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Cns vasculitis

Tanawan Riangwiwat, Chris Y Wu, Alberto S Santos-Ocampo, Randal J Liu, Aaron M McMurtray, Beau K Nakamoto
Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis...
2016: Case Reports in Neurological Medicine
Anna Lis-Święty, Ligia Brzezińska-Wcisło, Hubert Arasiewicz
INTRODUCTION: Localized scleroderma (LoS) of the face and head is often associated with neurologic manifestations and/or imaging abnormalities in the central nervous system (CNS). CASE SERIES: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e...
October 4, 2016: International Journal of Neuroscience
W Reith, C Kraus, N Harsch
CLINICAL/METHODICAL ISSUE: Vasculitis is a rare cause of diseases of the central nervous system (CNS). Vasculitis can be divided into primary and secondary forms, of which the vast majority can be manifested in various organ systems, including the CNS. Isolated vasculitis of the CNS is limited to the CNS and clinical neurological symptoms as with the other forms of vasculitis, are headaches, encephalopathy, focal deficits and seizures. A criterion of isolated CNS vasculitis is the clinical and laboratory diagnostic exclusion of other forms of vasculitis and the involvement of other organ systems...
October 2016: Der Radiologe
M Aringer, M Schneider
In the last few decades a number of small, often largely unrecognized steps have fundamentally changed the management of systemic lupus erythematosus (SLE). The current goal is to stop all disease activity without long-term use of more than 5 mg prednisolone per day. Remission, i.e. absence of activity in the SLE activity score of choice, is the defined target in the treat to target approach. The essential basic measures include life-long hydroxychloroquine as well as protection from sunlight (UV) and vitamin D substitution...
September 26, 2016: Der Internist
Sreeja Hareendranathan Pillai, Sapna Erat Sreedharan, Girish Menon, Santhosh Kannoth, Sylaja Pn
Primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Intracranial haemorrhages though less reported are in the form of parenchymal haemorrhage and subarachnoid haemorrhage. We report a case of PACNS with intraventricular haemorrhage due to aneurysms secondary to progression of vasculitis.
July 2016: Annals of Indian Academy of Neurology
Marco Felipe Silva, Ana Raquel Feitosa, José A Paz, Nádia Emi Aikawa, Clovis A Silva
Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation...
July 2016: Revista Brasileira de Reumatologia
Álex Rovira, Cristina Auger, Antoni Rovira
Idiopathic inflammatory-demyelinating diseases (IIDDs) represent a broad spectrum of central nervous system (CNS) disorders, including monophasic, multiphasic, and progressive disorders that range from highly localized forms to multifocal or diffuse variants. In addition to the classic multiple sclerosis (MS) phenotypes, several MS variants have been described, which can be differentiated on the basis of severity, clinical course, and lesion distribution. Other forms of IIDD are now recognized as distinct entities and not MS variants, such as acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders...
2016: Handbook of Clinical Neurology
David A Geier, Mark R Geier
Gardasil is a quadrivalent human papillomavirus (HPV4) vaccine that was approved for use by the US Food and Drug Administration in June 2006. HPV4 vaccine is routinely recommended for administration to women in the USA who are 11-12 years old by the Advisory Committee on Immunization Practices. Previous studies suggest HPV4 vaccine administration was associated with autoimmune diseases. As a consequence, an epidemiological assessment of the vaccine adverse event reporting system database was undertaken for adverse event reports associated with vaccines administered from 2006 to 2014 to 6-39 year-old recipients with a listed US residence and a specified female gender...
July 13, 2016: Immunologic Research
Jose Gavito-Higuera, Carola Birgit Mullins, Luis Ramos-Duran, Cristina Ivette Olivas Chacon, Nawar Hakim, Enrique Palacios
Fungal infections of the central nervous system (CNS) pose a threat to especially immunocompromised patients and their development is primarily determined by the immune status of the host. With an increasing number of organ transplants, chemotherapy, and human immunodeficiency virus infections, the number of immunocompromised patients as susceptible hosts is growing and fungal infections of the CNS are more frequently encountered. They may result in meningitis, cerebritis, abscess formation, cryptococcoma, and meningeal vasculitis with rapid disease progression and often overlapping symptoms...
2016: Journal of Clinical Imaging Science
Yoshiyuki Abe, Naoto Tamura, Kwang-Seok Yang, Joe Matsuoka, Takayuki Kon, Ken Yamaji, Hiroshi Hashimoto, Hiroshi Tsuda, Yoshinari Takasaki
PURPOSE: To determine mortality and its predictive factors in elderly Japanese patients with severe microscopic polyangiitis (MPA). METHOD: This retrospective single-center study determined the mortality of 52 patients with MPA who were admitted to our geriatric medical center from 2002 to 2014. The variables at baseline, including patient demographics, clinical characteristics, and treatment, were analyzed for their association with mortality. RESULT: Mean age at onset of MPA was 73...
July 11, 2016: Modern Rheumatology
Jose Torres, Caitlin Loomis, Brett Cucchiara, Michelle Smith, Steven Messé
BACKGROUND AND PURPOSE: The utility and safety of brain biopsy for suspected primary angiitis of the central nervous system (PACNS) are uncertain. Factors predictive of a positive biopsy have not been well described. Our aim was to evaluate the diagnostic yield and safety of brain biopsy in suspected PACNS and determine whether any prebiopsy variables are associated with a positive biopsy. METHODS: This is a retrospective study of consecutive patients who underwent diagnostic brain biopsy for PACNS at a single institution...
August 2016: Stroke; a Journal of Cerebral Circulation
Christoph Stretz, Xianyuan Song, Brendan D Killory, Martin D Ollenschleger, Amre M Nouh
A 75-year-old female with untreated rheumatoid arthritis presented with two weeks of behavioral changes and cognitive decline. A neurologic examination showed severe encephalopathy, brisk reflexes, and bilateral Babinski sign. A contrast-enhanced brain MRI demonstrated right meningeal enhancement and periventricular white matter disease. A computed tomographic angiogram (CTA) of the head and neck was negative for vasculitis. The cerebrospinal fluid (CSF) demonstrated lymphocytic pleocytosis. The patient's serum rheumatoid factor levels were elevated...
March 2016: Connecticut Medicine
Sniya Valsa Sudhakar, Karthik Muthusamy, Sunithi Mani, Sridhar Gibikote, Manohar Shroff
Imaging plays an important role in diagnosis, management, prognostication and follow up of pediatric demyelinating and inflammatory diseases of brain and forms an integral part of the diagnostic criteria. This article reviews the spectrum of aquaporinopathies with an in-depth discussion on present criteria and differentiation from other demyelinating diseases with clinical vignettes for illustration; the latter part of article deals with the spectrum of CNS vasculitis.
September 2016: Indian Journal of Pediatrics
Suzan A AlKhater
BACKGROUND: Primary immunodeficiency disorders associated with autoimmunity are poorly understood. Central nervous system (CNS) vasculitis can complicate the courses of such entities, but it is underappreciated. Deletion of the dedicator of cytokinesis 8 (DOCK8) gene is considered to be the autosomal recessive form of hyperimmunoglobulin E syndrome which is a rare type of primary immunodeficiency disease characterized by elevated levels of IgE antibody, eczema, and recurrent staphylococcal infections...
April 26, 2016: BMC Neurology
Osama Jamil, Philipp Taussky, Richard H Schmidt, Min S Park
BACKGROUND: Central nervous system vasculitis has multiple presentations, including stroke, seizures, cranial nerve palsies, and encephalopathy. CASE DESCRIPTION: We present the case of an unresponsive 45-year-old woman with vasculitis associated with fulminant intracranial vessel dissection and occlusion with ischemic strokes and subarachnoid hemorrhage secondary to aneurysm rupture. Imaging studies demonstrated both ischemic and hemorrhagic strokes. She had a ruptured right internal carotid artery dorsal variant aneurysm, right vertebral artery dissection with occlusion and posterior inferior cerebellar artery infarct, left vertebral artery dissection, and severe vasculitis involving intracranial and extracranial vessels...
July 2016: World Neurosurgery
Guillermo Carvajal Alegria, Dewi Guellec, Xavier Mariette, Jacques-Eric Gottenberg, Emmanuelle Dernis, Jean-Jacques Dubost, Anne-Priscille Trouvin, Eric Hachulla, Claire Larroche, Veronique Le Guern, Divi Cornec, Valérie Devauchelle-Pensec, Alain Saraux
OBJECTIVES: Neurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjögren's syndrome (ASSESS) cohort. METHODS: The ASSESS cohort, established in 2006, includes 395 patients fulfilling American-European Consensus Group criteria for pSS...
2016: RMD Open
Lauren Koffman, Richard Prayson, E M Manno
BACKGROUND: The neurological manifestations of Systemic Lupus Erythematosus (SLE) are varied and incompletely described. A few case series report a benign idiopathic intracranial hypertension (IIH) related to SLE, which is responsive to immunotherapy. There are limited reports of patients with malignant cerebral edema, and diffuse white matter changes in the absence of central nervous system (CNS) vasculitis. METHODS: Case series from our tertiary care center and review of the relevant literature...
May 15, 2016: Journal of the Neurological Sciences
Juan-Fang Liu, Yuan Li, Kai Li, Xiao Zhang, Yi-Ning Yang, Gang Zhao, Zhi-Rong Liu
Neuro-Sweet disease (NSD) is Sweet disease with central nervous system (CNS) involvement. To the best of our knowledge, the present case report is the first to describe NSD complicated by endogenous infection with Mycobacterium tuberculosis. The present case report describes a male patient who developed NSD-induced meningitis, which initially manifested as a fever, headache and neck stiffness. Painful erythematous plaques subsequently developed on his face, neck and upper trunk. Brain magnetic resonance imaging was performed and the results were normal, whereas modified acid-fast stain analysis of the cerebrospinal fluid (CSF) provided a positive result...
April 2016: Experimental and Therapeutic Medicine
Rashi I Mehta, Rupal I Mehta
Polymer substances are commonly applied as surface coatings on endovascular catheters and vascular devices. Adverse effects related to their use have been reported, although the overall clinical significance and appropriate methods of detection of these complications have been unclear. In this analysis, we systematically reviewed clinical and diagnostic features in 32 patients (age, 36-87years; mean, 59years) in whom intracranial polymer reactions were documented following vascular interventions. Associated neuroradiologic and neuropathologic findings were variable and included cerebral vasculitis or vasculopathy (63%), abscess or granuloma formation (38%), ischemic infarcts (28%), parenchymal hematomas (28%), white matter change (25%), and/or chemical meningitis (22%)...
July 2016: Human Pathology
Martha L Decker, Derek J Emery, Penelope S Smyth, Jian-Qiang Lu, Atilano Lacson, Elaine Yacyshyn
BACKGROUND: Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis (AAV; ANCA denotes antineutrophil cytoplasmic antibody) that causes necrotizing inflammation of small blood vessels. Renal and pulmonary manifestations are common whereas central nervous system (CNS) involvement, and in particular spinal disease, is rare. METHODS: We reviewed a case of MPA presenting with spinal intradural hemorrhage and intracerebral hemorrhage. We also summarized all reported cases of AAV with spinal cord involvement in the literature (database search included MEDLINE, Embase, Scopus, and Proquest with no date or language restriction)...
July 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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