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Superficial siderosis

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
Christopher Karayiannis, Cathy Soufan, Ronil V Chandra, Thanh G Phan, Kitty Wong, Shaloo Singhal, Lee-Anne Slater, John Ly, Chris Moran, Velandai Srikanth
BACKGROUND AND PURPOSE: Cerebral microbleeds (CMBs), cortical superficial siderosis, white matter lesions (WML), and cerebral atrophy may signify greater bleeding risk particularly in patients in whom anticoagulation is to be considered. We investigated their prevalence and associations with stroke type in patients with stroke and atrial fibrillation (AF). MATERIALS AND METHODS: Cross-sectional sample, Monash Medical Centre (Melbourne, Australia) between 2010 and 2013, with brain MRI...
2016: Frontiers in Neurology
Duangnapa Roongpiboonsopit, Andreas Charidimou, Christopher M William, Arne Lauer, Guido J Falcone, Sergi Martinez-Ramirez, Alessandro Biffi, Alison Ayres, Anastasia Vashkevich, Oluwole O Awosika, Jonathan Rosand, M Edip Gurol, Scott B Silverman, Steven M Greenberg, Anand Viswanathan
OBJECTIVE: To identify predictors of early lobar intracerebral hemorrhage (ICH) recurrence, defined as a new ICH within 6 months of the index event, in patients with cerebral amyloid angiopathy (CAA). METHODS: Participants were consecutive survivors (age ≥55 years) of spontaneous symptomatic probable or possible CAA-related lobar ICH according to the Boston criteria, drawn from an ongoing single-center cohort study. Neuroimaging markers ascertained in CT or MRI included focal (≤3 sulci) or disseminated (>3 sulci) cortical superficial siderosis (cSS), acute convexity subarachnoid hemorrhage (cSAH), cerebral microbleeds, white matter hyperintensities burden and location, and baseline ICH volume...
September 30, 2016: Neurology
T Abkur, S Looby, T Counihan
Herein, we describe a case of 69 year old man who presented with gradual bilateral hearing impairment and unsteadiness. Examination showed bilateral sensorineural deafness and cerebellar ataxia. Neuroimaging revealed a rim of hypointensity surrounding the brain stem, superficial cerebellar fissures and extending into the cervical spinal cord consistent with the diagnosis of superficial siderosis (SS). Investigations failed to identify any reversible cause for the condition.
2016: Irish Medical Journal
U Yilmaz, S Meyer, L Gortner, H Körner, M Türkyilmaz, A Simgen, W Reith, R Mühl-Benninghaus
BACKGROUND AND PURPOSE: Germinal matrix hemorrhage is a frequent complication of prematurity and can be associated with adverse neurodevelopmental outcome, depending on its severity. In addition to parenchymal damage, intraventricular residues of hemorrhage and hydrocephalus MR imaging findings include superficial siderosis. The purpose of this study was to investigate the prevalence and location of superficial siderosis in patients with a history of germinal matrix hemorrhage. MATERIALS AND METHODS: We retrospectively identified patients with a history of germinal matrix hemorrhage who underwent MR imaging in our institution between 2008 and 2016...
September 15, 2016: AJNR. American Journal of Neuroradiology
Arne Lauer, Susanne J van Veluw, Christopher M William, Andreas Charidimou, Duangnapa Roongpiboonsopit, Anastasia Vashkevich, Alison Ayres, Sergi Martinez-Ramirez, Edip M Gurol, Geert Jan Biessels, Matthew Frosch, Steven M Greenberg, Anand Viswanathan
OBJECTIVES: To identify in vivo MRI markers that might correlate with cerebral microinfarcts (CMIs) on autopsy in patients with cerebral amyloid angiopathy (CAA). METHODS: We included patients with neuropathologic evidence of CAA on autopsy and available antemortem brain MRI. Clinical characteristics and in vivo MRI markers of CAA-related small vessel disease were recorded, including white matter hyperintensities, cerebral microbleeds, cortical superficial siderosis, and centrum semiovale perivascular spaces...
October 4, 2016: Neurology
Dimitri Renard, Audrey Gabelle, Christophe Hirtz, Christophe Demattei, Eric Thouvenot, Sylvain Lehmann
We evaluated cerebrospinal fluid amyloid-β 1-40 (Aβ40), amyloid-β 1-42 (Aβ42), total and phosphorylated-tau (t-tau and p-tau) in patients with symptomatic isolated cortical supratentorial superficial siderosis (SS), by prospectively recruiting ten patients with SS in the absence of pre-existing cognitive dysfunction, and comparing biomarkers with lobar hematoma cerebral amyloid angiopathy patients (LH-CAA, n = 13), Alzheimer's disease patients (AD, n = 42), and controls (n = 16). Compared to controls, SS patients showed statistically significant higher t-tau (p = 0...
August 26, 2016: Journal of Alzheimer's Disease: JAD
Sara Shams, Juha Martola, Andreas Charidimou, Lena Cavallin, Tobias Granberg, Mana Shams, Yngve Forslin, Peter Aspelin, Maria Kristoffersen-Wiberg, Lars-Olof Wahlund
OBJECTIVE: To gain further insight into cortical superficial siderosis (cSS), a new hemorrhagic neuroimaging marker of cerebral amyloid angiopathy (CAA), and to investigate the clinical, neuroimaging, genetic, and CSF biomarker profile of cSS in a large, consecutive memory clinic series. METHODS: We included 1,504 memory clinic patients undergoing dementia investigation including a brain MRI in our center. Routine CSF biomarker analysis was performed in 1,039 patients and APOE genotyping in 520 patients...
September 13, 2016: Neurology
Charlotte Zerna, Jayesh Modi, Lisa Bilston, Ashkan Shoamanesh, Shelagh B Coutts, Eric E Smith
BACKGROUND AND PURPOSE: Transient focal neurological episodes occur in cerebral amyloid angiopathy (CAA) and can mimic transient ischemic attack (TIA). Risk factors and outcomes of minor ischemic stroke or TIA might differ in patients with and without cerebral microbleeds (CMBs), including CAA-consistent lobar CMB. METHODS: Baseline magnetic resonance imaging (MRI) was analyzed for CMBs and cortical superficial siderosis in 416 patients in the prospective computed tomography and MRI in the CATCH study (Triage of TIA and Minor Cerebrovascular Events to Identify High Risk Patients)...
September 2016: Stroke; a Journal of Cerebral Circulation
Samir Abu Rumeileh, Valentina Favoni, Francesco Toni, Giulia Pierangeli, Federico Oppi, Giovanna Calandra-Buonaura, David Milletti, Monica Maffei, Luigi Cirillo, Raffaele Agati, Giorgio Palandri, Pietro Cortelli
We describe the case of a man whose initial clinical presentation included sensorineural hearing loss and orthostatic hypotension. The patient was diagnosed with superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus.
August 6, 2016: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
Sung Mo Ryu, Eun-Sang Kim, Seung-Kook Kim, Sun-Ho Lee, Whan Eoh
Superficial siderosis of the central nervous system(SSCNS) is a rare disease characterized by hemosiderin deposition on the surface of the central nervous system. We report a case of SSCNS originating from the thoracic spine, presenting with neurological deficits including, sensorineuronal hearing loss, ataxia, and corticospinal and dorsal column tract signs. The patient underwent dural repair with an artificial dural patch. Clinical findings were elicited by neurological examination, imaging studies, and intraoperative findings, and these were addressed through literature review...
June 2016: Korean Journal of Spine
Hae-Won Koo, Kyung-Il Jo, Je-Young Yeon, Jong-Soo Kim, Seung-Chyul Hong
BACKGROUND: There is no doubt that cerebral amyloid angiopathy (CAA) is a key risk factor for recurrent lobar ICH, however, the exact mechanism and interaction with MRI markers of disease severity are less well known. Centrum semiovale-perivascular spaces (CSO-PVS) have been suggested as adjunctive diagnostic criteria in order to enhance diagnostic power. The purposes of this study were to investigate the prevalence of CSO-PVS and its association with other imaging signatures {lobar microbleeds (CMB), cortical superficial siderosis (CSS), white matter hyperintensity (WMH)} in lobar ICH patients as well as recurrent lobar ICH risk, especially in patients taking antithrombotic agents...
August 15, 2016: Journal of the Neurological Sciences
Luis Prats-Sánchez, Pol Camps-Renom, Javier Sotoca-Fernández, Raquel Delgado-Mederos, Alejandro Martínez-Domeño, Rebeca Marín, Miriam Almendrote, Laura Dorado, Meritxell Gomis, Javier Codas, Laura Llull, Alejandra Gómez González, Jaume Roquer, Francisco Purroy, Manuel Gómez-Choco, David Cánovas, Dolores Cocho, Moises Garces, Sonia Abilleira, Joan Martí-Fàbregas
BACKGROUND AND PURPOSE: Remote parenchymal hemorrhage (rPH) after intravenous thrombolysis with recombinant tissue-type plasminogen activator may be associated with cerebral amyloid angiopathy, although supportive data are limited. We aimed to investigate risk factors of rPH after intravenous thrombolysis with recombinant tissue-type plasminogen activator. METHODS: This is an observational study of patients with ischemic stroke who were treated with intravenous thrombolysis with recombinant tissue-type plasminogen activator and were included in a multicenter prospective registry...
August 2016: Stroke; a Journal of Cerebral Circulation
Andreas Charidimou, Sergi Martinez-Ramirez, Yael D Reijmer, Jamary Oliveira-Filho, Arne Lauer, Duangnapa Roongpiboonsopit, Matthew Frosch, Anastasia Vashkevich, Alison Ayres, Jonathan Rosand, Mahmut Edip Gurol, Steven M Greenberg, Anand Viswanathan
IMPORTANCE: Cerebral amyloid angiopathy (CAA) is characteristically associated with magnetic resonance imaging (MRI) biomarkers of small vessel brain injury, including strictly lobar cerebral microbleeds, cortical superficial siderosis, centrum semiovale perivascular spaces, and white matter hyperintensities. Although these neuroimaging markers reflect distinct pathophysiologic aspects in CAA, no studies to date have combined these structural imaging features to gauge total brain small vessel disease burden in CAA...
August 1, 2016: JAMA Neurology
Arifumi Matsumoto, Hiroyoshi Suzuki, Muneshige Tobita, Kinya Hisanaga
A 74-year-old female patient, who was diagnosed with neurofibromatosis type 1 (NF1) at the age of 40, was admitted with complaints of flickering vision and gait disturbance for the last 2 years. On admission, neurological examination revealed mild bilateral hearing loss and ataxia in the limb and trunk. Laboratory tests revealed anti-hepatitis C virus (HCV) antibody positivity and elevated HCV RNA by real-time polymerase chain reaction. The cerebrospinal fluid examination revealed a slightly yellowish appearance with elevated total protein levels...
July 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
D Renard, A Waconge, S Bouly, C Castelli, E Thouvenot
BACKGROUND: Density heterogeneity and fluid-blood levels (FBLs) are frequently seen on acute CT scans of deep brain hemorrhage. Our aim was to analyze the density heterogeneity and FBLs seen on acute/subacute CT in patients aged>55 with lobar haemorrhage (LH), and to study the relationship of these brain abnormalities with other parameters, including cerebral amyloid angiopathy (CAA)-related abnormalities. METHODS: This was an observational study and retrospective analysis of early CT scans (<7 days) in patients aged>55 years with acute lobar hemorrhage who, between 2012 and 2015, were entered into our stroke database...
June 2016: Revue Neurologique
Yuta Maetani, Dai Agari, Eiichi Nomura, Mitsuharu Ueda, Yukio Ando, Takemori Yamawaki
A 76-year-old woman was admitted to our hospital because of transthyretin-related familial amyloid polyneuropathy (TTR-FAP). She had developed bilateral vitreous opacity at the age of 58 and paroxysmal atrial fibrillation at the age of 62. She suffered gait disturbance and dysesthesia of the limbs at the age of 68 and was diagnosed with FAP involving a homozygous Val30Met mutation in the amyloidogenic transthyretin (ATTR) gene after a genetic test. Her parents were cousins, and her aunt's medical history included pacemaker implantation and polyneuropathy...
June 22, 2016: Rinshō Shinkeigaku, Clinical Neurology
Toshihiro Nonaka, Yusuke Yakushiji, Toshihiro Ide, Hiroshi Ito, Kazuhiro Kawamoto, Hideo Hara
An 85-year-old woman with untreated hypertension was admitted with a disturbance of consciousness. On admission, brain CT revealed a lobar intracerebral hemorrhage with a midline shift. An intracranial hematoma was evacuated via a life-saving craniotomy. Definite pathological findings of amyloid-β deposition in the excised hematoma (strong in anti-amyloid β40 immunostain, but weak in anti- amyloid β42) indicated cerebral amyloid angiopathy (CAA). She had been diagnosed with Alzheimer's disease at a regional memory clinic one month before symptom onset based on MRI findings of medial temporal lobe atrophy as well as CAA-related features of multiple strictly lobar cerebral microbleeds in the occipital lobe, cortical superficial siderosis and >20 enlarged perivascular spaces in the centrum semiovale...
May 31, 2016: Rinshō Shinkeigaku, Clinical Neurology
Solène Moulin, Julien Labreuche, Stéphanie Bombois, Costanza Rossi, Gregoire Boulouis, Hilde Hénon, Alain Duhamel, Didier Leys, Charlotte Cordonnier
BACKGROUND: Dementia occurs in at least 10% of patients within 1 year after stroke. However, the risk of dementia after spontaneous intracerebral haemorrhage that accounts for about 15% of all strokes has not been investigated in prospective studies. We aimed to determine the incidence of dementia and risk factors after an intracerebral haemorrhage. METHODS: We did a prospective observational cohort study in patients with spontaneous intracerebral haemorrhage from the Prognosis of Intracerebral Haemorrhage (PITCH) cohort who were admitted to Lille University Hospital, Lille, France...
July 2016: Lancet Neurology
Griffin R Baum, Nefize Turan, Ferdinando S Buonanno, Gustavo Pradilla, Raul G Nogueira
BACKGROUND: Superficial siderosis (SS) is the occult deposition of hemosiderin within the cerebral cortex due to repeat microhemorrhages within the central nervous system. The collection of hemosiderin within the pia and superficial cortical surface can lead to injury to the nervous tissue. The most common presentation is occult sensorineural hearing loss although many patients have been misdiagnosed with diseases such as multiple sclerosis and amyotrophic lateral sclerosis before being diagnosed with SS...
2016: Surgical Neurology International
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