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White matter AND disease

Gianvincenzo Sparacia, Francesco Agnello, Angelo Gambino, Martina Sciortino, Massimo Midiri
Purpose The aim of this study was to determine the occurrence and distribution of the 'central vein' sign in white matter lesions on susceptibility-weighted magnetic resonance images in patients with multiple sclerosis (MS) and cerebral small vessel disease (CSVD). Materials and methods T2-weighted and fluid-attenuated inversion recovery magnetic resonance images of 19 MS patients and 19 patients affected by CSVD were analysed for the presence and localisation of focal hyperintense white matter lesions. Lesions were subdivided into periventricular or non-periventricular (juxtacortical, subcortical, deep white matter and cerebellar) distributed...
January 1, 2018: Neuroradiology Journal
Anders Wallin, Gustavo C Román, Margaret Esiri, Petronella Kettunen, Johan Svensson, George P Paraskevas, Elisabeth Kapaki
Subcortical small-vessel disease (SSVD) is a disorder well characterized from the clinical, imaging, and neuropathological viewpoints. SSVD is considered the most prevalent ischemic brain disorder, increasing in frequency with age. Vascular risk factors include hypertension, diabetes, hyperlipidemia, elevated homocysteine, and obstructive sleep apnea. Ischemic white matter lesions are the hallmark of SSVD; other pathological lesions include arteriolosclerosis, dilatation of perivascular spaces, venous collagenosis, cerebral amyloid angiopathy, microbleeds, microinfarcts, lacunes, and large infarcts...
2018: Journal of Alzheimer's Disease: JAD
L A Kalashnikova, T S Gulevskaya, L A Dobrynina
Cerebral microangiopathy (small vessels disease) is a cause of diffuse changes of brain tissue (encephalopathy) denoted in Russian literature by the term dyscirculatory encephalopathy (DE). The main cause of microangiopathy leading to encephalopathy is arterial hypertension, less frequently - cerebral amyloid angiopathy and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. The diagnosis of encephalopathy in patients with microangiopathy is based on the combination of clinical manifestations (mainly, cognitive impairment of varying severity and disorders of gait) with the neuroimaging changes (white matter hyperintensity, multiple lacunar infarcts on MRI)...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
G V Ponomarev, T V Lalayan, S A Dambinova, A A Skoromets
The study aimed at assessing the level of glutamate receptors antibodies (Abs) in blood serum and cerebrospinal fluid (CSF) of patients with spinal cord ischemia along with traditional diagnostic approaches. MATERIAL AND METHODS: Forty patients with spinal cord ischemia (10 with spinal stroke and 30 with subacute and chronic course of the disease) were enrolled. After exclusion of some participants, 27 patients continued the study. Comparison groups included 30 patients with ischemic stroke and 30 patients with radiculopathy...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Jan Kassubek, Hans-Peter Müller, Kelly Del Tredici, Michael Hornberger, Matthias L Schroeter, Karsten Müller, Sarah Anderl-Straub, Ingo Uttner, Murray Grossman, Heiko Braak, John R Hodges, Olivier Piguet, Markus Otto, Albert C Ludolph
Objective: Recently, the characteristic longitudinal distribution pattern of the underlying phosphorylated TDP-43 (pTDP-43) pathology in the behavioral variant of frontotemporal dementia (bvFTD) excluding Pick's disease (PiD) across specific brain regions was described. The aim of the present study was to investigate whether in vivo investigations of bvFTD patients by use of diffusion tensor imaging (DTI) were consistent with these proposed patterns of progression. Methods: Sixty-two bvFTD patients and 47 controls underwent DTI in a multicenter study design...
2018: Frontiers in Aging Neuroscience
Mandip S Dhamoon, Ying-Kuen Cheung, Yeseon Moon, Janet DeRosa, Ralph Sacco, Mitchell S V Elkind, Clinton B Wright
BACKGROUND: Cerebral white matter hyperintensities (WMHs) on MRI are common and associated with vascular and functional outcomes. However, the relationship between WMHs and longitudinal trajectories of functional status is not well characterized. We hypothesized that whole brain WMHs are associated with functional decline independently of intervening clinical vascular events and other vascular risk factors. METHODS AND FINDINGS: In the Northern Manhattan Study (NOMAS), a population-based racially/ethnically diverse prospective cohort study, 1,290 stroke-free individuals underwent brain MRI and were followed afterwards for a mean 7...
March 2018: PLoS Medicine
A Ulate-Campos, J Petanas-Argemi, M Rebollo-Polo, C Jou, C Sierra, J Armstrong, M C Fons-Estupina
INTRODUCTION: X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disease. It is due to a mutation in the ABCD1 gene. The loss of functioning of ABCD1 triggers ineffective beta oxidation of very long-chain fatty acids, which gives rise to an accumulation of these fatty acids. The typical alteration revealed in neuroimaging scans in the cerebral form is symmetrical periventricular demyelination with posterior location. CASE REPORT: We report the case of a 10-year-old boy with right spastic hemiparesis and subacute cognitive impairment...
April 1, 2018: Revista de Neurologia
Rocío-Nur Villar-Quiles, Celia Delgado-Suárez, Manuela Jorquera-Moya, Javier Arpa-Gutiérrez, Gloria Ortega-Suero
No abstract text is available yet for this article.
March 20, 2018: Neurology
Hanna Cho, Sang Won Seo, Jae Yong Choi, Hye Sun Lee, Young Hoon Ryu, Myung Sik Lee, Duk L Na, Hee Jin Kim, Chul Hyoung Lyoo
Behavioral variant frontotemporal dementia (bvFTD) is the most common form of frontotemporal dementia, and tau pathology can be found in 40%-50% of bvFTD patients. In this study, we sought to investigate18 F-flortaucipir-binding patterns and their correlates in clinically diagnosed bvFTD patients by comparing with results for Alzheimer's disease (AD) patients. We enrolled 20 bvFTD, 20 AD, and 20 age-matched healthy subjects who underwent neuropsychological tests, magnetic resonance imaging, and tau positron emission tomography scans with18 F-flortaucipir...
February 21, 2018: Neurobiology of Aging
Isaac H Solomon, Kristyn M Spera, Sophia L Ryan, Jeffrey Helgager, Juliana Andrici, Sherif R Zaki, Henrikas Vaitkevicius, Kristoffer E Leon, Michael R Wilson, Joseph L DeRisi, Sophia Koo, Stelios M Smirnakis, Umberto De Girolami
Importance: Powassan virus is a rare but increasingly recognized cause of severe neurological disease. Objective: To highlight the diagnostic challenges and neuropathological findings in a fatal case of Powassan encephalitis caused by deer tick virus (lineage II) in a patient with follicular lymphoma receiving rituximab, with nonspecific anti-GAD65 antibodies, who was initially seen with fever and orchiepididymitis. Design, Setting, and Participants: Comparison of clinical, radiological, histological, and laboratory findings, including immunohistochemistry, real-time polymerase chain reaction, antibody detection, and unbiased sequencing assays, in a single case report (first seen in December 2016) at an academic medical center...
March 19, 2018: JAMA Neurology
Nicola Moscufo, Dorothy B Wakefield, Dominik S Meier, Michele Cavallari, Charles R G Guttmann, William B White, Leslie Wolfson
Mobility impairment in older persons is associated with brain white matter hyperintensities (WMH), a common finding in magnetic resonance images and one established imaging biomarker of small vessel disease. The contribution of possible microstructural abnormalities within normal-appearing white matter (NAWM) to mobility, however, remains unclear. We used diffusion tensor imaging (DTI) measures, i.e. fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD), to assess microstructural changes within supratentorial NAWM and WMH sub-compartments, and to investigate their association with changes in mobility performance, i...
2018: PloS One
Russell Ouellette, Åsa Bergendal, Sara Shams, Juha Martola, Caterina Mainero, Maria Kristoffersen Wiberg, Sten Fredrikson, Tobias Granberg
OBJECTIVE: To investigate the long-term progression of cognitive dysfunction and its neuroanatomical correlates and predictors in multiple sclerosis (MS). METHODS: A cohort of 37 MS patients reflecting five decades of disease duration and all subtypes was followed over 17.5 years. Matched controls were recruited at the last follow-up. Global cognitive functioning was assessed using a principal component cognitive index based on comprehensive neuropsychological testing...
March 6, 2018: Multiple Sclerosis and related Disorders
Timothy M Hughes, Lynne E Wagenknecht, Suzanne Craft, Akiva Mintz, Gerardo Heiss, Priya Palta, Dean Wong, Yun Zhou, David Knopman, Thomas H Mosley, Rebecca F Gottesman
OBJECTIVE: Arterial stiffness has been associated with evidence of cerebral small vessel disease (cSVD) and fibrillar β-amyloid (Aβ) deposition in the brain. These complex relationships have not been examined in racially and cognitively diverse cohorts. METHODS: The Atherosclerosis Risk in Communities (ARIC)-Neurocognitive Study collected detailed cognitive testing for adjudication of dementia and mild cognitive impairment (MCI), brain MRI, and arterial stiffness by pulse wave velocity (PWV, carotid-femoral [cfPWV] and heart-carotid [hcPWV])...
March 16, 2018: Neurology
Asaff Harel, Dylan Sperling, Maria Petracca, Achillefs Ntranos, Ilana Katz-Sand, Stephen Krieger, Fred Lublin, Zichen Wang, Yangbo Liu, Matilde Inglese
OBJECTIVES: The accuracy of 'no evidence of disease activity' (NEDA) in predicting long-term clinical outcome in patients with relapsing remitting multiple sclerosis (RRMS) is unproven, and there is growing evidence that NEDA does not rule out disease worsening. We used diffusion tensor imaging (DTI) to investigate whether ongoing brain microstructural injury occurs in patients with RRMS meeting NEDA criteria. METHODS: We performed a retrospective study to identify patients with RRMS visiting our centre over a 3-month period who had undergone prior longitudinal DTI evaluation at our facility spanning ≥2 years...
March 16, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
Chenhui Mao, Jing Gao, Liri Jin, Bin Peng, Yupu Guo
Neurosyphilis occurs in the late stage of systemic syphilis infection; early diagnosis and treatment are crucial to the prognosis. We review 3 autopsy cases with different subtypes of neurosyphilis, that is cases with meningovascular, general paresis, and a combination of the 2, respectively. We investigated the gross morphology and leptomeninges, vessels, cerebral cortex, white matter, brainstem, cerebellum, olfactory bulb and spinal cord microscopically. We found that meningovascular inflammation exists in both early and late phases of neurosyphilis, not only in the meningovascular subtype...
March 12, 2018: Journal of Neuropathology and Experimental Neurology
Sebhat Erqou, Jane E Clougherty, Oladipupo Olafiranye, Jared W Magnani, Aryan Aiyer, Sheila Tripathy, Ellen Kinnee, Kevin E Kip, Steven E Reis
OBJECTIVE: We aimed to assess racial differences in air pollution exposures to ambient fine particulate matter (particles with median aerodynamic diameter <2.5 µm [PM2.5 ]) and black carbon (BC) and their association with cardiovascular disease (CVD) risk factors, arterial endothelial function, incident CVD events, and all-cause mortality. APPROACH AND RESULTS: Data from the HeartSCORE study (Heart Strategies Concentrating on Risk Evaluation) were used to estimate 1-year average air pollution exposure to PM2...
March 15, 2018: Arteriosclerosis, Thrombosis, and Vascular Biology
Heather T Whittaker, Shenghua Zhu, Domenico L Di Curzio, Richard Buist, Xin-Min Li, Suzanna Noy, Frances K Wiseman, Jonathan D Thiessen, Melanie Martin
Alzheimer's disease (AD) pathology causes microstructural changes in the brain. These changes, if quantified with magnetic resonance imaging (MRI), could be studied for use as an early biomarker for AD. The aim of our study was to determine if T1 relaxation, diffusion tensor imaging (DTI), and quantitative magnetization transfer imaging (qMTI) metrics could reveal changes within the hippocampus and surrounding white matter structures in ex vivo transgenic mouse brains overexpressing human amyloid precursor protein with the Swedish mutation...
March 12, 2018: Magnetic Resonance Imaging
Sarah Caughlin, Shikhar Maheshwari, Yuksel Agca, Cansu Agca, Aaron J Harris, Kristina Jurcic, Ken K-C Yeung, David F Cechetto, Shawn N Whitehead
BACKGROUND: Accumulation of simple gangliosides GM2 and GM3, and gangliosides with longer long-chain bases (d20:1) have been linked to toxicity and the pathogenesis of Alzheimer's disease (AD). Conversely, complex gangliosides, such as GM1, have been shown to be neuroprotective. Recent evidence using matrix-assisted laser desorption ionization imaging mass spectrometry (MALDI-IMS) has demonstrated that a-series gangliosides are differentially altered during normal aging, yet it remains unclear how simple species are shifting relative to complex gangliosides in the prodromal stages of AD...
March 12, 2018: Biochimica et Biophysica Acta
Celeste Sassi, Michael A Nalls, Perry G Ridge, Jesse R Gibbs, Michelle K Lupton, Claire Troakes, Katie Lunnon, Safa Al-Sarraj, Kristelle S Brown, Christopher Medway, Jenny Lord, James Turton, Jose Bras, Sonja Blumenau, Mareike Thielke, Christa Josties, Dorette Freyer, Annette Dietrich, Monia Hammer, Michael Baier, Ulrich Dirnagl, Kevin Morgan, John F Powell, John S Kauwe, Carlos Cruchaga, Alison M Goate, Andrew B Singleton, Rita Guerreiro, Angela Hodges, John Hardy
Mendelian adult-onset leukodystrophies are a spectrum of rare inherited progressive neurodegenerative disorders affecting the white matter of the central nervous system. Among these, cerebral autosomal dominant and recessive arteriopathy with subcortical infarcts and leukoencephalopathy, cerebroretinal vasculopathy, metachromatic leukodystrophy, hereditary diffuse leukoencephalopathy with spheroids, and vanishing white matter disease present with rapidly progressive dementia as dominant feature and are caused by mutations in NOTCH3, HTRA1, TREX1, ARSA, CSF1R, EIF2B1, EIF2B2, EIF2B3, EIF2B4, and EIF2B5, respectively...
February 2, 2018: Neurobiology of Aging
G Fenu, L Lorefice, L Loi, V Sechi, F Contu, G Coghe, J Frau, G Spinicci, M A Barracciu, M G Marrosu, E Cocco
BACKGROUND: Paediatric onset multiple sclerosis (POMS) is associated with reduced brain and deep grey matter volume in comparison with that in healthy controls and individuals with adult onset multiple sclerosis (AOMS). The aim of our study was to evaluate the impact of POMS on adult brain volume with adjustment for other parameters, such as disease duration. PATIENTS AND METHODS: We recruited 20 POMS and 40 AOMS patients and 20 healthy controls matched for age and sex...
March 6, 2018: Multiple Sclerosis and related Disorders
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