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Superficial siderosis

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https://www.readbyqxmd.com/read/29451456/clival-meningocele-causing-bilateral-hearing-loss-in-a-child-due-to-superficial-siderosis-of-the-central-nervous-system-case-report
#1
Stephen J Johans, Kevin N Swong, Daniel J Burkett, Michael P Wemhoff, Sean M Lew, Chirag R Patel, Anand V Germanwala
Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated...
February 16, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29421784/complement-activation-in-capillary-cerebral-amyloid-angiopathy
#2
Ko Matsuo, Akihiro Shindo, Atsushi Niwa, Ken-Ichi Tabei, Hiroyasu Akatsu, Yoshio Hashizume, Haruhiko Akiyama, Takashi Ayaki, Takakuni Maki, Nobukatsu Sawamoto, Ryosuke Takahashi, Shinji Oikawa, Hidekazu Tomimoto
BACKGROUND: Cerebral amyloid angiopathy (CAA) is classified as type 1 with capillary amyloid β (Aβ) or type 2 without capillary Aβ. While it is known that CAA activates complement, an inflammatory mediator, there is no information on the relationship between capillary Aβ and complement activation. METHODS: We evaluated 34 autopsy brains, including 22 with CAA and 12 with other neurodegenerative diseases. We assessed the vascular density of CAA by analyzing the expression of complement (C1q, C3d, C6, C5b-9), macrophage scavenger receptor (MSR), and apolipoprotein E (ApoE)...
February 8, 2018: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/29367736/statins-do-not-increase-markers-of-cerebral-angiopathies-in-patients-with-cardioembolic-stroke
#3
Joan Martí-Fàbregas, Santiago Medrano-Martorell, Elisa Merino, Luis Prats-Sánchez, Rebeca Marín, Raquel Delgado-Mederos, Pol Camps-Renom, Alejandro Martínez-Domeño, Manuel Gómez-Choco, Lidia Lara, Ignacio Casado-Naranjo, David Cánovas, Maria José Torres, Marimar Freijo, Ana Calleja, Yolanda Bravo, Dolores Cocho, Ana Rodríguez-Campello, Beatriz Zandio, Blanca Fuentes, Alicia de Felipe, Laura Llull, José Maestre, María Hernández, Moisès Garcés, Ana Maria De Arce-Borda, Ernest Palomeras, Manuel Rodríguez-Yáñez, Inma Díaz-Maroto, Marta Serrano, Jéssica Fernández-Domínguez, Jordi Sanahuja, Francisco Purroy, Marialuisa Zedde, Jordi Delgado-Mengual, Ignasi Gich
We investigated whether pre-treatment with statins is associated with surrogate markers of amyloid and hypertensive angiopathies in patients who need to start long-term oral anticoagulation therapy. A prospective multicenter study of patients naive for oral anticoagulants, who had an acute cardioembolic stroke. MRI was performed at admission to evaluate microbleeds, leukoaraiosis and superficial siderosis. We collected data on the specific statin compound, the dose and the statin intensity. We performed bivariate analyses and a logistic regression to investigate variables associated with microbleeds...
January 24, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29348376/-benign-outcome-of-cochlear-implantation-in-a-patient-with-superficial-siderosis
#4
Kei Sato, Takekazu Ohi, Taisaku Shozaki, Shin Kariya
We report the case of a 38-year-old man with gait disorder and hearing loss. The patient had developed gait disorder due to a cervical meningioma since 4 year-old disappeared for 15 years after the surgical removal of the meningioma. However, at the age of 21 year-old, the gait disorder reappeared and worsened progressively. Sensorineural hearing loss (SNHL) and epileptic seizures developed during the disease course, and he was diagnosed with superficial siderosis (SS). When he was 37 years old, he experienced sudden-onset of right-side hearing impairment and was considered a candidate for cochlear implantation (CI) at the otorhinolaryngology clinic of the nearby University Hospital...
January 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29285884/two-year-observational-study-of-deferiprone-in-superficial-siderosis
#5
Remi A Kessler, Xu Li, Kateryna Schwartz, Hwa Huang, Maureen A Mealy, Michael Levy
INTRODUCTION: Superficial siderosis is a rare, neurodegenerative disease caused by toxic accumulation of hemosiderin on the surface of the brain and the spinal cord, most commonly from chronic subarachnoid hemorrhage. AIMS: The aim of this study was to assess the clinical and radiological outcomes of superficial siderosis patients using deferiprone, a cell permeant iron chelator. Subjects obtained pre- and post-treatment brain MRIs and weekly laboratory tests. Osirix software was used to develop a method of quantifying hemosiderin deposition...
December 28, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29249378/cerebral-amyloid-angiopathy-the-modified-boston-criteria-in-clinical-practice
#6
André Caetano, Filipa Ladeira, Raquel Barbosa, Sofia Calado, Miguel Viana-Baptista
BACKGROUND: Early identification of patients with cerebral amyloid angiopathy (CAA) is relevant considering the increased risk for cerebral hemorrhage. A new set of diagnostic criteria for CAA was recently proposed, which include the presence of superficial siderosis. We aimed to assess the impact of applying these criteria regarding use of antithrombotic therapy. METHODS: Review of consecutive patients admitted to a Neurology Department from 2014 to 2016, with acute parenchymal or subarachnoid hemorrhage and/or atypical transient focal neurological episodes...
January 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29247143/cognitive-impairment-before-intracerebral-hemorrhage-is-associated-with-cerebral-amyloid-angiopathy
#7
Gargi Banerjee, Duncan Wilson, Gareth Ambler, Karen Osei-Bonsu Appiah, Clare Shakeshaft, Surabhika Lunawat, Hannah Cohen, Tarek Yousry, Gregory Y H Lip, Keith W Muir, Martin M Brown, Rustam Al-Shahi Salman, Hans Rolf Jäger, David J Werring
BACKGROUND AND PURPOSE: Although the association between cerebral amyloid angiopathy (CAA) and cognitive impairment is increasingly recognized, it is not clear whether this is because of the impact of recurrent intracerebral hemorrhage (ICH) events, disruptions caused by cerebral small vessel damage, or both. We investigated this by considering whether cognitive impairment before ICH was associated with neuroimaging features of CAA on magnetic resonance imaging. METHODS: We studied 166 patients with neuroimaging-confirmed ICH recruited to a prospective multicentre observational study...
January 2018: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29204961/patterns-of-convexal-subarachnoid-haemorrhage-clinical-radiological-and-outcome-differences-between-cerebral-amyloid-angiopathy-and-other-causes
#8
Lionel Calviere, Nicolas Raposo, Victor Cuvinciuc, Christophe Cognard, Fabrice Bonneville, Alain Viguier
BACKGROUND: Cerebral amyloid angiopathy (CAA) is a common aetiology of convexal subarachnoid haemorrhage (cSAH) but little is known about its specific characteristics in comparison with cSAH from other causes. In this study we compared patients with CAA vs. non-CAA-related cSAH. METHODS: Retrospective review of baseline and follow-up data of consecutive patients admitted with a symptomatic acute cSAH. RESULTS: Sixty-two patients were included (mean age 66...
December 4, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29168672/acute-diffusion-weighted-imaging-lesions-in-cerebral-amyloid-angiopathy-related-convexal-subarachnoid-hemorrhage
#9
Markus Beitzke, Christian Enzinger, Alexander Pichler, Gerit Wünsch, Franz Fazekas
Small acute diffusion-weighted imaging (DWI) lesions can accompany intracerebral hemorrhage due to cerebral amyloid angiopathy (CAA). We therefore examined the occurrence of such lesions in the context of CAA-related convexal subarachnoid hemorrhage (cSAH) both in a cross-sectional and longitudinal manner. DWI lesions were noted in 14/29 (48%) patients at their index cSAH and 12/21 patients (57%) showed acute small DWI lesions at follow-up MRI. Forty-four of 71 (62%) DWI lesions were spatially related to areas of cortical superficial siderosis...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29167486/distinctive-clinical-effects-of-haemorrhagic-markers-in-cerebral-amyloid-angiopathy
#10
Young Kyoung Jang, Hee Jin Kim, Jin San Lee, Yeo Jin Kim, Ko Woon Kim, Yeshin Kim, Hyemin Jang, Juyoun Lee, Jong Min Lee, Seung-Joo Kim, Kyung-Ho Yu, Andreas Charidimou, David J Werring, Sung Tae Kim, Duk L Na, Sang Won Seo
Restricted lobar cerebral microbleeds (CMBs) and cortical superficial siderosis (CSS) are the characteristic markers of cerebral amyloid angiopathy (CAA). However, their effects on clinical features has not been evaluated well. The purpose of this study is to investigate the clinical implication of these markers in clinical-radiologically diagnosed CAA. A total of 372 patients with possible or probable CAA who met the modified Boston criteria were recruited in a memory clinic setting. Cortical thickness was measured using surface based methods...
November 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29160113/superficial-siderosis-of-central-nervous-system-with-unknown-cause-report-of-2-cases-and-review-of-the-literature
#11
Hao Chen, Hafiz Khuram Raza, Jia Jing, Xinchun Ye, Zuohui Zhang, Fang Hua, Guiyun Cui
OBJECTIVE: To report 2 cases of superficial siderosis of central nervous system (SS-CNS) and a review of the literature. METHODS: We have analyzed the clinical data and relevant features of two patients with SS-CNS who were presented with ataxia and slurred speech. Both patients undertook blood tests, lumbar puncture, head CT (computer tomography) scans, and brain and spinal cord magnetic resonance (MR) scans. In addition, the first patient also undewent enhanced susceptibility-weighted angiography (ESWAN) and the second patient undertook susceptibility weighted imaging (SWI) scan...
November 21, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29153366/papillary-glioneuronal-tumors-a-radiopathologic-correlation
#12
Nishtha Yadav, Shilpa Rao, Jitender Saini, Chandrajit Prasad, Anita Mahadevan, Nishanth Sadashiva
OBJECTIVE: Papillary glioneuronal tumors (PGNT) are a rare and recently recognized tumor entity. The neuroimaging findings were reviewed to determine if any specific findings emerge to assist a preoperative diagnosis of PGNT. MATERIALS AND METHODS: Seven histologically confirmed cases of PGNT were evaluated from 2004 to 2014. Clinical, neuroimaging and histological findings were reviewed and tabulated. RESULTS: Headache and seizures were observed in 4 patients (57...
December 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/29122310/idiopathic-infratentorial-superficial-siderosis-of-the-central-nervous-system-case-report-and-review-of-literature
#13
Andrea Stabile, Vincenzo Di Lazzaro, Cesare Colosimo, Fabrizio Piazza, Carlo Ferrarese, Jacopo C DiFrancesco
The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS has been divided into two distinct entities, according to the distribution of iron deposition in the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here we describe an adult case of iSS, with detailed clinical and radiological features...
October 27, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29120920/the-growing-clinical-spectrum-of-cerebral-amyloid-angiopathy
#14
Marieke J H Wermer, Steven M Greenberg
PURPOSE OF REVIEW: Cerebral amyloid angiopathy (CAA) is diagnosed primarily as a cause of lobar intracerebral hemorrhages (ICH) in elderly patients. With improving MRI techniques, however, the role of CAA in causing other symptoms has become clear. Recognizing the full clinical spectrum of CAA is important for diagnosis and treatment. In this review we summarize recent insights in clinical CAA features, MRI biomarkers, and management. RECENT FINDINGS: The rate of ICH recurrence in CAA is among the highest of all stroke subtypes...
February 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29110998/total-small-vessel-disease-burden-and-brain-network-efficiency-in-cerebral-amyloid-angiopathy
#15
Raffaella Valenti, Yael D Reijmer, Andreas Charidimou, Gregoire Boulouis, Sergi Ramirez Martinez, Li Xiong, Panagiotis Fotiadis, Michael Jessel, Alison Ayres, Grace Riley, Leonardo Pantoni, M Edip Gurol, Steven M Greenberg, Anand Viswanathan
BACKGROUND: Cerebral amyloid angiopathy (CAA) is associated with hemorrhagic and nonhemorrhagic markers small vessel disease (SVD). A composite score to quantify the total burden of SVD on MRI specifically for CAA patients was recently developed. Brain network alterations related to individual MRI markers of SVD in CAA were demonstrated. OBJECTIVES: Considering diffusion based network measures sensitive to detect different relevant SVD-related brain injury, we investigated if increased overall SVD injury on MRI corresponds to worse global brain connectivity in CAA...
November 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29070715/prevalence-and-natural-history-of-superficial-siderosis-a-population-based-study
#16
Michael Pichler, Prashanthi Vemuri, Alejandro A Rabinstein, Jeremiah Aakre, Kelly D Flemming, Robert D Brown, Neeraj Kumar, Kejal Kantarci, Walter Kremers, Michelle M Mielke, David S Knopman, Clifford R Jack, Ronald C Petersen, Val Lowe, Jonathan Graff-Radford
BACKGROUND AND PURPOSE: Superficial siderosis (SS) is characterized by hemosiderin deposition in the superficial layers of the central nervous system and can be seen during postmortem examination or with iron-sensitive magnetic resonance imaging techniques. The distribution of SS may predict the probable underlying cause. This study aimed to report the prevalence and natural history of SS in a population-based study. METHODS: Brain magnetic resonance imaging scans from the MCSA (Mayo Clinic Study of Aging), a population-based study of residents 50 to 89 years of age in Olmsted County, Minnesota, were reviewed...
December 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29070669/cortical-superficial-siderosis-multifocality-in-cerebral-amyloid-angiopathy-a-prospective-study
#17
Andreas Charidimou, Gregoire Boulouis, Duangnapa Roongpiboonsopit, Eitan Auriel, Marco Pasi, Kellen Haley, Ellis S van Etten, Sergi Martinez-Ramirez, Alison Ayres, Anastasia Vashkevich, Kristin M Schwab, Joshua N Goldstein, Jonathan Rosand, Anand Viswanathan, Steven M Greenberg, M Edip Gurol
OBJECTIVE: In order to explore the mechanisms of cortical superficial siderosis (cSS) multifocality and its clinical implications for recurrent intracerebral hemorrhage (ICH) risk in patients with cerebral amyloid angiopathy (CAA), we used a new rating method that we developed specifically to evaluate cSS extent at spatially separated foci. METHODS: Consecutive patients with CAA-related ICH according to Boston criteria from a single-center prospective cohort were analyzed...
November 21, 2017: Neurology
https://www.readbyqxmd.com/read/29070668/evolution-of-dwi-lesions-in-cerebral-amyloid-angiopathy-evidence-for-ischemia
#18
Susanne J van Veluw, Arne Lauer, Andreas Charidimou, Narimene Bounemia, Li Xiong, Gregoire Boulouis, Panagiotis Fotiadis, Alison Ayres, M Edip Gurol, Anand Viswanathan, Steven M Greenberg, Meike W Vernooij
OBJECTIVE: To address the pathophysiologic nature of small diffusion-weighted imaging (DWI) lesions in patients with cerebral amyloid angiopathy (CAA) who underwent serial MRI. Specifically, we tested (1) whether DWI lesions occurred preferentially in individuals with prior DWI lesions, (2) the cross-sectional association with chronic cortical cerebral microinfarcts (CMIs), and (3) the evolution of DWI lesions over time. METHODS: Patients with probable CAA (n = 79) who underwent at least 2 MRI sessions were included...
November 21, 2017: Neurology
https://www.readbyqxmd.com/read/29070660/ischemic-lesions-and-superficial-siderosis-in-caa-partners-in-crime-or-innocent-bystanders
#19
EDITORIAL
Randolph S Marshall, Vasileios-Arsenios Lioutas
No abstract text is available yet for this article.
November 21, 2017: Neurology
https://www.readbyqxmd.com/read/29063241/prevalence-and-clinical-characteristics-of-cortical-superficial-siderosis-in-patients-with-acute-stroke
#20
Satoshi Suda, Takashi Shimoyama, Shizuka Suzuki, Takahiro Ouchi, Masafumi Arakawa, Junya Aoki, Kentaro Suzuki, Yuki Sakamoto, Seiji Okubo, Yasuhiro Nishiyama, Chikako Nito, Masahiro Mishina, Kazumi Kimura
Cortical superficial siderosis (cSS) is a pathologic and radiologic diagnosis of hemosiderin deposition in subpial brain layers. However, cSS has not been fully studied in patients with acute stroke. Here, we investigated the prevalence of cSS in patients with acute stroke and analyzed the relationship between cSS and different clinical and neuroimaging characteristics. From September 2014 through June 2016, consecutive patients with acute stroke who were admitted to our department were retrospectively investigated...
December 2017: Journal of Neurology
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