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Cerebral small vascular disease

Kazuyuki Shimada
Stroke is known to frequently recur in patients with a history of cerebrovascular disease, and the control of hypertension is extremely important for the treatment of those patients. The robust relationship between the recurrent cerebrovascular disease and blood pressure control has been demonstrated in large-scale clinical studies. The antihypertensive drug therapy significantly reduces the recurrence rate of all types of cerebrovascular disease, incidences of myocardial infarction and all vascular events...
September 2016: Journal of Hypertension
Gregoire Boulouis, Ellis S van Etten, Andreas Charidimou, Eitan Auriel, Andrea Morotti, Marco Pasi, Kellen E Haley, H Bart Brouwers, Alison M Ayres, Anastasia Vashkevich, Michael J Jessel, Kristin M Schwab, Anand Viswanathan, Steven M Greenberg, Jonathan Rosand, Joshua N Goldstein, M Edip Gurol
Importance: Hematoma expansion is an important determinant of outcome in spontaneous intracerebral hemorrhage (ICH) due to small vessel disease (SVD), but the association between the severity of the underlying SVD and the extent of bleeding at the acute phase is unknown to date. Objective: To investigate the association between key magnetic resonance imaging (MRI) markers of SVD (as per the Standards for Reporting Vascular Changes on Neuroimaging [STRIVE] guidelines) and hematoma volume and expansion in patients with lobar or deep ICH...
October 10, 2016: JAMA Neurology
Elisabeth B Marsh, Wendy C Ziai, Rafael H Llinas
INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) typically affects young patients and left untreated can result in hemorrhage or ischemic stroke. Though the disorder has been well characterized in the literature, the most appropriate way to diagnose, treat, and evaluate therapeutic response remains unclear. In previous studies, transcranial Doppler ultrasound (TCD) has shown elevated velocities indicative of vasospasm. This imaging modality is noninvasive and inexpensive; an attractive option for diagnosis and therapeutic monitoring if it is sensitive enough to detect changes in the acute setting given that RCVS often affects the distal vessels early in the course of disease...
May 2016: Case Reports in Neurology
Stewart J Wiseman, Mark E Bastin, Charlotte L Jardine, Gayle Barclay, Iona F Hamilton, Elaine Sandeman, David Hunt, E Nicole Amft, Susan Thomson, Jill F F Belch, Stuart H Ralston, Joanna M Wardlaw
BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE) increases stroke risk, but the mechanism is uncertain. This study aimed to determine the association between SLE and features on neuroimaging of cerebral small vessel disease (SVD), a risk factor for stroke. METHODS: Consecutive patients attending a clinic for SLE were recruited. All patients underwent brain magnetic resonance imaging; had blood samples taken for markers of inflammation, endothelial dysfunction, cholesterol, and autoantibodies; and underwent cognitive and psychiatric testing...
October 4, 2016: Stroke; a Journal of Cerebral Circulation
Dana R Jorgensen, Caterina Rosano, Enrico M Novelli
Adults with homozygous sickle cell anemia have, on average, lower cognitive function than unaffected controls. The mechanisms underlying cognitive deterioration in this population are poorly understood, but cerebral small vessel disease (CSVD) is likely to be implicated. We conducted a systematic review using the Prisma Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines of articles that included both measures of cognitive function and magnetic resonance imaging (MRI) neuroimaging markers of small vessel disease...
September 30, 2016: Hemoglobin
Marianna Bugiani, Sietske H Kevelam, Hannah S Bakels, Quinten Waisfisz, Chantal Ceuterick-de Groote, Hans W M Niessen, Truus E M Abbink, Saskia A M J Lesnik Oberstein, Marjo S van der Knaap
OBJECTIVE: To characterize the clinical and MRI features of 2 families with adult-onset dominant leukoencephalopathy and strokes and identify the underlying genetic cause. METHODS: We applied MRI pattern recognition, whole-exome sequencing, and neuropathology. RESULTS: Based on brain imaging, 13 family members of 40 years or older from 2 families were diagnosed with the disease; in 11 family members of the same age, MRI was normal. In the affected family members, MRI showed a leukoencephalopathy that was disproportionately severe compared to the clinical disease...
September 24, 2016: Neurology
Kazuyuki Shimada
Stroke is known to frequently recur in patients with a history of cerebrovascular disease, and the control of hypertension is extremely important for the treatment of those patients. The robust relationship between the recurrent cerebrovascular disease and blood pressure control has been demonstrated in large-scale clinical studies. The antihypertensive drug therapy significantly reduces the recurrence rate of all types of cerebrovascular disease, incidences of myocardial infarction and all vascular events...
September 2016: Journal of Hypertension
Shinji Ito, Masaki Takao, Toshio Fukutake, Hiroyuki Hatsuta, Sayaka Funabe, Nobuo Ito, Yutaka Shimoe, Toshiro Niki, Imaharu Nakano, Masashi Fukayama, Shigeo Murayama
Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a nonhypertensive hereditary cerebral small vessel disease that is caused by mutations in a single gene, HTRA1 The HTRA1 protein normally represses transforming growth factor-β (TGF-β) signaling and its mutations result in vascular changes. Ten homozygous, 1 compound heterozygous, and 1 homozygous frameshift mutation have been identified in the HTRA1 gene of patients with genetically confirmed CARASIL...
September 15, 2016: Journal of Neuropathology and Experimental Neurology
Yeo Jin Kim, Hee Jin Kim, Jae-Hyun Park, Seonwoo Kim, Sook-Young Woo, Ki-Chang Kwak, Jong Min Lee, Na-Yeon Jung, Jae Seung Kim, Yearn Seong Choe, Kyung-Han Lee, Seung Hwan Moon, Jae-Hong Lee, Yun Joong Kim, David J Werring, Duk L Na, Sang Won Seo
OBJECTIVE: To determine whether amyloid and hypertensive cerebral small vessel disease (hCSVD) changes synergistically affect the progression of lobar microbleeds in patients with subcortical vascular mild cognitive impairment (svMCI). METHODS: Among 72 patients with svMCI who underwent brain MRI and [(11)C] Pittsburgh compound B (PiB)-PET, 52 (72.2%) completed the third year of follow-up. These patients were evaluated by annual neuropsychological testing, brain MRI, and follow-up PiB-PET...
September 14, 2016: Neurology
Rebecca L Brookes, Matthew J Hollocks, Rhea Y Y Tan, Robin G Morris, Hugh S Markus
BACKGROUND AND PURPOSE: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic form of cerebral small vessel disease leading to early-onset stroke and dementia, with younger patients frequently showing subclinical deficits in cognition. At present, there are no targeted cognitive screening measures for this population. However, the Brief Memory and Executive Test (BMET) and the Montreal Cognitive Assessment (MoCA) have shown utility in detecting cognitive impairment in sporadic small vessel disease...
October 2016: Stroke; a Journal of Cerebral Circulation
Nicolae Sarbu, Robert Y Shih, Robert V Jones, Iren Horkayne-Szakaly, Laura Oleaga, James G Smirniotopoulos
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease)...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Anine H Stam, Parul H Kothari, Aisha Shaikh, Andreas Gschwendter, Joanna C Jen, Suzanne Hodgkinson, Todd A Hardy, Michael Hayes, Peter A Kempster, Katya E Kotschet, Ingeborg M Bajema, Sjoerd G van Duinen, Marion L C Maat-Schieman, Paulus T V M de Jong, Marc D de Smet, Didi de Wolff-Rouendaal, Greet Dijkman, Nadine Pelzer, Grant R Kolar, Robert E Schmidt, JoAnne Lacey, Daniel Joseph, David R Fintak, M Gilbert Grand, Elizabeth M Brunt, Helen Liapis, Rula A Hajj-Ali, Mark C Kruit, Mark A van Buchem, Martin Dichgans, Rune R Frants, Arn M J M van den Maagdenberg, Joost Haan, Robert W Baloh, John P Atkinson, Gisela M Terwindt, Michel D Ferrari
Cerebroretinal vasculopathy, hereditary vascular retinopathy, and hereditary endotheliopathy, retinopathy, nephropathy and stroke are neurovascular syndromes initially described as distinct entities. Recently they were shown to be one disease caused by C-terminal frame-shift mutations in TREX1, which was termed 'retinal vasculopathy with cerebral leukodystrophy'. Here we defined the genetic and clinicopathologic spectrum of this clinically and pathophysiologically poorly characterized and frequently misdiagnosed fatal neurovascular disorder...
September 6, 2016: Brain: a Journal of Neurology
Saima Hilal, Emiel Sikking, Muhammad Amin Shaik, Qun Lin Chan, Susanne J van Veluw, Henri Vrooman, Ching-Yu Cheng, Charumathi Sabanayagam, Carol Y Cheung, Tien Yin Wong, Narayanaswamy Venketasubramanian, Geert Jan Biessels, Christopher Chen, Mohammad Kamran Ikram
OBJECTIVE: We examined the risk factors of cortical cerebral microinfarcts (CMIs) on 3T MRI and their association with cognitive impairment. METHODS: Participants (aged 60 years and older) from the multiethnic Epidemiology of Dementia In Singapore Study underwent detailed neuropsychological testing and 3T brain MRI. Cortical CMIs were graded using a previously validated protocol. Cognitive impairment was categorized into cognitive impairment, no dementia (CIND)-mild, CIND-moderate, and dementia...
September 2, 2016: Neurology
Printha Wijesinghe, S K Shankar, T C Yasha, Catherine Gorrie, Dhammika Amaratunga, Sanjayah Hulathduwa, K Sunil Kumara, Kamani Samarasinghe, Yoo-Hun Suh, Harry W M Steinbusch, K Ranil D De Silva
BACKGROUND: Evidence from various consortia on vascular contributions has been inconsistent in determining the etiology of sporadic Alzheimer's disease (AD). OBJECTIVE: To investigate vascular risk factors and cerebrovascular pathologies associated in manifestation of AD-related neuropathological changes of an elderly population. METHODS: Postmortem brain samples from 76 elderly subjects (≥50 years) were used to study genetic polymorphisms, intracranial atherosclerosis of the circle of Willis (IASCW), and microscopic infarcts in deep white matters...
September 2, 2016: Journal of Alzheimer's Disease: JAD
Andrew A Fanous, Andrew J Fabiano
BACKGROUND: Adverse radiation effect (ARE) is one of the complications of stereotactic radiosurgery. Its treatment with conventional medications, such as corticosteroids, vitamin E, and pentoxifylline carries a high risk of failure, with up to 20% of lesions refractory to such medications. In addition, deep lesions and those occurring in patients with significant medical comorbidities may not be suitable for surgical resection. Bevacizumab is an antiangiogenic monoclonal antibody against vascular endothelial growth factor, a known mediator of cerebral edema...
2016: Surgical Neurology International
Emma M Jenkinson, Mathieu P Rodero, Paul R Kasher, Carolina Uggenti, Anthony Oojageer, Laurence C Goosey, Yoann Rose, Christopher J Kershaw, Jill E Urquhart, Simon G Williams, Sanjeev S Bhaskar, James O'Sullivan, Gabriela M Baerlocher, Monika Haubitz, Geraldine Aubert, Kristin W Barañano, Angela J Barnicoat, Roberta Battini, Andrea Berger, Edward M Blair, Janice E Brunstrom-Hernandez, Johannes A Buckard, David M Cassiman, Rosaline Caumes, Duccio M Cordelli, Liesbeth M De Waele, Alexander J Fay, Patrick Ferreira, Nicholas A Fletcher, Alan E Fryer, Himanshu Goel, Cheryl A Hemingway, Marco Henneke, Imelda Hughes, Rosalind J Jefferson, Ram Kumar, Lieven Lagae, Pierre G Landrieu, Charles M Lourenço, Timothy J Malpas, Sarju G Mehta, Imke Metz, Sakkubai Naidu, Katrin Õunap, Axel Panzer, Prab Prabhakar, Gerardine Quaghebeur, Raphael Schiffmann, Elliott H Sherr, Kanaga R Sinnathuray, Calvin Soh, Helen S Stewart, John Stone, Hilde Van Esch, Christine E G Van Mol, Adeline Vanderver, Emma L Wakeling, Andrea Whitney, Graham D Pavitt, Sam Griffiths-Jones, Gillian I Rice, Patrick Revy, Marjo S van der Knaap, John H Livingston, Raymond T O'Keefe, Yanick J Crow
Although ribosomes are ubiquitous and essential for life, recent data indicate that monogenic causes of ribosomal dysfunction can confer a remarkable degree of specificity in terms of human disease phenotype. Box C/D small nucleolar RNAs (snoRNAs) are evolutionarily conserved non-protein-coding RNAs involved in ribosome biogenesis. Here we show that biallelic mutations in the gene SNORD118, encoding the box C/D snoRNA U8, cause the cerebral microangiopathy leukoencephalopathy with calcifications and cysts (LCC), presenting at any age from early childhood to late adulthood...
October 2016: Nature Genetics
Jun Hua, Allison S Brandt, SeungWook Lee, Nicholas I S Blair, Yuankui Wu, Su Lui, Jaymin Patel, Andreia V Faria, Issel Anne L Lim, Paul G Unschuld, James J Pekar, Peter C M van Zijl, Christopher A Ross, Russell L Margolis
Metabolic dysfunction and microvascular abnormality may contribute to the pathogenesis of schizophrenia. Most previous studies of cerebral perfusion in schizophrenia measured total cerebral blood volume (CBV) and cerebral blood flow (CBF) in the brain, which reflect the ensemble signal from the arteriolar, capillary, and venular compartments of the microvasculature. As the arterioles are the most actively regulated blood vessels among these compartments, they may be the most sensitive component of the microvasculature to metabolic disturbances...
August 18, 2016: Schizophrenia Bulletin
Junfeng Liu, Deren Wang, Jie Li, Yao Xiong, Bian Liu, Chenchen Wei, Simiao Wu, Ming Liu
Background and Objective Elevated alkaline phosphatase (ALP) levels are associated with cerebral small vascular diseases, such as silent brain infarction and cerebral white matter hyperintensity, but few prospective data are available for cerebral microbleeds (CMBs). The aim of the study was to investigate associations between serum ALP levels and CMBs in acute ischemic stroke patients with atrial fibrillation and/or rheumatic heart disease. Methods This prospective study involved consecutively recruited acute ischemic stroke patients with atrial fibrillation and/or rheumatic heart disease treated at two large tertiary care hospitals in southwestern China...
August 16, 2016: Current Neurovascular Research
Raffaella Valenti, Alessandra Del Bene, Anna Poggesi, Andrea Ginestroni, Emilia Salvadori, Giovanni Pracucci, Laura Ciolli, Sandro Marini, Serena Nannucci, Marco Pasi, Francesca Pescini, Stefano Diciotti, Giovanni Orlandi, Mirco Cosottini, Alberto Chiti, Mario Mascalchi, Ubaldo Bonuccelli, Domenico Inzitari, Leonardo Pantoni
BACKGROUND AND OBJECTIVES: Cerebral microbleeds (CMBs) are a neuroimaging expression of small vessel disease (SVD). We investigated in a cohort of SVD patients with mild cognitive impairment (MCI): 1) the reliability of the Microbleed Anatomical Rating Scale (MARS); 2) the burden and location of CMBs and their association with cognitive performances, independent of other clinical and neuroimaging features. METHODS: Patients underwent clinical, neuropsychological (4 cognitive domains), and MRI assessments...
September 15, 2016: Journal of the Neurological Sciences
Benjamin-Andreas Berk, Sandra Nagel, K Hering, S Paschke, Karl-Titus Hoffmann, Rolf-Dieter Kortmann, Chiara Gaudino, Clemens Seidel
Brain metastases are major complications of common cancers. Tumor type and proneness to the CNS are thought to define the number and size of brain metastases. It is not known if intrinsic vascular factors can also have an effect. Restricted perfusion due to cerebral small vessel disease is frequent in elderly patients and causes white matter lesions (WML). The aim of this analysis was to evaluate a possible negative effect of WML and patient age on the number and size of brain metastases (BM) of different tumor entities...
August 17, 2016: Journal of Neuro-oncology
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