focus on aging.
Abstract Age-related neurological disorders continue to pose a significant societal and economic burden. Aging is a complex phenomenon that affects many aspects of the human body. Specifically, aging can have detrimental effects on the progression of brain diseases and endogenous stem cells. Stem cell therapies possess promising potential to mitigate the neurological symptoms of such diseases. However, aging presents a major obstacle for maximum efficacy of these treatments. In this review, we discuss current preclinical and clinical literature to highlight the interactions between aging, stem cell therapy, and th...
Source: Neurobiology of Disease - September 13, 2018 Category: Neurology Authors: Nguyen H, Zarriello S, Coats A, Nelson C, Kingsbury C, Gorsky A, Rajani M, Neal EG, Borlongan CV Tags: Neurobiol Dis Source Type: research

Targeting pericytes for therapeutic approaches to neurological disorders
AbstractMany  central nervous system diseases currently lack effective treatment and are often associated with defects in microvascular function, including a failure to match the energy supplied by the blood to the energy used on neuronal computation, or a breakdown of the blood–brain barrier. Pericytes, an u nder-studied cell type located on capillaries, are of crucial importance in regulating diverse microvascular functions, such as angiogenesis, the blood–brain barrier, capillary blood flow and the movement of immune cells into the brain. They also form part of the “glial” scar isolating damaged parts of the CN...
Source: Acta Neuropathologica - October 1, 2018 Category: Neurology Source Type: research

Understanding the Role of Hypoxia Inducible Factor During Neurodegeneration for New Therapeutics Opportunities.
Abstract Neurodegeneration (NDG) is linked with the progressive loss of neural function with intellectual and/or motor impairment. Several diseases affecting older individuals, including Alzheimer's disease, Amyotrophic Lateral Sclerosis, Huntington's disease, Parkinson's disease, stroke, Multiple Sclerosis and many others, are the most relevant disorders associated with NDG. Since other pathologies such as refractory epilepsy, brain infections, or hereditary diseases such as "neurodegeneration with brain iron accumulation", also lead to chronic brain inflammation with loss of neural cells, NDG can be said to affe...
Source: Epilepsy Curr - November 28, 2018 Category: Neurology Authors: Merelli A, Rodríguez JCG, Folch J, Regueiro MR, Camins A, Lazarowski A Tags: Curr Neuropharmacol Source Type: research

Individual variations of the human corticospinal tract and its hand-related motor fibers using diffusion MRI tractography
AbstractThe corticospinal tract (CST) is one of the most well studied tracts in human neuroanatomy. Its clinical significance can be demonstrated in many notable traumatic conditions and diseases such as stroke, spinal cord injury (SCI) or amyotrophic lateral sclerosis (ALS). With the advent of diffusion MRI and tractography the computational representation of the human CST in a 3D model became available. However, the representation of the entire CST and, specifically, the hand motor area has remained elusive. In this paper we propose a novel method, using manually drawn ROIs based on robustly identifiable neuroanatomic st...
Source: Brain Imaging and Behavior - January 8, 2019 Category: Neurology Source Type: research

Edaravone for the treatment of amyotrophic lateral sclerosis.
Authors: Yoshino H Abstract INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a progressive fatal disorder that affects all skeletal muscles, leading to death, mostly within 2-4 years from onset. To date, the anti-glutamatergic drug riluzole is the only drug that has been approved for the treatment of this disease; however, its efficacy is modest. Oxidative stress is considered to be involved in the pathology of ALS, and in this regard, the free radical scavenger edaravone, which was originally developed for the treatment of acute ischemic stroke, has also been developed for the treatment of ALS. Areas covered: Thi...
Source: Expert Review of Neurotherapeutics - February 28, 2019 Category: Neurology Tags: Expert Rev Neurother Source Type: research

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