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Cerebral vasculopathy

Sven Haller, Meike W Vernooij, Joost P A Kuijer, Elna-Marie Larsson, Hans Rolf Jäger, Frederik Barkhof
Cerebral microbleeds (CMBs), also referred to as microhemorrhages, appear on magnetic resonance (MR) images as hypointense foci notably at T2*-weighted or susceptibility-weighted (SW) imaging. CMBs are detected with increasing frequency because of the more widespread use of high magnetic field strength and of newer dedicated MR imaging techniques such as three-dimensional gradient-echo T2*-weighted and SW imaging. The imaging appearance of CMBs is mainly because of changes in local magnetic susceptibility and reflects the pathologic iron accumulation, most often in perivascular macrophages, because of vasculopathy...
April 2018: Radiology
David E Mandelbaum, Amanda Arsenault, Barbara S Stonestreet, Stefan Kostadinov, Suzanne M de la Monte
A pregnant woman with new-onset type 1 diabetes and ketoacidosis delivered an infant at 28 weeks of gestation who died with multiple organ failure and severe cerebral vasculopathy with extensive hemorrhage, diffuse microgliosis, and edema. This illustrates that antenatal metabolic and inflammatory stressors may be associated with neonatal encephalopathy and cerebral hemorrhage.
March 16, 2018: Journal of Pediatrics
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
Alexander Pschibul, Wibke G Janzarik, Peter Franck, Markus Hufnagel, Christopher Beck, Rudolf Korinthenberg
Despite the availability of modern antibiotics, pneumococcal meningitis in both children and adults remains a severe disease-one known to frequently cause grave complications and residual disability. Although the appearance of arterial vasospasms in bacterial meningitis systematically has been investigated and reported on for adult patients, such research is lacking when it comes to infants. We report on a 4-week-old infant who, 6 days after onset of pneumococcal meningitis, suffered severe neurological deterioration with treatment-resistant seizures and coma...
March 9, 2018: Neuropediatrics
Nobuo Yamashiro, Takamura Nagasaka, Naoki Ooishi, Mai Tsuchiya, Ryusuke Takaki, Fumikazu Kobayashi, Kazumasa Shindo, Yoshihisa Takiyama
We report here the clinical presentation and subsequent autopsy of a 90-year-old man who developed small papules with pain and swelling in his right ear. On admission, he exhibited right facial nerve paralysis, neck stiffness and Kernig's sign. The cell count was elevated and the varicella-zoster virus-PCR was positive in the CSF. Brain magnetic resonance imaging showed hyperintense lesions in the left pons and left temporal lobe, in FLAIR images. We diagnosed the patient with Ramsay Hunt syndrome and meningoencephalitis due to varicella-zoster virus...
March 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
Brice Ongali, Nektaria Nicolakakis, Xin-Kang Tong, Clotilde Lecrux, Hans Imboden, Edith Eh Hamel
Transgenic mice constitutively overexpressing the cytokine transforming growth factor-β1 (TGF-β1) (TGF mice) display cerebrovascular alterations as seen in Alzheimer's disease (AD) and vascular cognitive impairment and dementia (VCID), but no or only subtle cognitive deficits. TGF-β1 may exert part of its deleterious effects through interactions with angiotensin II (AngII) type 1 receptor (AT1R) signaling pathways. We test such interactions in the brain and cerebral vessels of TGF mice by measuring cerebrovascular reactivity, levels of protein markers of vascular fibrosis, nitric oxide synthase activity, astrogliosis and mnemonic performance in mice treated (6 months) with the AT1R blocker, losartan (10 mg/kg/day), or the angiotensin converting enzyme inhibitor enalapril (ACEi,3 mg/kg/day)...
March 5, 2018: Canadian Journal of Physiology and Pharmacology
Satoshi Namitome, Seigo Shindo, Kuniyasu Wada, Tadashi Terasaki, Makoto Nakajima, Yukio Ando
A 14-year-old girl developed transient disturbance of consciousness, dysarthria, and clumsiness of the right upper limb 4 months after herpes zoster ophthalmicus. Brain MRI showed acute cerebral infarction in the left middle cerebral artery (MCA) territory. CT angiography demonstrated mild stenosis in the top of the left internal carotid artery and the proximal side of the MCA. Cerebrospinal fluid (CSF) examination showed slightly mononuclear pleocytosis (6/μl). Titer of the anti-varicella zoster virus (VZV) IgG antibodies in CSF was increased, and gadolinium-enhanced brain MRI (T1 -weighted imaging) revealed enhancement of the vessel walls at the stenotic lesions...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
Yang Liu, Yan-Hong Dong, Pei-Yuan Lyu, Wei-Hong Chen, Rui Li
Objective: Alzheimer's disease and vascular dementia are responsible for more than 80% of dementia cases. These two conditions share common risk factors including hypertension. Cerebral small vessel disease (CSVD) is strongly associated with both hypertension and cognitive impairment. In this review, we identify the pathophysiological changes in CSVD that are caused by hypertension and further explore the relationship between CSVD and cognitive impairment. Data Sources: We searched and scanned the PubMed database for recently published literatures up to December 2017...
March 5, 2018: Chinese Medical Journal
Huan T Nguyen, Satoru Noguchi, Kazuma Sugie, Yoshiyuki Matsuo, Chuyen T H Nguyen, Hitoshi Koito, Ichiro Shiojima, Ichizo Nishino, Hiroyasu Tsukaguchi
Lysosomal associated membrane protein 2 (LAMP2) is physiologically implicated in autophagy. A genetic LAMP2 defect causes Danon disease, which consists of two major phenotypes of myopathy and cardiomyopathy. In addition, arteriopathy may manifest on rare occasions but the pathological basis remains unknown. We encountered two Danon families that developed small-vessel vasculopathy in the coronary or cerebral arteries. To investigate the underlying mechanisms, we characterized the biological features of LAMP-2-deficient mice and cultured cells...
February 20, 2018: Scientific Reports
Ahmed Abdel Khalek Abdel Razek
The aim of this work is to review the MR imaging of neoplastic and non-neoplastic lesions of the brain and spine in neurofibromatosis type I. Neoplastic lesions are optic pathway gliomas, brain stem gliomas, other gliomas of the brain, and peripheral nerve sheath tumors. Structural changes in the brain include unidentified bright objects, macrocephaly, and enlarged corpus callosum. Bony dysplasia changes as sphenoid ridge dysplasia, spinal scalloping, dural ectasia, and meningoceles. Vasculopathy and cortical cerebral and cerebellar malformations of the brain have been reported...
February 17, 2018: Neurological Sciences
Steven D Brooks, Stan Hileman, Paul D Chantler, Samantha Milde, Kent C Lemaster, Stephanie Frisbee, J Kevin Shoemaker, Dwayne N Jackson, Jefferson C Frisbee
The increasing prevalence and severity of clinical depression is strongly correlated with vascular disease risk, creating a comorbid condition with poor outcomes, although demonstrating a sexual disparity where females are at lower risk than males for subsequent cardiovascular events. To determine potential mechanisms responsible for this protection against stress/depression-induced vasculopathy in females, we exposed male and female ({plus minus}ovariectomy) rats to the unpredictable chronic mild stress (UCMS) model for 8 weeks and determined depressive symptom severity, vascular reactivity in aortic rings (AR) and ex vivo middle cerebral arteries (MCA), and determined the profile of major metabolites regulating vascular tone...
February 16, 2018: American Journal of Physiology. Heart and Circulatory Physiology
Mohamed Shehabeldin, Doungporn Ruthirago, Yazan J Alderazi
No abstract text is available yet for this article.
December 2017: Journal of Vascular and Interventional Neurology
Yi-Yi Xuan, Tian-Fang Li, Lei Zhang, Sheng-Yun Liu
RATIOINALE: Relapsing polychondritis (RP) is a rare and heterogeneous disease complex of unknown origin which basically affects cartilaginous structures, 40% of which accompanied by rheumatic, hematologic, and endocrine disease. Among them, vasculitis is the most common accompanying type and usually presented with positive antineutrophilic cytoplasmic antibody (ANCA). The presence of ANCA could be primary or drug-induced like propylthiouracil (PTU). Central involvement of RP is very rare, and there is almost no report of cerebral vasculopathy manifested as moyamoya...
December 2017: Medicine (Baltimore)
Ryouhei Komaki, Takehiro Ueda, Yukio Tsuji, Toko Miyawaki, Sentaro Kusuhara, Shigeo Hara, Tatsushi Toda
A 40-year-old woman with renal dysfunction for 2 years was admitted to our hospital suffering from a headache. Family history revealed that her mother had a headache, renal dysfunction, and brain infarction in younger age. She had a retinal hemorrhage, a retinal atrophy, pitting edema in her lower extremities. Her neurological findings were unremarkable. Brain imaging showed multiple white matter lesions accompanied with calcifications and slightly enhancement. Kidney biopsy showed the thrombotic microangiopathy, Gene analysis demonstrated a causative mutation in three-prime repair exonuclease-1 (TREX1) gene, c...
January 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
Candice Fabre, Barthélémy Tosello, Estelle Pipon, Catherine Gire, Kathia Chaumoitre
BACKGROUND: Lenticulostriate vasculopathy (LSV) is a hyperechogenicity of the lenticulostriate branches of the basal ganglia and/or thalamus' middle cerebral arteries and is frequently seen in neonatology. Our study primarily describes the perinatal data and long-term follow-up of newborns with lenticulostriate vessel hyperechoic degeneration. Secondly, it describes the cerebral imaging data as a function of perinatal factors and neurodevelopmental follow-up of these newborns. METHODS: This retrospective study assesses the outcome of newborns with LSV hyperechogenicity on cerebral ultrasound (two grades)...
January 6, 2018: Pediatrics and Neonatology
Candice D Carpenter, Luke L Linscott, James L Leach, Sudhakar Vadivelu, Todd Abruzzo
BACKGROUND: Alagille syndrome is a pediatric multisystem disease with increased prevalence of cerebrovascular disease. The spectrum of cerebrovascular disease in Alagille syndrome includes cerebral aneurysms, moyamoya arteriopathy and dolichoectasia. The prevalence of cerebrovascular disease in Alagille syndrome varies widely in the literature. OBJECTIVE: To determine the prevalence of cerebrovascular disease in our institution's Alagille patient population by employing a full primary review of all available neuroimaging...
January 23, 2018: Pediatric Radiology
Jill M Roberts, Michael E Maniskas, Justin F Fraser, Gregory J Bix
Moyamoya is a cerebrovascular disorder characterized by progressive stenosis of the intracranial internal carotid arteries. There are two forms: Disease and Syndrome, with each characterized by the sub-population it affects. Moyamoya syndrome (MMS) is more prominent in adults in their 20's-40's, and is often associated with autoimmune diseases. Currently, there are no surgical models for inducing moyamoya syndrome, so our aim was to develop a new animal model to study this relatively unknown cerebrovascular disease...
2018: PloS One
Yutaka Tsubata, Takashi Morita, Tetsuo Morioka, Taiji Sasagawa, Kouzo Ikarashi, Noriko Saito, Hisaki Shimada, Shigeru Miyazaki, Shinji Sakai, Hajime Tanaka, Rie Saito, Yasuko Toyoshima, Hiroaki Nozaki, Ichiei Narita
Retinal vasculopathy with cerebral leukodystrophy (RVCL) is a rare autosomal dominant systemic microvascular disease. Neurological disorders and visual disturbance are highlighted as manifestations of RVCL; however, there are few reports focused on nephropathy. Herein, we describe detailed renal histopathological findings in a daughter and father with RVCL, proven by TREX1 genetic analysis. A kidney biopsy of the daughter, 35-year-old with asymptomatic proteinuria, revealed unique and various glomerular changes...
January 10, 2018: CEN Case Reports
Masoud Pourmoghaddas, Hamid Reza Rohani, Hamid Sanei, Afshin Amirpour
Background: The present study tries to compare the unintended outcomes of the Catania stent versus Xience stent in patients undergoing angioplasty. Materials and Methods: In a three month, follow-up, double-blinded, randomized controlled trial, 83 patients undergoing angioplasty, who met the inclusion criteria were entered into the study. After randomization 43 patients were treated with the Xience stent and 40 patients with the Catania stent. Stent-related outcomes such as Cardiac and Non-Cardiovascular Death, Myocardial Infarction (MI), Target Lesion Revascularization (TLR), Stent Thrombosis (ST), Coronary Artery Bypass Grafting (CABG), Peripheral vasculopathy, and Cerebral Vascular Accident (CVA) were compared between the groups...
2017: Advanced Biomedical Research
Kushak Suchdev, Gregory Norris, Imad Zak, Wazim Mohamed, Mohammed Ibrahim
Reversible cerebral vasoconstriction syndrome (RCVS) is increasingly being recognized as a diagnosis in patients presenting with thunderclap headaches. In the vast majority of the cases, the syndrome follows a benign course and patients recover completely without any significant therapeutic intervention. In the rarest of cases, RCVS follows a monophasic course with rapid worsening, severe neurological deficits, and poor outcomes. We present the case of a 25-year-old female who presented with headaches which were worsening over 1 week...
January 2018: Neurohospitalist
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