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Neurological disorders

Michael J Boivin, Horacio Ruiseñor-Escudero, Itziar Familiar-Lopez
There is growing concern that although the more severe forms of HIV-associated neurologic deficits are reduced following highly active anti-retroviral therapy (HAART), mild to moderate cognitive disorders may persist for years after HAART initiation and this may occur despite complete plasma viral suppression. According to the UNAIDS 2014 report, there were 3.2 million children living with HIV around the world at the end of 2013 and 91 % of these resided in sub-Saharan Africa. In the same year, only 24 % of children who needed antiretroviral treatment (ART) received it and 190,000 children died of AIDS-related illnesses...
October 25, 2016: Current HIV/AIDS Reports
Oscar Marín
There is a growing understanding that pathological genetic variation and environmental insults during sensitive periods in brain development have long-term consequences on brain function, which range from learning disabilities to complex psychiatric disorders such as schizophrenia. Furthermore, recent experiments in animal models suggest that therapeutic interventions during sensitive periods, typically before the onset of clear neurological and behavioral symptoms, might prevent or ameliorate the development of specific pathologies...
October 26, 2016: Nature Medicine
Mohammad A Kaisar, Ravi K Sajja, Shikha Prasad, Vinay V Abhyankar, Taylor Liles, Luca Cucullo
The blood-brain barrier (BBB) is a dynamic biological interface which actively controls the passage of substances between the blood and the central nervous system (CNS). From a biological and functional standpoint, the BBB plays a crucial role in maintaining brain homeostasis inasmuch that deterioration of BBB functions are prodromal to many CNS disorders. Conversely, the BBB hinders the delivery of drugs targeting the brain to treat a variety of neurological diseases. Area covered: This article reviews recent technological improvements and innovation in the field of BBB modeling including static and dynamic cell-based platforms, microfluidic systems and the use of stem cells and 3D printing technologies...
October 26, 2016: Expert Opinion on Drug Discovery
Beomsue Kim, Suihan Feng, Seong-Wook Yun, Cheryl Leong, Rudrakanta Satapathy, Si Yan Diana Wan, Young-Tae Chang
Selection of a specific neural stem/progenitor cells (NSPCs) has attracted broad attention in regenerative medicine for neurological disorders. Here, we report a fluorescent probe, CDg13, and its application for isolating strong neurogenic NSPCs. In comparison to the NSPCs isolated by other biomarkers, CDg13-stained NSPCs showed higher capability to differentiate into neurons. Target identification revealed that the fluorescence intensity of the probe within cells is inversely proportional to the expression levels of mouse and human Abcg2 transporters...
October 26, 2016: Chembiochem: a European Journal of Chemical Biology
Bharat Bhusan Subudhi, Pratap Kumar Sahu
Oxidative stress in brain underlies the major neurological disorders including Alzheimer's disease (AD) and Parkinson's disease (PD). Peripherally, Angiotensin-II is a major effector of inflammation. Identification of its capacity to access brain during hypertension, as well as location of central rennin angiotensin system have led to its recognition as the major effector of oxidative stress in brain. Clinical uses of antioxidants to antagonize this oxidative stress have mostly failed. In this scenario, AT1 blockers have been investigated to prevent neurodegeneration...
October 24, 2016: Mini Reviews in Medicinal Chemistry
Randall C Edgell, Ahmed E Sarhan, Jazba Soomro, Collin Einertson, Joanna Kemp, Peyman Shirani, Theodore K Malmstrom, Jeroen Coppens
BACKGROUND: Central nervous system vasculitis (CNSV) is a rare disorder, the pathophysiology of which is not fully understood. It involves a combination of inflammation and thrombosis. CNSV is most commonly associated with headache, gradual changes in mental status, and focal neurological symptoms. Diagnosis requires the effective use of history, laboratory testing, imaging, and biopsy. Catheter angiography can be a powerful tool in the diagnosis when common and low-frequency angiographic manifestations of CNSV are considered...
September 2016: Interventional Neurology
Elena Parrini, Valerio Conti, William B Dobyns, Renzo Guerrini
Malformations of cortical development (MCD) represent a major cause of developmental disabilities, severe epilepsy, and reproductive disadvantage. Genes that have been associated to MCD are mainly involved in cell proliferation and specification, neuronal migration, and late cortical organization. Lissencephaly-pachygyria-severe band heterotopia are diffuse neuronal migration disorders causing severe global neurological impairment. Abnormalities of the LIS1, DCX, ARX, RELN, VLDLR, ACTB, ACTG1, TUBG1, KIF5C, KIF2A, and CDK5 genes have been associated with these malformations...
September 2016: Molecular Syndromology
Ingo Helbig, Abou Ahmad N Tayoun
Epileptic encephalopathies are severe often intractable seizure disorders where epileptiform abnormalities contribute to a progressive disturbance in brain function. Often, epileptic encephalopathies start in childhood and are accompanied by developmental delay and various neurological and non-neurological comorbidities. In recent years, this concept has become virtually synonymous with a group of severe childhood epilepsies including West syndrome, Lennox-Gastaut syndrome, Dravet syndrome, and several other severe childhood epilepsies for which genetic factors are increasingly recognized...
September 2016: Molecular Syndromology
Shahid Bashir, Woo-Kyoung Yoo
The field of neuromodulation encompasses a wide spectrum of interventional technologies that modify the pathological activity within the nervous system to achieve a therapeutic effect. Therapy, including transcranial direct current stimulation, has shown promising results across a range of neurological and neuropsychiatric disorders. This article reviews the state-of-the-art of neuromodulation for addiction and discusses the opportunities and challenges available for clinicians and researchers interested in advancing the neuromodulation therapy...
October 2016: Annals of Neurosciences
Brit Long, Alex Koyfman
BACKGROUND: Stroke is a leading cause of death and disability and most commonly presents with focal neurologic deficit within a specific vascular distribution. Several other conditions may present in a similar manner. OBJECTIVES: This review provides emergency providers with an understanding of stroke mimics, use of thrombolytics in these mimics, and keys to differentiate true stroke from mimic. DISCUSSION: Stroke has significant morbidity and mortality, and the American Heart Association emphasizes rapid recognition and aggressive treatment for patients with possible stroke-like symptoms, including thrombolytics...
October 22, 2016: Journal of Emergency Medicine
Yoshimichi Hirayama, Yoshiaki Saito, Yoshihiro Maegaki
BACKGROUND: Development of infection-associated acute encephalopathy (AE) is precipitated by several factors, including viral agents, age, and genetic polymorphisms. In addition, children with prior underlying neurological disorders can also present with AE. METHOD: We reviewed 55 children with AE who were referred to hospitals participating in the Status Epilepticus Study Group from 1988 to 2013. AE was classified into eight subtypes: acute encephalopathy with biphasic seizures and late reduced diffusion (AESD); hemiconvulsion-hemiplegia syndrome (HH); acute necrotizing encephalopathy; hemorrhagic shock and encephalopathy syndrome (HSES); clinically mild encephalitis/encephalopathy with a reversible splenial lesion; acute encephalitis with refractory, repetitive partial seizures; Reye-like syndrome; and unclassified...
October 22, 2016: Brain & Development
Fary Khan, Bhasker Amatya, Mary P Galea, Roman Gonzenbach, Jürg Kesselring
The prevalence of disability due to neurological conditions is escalating worldwide. Neurological disorders have significant disability-burden with long-term functional and psychosocial issues, requiring specialized rehabilitation services for comprehensive management, especially treatments tapping into brain recovery 'neuroplastic' processes. Neurorehabilitation is interdisciplinary and cross-sectorial, requiring coordinated effort of diverse sectors, professions, patients and community to manage complex condition-related disability...
October 24, 2016: Journal of Neurology
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
Qian Jiao, Xi-Xun DU, Jun-Xia Xie, Hong Jiang
Neural stem cells (NSCs) offer great promise for the treatment of multiple neurodegenerative diseases. However, the survival and differentiation rates of grafted cells in the host brain need to be enhanced. In this regard, understanding of the underlying mechanism of NSCs survival and death is of great importance for the implications of stem cell-based therapeutic application in the treatments of neurological disorders. Autophagy is a conserved proteolytic mechanism required for maintaining cellular homeostasis, which can affect NSCs fate through regulating their biological behaviors, such as survival and proliferation...
October 25, 2016: Sheng Li Xue Bao: [Acta Physiologica Sinica]
Anna Nolte, Aimara Bello, Michaela Drögemüller, Tosso Leeb, Eva Brockhaus, Wolfgang Baumgärtner, Peter Wohlsein
A female, 5-year-old American Staffordshire Terrier with severe progressive neurological deficits, particularly in terms of ataxia and keeping balance, was examined pathomorphologically and a genetic analysis was performed. In neurons of various localizations of the central nervous system an accumulation of a finely granular pale eosinophilic or light brown material was found. In addition, the cerebellum revealed marked degeneration and loss of Purkinje and inner granule cells. The accumulated PAS-positive, argyrophilic, autofluorescent material showed ultrastructurally a lamellar appearance suggestive of lipofuscin...
October 25, 2016: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
Vellingiri Balachandar, Venkatesan Dhivya, Mohan Gomathi, Subramaniam Mohanadevi, Balasubramanian Venkatesh, Bharathi Geetha
Human induced pluripotent stem cells (hiPSCs) are pluripotent stem cells generated from somatic cells by the introduction of a combination of pluripotency-associated genes such as OCT4, SOX2, along with either KLF4 and c-MYC or NANOG and LIN28 via retroviral or lentiviral vectors. Most importantly, hiPSCs are similar to human embryonic stem cells (hESCs) functionally as they are pluripotent and can potentially differentiate into any desired cell type when provided with the appropriate cues, but do not have the ethical issues surrounding hESCs...
2016: Stem Cell Investigation
Dorota Tassigny, Basel Abu-Serieh, Daniella Tsague Fofe, Jacques Born, Germain Milbouw
BACKGROUND AND IMPORTANCE: Syringomyelia is a progressive cerebrospinal fluid disorder which can lead to irreversible spinal cord injury. To date, the optimal management of syringomyelic cavities remains controversial. Multiple studies have emphasized the importance of the cranio-cervical decompression or shunting procedures. However, excepted for syrinx related to Arnold Chiari malformation, nearly half of patients need to be re-operated. The purpose of the present study was to describe a simple and efficient surgical technique and to report the long-term radio-clinical outcomes...
October 21, 2016: World Neurosurgery
Junmin Zhang, Juan Yao, Shoujiao Peng, Xinming Li, Jianguo Fang
Thioredoxin reductase (TrxR) and thioredoxin (Trx) are two major components of the thioredoxin system, which plays essential roles in regulating cellular redox signaling. Mammalian TrxRs are essential seleno-flavoenzymes with a conserved penultimate selenocysteine (Sec) residue at the C-terminus, and have attracted considerable interests as promising targets for anticancer drugs. Securinine (SCR), a major active alkaloid lactone from the Chinese herbal medicine Securinega suffruticosa, has been established clinical success in treatment of neurological disorders...
October 21, 2016: Biochimica et Biophysica Acta
Mengxuan Tang, Davis C Ryman, Eric McDade, Mateusz S Jasielec, Virginia D Buckles, Nigel J Cairns, Anne M Fagan, Alison Goate, Daniel S Marcus, Chengjie Xiong, Ricardo F Allegri, Jasmeer P Chhatwal, Adrian Danek, Martin R Farlow, Nick C Fox, Bernardino Ghetti, Neill R Graff-Radford, Christopher Laske, Ralph N Martins, Colin L Masters, Richard P Mayeux, John M Ringman, Martin N Rossor, Stephen P Salloway, Peter R Schofield, John C Morris, Randall J Bateman
BACKGROUND: Autosomal dominant familial Alzheimer's disease (ADAD) is a rare disorder with non-amnestic neurological symptoms in some clinical presentations. We aimed to compile and compare data from symptomatic participants in the Dominantly Inherited Alzheimer Network observational study (DIAN-OBS) with those reported in the literature to estimate the prevalences of non-amnestic neurological symptoms in participants with ADAD. METHODS: We prospectively collected data from the DIAN-OBS database, which recruited participants from study centres in the USA, Europe, and Australia, between Feb 29, 2008, and July 1, 2014...
October 21, 2016: Lancet Neurology
H Rigole, N Senal, M Damphousse, C Brochard, A Manunta, J Kerdraon, L Tondut, Q Alimi, J Hascoet, L Siproudhis, B Peyronnet, I Bonan
AIMS: The presence of stools in the rectum might affect the quality of the abdominal pressure curve during filling cystometry, but, to date, no study has evaluated the impact of bowel preparation before urodynamics. We evaluated the influence of a sodium phosphate enema before urodynamics on the quality of the abdominal pressure curve. METHODS: A prospective, controlled, single-blind study was conducted in a single center from May to June 2013. The patients were divided into 2 consecutive groups: patients seen in outpatient clinics during the first 6 weeks (group A) who underwent urodynamics without bowel preparation and patients seen in outpatient clinics during the second 6 weeks (group B) who had a prescription of sodium phosphate enema before urodynamics...
October 21, 2016: Progrès en Urologie
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