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Neurological disorders

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https://www.readbyqxmd.com/read/29351245/exposure-to-ambient-air-particles-increases-the-risk-of-mental-disorder-findings-from-a-natural-experiment-in-beijing
#1
Zhen Jia, Yongjie Wei, Xiaoqian Li, Lixin Yang, Huijie Liu, Chen Guo, Lulu Zhang, Nannan Li, Shaojuan Guo, Yan Qian, Zhigang Li
Epidemiology studies indicated that air pollution has been associated with adverse neurological effects in human. Moreover, the secretion of glucocorticoid (GC) affects the mood regulation, and the negative feedback of hippocampal glucocorticoid receptors (GR) inhibits the GC secretion. Meanwhile, the over secretion of GC can interfere the immune system and induce neurotoxicity. In the present study, the human test showed that the secretion of the cortisol in plasma was elevated after exposure in heavy air pollution...
January 19, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29350350/biochemical-changes-and-clinical-outcomes-in-34-patients-with-classic-galactosemia
#2
Tatiana Yuzyuk, Krista Viau, Ashley Andrews, Marzia Pasquali, Nicola Longo
Impaired activity of galactose-1-phosphate uridyltransferase (GALT) causes galactosemia, an autosomal recessive disorder of galactose metabolism. Early initiation of a galactose-restricted diet can prevent or resolve neonatal complications. Despite therapy, patients often experience long-term complications including speech impairment, learning disabilities, and premature ovarian insufficiency in females. This study evaluates clinical outcomes in 34 galactosemia patients with markedly reduced GALT activity and compares outcomes between patients with different levels of mean galactose-1-phosphate in red blood cells (GAL1P) using logistic regression: group 1 (n = 13) GAL1P ≤1...
January 19, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29349556/neuropathology-correlates-of-cognitive-assessments
#3
Francesca M Brett, Hugh Kearney
BACKGROUND: Alzheimer's disease and other dementias are the fourth largest contributors to neurological disability and the second largest contributor to deaths from neurological disease. Described in the 1980s as 'the silent epidemic' these disorders principally, though not exclusively, affect persons 80 years or older, and in developed countries, this 'old old' population continues to grow. Definitive diagnosis of the underlying cause of the neurodegenerative disease relies on neuropathological evaluation...
January 18, 2018: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/29348136/resting-state-functional-mri-everything-that-nonexperts-have-always-wanted-to-know
#4
REVIEW
H Lv, Z Wang, E Tong, L M Williams, G Zaharchuk, M Zeineh, A N Goldstein-Piekarski, T M Ball, C Liao, M Wintermark
Resting-state fMRI was first described by Biswal et al in 1995 and has since then been widely used in both healthy subjects and patients with various neurologic, neurosurgical, and psychiatric disorders. As opposed to paradigm- or task-based functional MR imaging, resting-state fMRI does not require subjects to perform any specific task. The low-frequency oscillations of the resting-state fMRI signal have been shown to relate to the spontaneous neural activity. There are many ways to analyze resting-state fMRI data...
January 18, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29346777/prrt2-regulates-synaptic-fusion-by-directly-modulating-snare-complex-assembly
#5
Jeff Coleman, Ouardane Jouannot, Sathish K Ramakrishnan, Maria N Zanetti, Jing Wang, Vincenzo Salpietro, Henry Houlden, James E Rothman, Shyam S Krishnakumar
Mutations in proline-rich transmembrane protein 2 (PRRT2) are associated with a range of paroxysmal neurological disorders. PRRT2 predominantly localizes to the pre-synaptic terminals and is believed to regulate neurotransmitter release. However, the mechanism of action is unclear. Here, we use reconstituted single vesicle and bulk fusion assays, combined with live cell imaging of single exocytotic events in PC12 cells and biophysical analysis, to delineate the physiological role of PRRT2. We report that PRRT2 selectively blocks the trans SNARE complex assembly and thus negatively regulates synaptic vesicle priming...
January 16, 2018: Cell Reports
https://www.readbyqxmd.com/read/29346658/potential-for-diet-to-prevent-and-remediate-cognitive-deficits-in-neurological-disorders
#6
Heather M Francis, Richard J Stevenson
The pathophysiology of many neurological disorders involves oxidative stress, neuroinflammation, and mitochondrial dysfunction. There is now substantial evidence that diet can decrease these forms of pathophysiology, and an emerging body of literature relatedly suggests that diet can also prevent or even remediate the cognitive deficits observed in neurological disorders that exhibit such pathology (eg, Alzheimer's disease, multiple sclerosis, age-related cognitive decline, epilepsy). The current review summarizes the emerging evidence in relation to whole diets prominent in the scientific literature-ketogenic, caloric restriction, high polyphenol, and Mediterranean diets-and provides a discussion of the possible underlying neurophysiological mechanisms...
January 15, 2018: Nutrition Reviews
https://www.readbyqxmd.com/read/29346380/rapidly-progressive-dementia-an-eight-year-2008-2016-retrospective-study
#7
Patil Anuja, Vishnu Venugopalan, Naheed Darakhshan, Pandit Awadh, Vinny Wilson, Goyal Manoj, Modi Manish, Lal Vivek
BACKGROUND AND PURPOSE: Rapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation. METHODS: Retrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016...
2018: PloS One
https://www.readbyqxmd.com/read/29346110/a-three-dimensional-arrayed-microfluidic-blood-brain-barrier-model-with-integrated-electrical-sensor-array
#8
Sehoon Jeong, Sunja Kim, John Buonocore, Jaewon Park, C Jane Welsh, Jianrong Li, Arum Han
OBJECTIVE: The blood-brain barrier (BBB) poses a unique challenge to the development of therapeutics against neurological disorders due to its impermeabi-lity to most of the chemical compounds. Most in vitro BBB models have limitations in mimicking in vivo conditions and functions. Here, we show a co-culture microfluidic BBB-on-a-chip that provides interactions between neurovascular endothelial cells and neuronal cells across a porous polycarbonate membrane, which better mimics the in vivo conditions, as well as allows in vivo level shear stress to be applied...
February 2018: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/29345437/antioxidant-and-acetylcholinesterase-inhibitory-activities-of-ginger-root-zingiber-officinale-roscoe-extract
#9
Bui Thanh Tung, Dang Kim Thu, Nguyen Thi Kim Thu, Nguyen Thanh Hai
Background Zingiber officinale Roscoe has been used in traditional medicine for the treatment of neurological disorder. This study aimed to investigate the phenolic contents, antioxidant, acetylcholinesterase enzyme (AChE) inhibitory activities of different fraction of Z. officinale root grown in Vietnam. Methods The roots of Z. officinale are extracted with ethanol 96 % and fractionated with n-hexane, ethyl acetate (EtOAc) and butanol (BuOH) solvents. These fractions evaluated the antioxidant activity by 1,1-Diphenyl -2-picrylhydrazyl (DPPH) assay and AChE inhibitory activity by Ellman's colorimetric method...
May 4, 2017: Journal of Complementary & Integrative Medicine
https://www.readbyqxmd.com/read/29344929/pla2g6-deficiency-in-zebrafish-leads-to-dopaminergic-cell-death-axonal-degeneration-increased-%C3%AE-synuclein-expression-and-defects-in-brain-functions-and-pathways
#10
Elena Sánchez, Luis J Azcona, Coro Paisán-Ruiz
This study aimed to gain insights into the pathophysiology underlying PLA2G6-associated neurodegeneration that is implicated in three different neurological disorders, suggesting that other, unknown genetic or environmental factors might contribute to its wide phenotypic expression. To accomplish this, we downregulated the function of pla2g6 in the zebrafish nervous system, performed parkinsonism-related phenotypic characterization, and determined the effects of gene regulation upon the loss of pla2g6 function by using RNA sequencing and downstream analyses...
January 17, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29344584/early-and-late-outcomes-after-cord-blood-transplantation-for-pediatric-patients-with-inherited-leukodystrophies
#11
Brigitte T A van den Broek, Kristin Page, Annalisa Paviglianiti, Janna Hol, Heather Allewelt, Fernanda Volt, Gerard Michel, Miguel Angel Diaz, Victoria Bordon, Tracey O'Brien, Peter J Shaw, Chantal Kenzey, Amal Al-Seraihy, Peter M van Hasselt, Andrew R Gennery, Eliane Gluckman, Vanderson Rocha, Annalisa Ruggeri, Joanne Kurtzberg, Jaap Jan Boelens
Leukodystrophies (LD) are devastating inherited disorders leading to rapid neurological deterioration and premature death. Hematopoietic stem cell transplantation (HSCT) can halt disease progression for selected LD. Cord blood is a common donor source for transplantation of these patients because it is rapidly available and can be used without full HLA matching. However, precise recommendations allowing care providers to identify patients who benefit from HSCT are lacking. In this study, we define risk factors and describe the early and late outcomes of 169 patients with globoid cell leukodystrophy, X-linked adrenoleukodystrophy, and metachromatic leukodystrophy undergoing cord blood transplantation (CBT) at an European Society for Blood and Marrow Transplantation center or at Duke University Medical Center from 1996 to 2013...
January 9, 2018: Blood Advances
https://www.readbyqxmd.com/read/29344472/status-epilepticus-as-an-unusual-manifestation-of-heat-stroke
#12
Won Gu Lee, So-Young Huh, Jin-Hyung Lee, Bong Goo Yoo, Meyung Kug Kim
Heat stroke (HS) is a medical emergency and life threatening condition, characterized by body temperature over 40°C. This can lead to dysfunction of multiple organs such as the heart, liver, kidneys, lungs, blood coagulation system, and central nervous system. Neurological complications include change in consciousness, cerebellar dysfunction, convulsions, aphasia, muscular weakness, and parkinsonism. Cerebellar syndrome is the most common neurological finding in HS. We report a case of HS presenting with status epilepticus, without any other neurological manifestations...
December 2017: Journal of Epilepsy Research
https://www.readbyqxmd.com/read/29344071/acetazolamide-a-new-treatment-for-visual-vertigo
#13
Ilya M Sluch, Michael S Elliott, Justin Dvorak, Kai Ding, Bradley K Farris
Visual vertigo is a disorder characterised by symptoms of dizziness, vertigo, unsteadiness, disorientation, and general discomfort induced by visual triggers. It is currently treated with vestibular rehabilitation therapy, with no effective pharmacotherapy available for treatment-resistant cases. The objective of this study was to evaluate the efficacy of oral acetazolamide in improving symptoms of visual vertigo. A comparative case series of adult patients clinically diagnosed with visual vertigo was conducted from January 1992 to May 2015...
December 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/29343288/changes-in-the-secretome-of-tri-dimensional-spheroid-cultured-human-mesenchymal-stem-cells-in-vitro-by-interleukin-1-priming
#14
Elena Redondo-Castro, Catriona J Cunningham, Jonjo Miller, Helena Brown, Stuart M Allan, Emmanuel Pinteaux
BACKGROUND: Mesenchymal stem cells (MSCs) are one of the most promising candidates for the treatment of major neurological disorders. Desirable therapeutic properties of MSCs include reparative and regenerative potential but, despite their proven safety, the efficacy of MSCs remains controversial. Therefore, it is essential to optimise culture protocols to enhance the therapeutic potential of the MSC secretome. Here we aimed to: assess the increase in secretion of cytokines that may induce repair, regeneration, or immunomodulation when cultured in three dimensions; study the effect of interleukin (IL)-1 priming on two- (2D) and three-dimensional (3D) cultures of MSC; and evaluate the potential use of the modified secretome using microglial-MSC co-cultures...
January 17, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29342881/development-of-self-assembled-nanoribbon-bound-peptide-polyaniline-composite-scaffolds-and-their-interactions-with-neural-cortical-cells
#15
Andrew M Smith, Harrison T Pajovich, Ipsita A Banerjee
Degenerative neurological disorders and traumatic brain injuries cause significant damage to quality of life and often impact survival. As a result, novel treatments are necessary that can allow for the regeneration of neural tissue. In this work, a new biomimetic scaffold was designed with potential for applications in neural tissue regeneration. To develop the scaffold, we first prepared a new bolaamphiphile that was capable of undergoing self-assembly into nanoribbons at pH 7. Those nanoribbons were then utilized as templates for conjugation with specific proteins known to play a critical role in neural tissue growth...
January 13, 2018: Bioengineering
https://www.readbyqxmd.com/read/29342116/vegf-signaling-in-neurological-disorders
#16
REVIEW
Joon W Shim, Joseph R Madsen
Vascular endothelial growth factor (VEGF) is a potent growth factor playing diverse roles in vasculogenesis and angiogenesis. In the brain, VEGF mediates angiogenesis, neural migration and neuroprotection. As a permeability factor, excessive VEGF disrupts intracellular barriers, increases leakage of the choroid plexus endothelia, evokes edema, and activates the inflammatory pathway. Recently, we discovered that a heparin binding epidermal growth factor like growth factor (HB-EGF)-a class of EGF receptor (EGFR) family ligands-contributes to the development of hydrocephalus with subarachnoid hemorrhage through activation of VEGF signaling...
January 17, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29341839/activation-of-frataxin-protein-expression-by-antisense-oligonucleotides-targeting-the-mutant-expanded-repeat
#17
Liande Li, Xiulong Shen, Zhongtian Liu, Michaela Norrbom, Thazha P Prakash, Daniel O'Reilly, Vivek K Sharma, Masad J Damha, Jonathan K Watts, Frank Rigo, David R Corey
Friedreich's Ataxia (FA) is an inherited neurologic disorder caused by an expanded GAA repeat within intron 1 of the frataxin (FXN) gene that reduces expression of FXN protein. Agents that increase expression of FXN have the potential to alleviate the disease. We previously reported that duplex RNAs (dsRNAs) and antisense oligonucleotides (ASOs) complementary to the GAA repeat could enhance expression of FXN protein. We now explore the potential of a diverse group of chemically modified dsRNAs and ASOs to define the breadth of repeat-targeted synthetic nucleic acids as a platform for therapeutic development for FA...
January 17, 2018: Nucleic Acid Therapeutics
https://www.readbyqxmd.com/read/29340960/higher-levels-of-progranulin-in-cerebrospinal-fluid-of-patients-with-lymphoma-and-carcinoma-with-cns-metastasis
#18
Akio Kimura, Masao Takemura, Ginette Serrero, Nobuaki Yoshikura, Yuichi Hayashi, Kuniaki Saito, Takashi Inuzuka
Assessing central nervous system (CNS) involvement in patients with lymphoma or carcinoma is important in determining therapy and prognosis. Progranulin (PGRN) is a secreted glycosylated protein with roles in cancer growth and survival; it is highly expressed in aggressive cancer cell lines and specimens from many cancer types. We examined PRGN levels by Enzyme Immuno-Assay (EIA) in cerebrospinal fluid (CSF) samples from 230 patients, including 18 with lymphoma [12 with CNS metastasis (CNS+); 6 without CNS metastasis (CNS-)], 21 with carcinomas (10 CNS+; 11 CNS-), and 191 control patients with non-cancer neurological diseases, and compared PRGN levels among these disease groups...
January 16, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29340766/wanted-a%C3%A2-better-cut-off-value-for-the-epworth-sleepiness-scale
#19
Karin Trimmel, Magdalena Żebrowska, Marion Böck, Andrijana Stefanic, Daniel Mayer, Gerhard Klösch, Eduard Auff, Stefan Seidel
BACKGROUND: Excessive daytime sleepiness (EDS) is the main complaint in many neurological sleep disorders, such as idiopathic hypersomnia, narcolepsy, or obstructive sleep apnea/hypopnea syndrome (OSAS). The validity of the Epworth Sleepiness Scale (ESS) as a screening tool for EDS remains controversial. We therefore investigated (1) the interrelation of the ESS total score and the mean sleep latency (MSL) during the multiple sleep latency test (MSLT) and (2) the diagnostic accuracy of the ESS total score to detect EDS in patients with the chief complaint of subjective EDS...
January 16, 2018: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/29340742/-wilson-disease
#20
D Huster
Wilson disease is a rare hereditary disorder of copper metabolism. The genetic defect is caused by various mutations in the copper-transporting enzyme ATP7B, located mainly in the liver and brain. Clinical symptoms are highly variable, with any combination of hepatic and/or neurological or psychiatric manifestations. The age of onset varies from early childhood to young adults and can even be manifested in later ages. The clinical diagnosis is based on a combination of clinical, biochemical and molecular markers...
January 16, 2018: Der Internist
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