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primary cns vasculitis

Carmine Franco Muccio, Enrico Tedeschi, Andrea Elefante, Ferdinando Caranci, Alfonso Cerase
No abstract text is available yet for this article.
March 7, 2018: Acta Neurologica Belgica
(no author information available yet)
[This corrects the article DOI: 10.1093/pch/pxx086.079.][This corrects the article DOI: 10.1093/pch/pxx086.079.].
November 2017: Paediatrics & Child Health
Carlo Salvarani, Robert D Brown, Teresa J H Christianson, John Huston, Stephen M Ansell, Caterina Giannini, Gene G Hunder
OBJECTIVES: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma. PATIENTS AND METHODS: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. We compared the findings in these 10 patients with those from 158 patients with PCNSV without lymphoma seen over 29 years...
February 2, 2018: Neurology
Amy Kunchok, Helen C Castley, Lucie Aldous, Simon H Hawke, Emma Torzillo, Geoffrey D Parker, G Michael Halmagyi
We report four fatal cases of fulminant reversible cerebral vasoconstriction syndrome, all initially diagnosed as primary central nervous system vasculitis and treated with corticosteroids. Although reversible cerebral vasoconstriction syndrome is usually self-limiting without permanent neurologic deficits, rarely it can be fatal and worse outcomes have been associated with corticosteroid treatment.
February 15, 2018: Journal of the Neurological Sciences
Leila Chimelli
Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Despite the fact that the need for biopsy has decreased in the era of new neuroimaging methods, biopsies that include small leptomeningeal and parenchymal arterial vessels still remain the gold standard to diagnose PACNS and other CSVD, and to exclude mimics such as infections and malignancies...
October 2017: Dementia & Neuropsychologia
Sikawat Thanaviratananich, Bashar Katirji
Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters...
June 2017: ENeurologicalSci
Sae Aratani, Yukinao Sakai, Shuichi Tsuruoka
Microscopic polyangiitis (MPA) is a primary systemic vasculitis that predominantly affects small and medium vessels. MPA is rarely complicated with central nervous system or cardiovascular disease. We report a very rare case of MPA complicated with cerebral infarction, cardiovascular disease, and fatal subarachnoid hemorrhage in a 54-year-old man. During the first six days of hospitalization the patient was diagnosed with rapid progressive glomerulonephritis (RPGN), cerebral infarction, and unstable angina...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
Rawan K Matar, Bader Alshamsan, Saleh Alsaleh, Hindi Alhindi, Khalid O Alahmedi, Sami Khairy, Salah Baz
Primary Angiitis of the central nervous system is a rare and poorly understood variant of vasculitis. We narrate a case of a 46-year-old male who presented with new onset refractory status epilepticus mimicking autoimmune encephalitis. In this case we are reporting clues that could be useful for diagnosis and extensive literature review on the topic.
2017: Epilepsy & Behavior Case Reports
Jonathan Graf
Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease...
November 2017: Rheumatic Diseases Clinics of North America
Dimitri Renard, Anne Wacongne, Eric Thouvenot
In amyloid β-related angiitis of the central nervous system (also called cerebral amyloid angiopathy-related inflammation), cerebral amyloid angiopathy occurs in association with primary vasculitis of small- and medium-sized leptomeningeal and cortical arteries. To avoid brain biopsy, clinicoradiological criteria (including clinical features due to inflammation-related uni/multifocal white matter hyperintensities) for the diagnosis of cerebral amyloid angiopathy-related inflammation have been validated recently...
November 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Bayan Al Share, Ali Zakaria, Evan Hiner, Ziyad Iskenderian, Nader Warra
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis involving medium and small blood vessels of the brain, spinal cord, and meninges, without systemic involvement. The diffuse and patchy nature of its pathology is reflected by a wide spectrum of nonspecific clinical symptoms. Diagnosis is challenging due to lack of defined clinical criteria or specific imaging findings. Specific workup should be done only after exclusion of other etiologies, including infectious, neoplastic, toxic, and other vascular etiologies including systemic vasculitis...
2017: Case Reports in Neurological Medicine
H de Boysson, G Boulouis, J-J Parienti, E Touzé, M Zuber, C Arquizan, N Dequatre, O Detante, B Bienvenu, A Aouba, L Guillevin, C Pagnoux, O Naggara
BACKGROUND AND PURPOSE: 3D-TOF-MRA and DSA are 2 available tools to demonstrate neurovascular involvement in primary central nervous system vasculitis. We aimed to compare the diagnostic concordance of vessel imaging using 3D-TOF-MRA and DSA in patients with primary central nervous system vasculitis. MATERIALS AND METHODS: We retrospectively identified all patients included in the French primary central nervous system vasculitis cohort of 85 patients who underwent, at baseline, both intracranial 3D-TOF-MRA and DSA in an interval of no more than 2 weeks and before treatment initiation...
October 2017: AJNR. American Journal of Neuroradiology
Markus Kraemer, Jana Becker, Peter A Horn, Jan Claudius Schwitalla, Kathy Keyvani, Imke Metz, Christiane Wegner, Wolfgang Brück, Marc Schlamann, Falko M Heinemann, Peter Berlit
OBJECTIVES: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear. PATIENTS AND METHODS: We analyzed the DNA of 25 Caucasian patients with PCNSV for human leucocyte antigen genes HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1, respectively. HLA-frequencies of the 25 patients with PCNSV were compared with HLA-frequencies of matched Caucasian controls. RESULTS: No statistically significant associations were found for HLA-B, HLA-DR1 and HLA-DQB1 variant...
September 2017: Clinical Neurology and Neurosurgery
Saeed Arif, Jahanzeb Liaqat, Khuram Haq Nawaz, Asif Hashmat
No abstract text is available yet for this article.
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
Joo-Seok Lee, Tae-Young Jung, Kyung-Hwa Lee, Seul-Kee Kim
We report a case of primary central nervous system vasculitis (PCNSV) mimicking a cortical brain tumor. A 25-year-old woman presented with a 2-week history of headache and transient right hemiparesis. Brain magnetic resonance imaging (MRI) revealed a cortical-involving lesion on the left frontal lobe. The 6-cm sized lesion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion had continual linear enhancement on the subcortical white matter and leptomeninges...
April 2017: Brain Tumor Research and Treatment
Pelluru Pavan Kumar, Alugolu Rajesh, Rukmini Mrudula Kandadai, Aniruddh Kumar Purohit, Challa Sundaram
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup...
January 2017: Asian Journal of Neurosurgery
Sonja Walsh, Ralf Knöfler, Gabriele Hahn, Judith Lohse, Reinhard Berner, Sebastian Brenner, Martin Smitka, Maja von der Hagen, Christian M Hedrich
Ischaemic brain injuries are rare conditions in the paediatric age group. Main causes include non-arteriosclerotic arteriopathies, which in childhood usually result from primary vasculitis of large or small vessels and lead to impaired perfusion and subsequent ischaemic brain lesions. In accordance with the nomenclature of systemic forms, CNS vasculitis is subdivided into groups, based on the size of affected vessels: angiography-positive primary angiitis of medium-sized and large vessels (pPACNS), and angiography-negative angiitis of small vessels (svPACNS)...
March 2017: Clinical and Experimental Rheumatology
Grégoire Boulouis, Hubert de Boysson, Mathieu Zuber, Loïc Guillevin, Eric Meary, Vincent Costalat, Christian Pagnoux, Olivier Naggara
BACKGROUND AND PURPOSE: Primary angiitis of the central nervous system remains challenging. To report an overview and pictorial review of brain magnetic resonance imaging findings in adult primary angiitis of the central nervous system and to determine the distribution of parenchymal, meningeal, and vascular lesions in a large multicentric cohort. METHODS: Adult patients from the French COVAC cohort (Cohort of Patients With Primary Vasculitis of the Central Nervous System), with biopsy or angiographically proven primary angiitis of the central nervous system and brain magnetic resonance imaging available at the time of diagnosis were included...
May 2017: Stroke; a Journal of Cerebral Circulation
J Becker, P A Horn, K Keyvani, I Metz, C Wegner, W Brück, F M Heinemann, J C Schwitalla, P Berlit, M Kraemer
OBJECTIVES: To compare clinical features and outcome, imaging characteristics, biopsy results and laboratory findings in a cohort of 69 patients with suspected or diagnosed primary central nervous system vasculitis (PCNSV) in adults; to identify risk factors and predictive features for PCNSV. PATIENTS AND METHODS: We performed a case-control-study including 69 patients referred with suspected PCNSV from whom 25 were confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%)...
May 2017: Clinical Neurology and Neurosurgery
Aashima Dabas, Sangeeta Yadav
Childhood primary angiitis of the central nervous system (cPACNS) is a rare and a potentially fatal cause of childhood stroke. The disease poses a diagnostic dilemma for the clinicians due to overlapping and varied clinical manifestations such as headache, focal acute neurological deficits, cognitive impairment, or encephalopathy. We report a young boy who presented with low-grade fever and headache but rapidly progressed to develop acute encephalopathy and quadriparesis with multiple cranial nerve palsies, masquerading as acute disseminated encephalomyelitis...
October 2016: Journal of Pediatric Neurosciences
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