80 results match your criteria Journal of Central Nervous System Disease [Journal]


Rotatory Dislocation of the Spine in Dystrophic Kyphoscoliosis Secondary to Neurofibromatosis Type 1.

J Cent Nerv Syst Dis 2018 18;10:1179573518819484. Epub 2018 Dec 18.

Scottish National Spine Deformity Service, Royal Hospital for Sick Children, Edinburgh, UK.

Aim Of The Study: Neurofibromatosis type 1 (NF-1) is associated with the development of scoliosis or kyphoscoliosis. The deformity is rapidly progressive in the presence of dystrophic changes producing bone erosion. Vertebral subluxation or dislocation can occur in children with highly dystrophic kyphoscoliosis and carries an increased risk of paralysis. Read More

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http://dx.doi.org/10.1177/1179573518819484DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309030PMC
December 2018
2 Reads

Cryptogenic Stroke: To Close a Patent Foramen Ovale or Not to Close?

J Cent Nerv Syst Dis 2018 16;10:1179573518819476. Epub 2018 Dec 16.

Department of Medicine, The Chicago Medical School, Rosalind Franklin University, North Chicago, IL, USA.

A patent foramen ovale (PFO) has been shown to be highly prevalent in patients diagnosed with strokes of unknown cause, which are also called cryptogenic strokes (CSs). It has been a long-running controversy as to whether a PFO should be closed or not to prevent recurrent strokes in patients diagnosed with CS. A paradoxical embolism that is produced through a PFO is hypothesized to be a leading cause of CS, especially in younger patients with low risk factors for stroke. Read More

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http://dx.doi.org/10.1177/1179573518819476DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297885PMC
December 2018
2 Reads

The Clinical Utility of Virtual Reality in Neurorehabilitation: A Systematic Review.

J Cent Nerv Syst Dis 2018 27;10:1179573518813541. Epub 2018 Nov 27.

Graduate Program in Rehabilitation Sciences, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Background: Virtual reality (VR) experiences (through games and virtual environments) are increasingly being used in physical, cognitive, and psychological interventions. However, the impact of VR as an approach to rehabilitation is not fully understood, and its advantages over traditional rehabilitation techniques are yet to be established.

Method: We present a systematic review which was conducted according to (PRISMA). Read More

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http://dx.doi.org/10.1177/1179573518813541DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262495PMC
November 2018
11 Reads

Evaluation of Serum Apolipoprotein E as a Potential Biomarker for Pharmacological Therapeutic Efficacy Monitoring in Dopamine Dictated Disease Spectrum of Schizophrenia and Parkinson's disease: A Preliminary Study.

J Cent Nerv Syst Dis 2018 9;10:1179573518803585. Epub 2018 Oct 9.

Department of Biophysics, All India Institute of Medical Sciences, New Delhi, New Delhi, India.

Aim Of The Study: Parkinson's disease and schizophrenia are disease end points of dopaminergic deficit and hyperactivity, respectively, in the mid brain. Accordingly, current medications aim to restore normal dopamine levels, overshooting of which results in adverse effects of psychosis and extra-pyramidal symptoms, respectively. There are currently no available laboratory tests to guide treatment decisions or help predict adverse side effects of the drugs. Read More

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http://dx.doi.org/10.1177/1179573518803585DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6178121PMC
October 2018
2 Reads

Unraveling of Central Nervous System Disease Mechanisms Using CRISPR Genome Manipulation.

Authors:
Aino Vesikansa

J Cent Nerv Syst Dis 2018 10;10:1179573518787469. Epub 2018 Jul 10.

Department of Veterinary Biosciences, Faculty of Veterinary Medicine, University of Helsinki, Helsinki, Finland.

The complex structure and highly variable gene expression profile of the brain makes it among the most challenging fields to study in both basic and translational biological research. Most of the brain diseases are multifactorial and despite the rapidly increasing genomic data, molecular pathways and causal links between genes and central nervous system (CNS) diseases are largely unknown. The advent of an easy and flexible CRISPR-Cas genome editing technology has rapidly revolutionized the field of functional genomics and opened unprecedented possibilities to dissect the mechanisms of CNS disease. Read More

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http://dx.doi.org/10.1177/1179573518787469DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6043941PMC
July 2018
1 Read

Pharmacotherapy of Insomnia.

J Cent Nerv Syst Dis 2018 19;10:1179573518770672. Epub 2018 Apr 19.

Department of Pharmacology and Therapeutics, School of Medicine Clinics Hospital, University of the Republic, Montevideo, Uruguay.

Insomnia remains a common clinical concern that is associated with negative daytime consequences for patients and represents a significant public health problem for our society. Although a variety of therapies may be employed to treat insomnia, the use of medications has been a dominant approach. Regulatory agencies have now classified insomnia medications into 4 distinct pharmacodynamics classes. Read More

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http://dx.doi.org/10.1177/1179573518770672DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5987897PMC
April 2018
1 Read

Small Fiber Neuropathy: Disease Classification Beyond Pain and Burning.

Authors:
Todd D Levine

J Cent Nerv Syst Dis 2018 18;10:1179573518771703. Epub 2018 Apr 18.

Honor Health Neurology Department, Phoenix Neurological Associates, Phoenix, AZ, USA.

Small fiber neuropathy (SFN) has a poorly understood pathology, but patients would benefit from determination of clinical phenotypes that allows for better diagnosis and treatment planning. I propose that patients should be classified dependent on whether there is sodium channel dysfunction, classic neurologic symptoms only, widespread neuropathic pain, or autonomic symptoms. Patients with SFN can then be considered in light of their clinical phenotype, allowing for focus on subsets of patients who might have diagnosable conditions or be more prone to responding to a particular type of therapy that may not be efficacious in the broader patient population with SFN. Read More

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http://dx.doi.org/10.1177/1179573518771703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5912271PMC

Further Delineation of the Clinical Phenotype of Cerebellar Ataxia, Mental Retardation, and Disequilibrium Syndrome Type 4.

J Cent Nerv Syst Dis 2018 28;10:1179573518759682. Epub 2018 Feb 28.

King Abdullah International Medical Research Center (KAIMRC), King Saud bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs (MNGHA), Riyadh, Saudi Arabia.

Background: Cerebellar ataxia, mental retardation, and disequilibrium syndrome (CAMRQ) is a heterogeneous group of genetic disorders that have been grouped by shared clinical features; all of these features are transmitted via an autosomal recessive mechanism. Four variants of this syndrome have been identified so far, and each one differs in terms of both clinical and genotypical features. CAMRQ4 is a rare genetic disorder characterized by mental retardation, ataxia or an inability to walk, dysarthria and, in some patients, quadrupedal gait. Read More

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http://dx.doi.org/10.1177/1179573518759682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5843099PMC
February 2018
17 Reads

The Need for Antiepileptic Drug Chronotherapy to Treat Selected Childhood Epilepsy Syndromes and Avert the Harmful Consequences of Drug Resistance.

J Cent Nerv Syst Dis 2017 20;9:1179573516685883. Epub 2017 Dec 20.

Harvard Medical School, Boston, MA, US.

Antiepileptic drug (AED) chronotherapy involves the delivery of a greater AED dose at the time of greatest seizure susceptibility usually associated with predictable seizure peaks. Although research has proven AED chronotherapy, commonly known as differential dosing, to be safe, well tolerated, and highly effective in managing cyclic seizure patterns in selected childhood epilepsies, conventional, equally divided AED dosing remains the standard of care. Differential dosing is more often applied in the emergency management of acute seizure clustering resulting from drug resistance-a harmful epilepsy-related consequence that affects 30% of children. Read More

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http://dx.doi.org/10.1177/1179573516685883DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5751908PMC
December 2017
2 Reads

Early Infantile Leigh-like Gene Defects Have a Poor Prognosis: Report and Review.

Authors:
Majid Alfadhel

J Cent Nerv Syst Dis 2017 27;9:1179573517737521. Epub 2017 Oct 27.

King Abdullah International Medical Research Centre, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

Solute carrier family 19 (thiamine transporter), member 3 () gene defect produces an autosomal recessive neurodegenerative disorder associated with different phenotypes and acronyms. One of the common presentations is early infantile lethal Leigh-like syndrome. We report a case of early infantile Leigh-like gene defects of patients who died at 4 months of age with no response to a high dose of biotin and thiamine. Read More

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http://dx.doi.org/10.1177/1179573517737521DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5661663PMC
October 2017
3 Reads

Review of Lisdexamfetamine Dimesylate in Adults With Attention-Deficit/Hyperactivity Disorder.

J Cent Nerv Syst Dis 2017 23;9:1179573517728090. Epub 2017 Aug 23.

Arnold & Marie Schwartz College of Pharmacy and Health Sciences, Long Island University, Brooklyn, NY, USA.

Lisdexamfetamine dimesylate (LDX) is the first prodrug stimulant used for the treatment of attention-deficit/hyperactivity disorder (ADHD) dosed once daily. Due to its long-acting properties, LDX remains pharmacologically inactive until an enzymatic process predominantly associated with red blood cells converts it to the active ingredient, d-amphetamine and the amino acid lysine. The efficacy of LDX over placebo has been demonstrated in several studies in adults with moderate to severe ADHD with significant improvements noted in ADHD rating scales, Clinical Global Improvement scores, and assessments of executive function, for all doses of LDX (30-70 mg daily). Read More

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http://dx.doi.org/10.1177/1179573517728090DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5571766PMC
August 2017
21 Reads

Abnormalities of Dopamine D Receptor Signaling in the Diseased Brain.

Authors:
G Aleph Prieto

J Cent Nerv Syst Dis 2017 16;9:1179573517726335. Epub 2017 Aug 16.

Institute for Memory Impairments and Neurological Disorders, University of California, Irvine, CA, USA.

Dopamine D receptors (DR) modulate neuronal activity in several brain regions including cortex, striatum, cerebellum, and hippocampus. A growing body of evidence suggests that aberrant DR signaling contributes to multiple brain diseases, such as Parkinson's disease, essential tremor, schizophrenia, and addiction. In line with these findings, DR has emerged as a potential target in the treatment of neurological disorders. Read More

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http://dx.doi.org/10.1177/1179573517726335DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5562332PMC
August 2017
1 Read

Inhibition of Inflammation Mediated Through the Tumor Necrosis Factor α Biochemical Pathway Can Lead to Favorable Outcomes in Alzheimer Disease.

J Cent Nerv Syst Dis 2017 28;9:1179573517722512. Epub 2017 Jul 28.

Saba University School of Medicine, The Bottom, Dutch Caribbean.

Tumor necrosis factor α (TNF-α) inhibitors have long been used as disease-modifying agents in immune disorders. Recently, research has shown a role of chronic neuroinflammation in the pathophysiology of neurodegenerative diseases such as Alzheimer disease, and interest has been generated in the use of anti-TNF agents and TNF-modulating agents for prevention and treatment. This article extensively reviewed literature on animal studies testing these agents. Read More

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http://journals.sagepub.com/doi/10.1177/1179573517722512
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http://dx.doi.org/10.1177/1179573517722512DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5536370PMC
July 2017
9 Reads

Treatment of Movement Disorders With Focused Ultrasound.

J Cent Nerv Syst Dis 2017 6;9:1179573517705670. Epub 2017 Jun 6.

Department of Diagnostic Radiology and Nuclear Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.

Although the use of ultrasound as a potential therapeutic modality in the brain has been under study for several decades, relatively few neuroscientists or neurologists are familiar with this technology. Stereotactic brain lesioning had been widely used as a treatment for medically refractory patients with essential tremor (ET), Parkinson disease (PD), and dystonia but has been largely replaced by deep brain stimulation (DBS) surgery, with advantages both in safety and efficacy. However, DBS is associated with complications including intracerebral hemorrhage, infection, and hardware malfunction. Read More

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http://dx.doi.org/10.1177/1179573517705670DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5462491PMC
June 2017
22 Reads

Tumor necrosis factor α Inhibition for Alzheimer's Disease.

J Cent Nerv Syst Dis 2017 15;9:1179573517709278. Epub 2017 May 15.

Department of Biopharmaceutical Sciences, School of Pharmacy, Keck Graduate Institute, Claremont, CA, USA.

Tumor necrosis factor α (TNF-α) plays a central role in the pathophysiology of Alzheimer's disease (AD). Food and Drug Administration-approved biologic TNF-α inhibitors are thus a potential treatment for AD, but they do not cross the blood-brain barrier. In this short review, we discuss the involvement of TNF-α in AD, challenges associated with the development of existing biologic TNF-α inhibitors for AD, and potential therapeutic strategies for targeting TNF-α for AD therapy. Read More

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http://dx.doi.org/10.1177/1179573517709278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5436834PMC
May 2017
26 Reads

Management of Viral Central Nervous System Infections: A Primer for Clinicians.

J Cent Nerv Syst Dis 2017 1;9:1179573517703342. Epub 2017 May 1.

Division of Infectious Diseases, Department of Internal Medicine, School of Medicine, University of South Carolina, Columbia, SC, USA.

Viruses are a common cause of central nervous system (CNS) infections with many host, agent, and environmental factors influencing the expression of viral diseases. Viruses can be responsible for CNS disease through a variety of mechanisms including direct infection and replication within the CNS resulting in encephalitis, infection limited to the meninges, or immune-related processes such as acute disseminated encephalomyelitis. Common pathogens including herpes simplex virus, varicella zoster, and enterovirus are responsible for the greatest number of cases in immunocompetent hosts. Read More

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http://dx.doi.org/10.1177/1179573517703342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5415352PMC
May 2017
24 Reads

Evidence-Based Brief Obsessive-Compulsive Scale.

J Cent Nerv Syst Dis 2017 15;9:1179573517702867. Epub 2017 May 15.

School of Medical Sciences, Örebro University, Örebro, Sweden.

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http://dx.doi.org/10.1177/1179573517702867DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5436836PMC
May 2017
6 Reads

Olfactory Neuroepithelial Neural Progenitor Cells from Subjects with Bipolar I Disorder.

J Cent Nerv Syst Dis 2017 15;9:1179573517694529. Epub 2017 Mar 15.

Professor and Director, Mood Disorders Research Program, Department of Psychiatry and Behavioral Sciences, University of Louisville School of Medicine, Louisville, KY, USA.

Background: Research into the pathophysiology of bipolar disorder (BD) is limited by the inability to examine brain cellular processes in subjects with the illness.

Methods: Endoscopic biopsy was performed in subjects with bipolar I disorder to establish olfactory neural progenitor (ONP) cell lines. Olfactory function was assessed prebiopsy and postbiopsy using the University of Pennsylvania Smell Identification Test (UPSIT). Read More

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http://insights.sagepub.com/redirect_file.php?fileId=8390&fi
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http://insights.sagepub.com/olfactory-neuroepithelial-neural
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http://dx.doi.org/10.1177/1179573517694529DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5392049PMC
March 2017
28 Reads

Role of Transporters in Central Nervous System Drug Delivery and Blood-Brain Barrier Protection: Relevance to Treatment of Stroke.

J Cent Nerv Syst Dis 2017 6;9:1179573517693802. Epub 2017 Apr 6.

Department of Pharmacology, College of Medicine, The University of Arizona, Tucson, AZ, USA.

Ischemic stroke is a leading cause of morbidity and mortality in the United States. The only approved pharmacologic treatment for ischemic stroke is thrombolysis via recombinant tissue plasminogen activator (r-tPA). A short therapeutic window and serious adverse events (ie, hemorrhage, excitotoxicity) greatly limit r-tPA therapy, which indicates an essential need to develop novel stroke treatment paradigms. Read More

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http://dx.doi.org/10.1177/1179573517693802DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5392046PMC
April 2017
41 Reads

Unmasking the Role of Uptake Transporters for Digoxin Uptake Across the Barriers of the Central Nervous System in Rat.

J Cent Nerv Syst Dis 2017 15;9:1179573517693596. Epub 2017 Mar 15.

Pharmaceutical Candidate Optimization, Biocon Bristol-Myers Squibb Research & Development Center (BBRC), Bristol-Myers Squibb India Ltd, Bangalore, India.

The role of uptake transporter (organic anion-transporting polypeptide [Oatp]) in the disposition of a P-glycoprotein (P-gp) substrate (digoxin) at the barriers of central nervous system, namely, the blood-brain barrier (BBB), blood-spinal cord barrier (BSCB), and brain-cerebrospinal fluid barrier (BCSFB), was studied using rat as a preclinical species. In vivo chemical inhibition of P-gp and Oatp was achieved using elacridar and rifampicin, respectively. Our findings show that (1) digoxin had a low brain-to-plasma concentration ratio (B/P) (0. Read More

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http://dx.doi.org/10.1177/1179573517693596DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5392048PMC
March 2017
21 Reads

Evidence-Based Assessment of Obsessive-Compulsive Disorder.

J Cent Nerv Syst Dis 2016 21;8:13-29. Epub 2016 Aug 21.

Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, CA, USA.

Obsessive-compulsive disorder (OCD) is a neuropsychiatric illness that often develops in childhood, affects 1%-2% of the population, and causes significant impairment across the lifespan. The first step in identifying and treating OCD is a thorough evidence-based assessment. This paper reviews the administration pragmatics, psychometric properties, and limitations of commonly used assessment measures for adults and youths with OCD. Read More

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http://dx.doi.org/10.4137/JCNSD.S38359DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4994744PMC
September 2016
8 Reads

Molecular Mechanism of Adult Neurogenesis and its Association with Human Brain Diseases.

Authors:
He Liu Ni Song

J Cent Nerv Syst Dis 2016 20;8:5-11. Epub 2016 Jun 20.

Division of Health Sciences & Workforce Technology, Lamar State College-Orange, Orange, TX, USA.

Recent advances in neuroscience challenge the old dogma that neurogenesis occurs only during embryonic development. Mounting evidence suggests that functional neurogenesis occurs throughout adulthood. This review article discusses molecular factors that affect adult neurogenesis, including morphogens, growth factors, neurotransmitters, transcription factors, and epigenetic factors. Read More

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http://dx.doi.org/10.4137/JCNSD.S32204DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4915785PMC
July 2016
6 Reads

Benign Sphenoid Wing Meningioma Presenting with an Acute Intracerebral Hemorrhage - A Case Report.

J Cent Nerv Syst Dis 2016 21;8:1-4. Epub 2016 Apr 21.

Department of Pathology, Oslo University Hospital - Rikshospitalet, Oslo, Norway.

Background And Study Object: We report an unusual case of a benign lateral sphenoid wing meningioma that presented with, and was masked by, an acute intracerebral hemorrhage.

Case Report: A 68-year-old woman was admitted after sudden onset of coma. Computed tomography (CT) revealed an intracerebral hemorrhage, without any underlying vascular pathology on CT angiography. Read More

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http://dx.doi.org/10.4137/JCNSD.S33653DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841328PMC
April 2016
5 Reads

Bioinformatics Data Mining Approach Suggests Coexpression of AGTPBP1 with an ALS-linked Gene C9orf72.

J Cent Nerv Syst Dis 2015 8;7:15-26. Epub 2015 Jun 8.

Department of Bioinformatics and Molecular Neuropathology, Meiji Pharmaceutical University, Kiyose, Tokyo, Japan.

Background: Expanded GGGGCC hexanucleotide repeats located in the noncoding region of the chromosome 9 open reading frame 72 (C9orf72) gene represent the most common genetic abnormality for familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Formation of nuclear RNA foci, accumulation of repeat-associated non-ATG-translated dipeptide-repeat proteins, and haploinsufficiency of C9orf72 are proposed for pathological mechanisms of C9ALS/FTD. However, at present, the physiological function of C9orf72 remains largely unknown. Read More

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http://dx.doi.org/10.4137/JCNSD.S24317DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4467204PMC
June 2015
4 Reads

A Comprehensive Profile of ChIP-Seq-Based Olig2 Target Genes in Motor Neuron Progenitor Cells Suggests the Possible Involvement of Olig2 in the Pathogenesis of Amyotrophic Lateral Sclerosis.

J Cent Nerv Syst Dis 2015 18;7:1-14. Epub 2015 May 18.

Department of Bioinformatics and Molecular Neuropathology, Meiji Pharmaceutical University, Kiyose, Tokyo, Japan.

Background: Amyotrophic lateral sclerosis (ALS) is an intractable neurodegenerative disease that primarily affects motor neurons in the cerebral cortex and the spinal cord. Recent evidence indicates that dysfunction of oligodendrocytes is implicated in the pathogenesis of ALS. The basic helix-loop-helix (bHLH) transcription factor Olig2 plays a pivotal role in the development of both motor neurons and oligodendrocytes in the progenitor of motor neuron (pMN) domain of the spinal cord, supporting evidence for the shared motor neuron/oligodendrocyte lineage. Read More

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http://dx.doi.org/10.4137/JCNSD.S23210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4437538PMC
May 2015
6 Reads

Administration of an amino Acid-based regimen for the management of autonomic nervous system dysfunction related to combat-induced illness.

J Cent Nerv Syst Dis 2014 8;6:93-8. Epub 2014 Oct 8.

Targeted Medical Pharma, Los Angeles, CA, USA.

The etiology and pathophysiology of posttraumatic stress disorder (PTSD) remains poorly understood. The nutritional deficiencies associated with the altered metabolic processes of PTSD have not previously been studied in detail. This pilot study measured the reduction in symptoms in 21 military veterans reporting moderate to severe symptoms associated with PTSD. Read More

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http://dx.doi.org/10.4137/JCNSD.S13793DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4197905PMC
October 2014
5 Reads

Idiopathic ventral spinal cord herniation: an increasingly recognized cause of thoracic myelopathy.

J Cent Nerv Syst Dis 2014 1;6:85-91. Epub 2014 Oct 1.

Department of Neurosurgery, Oslo University Hospital, Rikshospitalet, Oslo, Norway. ; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

Idiopathic spinal cord herniation (ISCH), where a segment of the spinal cord has herniated through a ventral defect in the dura, is a rarely encountered cause of thoracic myelopathy. The purpose of our study was to increase the clinical awareness of this condition by presenting our experience with seven consecutive cases treated in our department since 2005. All the patients developed pronounced spastic paraparesis or Brown-Séquard syndrome for several years (mean, 4. Read More

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http://dx.doi.org/10.4137/JCNSD.S16180DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4196882PMC
October 2014
3 Reads

Increased Epidermal Growth Factor Receptor (EGFR) Associated with Hepatocyte Growth Factor (HGF) and Symptom Severity in Children with Autism Spectrum Disorders (ASDs).

Authors:
Anthony J Russo

J Cent Nerv Syst Dis 2014 9;6:79-83. Epub 2014 Sep 9.

Hartwick College, Oneonta, NY, Health Research Institute and Pfeiffer Medical Center, Warrenville, IL.

Background: One in 88 children in the US is thought to have one of the autism spectrum disorders (ASDs). ASDs are characterized by social impairments and communication problems. Growth factors and their receptors may play a role in the etiology of ASDs. Read More

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http://dx.doi.org/10.4137/JCNSD.S13767DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4167315PMC
September 2014
9 Reads

Molecular network analysis suggests a logical hypothesis for the pathological role of c9orf72 in amyotrophic lateral sclerosis/frontotemporal dementia.

J Cent Nerv Syst Dis 2014 24;6:69-78. Epub 2014 Aug 24.

Department of Bioinformatics and Molecular Neuropathology, Meiji Pharmaceutical University, Kiyose, Tokyo, Japan.

Background: Expanded GGGGCC hexanucleotide repeats, ranging from hundreds to thousands in number, located in the noncoding region of the chromosome 9 open reading frame 72 (C9orf72) gene represent the most common genetic abnormality for familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (abbreviated as C9ALS). Currently, three pathological mechanisms, such as haplo insufficiency of C9orf72, formation of nuclear RNA foci composed of sense and antisense repeats, and accumulation of unconventionally transcribed dipeptide-repeat (DPR) proteins, are proposed for C9ALS. However, at present, the central mechanism underlying neurodegeneration in C9ALS remains largely unknown. Read More

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http://dx.doi.org/10.4137/JCNSD.S18103DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4149381PMC
September 2014
15 Reads

Vanishing white matter disease in a spanish population.

J Cent Nerv Syst Dis 2014 13;6:59-68. Epub 2014 Jul 13.

Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, Spain. ; Molecular Genetics Department, Hospital Sant Joan de Déu, Barcelona, Spain.

Vanishing white matter (VWM) leukoencephalopathy is one of the most prevalent hereditary white matter diseases. It has been associated with mutations in genes encoding eukaryotic translation initiation factor (eIF2B). We have compiled a list of all the patients diagnosed with VWM in Spain; we found 21 children. Read More

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http://dx.doi.org/10.4137/JCNSD.S13540DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4116383PMC
August 2014
13 Reads

Neuronal and glia-related biomarkers in cerebrospinal fluid of patients with acute ischemic stroke.

J Cent Nerv Syst Dis 2014 19;6:51-8. Epub 2014 May 19.

Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, University of Gothenburg, The Sahlgrenska Academy, Gothenburg, Sweden.

Background: Cerebral ischemia promotes morphological reactions of the neurons, astrocytes, oligodendrocytes, and microglia in experimental studies. Our aim was to examine the profile of CSF (cerebrospinal fluid) biomarkers and their relation to stroke severity and degree of white matter lesions (WML).

Methods: A total of 20 patients (mean age 76 years) were included within 5-10 days after acute ischemic stroke (AIS) onset. Read More

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http://dx.doi.org/10.4137/JCNSD.S13821DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4039292PMC
June 2014
5 Reads

Efficacy and side effects of natalizumab therapy in patients with multiple sclerosis.

J Cent Nerv Syst Dis 2014 28;6:41-9. Epub 2014 Apr 28.

Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Germany.

Natalizumab (Nat) is a humanized monoclonal antibody used for the treatment of relapsing multiple sclerosis (MS). Nat inhibits lymphocyte migration via the blood brain barrier (BBB) by blockage of an integrin adhesion molecule, very late antigen 4. During the phase III clinical trials, it was shown that Nat reduces disease activity and prevents disability progression. Read More

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http://dx.doi.org/10.4137/JCNSD.S14049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4011812PMC
June 2014
5 Reads

Medical treatment of essential tremor.

J Cent Nerv Syst Dis 2014 21;6:29-39. Epub 2014 Apr 21.

University of Saskatchewan, Canada. ; Saskatoon Health Region, Canada.

Essential tremor (ET) is the most common pathological tremor characterized by upper limb action-postural tremor (PT)/kinetic tremor (KT). There are no specific neuropathological or biochemical abnormalities in ET. The disability is consequent to amplitude of KT, which may remain mild without handicap or may become disabling. Read More

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http://dx.doi.org/10.4137/JCNSD.S13570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3999812PMC
June 2014
7 Reads

Temporal- and Location-Specific Alterations of the GABA Recycling System in Mecp2 KO Mouse Brains.

J Cent Nerv Syst Dis 2014 7;6:21-8. Epub 2014 Apr 7.

Neuroscience Laboratory, Hugo Moser Research Institute at Kennedy Krieger. ; Departments of Neurology, Johns Hopkins University School of Medicine; Baltimore, USA.

Rett syndrome (RTT), associated with mutations in methyl-CpG-binding protein 2 (Mecp2), is linked to diverse neurological symptoms such as seizures, motor disabilities, and cognitive impairments. An altered GABAergic system has been proposed as one of many underlying pathologies of progressive neurodegeneration in several RTT studies. This study for the first time investigated the temporal- and location-specific alterations in the expression of γ-amino butyric acid (GABA) transporter 1 (GAT-1), vesicular GABA transporter (vGAT), and glutamic acid decarboxylase 67kD (GAD67) in wild type (WT) and knockout (KO) mice in the Mecp2(tm1. Read More

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http://dx.doi.org/10.4137/JCNSD.S14012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3981570PMC
April 2014
2 Reads

Spontaneous spinal epidural hematoma as a potentially important stroke mimic.

J Cent Nerv Syst Dis 2014 6;6:15-20. Epub 2014 Feb 6.

Department of Internal Medicine, Jichi Medical University, Shimotsuke, Japan.

Hemiparesis develops in response to a wide range of neurological disorders, such as stroke, neoplasms and several inflammatory processes. Occasionally, it may also occur due to a lesion located in the high cervical spinal cord. In this concise review, we describe the features of spontaneous spinal epidural hematoma, which should be included in the large list of stroke mimics. Read More

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http://dx.doi.org/10.4137/JCNSD.S13252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921023PMC
June 2014
1 Read

Manual versus Automated Rodent Behavioral Assessment: Comparing Efficacy and Ease of Bederson and Garcia Neurological Deficit Scores to an Open Field Video-Tracking System.

J Cent Nerv Syst Dis 2014 22;6:7-14. Epub 2014 Jan 22.

Department of Neurological Surgery, Vanderbilt University, Nashville, TN, USA.

Animal models of stroke have been crucial in advancing our understanding of the pathophysiology of cerebral ischemia. Currently, the standards for determining neurological deficit in rodents are the Bederson and Garcia scales, manual assessments scoring animals based on parameters ranked on a narrow scale of severity. Automated open field analysis of a live-video tracking system that analyzes animal behavior may provide a more sensitive test. Read More

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http://dx.doi.org/10.4137/JCNSD.S13194DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921024PMC
February 2014
5 Reads

Aromatic amino Acid decarboxylase deficiency not responding to pyridoxine and bromocriptine therapy: case report and review of response to treatment.

J Cent Nerv Syst Dis 2014 Jan 7;6:1-5. Epub 2014 Jan 7.

Division of General Pediatrics, Department of Pediatrics, King Saud bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Riyadh, Saudi Arabia.

Aromatic L-amino acid decarboxylase (AADC) deficiency (MIM #608643) is an autosomal recessive inborn error of monoamines. It is caused by a mutation in the DDC gene that leads to a deficiency in the AADC enzyme. The clinical features of this condition include a combination of dopamine, noradrenaline, and serotonin deficiencies, and a patient may present with hypotonia, oculogyric crises, sweating, hypersalivation, autonomic dysfunction, and progressive encephalopathy with severe developmental delay. Read More

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http://dx.doi.org/10.4137/JCNSD.S12938DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3891626PMC
January 2014
2 Reads

Pharmacotherapy of essential tremor.

J Cent Nerv Syst Dis 2013 Dec 22;5:43-55. Epub 2013 Dec 22.

Department of Neurology, Vanderbilt University, Nashville, TN.

Essential tremor (ET) is a common movement disorder but its pathogenesis remains poorly understood. This has limited the development of effective pharmacotherapy. The current therapeutic armamentaria for ET represent the product of careful clinical observation rather than targeted molecular modeling. Read More

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http://dx.doi.org/10.4137/JCNSD.S6561DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3873223PMC
December 2013
1 Read

Efficacy of retigabine in adjunctive treatment of partial onset seizures in adults.

J Cent Nerv Syst Dis 2013 Oct 23;5:31-41. Epub 2013 Oct 23.

University of Oklahoma Health Sciences Center, College of Pharmacy, Department of Pharmacy: Clinical and Administrative Sciences, Oklahoma City, OK.

Objective: To evaluate efficacy and tolerability of retigabine (ezogabine, US adopted name) in the adjunctive treatment of partial-onset seizures in adults. Retigabine is the first anticonvulsant in its class, decreasing neuronal excitability by opening voltage-gated potassium channels.

Methods: MEDLINE and EMBASE were systematically searched using search terms retigabine and ezogabine for randomized controlled trials published from 1980 through August 17, 2013. Read More

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http://dx.doi.org/10.4137/JCNSD.S9299DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3825677PMC
October 2013
3 Reads

Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy.

J Cent Nerv Syst Dis 2013 4;5:25-30. Epub 2013 Jul 4.

Departments of Neurology and Pediatrics, Robert Wood Johnson Medical School.

Metachromatic leukodystrophy (MLD) is characterized by the accumulation of sulfatide sphingolipids in the brain and peripheral nerves. We report metabolite alterations recorded using multi-voxel proton spectroscopy of the brain in four children with MLD. The data revealed elevated myoinositol/creatine and lactate/creatine ratios as well as decreased N-acetyl aspartate/creatine ratios. Read More

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http://dx.doi.org/10.4137/JCNSD.S11861DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3728201PMC
August 2013
4 Reads

Prefabricated patient-matched cranial implants for reconstruction of large skull defects.

J Cent Nerv Syst Dis 2013 20;5:19-24. Epub 2013 Feb 20.

Department of Neurosurgery, Oslo University Hospital Rikshospitalet, Oslo, Norway.

Cranial defects can be caused by injury, infection, or tumor invasion. Large defects should be reconstructed to protect the brain and normalize the cerebral hemodynamics. The conventional method is to cover the defect with bone cement. Read More

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http://dx.doi.org/10.4137/JCNSD.S11106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616596PMC
May 2013
19 Reads

Pharmacological treatment of attention deficit hyperactivity disorder in children and adolescents: clinical strategies.

J Cent Nerv Syst Dis 2013 20;5:1-17. Epub 2012 Dec 20.

Attention deficit hyperactivity disorder (ADHD) is a common neurobehavioral disorder of childhood that can result in significant functional impairment, and if not adequately treated can lead to impaired quality of life. Pharmacotherapy is considered the first-line treatment for ADHD in children and adolescents. We review both recent literature and seminal studies regarding the pharmacological treatment of ADHD in children and adolescents. Read More

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http://dx.doi.org/10.4137/JCNSD.S6691DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616598PMC
May 2013
9 Reads

Approved Beta interferons in relapsing-remitting multiple sclerosis: is there an odd one out?

Authors:
Alexis Clapin

J Cent Nerv Syst Dis 2012 3;4:135-45. Epub 2012 Sep 3.

Three interferons are marketed for the treatment of relapsing-remitting multiple sclerosis. In its pivotal trial, one of them demonstrated impressive efficacy as a once-weekly regimen, but later head-to-head studies and reviews questioned its superiority. Analysis of this pivotal trial in publications and health authority reviews has shown that its early termination might have caused attrition bias. Read More

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http://dx.doi.org/10.4137/JCNSD.S10150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619495PMC
May 2013
7 Reads

Review of the treatment of restless legs syndrome: focus on gabapentin enacarbil.

J Cent Nerv Syst Dis 2012 17;4:147-56. Epub 2012 Sep 17.

Creighton University School of Pharmacy and Health Professions, Omaha, Nebraska, USA.

The FDA approved gabapentin enacarbil in 2011 as the first non-dopaminergic agent for the treatment of restless legs syndrome (RLS) symptoms. Although gabapentin enacarbil is a pro-drug of gabapentin, its pharmacokinetics differ. Absorption of gabapentin enacarbil is more predictable, and inter-patient variability in bioavailability is lower than that of gabapentin. Read More

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http://dx.doi.org/10.4137/JCNSD.S9107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619699PMC
May 2013
3 Reads

Clinical utility of glatiramer acetate in the management of relapse frequency in multiple sclerosis.

Authors:
Oscar Fernández

J Cent Nerv Syst Dis 2012 29;4:117-33. Epub 2012 Aug 29.

Department of Neurology, Hospital Regional Universitario Carlos Haya, Málaga, Spain.

Glatiramer acetate (GA) represents one of the most common disease-modifying therapies for multiple sclerosis. GA is currently approved for patients at high risk of developing clinically definite multiple sclerosis (CDMS) after having experienced a well-defined first clinical episode (clinically isolated syndrome or CIS) and for patients with relapsing-remitting multiple sclerosis (RRMS). GA's efficacy and effectiveness to reduce relapse frequency have been proved in placebo-controlled and observational studies. Read More

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http://dx.doi.org/10.4137/JCNSD.S8755DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619555PMC
May 2013
1 Read

Pharmacotherapeutic and Non-Pharmacological Options for Refractory and Difficult-to-Treat Seizures.

J Cent Nerv Syst Dis 2012 19;4:105-15. Epub 2012 Jun 19.

Department of Neuropsychiatry, University of Birmingham and BSMHFT, Birmingham, United Kingdom.

It is currently estimated that about 20%-30% of adults and 10%-40% of children diagnosed with epilepsy suffer from uncontrolled or poorly controlled seizures, despite optimal medical management. In addition to its huge economic costs, treatment-refractory epilepsy has a widespread impact on patients' health-related quality of life. The present paper focuses on the concepts of refractory and difficult-to-treat seizures and their pharmacological management. Read More

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http://dx.doi.org/10.4137/JCNSD.S8315DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619658PMC
May 2013
5 Reads

New and Emerging Disease-Modifying Therapies for Relapsing-Remitting Multiple Sclerosis: What is New and What is to Come.

J Cent Nerv Syst Dis 2012 16;4:81-103. Epub 2012 May 16.

The Ohio State University Medical Center, Department of Neurology, Division of Neuro-immunology, Columbus, Ohio.

The therapeutic landscape for multiple sclerosis (MS) is rapidly changing. Currently, there are eight FDA approved disease modifying therapies for MS including: IFN-β-1a (Avonex, Rebif), IFN-β-1b (Betaseron, Extavia), glatiramer acetate (Copaxone), mitoxantrone (Novantrone), natalizumab (Tysabri), and fingolimod (Gilenya). This review will highlight the experience to date and key clinical trials of the newest FDA approved agents, natalizumab and fingolimod. Read More

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http://dx.doi.org/10.4137/JCNSD.S6692DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619700PMC
May 2013
2 Reads

Vitamin k antagonist warfarin for palliative treatment of metachromatic leukodystrophy, a compassionate study of four subjects.

J Cent Nerv Syst Dis 2012 26;4:73-9. Epub 2012 Apr 26.

Robert Wood Johnson Medical School.

MLD is characterized by accumulation of sulfatides in the brain. Vitamin K regulates two enzymes in sphingolipid biosynthesis and warfarin is known to lower brain sulfatides in rats and mice. We hypothesized that warfarin may mitigate the MLD phenotype by reducing the formation of sulfatides. Read More

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http://dx.doi.org/10.4137/JCNSD.S9355DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619661PMC
May 2013
10 Reads

Sentra PM (a Medical Food) and Trazodone in the Management of Sleep Disorders.

J Cent Nerv Syst Dis 2012 23;4:65-72. Epub 2012 Apr 23.

Sleep disorders are a common and poorly treated disease state. This double blind, four arm placebo-controlled, randomized trial compared (1) low dose trazodone, (2) Sentra PM, a neurotransmitter based medical food, (3) the joint administration of trazodone and the medical food Sentra PM and (4) placebo. There were 111 subjects studied in 12 independent sites. Read More

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http://dx.doi.org/10.4137/JCNSD.S9381DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619436PMC
May 2013
9 Reads

The safety and tolerability of newer antiepileptic drugs in children and adolescents.

J Cent Nerv Syst Dis 2012 8;4:51-63. Epub 2012 Mar 8.

Department of Pediatric Neurology, University of Texas South Western Medical Center, Dallas, Texas.

Childhood epilepsy continues to be intractable in more than 25% of patients diagnosed with epilepsy. The introduction of new anti-epileptic drugs (AEDs) provides more options for treatment of children with epilepsy. We review the safety and tolerability of seven new AEDs (levetiracetam, lamotrigine, oxcarbazepine, rufinamide, topiramate, vigabatrin and zonisamide) focusing on their side effect profiles and safety in children and adolescents. Read More

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http://dx.doi.org/10.4137/JCNSD.S5097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3619554PMC
May 2013
3 Reads