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Subcortical white matter abnormalities of the brain

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https://www.readbyqxmd.com/read/28414663/not-another-acs-rule-out
#1
S Preston, R Nelson, M Watts, D Smith, T Dewenter, D Spruill
CASE: A 50 year old African-American woman with diabetes, hypertension, and hyperlipidemia presented with progressively worsening retro-sternal chest pain, exacerbated by activity and relieved by rest. She also endorsed a thirty-pound unintentional weight loss, and dysphagia. She was dysarthric with left-sided Bell's Palsy and a palpable left axillary lymph node. She had been evaluated at several hospitals in the previous months for similar typical chest pain. Her troponin values were normal, and an EKG showed T-wave inversions in leads I and aVL...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28413125/transient-dysautonomia-in-an-acute-phase-of-encephalopathy-with-biphasic-seizures-and-late-reduced-diffusion
#2
Yuko Ichimiya, Noriyuki Kaku, Yasunari Sakai, Fumiya Yamashita, Wakato Matsuoka, Mamoru Muraoka, Satoshi Akamine, Soichi Mizuguchi, Michiko Torio, Yoshitomo Motomura, Yuichiro Hirata, Yoshito Ishizaki, Masafumi Sanefuji, Hiroyuki Torisu, Hidetoshi Takada, Yoshihiko Maehara, Shouichi Ohga
Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection...
April 13, 2017: Brain & Development
https://www.readbyqxmd.com/read/28324197/the-spectrum-of-magnetic-resonance-findings-in-cerebrotendinous-xanthomatosis-redefinition-and-evidence-of-new-markers-of-disease-progression
#3
Andrea Mignarri, Maria Teresa Dotti, Antonio Federico, Nicola De Stefano, Marco Battaglini, Irene Grazzini, Paolo Galluzzi, Lucia Monti
Cerebrotendinous xanthomatosis (CTX) is a metabolic disease characterized by systemic signs and neurological impairment, which can be prevented if chenodeoxycholic acid (CDCA) treatment is started early. Despite brain MRI represents an essential diagnostic tool, the spectrum of findings is worth to be reappraised, and follow-up data are needed. We performed clinical evaluation and brain MRI in 38 CTX patients. Sixteen of them who were untreated at baseline examination underwent clinical and MRI follow-up after long-term treatment with CDCA...
March 21, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28222523/anomalous-white-matter-structure-and-the-effect-of-age-in-down-syndrome-patients
#4
Raquel Fenoll, Jesus Pujol, Susanna Esteba-Castillo, Susana de Sola, Núria Ribas-Vidal, Javier García-Alba, Gonzalo Sánchez-Benavides, Gerard Martínez-Vilavella, Joan Deus, Mara Dierssen, Ramón Novell-Alsina, Rafael de la Torre
BACKGROUND: Neural tissue alterations in Down syndrome are fully expressed at relatively late developmental stages. In addition, there is an early presence of neurodegenerative changes in the late life stages. OBJECTIVE: The aims of this study were both to characterize white matter abnormalities in the brain of adult Down syndrome patients using diffusion tensor imaging (DTI) and to investigate whether degenerative alterations in white matter structure are detectable before dementia is clinically evident...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28159909/organization-of-the-anterior-limb-of-the-internal-capsule-in-the-rat
#5
Veronique Coizet, Sarah R Heilbronner, Carole Carcenac, Philippe Mailly, Julia F Lehman, Marc Savasta, Oivier David, Jean-Michel Deniau, Henk J Groenewegen, Suzanne N Haber
Dysfunction of the orbitofrontal (OFC) and anterior cingulate (ACC) cortices has been linked with several psychiatric disorders, including obsessive-compulsive disorder, major depressive disorder, posttraumatic stress disorder, and addiction. These conditions are also associated with abnormalities in the anterior limb of the internal capsule, the white matter (WM) bundle carrying ascending and descending fibers from the OFC and ACC. Furthermore, deep-brain stimulation (DBS) for psychiatric disorders targets these fibers...
March 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28132975/a-case-of-neuromyelitis-optica-spectrum-disorder-nmosd-with-sj%C3%A3-gren-s-syndrome-manifested-only-brain-involvement-by-preceding-parotitis
#6
Takahiro Furukawa, Naoko Matsui, Keiko Tanaka, Yuishin Izumi, Ryuji Kaji
A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Immunoserological study showed that she was positive for anti-SS-A, aquaporin 4 (AQP4), and AQP5 antibodies. She clinically showed not only Sjögren's syndrome but also neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis or myelitis...
February 25, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28105707/binge-drinking-differentially-affects-cortical-and-subcortical-microstructure
#7
Laurel S Morris, Nicholas G Dowell, Mara Cercignani, Neil A Harrison, Valerie Voon
Young adult binge drinkers represent a model for endophenotypic risk factors for alcohol misuse and early exposure to repeated binge cycles. Chronic or harmful alcohol use leads to neurochemical, structural and morphological neuroplastic changes, particularly in regions associated with reward processing and motivation. We investigated neural microstructure in 28 binge drinkers compared with 38 matched healthy controls. We used a recently developed diffusion magnetic resonance imaging acquisition and analysis, which uses three-compartment modelling (of intracellular, extracellular and cerebrospinal fluid) to determine brain tissue microstructure features including neurite density and orientation dispersion index (ODI)...
January 20, 2017: Addiction Biology
https://www.readbyqxmd.com/read/28059798/sporadic-cases-with-novel-mutations-and-pedigree-in-hereditary-leukoencephalopathy-with-axonal-spheroids
#8
Liyong Wu, Jia Liu, Longze Sha, Xianling Wang, Jieying Li, Jing Dong, Jianping Jia
Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal dominant hereditary disease, featured by cerebral white matter degeneration with demyelination and axonal spheroids. We collected three gene-confirmed HDLS cases in our neurodegenerative clinic. Two HDLS cases were sporadic with novel mutations, while another case had a family history with previously described mutations. All three cases suffered memory problems with white matter lesions and pyramid signs. No obvious clinical differences were observed between sporadic and familial HDLS cases...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28046822/su-f-i-17-white-matter-track-based-analysis-at-limbic-abnormalities-in-cognitive-impairment
#9
R Juh, T Suh, J Han, C Kim, C Oh
PURPOSE: Mild cognitive impairment with depression (MCID) is common and associated with disability and cognitive impairment, with high probability of relapse. Hypothesize that a sign of WM disintegration would be observed in MCID than MCI nondepression (MCIND), especially in frontal and limbic regions and patients with depression would show reduced GM density in the hippocampus, amygdala, anterior gyrus cingulate, and dorsolateral prefrontal cortex (DLPFC) and dorsomedial prefrontal cortex (DMPFC) the abnormalities of long association fiber tracts integrity are correlated with geriatric depression...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28019685/tauopathy-with-hippocampal-4-repeat-tau-immunoreactive-spherical-inclusions-a-report-of-three-cases
#10
Gabor G Kovacs, Linda K Kwong, Murray Grossman, David J Irwin, Edward B Lee, John L Robinson, Eunran Suh, Vivianna M Van Deerlin, Virginia M Lee, John Q Trojanowski
Tauopathies are a major group of neurodegenerative proteinopathies characterized by the accumulation of abnormal and hyperphosphorylated tau proteins in the brain. Tau pathology is characterized as 3R (repeat) or 4R predominant or mixed 3R and 4R type. Here we report three cases lacking mutations in the microtubule associated protein tau (MAPT) gene with unusual tau pathology. The age at onset and duration of illness, respectively, were 63 and 20 years (male), 67 and 5 years (female) and 72 and 20 years (female)...
December 26, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27942447/altered-structural-and-functional-thalamocortical-networks-in-secondarily-generalized-extratemporal-lobe-seizures
#11
Syu-Jyun Peng, Yue-Loong Hsin
Structural and functional abnormalities in the thalamocortical network in primary generalized epilepsies or mesial temporal lobe epilepsy have recently been identified by voxel-wise analyses of neuroimaging. However, evidence is needed regarding the profiles of the thalamocortical network in patients with secondarily generalized seizures from focal neocortical sources. We used high-resolution T1-weighted, diffusion-tensor and resting-state functional MR imaging (rs-fMRI) to examine 16 patients with secondarily generalized extratemporal lobe seizures and 16 healthy controls...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27913960/neuroimaging-abnormalities-in-clade-c-hiv-are-independent-of-tat-genetic-diversity
#12
Robert H Paul, Sarah Phillips, Jacqueline Hoare, David H Laidlaw, Ryan Cabeen, Gayla R Olbricht, Yuqing Su, Dan J Stein, Susan Engelbrecht, Soraya Seedat, Lauren E Salminen, Laurie M Baker, Jodi Heaps, John Joska
Controversy remains regarding the neurotoxicity of clade C human immunodeficiency virus (HIV-C). When examined in preclinical studies, a cysteine to serine substitution in the C31 dicysteine motif of the HIV-C Tat protein (C31S) results in less severe brain injury compared to other viral clades. By contrast, patient cohort studies identify significant neuropsychological impairment among HIV-C individuals independent of Tat variability. The present study clarified this discrepancy by examining neuroimaging markers of brain integrity among HIV-C individuals with and without the Tat substitution...
April 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/27899912/specific-mri-abnormalities-reveal-severe-perrault-syndrome-due-to-clpp-defects
#13
Tom E J Theunissen, Radek Szklarczyk, Mike Gerards, Debby M E I Hellebrekers, Elvira N M Mulder-Den Hartog, Jo Vanoevelen, Rick Kamps, Bart de Koning, S Lane Rutledge, Thomas Schmitt-Mechelke, Carola G M van Berkel, Marjo S van der Knaap, Irenaeus F M de Coo, Hubert J M Smeets
In establishing a genetic diagnosis in heterogeneous neurological disease, clinical characterization and whole exome sequencing (WES) go hand-in-hand. Clinical data are essential, not only to guide WES variant selection and define the clinical severity of a genetic defect but also to identify other patients with defects in the same gene. In an infant patient with sensorineural hearing loss, psychomotor retardation, and epilepsy, WES resulted in identification of a novel homozygous CLPP frameshift mutation (c...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/27862544/brain-morphology-and-cortical-thickness-variations-in-systemic-lupus-erythematosus-patients-differences-among-neurological-psychiatric-and-nonneuropsychiatric-manifestations
#14
Nicolle Zimmermann, Diogo Goulart Corrêa, Gustavo Tukamoto, Tania Netto, Denis Batista Pereira, Rochele Paz Fonseca, Emerson Leandro Gasparetto
PURPOSE: To determine whether systemic lupus erythematosus (SLE) affecting subcortical white matter volumes, deep gray matter volumes, and cortical thickness differ between groups of SLE patients with psychiatric (P-SLE), neurological (N-SLE), or nonneuropsychiatric (non-NPSLE) presentations. MATERIALS AND METHODS: Sixty-seven participants were divided into three groups (P-SLE [n = 19], N-SLE [n = 12], and non-NPSLE [n = 36]) and examined with a 1.5T MRI scanner...
November 8, 2016: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/27829078/sex-specific-alterations-of-white-matter-developmental-trajectories-in-infants-with-prenatal-exposure-to-methamphetamine-and-tobacco
#15
Linda Chang, Kenichi Oishi, Jon Skranes, Steven Buchthal, Eric Cunningham, Robyn Yamakawa, Sara Hayama, Caroline S Jiang, Daniel Alicata, Antonette Hernandez, Christine Cloak, Tricia Wright, Thomas Ernst
Importance: Methamphetamine is a common illicit drug used worldwide. Methamphetamine and/or tobacco use by pregnant women remains prevalent. However, little is known about the effect of comorbid methamphetamine and tobacco use on human fetal brain development. Objective: To investigate whether microstructural brain abnormalities reported in children with prenatal methamphetamine and/or tobacco exposure are present at birth before childhood environmental influences...
December 1, 2016: JAMA Psychiatry
https://www.readbyqxmd.com/read/27815774/the-topological-organization-of-white-matter-network-in-internet-gaming-disorder-individuals
#16
Jinquan Zhai, Lin Luo, Lijun Qiu, Yongqiang Kang, Bo Liu, Dahua Yu, Xiaoqi Lu, Kai Yuan
White matter (WM) integrity abnormalities had been reported in Internet gaming disorder (IGD). Diffusion tensor imaging (DTI) tractography allows identification of WM tracts, potentially providing information about the integrity and organization of relevant underlying WM fiber tracts' architectures, which has been used to investigate the connectivity of cortical and subcortical structures in several brain disorders. Unfortunately, relatively little is known about the thoroughly circuit-level characterization of topological property changes of WM network with IGD...
November 4, 2016: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/27807409/structural-magnetic-resonance-imaging-can-identify-trigeminal-system-abnormalities-in-classical-trigeminal-neuralgia
#17
REVIEW
Danielle D DeSouza, Mojgan Hodaie, Karen D Davis
Classical trigeminal neuralgia (TN) is a chronic pain disorder that has been described as one of the most severe pains one can suffer. The most prevalent theory of TN etiology is that the trigeminal nerve is compressed at the root entry zone (REZ) by blood vessels. However, there is significant evidence showing a lack of neurovascular compression (NVC) for many cases of classical TN. Furthermore, a considerable number of patients who are asymptomatic have MR evidence of NVC. Since there is no validated animal model that reproduces the clinical features of TN, our understanding of TN pathology mainly comes from biopsy studies that have limitations...
2016: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/27798653/progression-of-regional-microstructural-degeneration-in-parkinson-s-disease-a-multicenter-diffusion-tensor-imaging-study
#18
Yu Zhang, I-Wei Wu, Duygu Tosun, Eric Foster, Norbert Schuff
This study aimed to identify the utility of diffusion tensor imaging (DTI) in measuring the regional distribution of abnormal microstructural progression in patients with Parkinson's disease who were enrolled in the Parkinson's progression marker initiative (PPMI). One hundred and twenty two de-novo PD patients (age = 60.5±9) and 50 healthy controls (age = 60.6±11) had DTI scans at baseline and 12.6±1 months later. Automated image processing included an intra-subject registration of all time points and an inter-subjects registration to a brain atlas...
2016: PloS One
https://www.readbyqxmd.com/read/27785698/association-between-falls-and-brain-subvolumes-results-from-a-cross-sectional-analysis-in-healthy-older-adults
#19
Olivier Beauchet, Cyrille P Launay, John Barden, Teresa Liu-Ambrose, Victoria L Chester, Tony Szturm, Sébastien Grenier, Guillaume Léonard, Louis Bherer, Cédric Annweiler, Jorunn L Helbostad, Joe Verghese, Gilles Allali
Falls are a consequence of gait instability. Cortical and subcortical abnormalities have been associated with gait instability but not yet with falls. This study aims to compare the global and regional brain subvolumes between healthy older fallers and non-fallers. A total of 77 healthy older individuals (23 fallers and 54 non-fallers, 69.8 ± 3.5 years; 45.5 % female) were included in this study using a cross-sectional design. Based on an a priori hypothesis, the following brain subvolumes were quantified from three-dimensional T1-weighted MRI using FreeSurfer software: total white matter abnormalities, total white matter, total cortical and subcortical gray matter, hippocampus, motor cortex, somatosensory cortex, premotor cortex, prefrontal cortex and parietal cortex volumes...
October 26, 2016: Brain Topography
https://www.readbyqxmd.com/read/27748972/astrocyte-mediated-infantile-onset-leukoencephalopathy-mouse-model
#20
Shouta Sugio, Koujiro Tohyama, Shinichiro Oku, Kanehiro Fujiyoshi, Takeshi Yoshimura, Keigo Hikishima, Ryutaro Yano, Takahiro Fukuda, Masaya Nakamura, Hideyuki Okano, Masahiko Watanabe, Masaki Fukata, Kazuhiro Ikenaka, Kenji F Tanaka
Astrocytes have recently been shown to provide physiological support for various brain functions, although little is known about their involvement in white matter integrity. Several inherited infantile-onset leukoencephalopathies, such as Alexander disease and megalencephalic leukoencephalopathy with subcortical cysts (MLC), implicate astrocytic involvement in the formation of white matter. Several mouse models of MLC had been generated by knocking out the Mlc1 gene; however, none of those models was reported to show myelin abnormalities prior to formation of the myelin sheath...
October 17, 2016: Glia
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