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Vasculitis associated with adenosine deaminase 2 deficiency: at the crossroads between Beh çet's disease and autoinflammation. A viewpoint
Reumatismo. 2023 Sep 18;75(3). doi: 10.4081/reumatismo.2023.1578.ABSTRACTAdenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke...
Source: Reumatismo - September 18, 2023 Category: Rheumatology Authors: A Colangelo F Tromby G Cafaro R Gerli E Bartoloni C Perricone Source Type: research

Assessment of glucocorticoid tapering in large vessel and anti-neutrophil cytoplasmic antibody-associated vasculitides.
CONCLUSIONS: In one quarter of patients referred for LVV or AAV taking GC, tapering was slower than recommended. Promoting timely tapering may reduce GC toxicity. PMID: 33338004 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - December 19, 2020 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research

Strategies for treatment of childhood primary angiitis of the central nervous system
Conclusion No grade-A evidence exists; however, this review provides recommendations for treatment of cPACNS.
Source: Neurology Neuroimmunology and Neuroinflammation - May 2, 2019 Category: Neurology Authors: Beelen, J., Benseler, S. M., Dropol, A., Ghali, B., Twilt, M. Tags: Autoimmune diseases, Vasculitis, All Pediatric, Pediatric stroke; see Cerebrovascular Disease/ Childhood stroke Views [amp ] Reviews Source Type: research

An Update of the Mayo Clinic Cohort of Patients With Adult Primary Central Nervous System Vasculitis: Description of 163 Patients
This study is based on a cohort of 163 consecutive patients with PCNSV who were examined at the Mayo Clinic over a 29-year period from 1983 to 2011. The aim of the study was to define the characteristics of these patients, which represents the largest series in adults reported to date. A total of 105 patients were diagnosed by angiographic findings and 58 by biopsy results. The patients diagnosed by biopsy more frequently had at presentation cognitive dysfunction, greater cerebrospinal fluid total protein concentrations, less frequent cerebral infarcts, and more frequent leptomeningeal gadolinium-enhanced lesions on magnet...
Source: Medicine - May 1, 2015 Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research

Fluctuating Mental Status and Fever of Unknown Origin (P1.030)
Conclusion: Central nervous system involvement as the initial manifestation of microscopic polyangiitis is rare and can result in a challenging clinical picture including recurrent ischemic strokes.Disclosure: Dr. Daniel has nothing to disclose. Dr. Brink has nothing to disclose. Dr. Hosley has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Daniel, A., Brink, J., Hosley, C. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

Primary Angiitis of the Central Nervous System (PACNS) Presenting with Intracerebral Hemorrhage (P2.293)
Conclusion: Primary CNS vasculitis should be suspected even in patients presenting initially with intracerebral hemorrhage in association with only moderate headaches.Disclosure: Dr. Alkhalifah has nothing to disclose. Dr. Fayad has received research support from the National Institute of Neurological Disorders and Stroke and St. Jude Medical. Dr. Omojola has nothing to disclose. Dr. Hearth-Holmes has nothing to disclose. Dr. McComb has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Alkhalifah, M., Fayad, P., Omojola, M., Hearth-Holmes, M., McComb, R. Tags: Cerebrovascular Disease and Interventional Neurology: Inflammation and Immunology Source Type: research

Primary Angiitis of the Central Nervous System (PACNS) presenting with intracerebral hemorrhage (ICH): three cases associated with chronic use of over-the-counter vasoconstrictive drugs (P7.128)
CONCLUSIONS: While the FDA withdrew phenylpropanolamine due to its association with ICH, other vasoconstrictive agents remain ingredients of nasal decongestants/diet pills. These may induce chronic vasoconstriction leading to secondary inflammation and arterial rupture, culminating in atypical PACNS presentations such as multifocal ICH or ICH with acute infarcts (patients 1,2).Disclosure: Dr. Jha has nothing to disclose. Dr. George has nothing to disclose. Dr. Singhal has received personal compensation for activities as a medicolegal expert witness. Dr. Singhal's spouse holds stock and/or stock options in Biogen Idec.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Jha, R., George, J., Singhal, A. Tags: Cerebrovascular Disease and Interventional Neurology: Stroke in Young Source Type: research

Spinal Cord Infarction as the Initial Presentation of Systemic Lupus Erythematosus (P2.077)
CONCLUSIONS:Aggressive therapy with high dose corticosteroids and intravenous cyclophosphamide followed by oral therapy may be a successful therapeutic approach to spinal cord infarcts secondary to systemic lupus erythematosus. Study Supported by: N/ADisclosure: Dr. Michael has nothing to disclose. Dr. Hayat has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Michael, A., Hayat, G. Tags: Neurological Consequences of Autoimmune Disease Source Type: research

A Rare Case of Churg Strauss Syndrome Associated with Cervical Spine and Brain Involvement (P4.037)
CONCLUSIONS:This patient meets four major criteria for diagnosis of CSS ( presence of sinusitis, histologic evidence of eosinophilic vasculitis, peripheral eosinophilia and pulmonary infiltrates). CSS associated with spine vasculitis is a rare but potentially serious clinical entity that may also lead to vascular/granulomatous compressive complications leading to permanent neurological damage. This may be only the second case of spinal cord non-hemorrhagic myelitis associated with CSS.Study Supported by:Disclosure: Dr. Mittal has nothing to disclose. Dr. Acsadi has received personal compensation for activities with Talecri...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Mittal, S., Acsadi, A., Cruz, L., Aglio, T., Ek, K., Lakshminarayanan, S., Logee, K. Tags: General Neurology III Source Type: research

Internal Carotid Artery Floating Thrombus in Relapsing Polychondritis
We present a 52‐year‐old man without any known risk factors for stroke, treated with prednisone and azathioprine for relapsing polychondritis, who presented a minor left hemisphere stroke. Ultrasound of the neck vessels revealed an isoechogenic thrombus in the left internal carotid artery superimposed on a smooth moderately stenosing isoechogenic atheroma of the carotid bulb. The patient was treated with high‐dose tinzaparin and was followed with serial ultrasound. After 16 days, the thrombus demonstrated a hypoechogenic core surrounded by a hyperechogenic rim and the following day it resolved completely. Thrombus fo...
Source: Journal of Neuroimaging - February 23, 2014 Category: Radiology Authors: Theodoros Karapanayiotides, Konstantinos Kouskouras, Panagiotis Ioannidis, Eleni Polychroniadou, Nikolaos Grigoriadis, Dimitrios Karacostas Tags: Case Report Source Type: research

Ischemic stroke as a presenting feature of Wegener's granulomatosis.
We describe a 51-old-male who presented with complaints of slurred speech and left-sided weakness, along with bilateral pulmonary nodules on initial chest x-ray, which were associated with pulmonary symptoms in the past. A repeated computed tomography (CT) scan showed ischemia in the distribution of the right-middle cerebral artery. An open lung biopsy showed vasculitis with necrosis of a vessel wall, with a multinucleated giant cell, confirming the diagnosis of WG. The patient was successfully treated with cyclophosphamide and oral prednisone and he has continued in remission for one year. PMID: 24266130 [PubMed - in process]
Source: Connecticut Medicine - October 1, 2013 Category: Journals (General) Authors: Jethava A, Ali S Tags: Conn Med Source Type: research

Primary Central Nervous System Vasculitis Triggered by Cytomegalovirus Encephalitis (P01.231)
CONCLUSIONS: Our case is the first reported case in the literature of proven CMV encephalitis triggering a subsequent non-infectious CNS vasculitis probably secondary to an autoimmune response. The absence of findings in the initial MRIs of the brain in spite of an intermittently symptomatic patient and the subsequent development of ischemic infarcts supports the theory of intermittent focal hypoperfusion, finally leading to infarction. The high clinical suspicion even with lack of clear evidence of the disease process and empirical treatment led to a successful clinical recovery.Disclosure: Dr. Rosales has nothing to disc...
Source: Neurology - February 14, 2013 Category: Neurology Authors: Rosales, D., Garcia-Gracia, C., Salgado, E., Salanga, V. Tags: P01 Cerebrovascular Disease I Source Type: research