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https://www.readbyqxmd.com/read/27913587/suppressing-n-acetyl-l-aspartate-naa-synthesis-prevents-loss-of-neurons-in-a-murine-model-of-canavan-leukodystrophy
#1
Jiho Sohn, Peter Bannerman, Fuzheng Guo, Travis Burns, Laird Miers, Christopher Croteau, Naveen K Singhal, Jennifer A McDonough, David Pleasure
Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves N-acetyl-L-aspartate (NAA) to acetate and L-aspartic acid, elevates brain NAA and causes "spongiform" vacuolation of superficial brain white matter and neighboring gray matter. In children with Canavan disease, neuroimaging shows early-onset dysmyelination and progressive brain atrophy. Neuron loss has been documented at autopsy in some cases. Prior studies have shown that mice homozygous for the Aspa nonsense mutation Nur7 also develop brain vacuolation...
December 2, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27913156/the-oral-spelling-profile-of-posterior-cortical-atrophy-and-the-nature-of-the-graphemic-representation
#2
Silvia Primativo, Keir X X Yong, Timothy J Shakespeare, Sebastian J Crutch
Spelling is a complex cognitive task where central and peripheral components are involved in engaging resources from many different cognitive processes. The present paper aims to both characterize the oral spelling deficit in a population of patients affected by a neurodegenerative condition and to clarify the nature of the graphemic representation within the currently available spelling models. Indeed, the nature of graphemic representation as a linear or multi-componential structure is still debated. Different hypotheses have been raised about its nature in the orthographic lexicon, with one positing that graphemes are complex objects whereby quantity and identity are separately represented in orthographic representations and can thus be selectively impaired...
November 29, 2016: Neuropsychologia
https://www.readbyqxmd.com/read/27911306/cortical-thickness-and-microstructural-white-matter-changes-detect-amnestic-mild-cognitive-impairment
#3
Zan Wang, Zhengjia Dai, Hao Shu, Duan Liu, Qihao Guo, Yong He, Zhijun Zhang
Both the apolipoprotein E (APOE) ɛ4 allele and amnestic mild cognitive impairment (aMCI) are considered to be risk factors for Alzheimer's disease (AD). The primary aim of this study was to determine whether the aMCI-related abnormality in gray matter (GM) cortical thickness and white matter (WM) tracts integrity would be modified by the APOE genotype. A total of 146 older adults, including 64 aMCI patients (28 ɛ4 carriers and 36 non-carriers) and 82 healthy controls (39 ɛ4 carriers and 43 non-carriers), underwent a standardized clinical interview, neuropsychological battery assessment, and multi-modal brain magnetic resonance imaging scans...
November 29, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27898404/prolonged-fasting-improves-endothelial-progenitor-cell-mediated-ischemic-angiogenesis-in-mice
#4
Bao Xin, Chun-Long Liu, Hong Yang, Cheng Peng, Xiao-Hui Dong, Chuan Zhang, Alex F Chen, He-Hui Xie
BACKGROUND/AIMS: Prolonged fasting (PF) was shown to be of great potency to promote optimal health and reduce the risk of many chronic diseases. This study sought to determine the effect of PF on the endothelial progenitor cell (EPC)-mediated angiogenesis in the ischemic brain and cerebral ischemic injury in mice. METHODS: Mice were subjected to PF or periodic PF after cerebral ischemia, and histological analysis and behavioral tests were performed. Mouse EPCs were isolated and examined, and the effects of EPC transplantation on cerebral ischemic injury were investigated in mice...
November 30, 2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27896492/hemianopic-line-bisection-error-in-a-patient-with-alzheimer-s-disease
#5
Heeyoung Kang, Soo-Kyoung Kim, Ki-Jong Park, Nack-Cheon Choi, Oh-Young Kwon, Byeong Hoon Lim
Visual symptoms are frequently observed in posterior cortical atrophy compared to typical amnestic Alzheimer's disease (AD). We report the case of a patient with amnestic AD with long-lasting homonymous hemianopia. A 62-year-old woman, who was diagnosed in amnestic AD, complained of visual disturbance. She had left homonymous hemianopia in the perimetry test and left tactile extinction. Regardless of posture, retinotopically leftward error was observed on 21 cm line bisection test. Brain single photon emission computerized tomography (SPECT) showed bilateral temporoparietal hypoperfusion, which was more severe to the right than left side...
November 28, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27893883/use-of-magnetic-resonance-imaging-to-visualize-leptomeningeal-inflammation-in-patients-with-multiple-sclerosis-a-review
#6
Jonathan Zurawski, Hans Lassmann, Rohit Bakshi
Importance: Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system traditionally characterized by an initial relapsing-remitting clinical course and focal inflammatory lesions that have a predilection for the periventricular white matter. Recently, however, histopathologic and imaging studies have illustrated a more complex pathologic substrate involving cortical demyelination, gray matter atrophy, and meningeal inflammation. Neuroimaging advances have facilitated improved detection of cortical pathology, but our understanding of the pathogenesis of cortical disease remains incomplete...
November 28, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27893017/evaluation-of-cognitive-deficits-and-structural-hippocampal-damage-in-encephalitis-with-leucine-rich-glioma-inactivated-1-antibodies
#7
Carsten Finke, Harald Prüss, Josephine Heine, Sigrid Reuter, Ute A Kopp, Florian Wegner, Florian Then Bergh, Sebastian Koch, Olav Jansen, Thomas Münte, Günther Deuschl, Klemens Ruprecht, Winfried Stöcker, Klaus-Peter Wandinger, Friedemann Paul, Thorsten Bartsch
Importance: Limbic encephalitis with leucine-rich, glioma-inactivated 1 (LGI1) antibodies is one of the most frequent variants of autoimmune encephalitis with antibodies targeting neuronal surface antigens. However, the neuroimaging pattern and long-term cognitive outcome are not well understood. Objective: To study cognitive outcome and structural magnetic resonance imaging (MRI) alterations in patients with anti-LGI1 encephalitis. Design, Setting, and Participants: A cross-sectional study was conducted at the Departments of Neurology at Charité-Universitätsmedizin Berlin and University Hospital Schleswig-Holstein, Kiel, Germany...
November 21, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27891071/chronic-progressive-neurodegeneration-in-a-transgenic-mouse-model-of-prion-disease
#8
Nina Fainstein, Dvir Dori, Kati Frid, Alexa T Fritz, Ilona Shapiro, Ruth Gabizon, Tamir Ben-Hur
Neurodegenerative diseases present pathologically with progressive structural destruction of neurons and accumulation of mis-folded proteins specific for each condition leading to brain atrophy and functional disability. Many animal models exert deposition of pathogenic proteins without an accompanying neurodegeneration pattern. The lack of a comprehensive model hinders efforts to develop treatment. We performed longitudinal quantification of cellular, neuronal and synaptic density, as well as of neurogenesis in brains of mice mimicking for genetic Creutzfeldt-Jacob disease as compared to age-matched wild-type mice...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27884179/factors-related-to-renal-cortical-atrophy-development-after-glucocorticoid-therapy-in-igg4-related-kidney-disease-a-retrospective-multicenter-study
#9
Ichiro Mizushima, Motohisa Yamamoto, Dai Inoue, Shinichi Nishi, Yoshinori Taniguchi, Yoshifumi Ubara, Shoko Matsui, Tetsuhiko Yasuno, Hitoshi Nakashima, Hiroki Takahashi, Kazunori Yamada, Hideki Nomura, Masakazu Yamagishi, Takao Saito, Mitsuhiro Kawano
BACKGROUND: In immunoglobulin G4-related kidney disease (IgG4-RKD), focal or diffuse renal cortical atrophy is often observed in the clinical course after glucocorticoid therapy. This study aimed to clarify the factors related to renal atrophy after glucocorticoid therapy in IgG4-RKD. METHODS: We retrospectively evaluated clinical features including laboratory data and computed tomography (CT) findings before and after glucocorticoid therapy in 23 patients diagnosed with IgG4-RKD, all of whom were followed up for more than 24 months...
November 25, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27884107/association-between-changes-in-cerebral-grey-matter-volume-and-postoperative-cognitive-dysfunction-in-elderly-patients-study-protocol-for-a-prospective-observational-cohort-study
#10
Nicolai Goettel, Panagiota Mistridis, Manfred Berres, Julia Reinhardt, Christoph Stippich, Andreas U Monsch, Luzius A Steiner
BACKGROUND: Cognitive decline is frequently observed in elderly patients after major surgery. The pathophysiology of postoperative cognitive dysfunction (POCD) remains unclear. The aim of our investigation is to identify potential associations between brain volume change and POCD in elderly patients undergoing major surgery. METHODS: This is a prospective observational cohort study approved by the regional ethics board. We intend to compare specific brain volumes (hippocampus, lateral ventricle, total grey matter volume, regional cortical thickness) on magnetic resonance imaging and cognitive functions determined by a neuropsychological assessment battery in 70 study participants aged ≥65 years before and 3 and 12 months after major noncardiac surgery...
November 25, 2016: BMC Anesthesiology
https://www.readbyqxmd.com/read/27878614/associations-of-intracranial-pressure-with-brain-biopsy-radiological-findings-and-shunt-surgery-outcome-in-patients-with-suspected-idiopathic-normal-pressure-hydrocephalus
#11
Maria Kojoukhova, Krista-Irina Vanha, Matti Timonen, Anne M Koivisto, Ossi Nerg, Jaana Rummukainen, Tuomas Rauramaa, Ritva Vanninen, Juha E Jääskeläinen, Anna Sutela, Ville Leinonen
BACKGROUND: It remains unclear how intracranial pressure (ICP) measures are associated with brain biopsies and radiological markers. Here, we aim to investigate associations between ICP and radiological findings, brain biopsies, and shunt surgery outcome in patients with suspected idiopathic normal pressure hydrocephalus (iNPH). METHOD: In this study, we retrospectively analyzed data from 73 patients admitted with suspected iNPH to Kuopio University Hospital. Of these patients, 71% underwent shunt surgery...
November 23, 2016: Acta Neurochirurgica
https://www.readbyqxmd.com/read/27876089/rapid-onset-obesity-hypoventilation-hypothalamic-dysfunction-autonomic-dysregulation-and-neuroendocrine-tumor-syndrome-with-a-homogenous-enlargement-of-the-pituitary-gland-a-case-report
#12
Lama Aljabban, Lina Kassab, Nour Alhuda Bakoura, Mohammad Fayez Alsalka, Ismaeil Maksoud
BACKGROUND: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome is a rare pediatric disorder with a variable sequence of clinical presentations, undefined etiology, and high risk of mortality. Our patient presented an unusual course of the disease accompanied by a homogenous mild enlargement of her pituitary gland with an intact pituitary-endocrine axis which, to the best of our knowledge, represents a new finding in rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome...
November 22, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27875715/common-and-divergent-neural-correlates-of-anomia-in-amnestic-and-logopenic-presentations-of-alzheimer-s-disease
#13
Cristian E Leyton, John R Hodges, Olivier Piguet, Kirrie J Ballard
The majority of logopenic variant primary progressive aphasia (lv-PPA) cases harbour Alzheimer pathology, suggesting that lv-PPA constitutes an atypical presentation of Alzheimer's disease (AD). However, even if caused by Alzheimer pathology, the clinical manifestations of lv-PPA differ from those observed in the typical or amnestic AD presentation: in lv-PPA, aphasia is the main feature while amnestic AD is characterised by impaired episodic memory. Anomia or impaired naming, however, is present in both AD presentations...
November 5, 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27875637/ambra1-a-novel-%C3%AE-synuclein-binding-protein-is-implicated-in-the-pathogenesis-of-multiple-system-atrophy
#14
Yasuo Miki, Kunikazu Tanji, Fumiaki Mori, Yota Tatara, Jun Utsumi, Hidenao Sasaki, Akiyoshi Kakita, Hitoshi Takahashi, Gian Maria Fimia, Koichi Wakabayashi
The accumulation of abnormal α-synuclein is the major histopathological feature of Lewy body disease and multiple system atrophy (MSA), which are referred to as synucleinopathies. Although the pathomechanisms underlying synucleinopathies are still unknown, mounting evidence indicates that cytoplasmic degradation systems, such as the autophagy-lysosome and proteasome pathways, are strongly involved in their pathogenesis. Autophagy is tightly regulated by several upstream proteins including UNC-51-like kinase 1/2, beclin1, vacuolar protein sorting-associated protein 34, and autophagy/beclin1 regulator 1 (AMBRA1)...
November 22, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27864109/selective-cytotoxicity-of-microcystins-lr-lw-and-lf-in-rat-astrocytes
#15
Klara Bulc Rozman, Damijana Mojca Jurič, Dušan Šuput
Microcystins (MCs) comprise a group of cyanobacterial toxins with hepatotoxic, nephrotoxic and, possibly, neurotoxic activity in mammals. In order to understand the development of their neurotoxicity we investigated the toxic effects of MC variants, MC-LR, MC-LW and MC-LF, in astrocytes that play a central role in maintaining brain homeostasis. 24h exposure of cultured rat cortical astrocytes to MCs revealed dose-dependent toxicity of MC-LF and MC-LW, but not of MC-LR, observed by significant reduction in cell number, declined viability monitored by MTT test and an increased percentage of apoptotic cells, confirmed by Annexin-V labelling...
November 15, 2016: Toxicology Letters
https://www.readbyqxmd.com/read/27862549/diffusion-tensor-imaging-defined-sulcal-enlargement-is-related-to-cognitive-impairment-in-multiple-sclerosis
#16
Zafer Keser, Khader M Hasan, Benson Mwangi, Refaat E Gabr, Flavia M Nelson
BACKGROUND AND PURPOSE: Cerebrospinal fluid (CSF) in the brain can be compartmentalized into two main divisions: ventricular CSF and subarachnoid space (sulcal CSF). Changes in CSF volumetry are seen in many neurological conditions including multiple sclerosis (MS) and found to correlate with clinical outcomes. We aimed to test the relation between the volumetry of sulcal and ventricular CSF and cognitive impairment (CI) based on the minimal assessment of cognitive function in MS (MACFIMS) in patients with MS...
November 7, 2016: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/27859539/clinicopathologic-heterogeneity-in-ftdp-17-due-to-mapt-p-p301l-mutation-including-a-patient-with-globular-glial-tauopathy
#17
Pawel Tacik, Monica Sanchez-Contreras, Michael DeTure, Melissa E Murray, Rosa Rademakers, Owen A Ross, Zbigniew K Wszolek, Joseph E Parisi, David S Knopman, Ronald C Petersen, Dennis W Dickson
AIM: The p.P301L mutation in microtubule-associated protein tau (MAPT) is a common cause of frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17). We compare clinicopathologic features of five unrelated and three related (brother, sister and cousin) patients with FTDP-17 due to p.P301L mutation. METHODS: Genealogical, clinical, neuropathologic, and genetic data were reviewed from eight individuals RESULTS: The series consisted of five men and three women with an average age of death of 58 years (52-65 years) and average disease duration of 9 years (3-14 years)...
November 8, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27857918/effectiveness-of-cortical-atrophy-scale-and-indirect-indices-of-brain-atrophy-to-predict-chronic-subdural-hematoma-in-older-patients
#18
Eun-Oh Jeong, Seung-Won Choi, Jeong-Wook Lim, Hyon-Jo Kwon, Seon-Hwan Kim, Hyeon-Song Koh, Jin-Young Youm, Shi-Hun Song
OBJECTIVE: To determine whether baseline cerebral atrophy can predict the rate of future chronic subdural hematoma (CSDH) after head trauma and compare indirect markers of brain atrophy with volumetric analysis of computed tomography (CT). METHODS: Single institution case-control study involving 1,476 patients who visited our hospital after head trauma from January 2009 to December 2014. Forty-one patients with delayed CSDH were identified and age, gender matched 41 patients were selected as control group...
October 2016: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/27853938/differential-pattern-of-cerebellar-atrophy-in-tremor-predominant-and-akinetic-rigidity-predominant-parkinson-s-disease
#19
Camila Callegari Piccinin, Lidiane Soares Campos, Rachel Paes Guimarães, Luiza Gonzaga Piovesana, Maria Cristina Arci Dos Santos, Paula Christina Azevedo, Brunno Machado Campos, Thiago Junqueira Ribeiro de Rezende, Augusto Amato-Filho, Fernando Cendes, Anelyssa D'Abreu
Parkinson's disease (PD) is an akinetic-rigid disorder characterized by basal ganglia dysfunction and a possible cerebello-thalamo-cortical circuit involvement. This study aims to investigate the pattern of cerebellar involvement in PD and to assess whether it correlates with clinical parameters. MRI scans were acquired from 50 healthy controls (HC) and 63 patients; 44 were classified as tremor-predominant-PD (PDT) and 19 as akinetic/rigidity-predominant-PD (PDAR). We designed an analysis of covariance including the three groups and contrasted as follows: (1) all 63 PD vs HC, (2) PDT vs HC, (3) PDAR vs HC, and (4) PDT vs PDAR...
November 16, 2016: Cerebellum
https://www.readbyqxmd.com/read/27843694/grey-matter-abnormalities-in-methcathinone-abusers-with-a-parkinsonian-syndrome
#20
Julius Juurmaa, Ricarda A L Menke, Pierre Vila, Andreas Müürsepp, Tiiu Tomberg, Pilvi Ilves, Mait Nigul, Heidi Johansen-Berg, Michael Donaghy, Charlotte J Stagg, Ainārs Stepens, Pille Taba
BACKGROUND: A permanent Parkinsonian syndrome occurs in intravenous abusers of the designer psychostimulant methcathinone (ephedrone). It is attributed to deposition of contaminant manganese, as reflected by characteristic globus pallidus hyperintensity on T1-weighted MRI. METHODS: We have investigated brain structure and function in methcathinone abusers (n = 12) compared to matched control subjects (n = 12) using T1-weighted structural and resting-state functional MRI...
November 2016: Brain and Behavior
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