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Cerebral vasculitis

Abhinav Anand, Kruthi Malur, Juhi Kawale, Milind Y Nadkar
We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. 38 years old female who had earlier polyserositis and cerebral venous thrombosis was admitted with provisional diagnosis of SLE and developed acute abdominal pain during hospital stay. She was diagnosed as mesenteric vasculitis and initially responded to pulse methylprednisolone. However, she had relapse which subsequently responded to pulse cyclophosphamide and steroids...
July 2016: Journal of the Association of Physicians of India
Andrea Streng, Veit Grote, Anita Rack-Hoch, Johannes G Liese
BACKGROUND: Universal varicella vaccination for one-year-old children was introduced in Germany in 2004. We investigated changes in incidence and type of varicella-associated neurologic complications in children during the first 7 years after universal vaccination recommendation. METHODS: Surveillance study based on patients <17 years of age with an International Classification of Diseases (10 Revision, ICD-10) discharge diagnosis of varicella, annually reported by 22-29 pediatric hospitals in Bavaria (Germany), 2005 to 2011...
October 3, 2016: Pediatric Infectious Disease Journal
Kazuki Fukuma, Hisanori Kowa, Hiroyuki Nakayasu, Kenji Nakashima
We presented a 38-year-old woman suffering from acute cerebral infarction due to arteritis limited to bilateral internal carotid arteries without a condition of giant cell arteritis or granulomatosis with polyangitis. Our case is unprecedented and characterized by a young woman with wall enhancement in the internal carotid arteries on contrast-enhanced magnetic resonance imaging (MRI), therapeutic effects of steroids, and positive status for human leucocyte antigen-B39, -B51 and -DR4. These disease characteristics were not in accordance with existing diagnostic criteria of vasculitis, such as Takayasu's arteritis, giant cell arteritis, granulomatosis with polyangiitis, and Behcet's disease...
September 2016: Yonago Acta Medica
Paolo Cariati, Almudena Cabello-Serrano, Fernando Monsalve-Iglesias, Maria Roman-Ramos, Blas Garcia-Medina
Complication of dental infections might be various and heterogeneous. The most common complications are represented by maxilar celulitis, canine space celulitis, infratemporal space celulitis, temporal celulitis and bacteremia. Among rarest complications we found: sepsis, bacterial endocarditis, mediastinitis, intracranial complications, osteomyelitis, etc. Although dental infections are often considered trivial entities, sometimes they can reach an impressive gravity. In this regard, the present study describes a case of dental infection complicated by meningitis, subdural empiema and cerebral vasculitis...
October 2016: Journal of Clinical and Experimental Dentistry
Fei Han, Ding-Rong Zhong, Hong-Lin Hao, Wei-Ze Kong, Yi-Cheng Zhu, Hong-Zhi Guan, Li-Ying Cui
BACKGROUND: Hypertrophic pachymeningitis (HP) is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. It can be divided into cranial and spinal forms depending on the location of the lesion. HP involving 2 separate sites simultaneously is quite uncommon. CASE SUMMARY: This study presents a case of a 49-year-old woman with pathologically confirmed cranial and lumbosacral hypertrophic pachymeningitis associated with systemic lupus erythematosus (SLE), which is a rare etiology of HP...
September 2016: Medicine (Baltimore)
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
Anne Laure Bernat, Etienne Lefevre, Damien Sène, Philippe Herman, Homa Adle Biassette, Sebastien Froelich
Granulomatosis with polyangitis (Wegener's disease) is a rare ANCA (anti-neutrophil cytoplasmic antibody) positive vasculitis that seldom involves brain and meninges. We herein present a case of a 35-year-old woman presenting with granulomatosis with polyangitis involving the brain and the meninges. The histological diagnosis required a complete resection of the lesion. Despite the possibility of post operative wound infection surgery was mandatory to ascertain the diagnostic and to treat locally.
September 23, 2016: World Neurosurgery
Vance T Lehman, Waleed Brinjikji, David F Kallmes, John Huston, Giussepe Lanzino, Rabinstein A Alejandro, Ashima Makol, Mahmud Mossa-Bosha
Intracranial arterial pathology has traditionally been evaluated with luminal imaging. Recently, high-resolution vessel wall imaging (HR-VWI) with MRI has facilitated sub-millimeter evaluation of the arterial walls. This technique can help differentiate various causes of intracranial steno-occlusive disease, identify culprit atherosclerotic plaques with a recent cerebral infarct, locate vessel wall pathology in areas with minimal or no narrowing on luminal imaging, predict aneurysm stability, and identify a ruptured aneurysm when multiple aneurysms are present...
September 2, 2016: British Journal of Radiology
T R Spraker, P A White
A previously unrecognized condition is described in wild free-ranging Pribilof arctic foxes (Alopex lagopus pribilofensis) from the Pribilof Islands, Alaska, USA. This condition is called shaggy lame fox syndrome (SLFS) denoting the primary clinical signs first observed. Criteria used to suspect SLFS on gross examination included emaciation, failure to shed winter pelage and moderate to severe polyarthritis. Criteria used to confirm SLFS histologically included polyarthritis (characterized by lymphoplasmacytic synovitis, tenosynovitis, bursitis, periosteal bony proliferation, and periarticular lymphoplasmacytic vasculitis) and systemic leukocytoclastic vasculitis...
August 19, 2016: Veterinary Pathology
Yeon-Jung Kim, Joo Kyung Lee, Sung-Ho Ahn, Bum Joon Kim, Dong-Wha Kang, Jong S Kim, Sun U Kwon
BACKGROUND AND PURPOSE: Middle cerebral artery steno-occlusive disease (MCAD) is not an uncommon cause of ischemic stroke in young Asians. Aside from atherosclerosis, the pathogenesis of MCAD include various nonatherosclerotic vasculopathies, most of which are yet to be defined. This study investigated the pathogenesis of symptomatic isolated MCAD in young Asian patients using high-resolution magnetic resonance imaging (HR-MRI) and mutation analysis of RNF213. METHODS: Patients aged <60 years with stroke or transient ischemic attack caused by MCAD were prospectively enrolled...
September 2016: Stroke; a Journal of Cerebral Circulation
Hubert de Boysson, Grégoire Boulouis, Nelly Dequatre, Sophie Godard, Antoine Néel, Caroline Arquizan, Olivier Detante, Coralie Bloch-Queyrat, Mathieu Zuber, Emmanuel Touzé, Boris Bienvenu, Achille Aouba, Loïc Guillevin, Olivier Naggara, Christian Pagnoux
BACKGROUND AND PURPOSE: We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. METHODS: We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. RESULTS: The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0...
September 2016: Stroke; a Journal of Cerebral Circulation
Bertrand Lioger, Nicole Ferreira-Maldent, Jean Philippe Cottier, Séverine Debiais, Emmanuel Gyan, François Maillot
No abstract text is available yet for this article.
August 2016: Neurology® Neuroimmunology & Neuroinflammation
Ji Young Lee, Young Seo Kim, Hyun Young Kim, Dong Woo Park, Sang-Cheol Bae, Young-Jun Lee
We describe two cases of non-aneurysmal subarachnoid hemorrhage (SAH) and multifocal stenosis of the intracranial arteries. The patients' histories together with magnetic resonance angiography, vessel wall imaging and transcranial Doppler (TCD) indicated that the SAH was due to vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Differential diagnosis of vasculitis and RCVS is important because the treatment strategies are different: immunosuppressants in vasculitis and calcium channel blockers in RCVS...
July 25, 2016: International Journal of Rheumatic Diseases
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
James Monroe Wright, Berje Haroutuon Shammassian, Jeffrey Tait Nelson, Christina Huang Wright
INTRODUCTION: The gold standard for diagnosis of central nervous system vasculitis (CNSV) is cerebral biopsy. Cerebral angiography, however, has become a common diagnostic tool for workup of this disease. Few analyses have been performed which examine the value of angiography as a stand-alone diagnostic entity or the value of cerebral biopsy in the setting of positive angiography. METHODS: A retrospective review was performed for all patients who underwent both cerebral angiography and cerebral biopsy for the workup of a presumptive diagnosis of CNSV at a single center from 2005 to 2016...
August 2016: Neurosurgery
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
Nina Lenherr, Kathi Walther, Jacques Schneider, Andreas Woerner, Melanie Hess
No abstract text is available yet for this article.
2015: Global Pediatric Health
J Jade, K Chung, M Arendse, Z Hussain, D White
We describe a patient with neuro-Behçets disease (NBD) that presented with symptoms of raised intracranial pressure including papilloedema. MRI revealed tumour-like lesions which, on biopsy, confirmed an active vasculitis. Treatment was commenced with prednisone and cyclophosphamide which proved unsuccessful with enlargement of the cerebral mass lesions. Infliximab and mycophenolate were trialled also without benefit. The patient required ventriculoperitoneal shunts to relieve the symptoms of hydrocephalus...
October 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
F Uettwiller, G Sarrabay, M P Rodero, G I Rice, E Lagrue, Y Marot, K Deiva, I Touitou, Y J Crow, P Quartier
The objective of this paper is to: describe the phenotype compound heterozygote for mutations in CECR1 in two children. We describe the clinical and immunological phenotype, including the assessment of ADA2 activity, cytokine expression, interferon-stimulated and neutrophil-stimulated gene signatures, and the results of CECR1 sequencing. The first patient presented with intermittent fever, cutaneous vasculitis, myalgia and muscle inflammation on MRI leading to a provisional diagnosis of periarteritis nodosa...
2016: RMD Open
Nandhakumar Balakrishnan, Marna Ericson, Ricardo Maggi, Edward B Breitschwerdt
BACKGROUND: The genus Bartonella is comprised of a rapidly increasing number of pathogenic species that induce a seemingly diverse spectrum of neurological symptoms. During the 12 year period that followed the initial onset of neurological and gastrointestinal symptoms, an 11 year-old girl experienced a spectrum of neurological complaints including frequent headaches, visual and auditory hallucinations, anxiety, vision loss involving the lower left quadrant of both eyes, episodic bouts of generalized paralysis, facial palsy, chronic insomnia, seizures, dizziness, cognitive dysfunction, and memory loss...
2016: Parasites & Vectors
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