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Superficial siderosis

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https://www.readbyqxmd.com/read/29928256/bilateral-vestibulopathy-in-superficial-siderosis
#1
Sang-Yeon Lee, Dong-Han Lee, Yun Jung Bae, Jae-Jin Song, Ji Soo Kim, Ja-Won Koo
Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS. Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29903393/one-year-prognosis-of-non-traumatic-cortical-subarachnoid-haemorrhage-a-prospective-series-of-34-patients
#2
R F Galiano Blancart, G Fortea, A Pampliega Pérez, S Martí, V Parkhutik, A V Sánchez Cruz, C Soriano, D Geffner Sclarsky, M T Pérez Saldaña, N López Hernández, I Beltrán, A Lago Martín
INTRODUCTION: Cortical subarachnoid haemorrhage (cSAH) has multiple aetiologies. No prospective study has reported the long-term progression of the condition. The objective of this study is to describe the clinical and aetiological characteristics of patients with cSAH and to gain insight into prognosis. METHODS: We performed a prospective, observational, multi-centre study. Data on clinical and radiological variables were collected; during a one-year follow-up period, we recorded data on mortality, dependence, rebleeding, and the appearance of dementia...
June 11, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29892257/focused-neuro-otological-review-of-superficial-siderosis-of-the-central-nervous-system
#3
Aran Yoo, Jonathan Jou, Jeffrey D Klopfenstein, Jorge C Kattah
Background: Infratentorial siderosis (iSS) is a progressive degenerative disorder targeting primarily the cerebellum and cranial nerve eighth; therefore, progressive ataxia and its neuro-otological findings are common. Toxicity from hemosiderin involves selectively vulnerable neurons and glia in these posterior fossa structures. Other neurologic findings may be present, though our focus relates to the cochlea-vestibular cerebellar involvement. Radiographic evidence of siderosis may be the result of recurrent, albeit covert bleeding in the subarachnoid space, or the consequence of an overt post-traumatic or aneurysmal subarachnoid hemorrhage (SAH)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29889788/vestibular-dysfunction-in-patients-with-superficial-siderosis-of-the-central-nervous-system
#4
Takamori Takeda, Yoshiyuki Kawashima, Chiaki Hirai, Ayane Makabe, Taku Ito, Taro Fujikawa, Katsura Yamamoto, Ayako Maruyama, Takeshi Tsutsumi
OBJECTIVE: To describe the vestibular function in patients with superficial siderosis of the central nervous system (SSCN). STUDY DESIGN: Retrospective analysis. SETTING: Tertiary referral center. PATIENTS: Ten consecutive patients with SSCN. This study is the largest case series of SSCN in which detailed neuro-otological findings, including electronystagmography recording, video head impulse test (vHIT), and posturography, were described...
July 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29884737/cortical-superficial-siderosis-a-prospective-observational-cohort-study
#5
Solène Moulin, Barbara Casolla, Grégory Kuchcinski, Gregoire Boulouis, Costanza Rossi, Hilde Hénon, Didier Leys, Charlotte Cordonnier
OBJECTIVE: To determine the prevalence of cortical superficial siderosis (cSS), its clinical and neuroimaging associated markers, and its influence on the risk of recurrent intracerebral hemorrhage (ICH) in a prospective observational ICH cohort. METHODS: We investigated clinical and radiologic markers associated with cSS using multivariable analysis. In survival analyses, we used Cox models to identify predictors of recurrent ICH after adjusting for potential confounders...
June 8, 2018: Neurology
https://www.readbyqxmd.com/read/29878907/cerebrovascular-disorders
#6
Franz Fazekas, Thomas Gattringer, Christian Enzinger
PURPOSE OF REVIEW: Not unexpected from the variety of cerebrovascular disorders and their morphologic and clinical consequences the contributions of neuroimaging are also quite diverse and go with different approaches in different directions. By reviewing the recent advances in these various areas, we will attempt to highlight those contributions, which may be most meaningful both for the clinician and the researcher dealing with cerebrovascular disorders. RECENT FINDINGS: After long expectations of the utility of mismatch concepts, such patient selection has proven successful in extending the benefit of recanalization of acute ischaemic stroke by mechanical thrombectomy beyond 6 h and the importance of infarct size and collaterals has been documented...
June 6, 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29871026/continuous-minor-bleeding-from-tumor-surface-in-patients-with-craniopharyngiomas-case-series-of-nonobstructive-hydrocephalus
#7
Takuhiro Shoji, Tomohiro Kawaguchi, Yoshikazu Ogawa, Mika Watanabe, Miki Fujimura, Teiji Tominaga
Nonobstructive hydrocephalus in patients with craniopharyngiomas is uncommon. We describe our surgical series of 25 consecutive patients with craniopharyngioma who presented with hydrocephalus. Obstructive hydrocephalus was evident in most cases, and nonobstructive hydrocephalus was revealed in three cases. Even after improvement of cerebrospinal fluid (CSF) pathway obstruction by tumor removal, 10 patients (40%) required CSF diversion. Preoperative imaging study revealed thin intraventricular hemorrhage or superficial siderosis in five cases, and CSF examination revealed hemosiderin-laden phagocytes in one case...
June 5, 2018: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/29678935/atrophy-patterns-in-cerebral-amyloid-angiopathy-with-and-without-cortical-superficial-siderosis
#8
Joonho Kim, Han Kyu Na, Jeong-Hyeon Shin, Hee Jin Kim, Sang Won Seo, Joon-Kyung Seong, Duk L Na
OBJECTIVE: To investigate differential atrophy patterns based on the presence of cortical superficial siderosis (cSS) and the role of cSS in predicting amyloid positivity in memory clinic patients fulfilling the diagnostic criteria for probable cerebral amyloid angiopathy (CAA). METHODS: We retrospectively collected data from 44 cognitively impaired patients with probable CAA who underwent 3-dimensional, T1-weighted MRIs (cSS+, n = 27; cSS-, n = 17). Amyloid-positive patients with Alzheimer disease (AD) (n = 56) and amyloid-negative cognitively normal participants (n = 34) were recruited as controls...
May 15, 2018: Neurology
https://www.readbyqxmd.com/read/29604173/cerebral-amyloid-angiopathy-review-of-clinico-radiological-features-and-mimics
#9
REVIEW
Rohit Sharma, Stephanie Dearaugo, Bernard Infeld, Richard O'Sullivan, Richard P Gerraty
Cerebral amyloid angiopathy (CAA) is an important cause of lobar intracerebral haemorrhage (ICH) in the elderly, but has other clinico-radiological manifestations. In the last two decades, certain magnetic resonance imaging (MRI) sequences, namely gradient-recalled echo imaging and the newer and more sensitive susceptibility-weighted imaging, have been utilised to detect susceptibility-sensitive lesions such as cerebral microbleeds and cortical superficial siderosis. These can be utilised sensitively and specifically by the Modified Boston Criteria to make a diagnosis of CAA without the need for 'gold-standard' histopathology from biopsy...
March 31, 2018: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/29545765/clinical-characteristics-and-etiology-of-bilateral-vestibular-loss-in-a-cohort-from-central-illinois
#10
Jorge C Kattah
Background: Previous series of bilateral vestibular loss (BVL) identified numerous etiologies, but surprisingly, a cause in a significant number of cases remains unknown. In an effort to understand possible etiology and management strategies, a global effort is currently in progress. Here, I contribute my 10-year experience with both acute and chronic BVL during the 2007-2017 decade. Methods: This is a retrospective review of the charts and EMR of patients diagnosed with BVL in the last 10 years...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29526127/-radiological-forms-of-cerebral-amyloid-angiopathy
#11
Sándor Csizmadia, Erika Vörös
Cerebral amyloid angiopathy (CAA) is most commonly recognized by β-amyloid deposition in the small and medium sized vessels of the brain. The 71-year-old female presented with a sudden onset of vertigo and headache. By native computer tomography (CT) examination we found cerebral atrophy and the sign of chronic vascular injury. The complaints of the patient worsened, thus magnetic resonance imaging (MRI) was performed. The MRI scan revealed a bleeding transformation of an ischemic lesion in the right occipital region...
March 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/29468260/superficial-siderosis-and-dural-ectasia-in-a-patient-with-marfan-syndrome
#12
Matthew Bower, Mark Farag, Nicolas Phielipp
No abstract text is available yet for this article.
February 21, 2018: Clinical Neuroradiology
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
#13
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...
May 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29421784/complement-activation-in-capillary-cerebral-amyloid-angiopathy
#14
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)...
2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/29367736/statins-do-not-increase-markers-of-cerebral-angiopathies-in-patients-with-cardioembolic-stroke
#15
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
#16
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
#17
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...
March 2018: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29249378/cerebral-amyloid-angiopathy-the-modified-boston-criteria-in-clinical-practice
#18
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
#19
MULTICENTER STUDY
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
#20
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...
January 2018: Journal of Neurology
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