Primary angiitis of the central nervous system: 2 atypical cases

Pagni, Fabio; Isimbaldi, Giuseppe; Vergani, Francesco; Casiraghi, Paolo; Marzorati, L.; Migliorino, Guglielmo Marco; Cattoretti, Giorgio

doi:10.5114/fn.2012.30530

Cited by 11 publications

(4 citation statements)

References 22 publications

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“…Angiitis of the CNS may have previously been categorized as primary angiitis of the CNS (PACNS) when its cause was unknown; however, Pagni et al reported two cases of PACNS, among which one had a possible EBV relationship. 11 These findings suggest that testing for EBV needs to be considered for the accurate diagnosis and classification of CNS vasculitis. Only one study has reported a case of chronic active EBV infection with the features of granulomatosis with polyangiitis because it is very rare.…”

Section: Discussionmentioning

confidence: 99%

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

et al. 2016

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A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis.

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Section: Discussionmentioning

confidence: 99%

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

et al. 2016

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“…During exome sequencing of genes in DNA samples from BEN patients, possible deleterious/damaging variants— CELA1 , HSPG2 , and KCNK5 —are detected. These genes could encode proteins connected to the process of angiogenesis (Toncheva et al, 2014), which is tightly correlated with BEN and UTUC (Jankovic-Velickovic et al, 2012).…”

Section: Toxicological Properties Of Aristolochic Acidsmentioning

confidence: 99%

Systematic Overview of Aristolochic Acids: Nephrotoxicity, Carcinogenicity, and Underlying Mechanisms

et al. 2019

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Aristolochic acids (AAs) are a group of toxins commonly present in the plants of genus Aristolochia and Asarum , which are spread all over the world. Since the 1990s, AA-induced nephropathy (AAN) and upper tract urothelial carcinoma (UTUC) have been reported in many countries. The underlying mechanisms of AAN and AA-induced UTUC have been extensively investigated. AA-derived DNA adducts are recognized as specific biomarkers of AA exposure, and a mutational signature predominantly characterized by A→T transversions has been detected in AA-induced UTUC tumor tissues. In addition, various enzymes and organic anion transporters are involved in AA-induced adverse reactions. The progressive lesions and mutational events initiated by AAs are irreversible, and no effective therapeutic regimen for AAN and AA-induced UTUC has been established until now. Because of several warnings on the toxic effects of AAs by the US Food and Drug Administration and the regulatory authorities of some other countries, the sale and use of AA-containing products have been banned or restricted in most countries. However, AA-related adverse events still occur, especially in the Asian and Balkan regions. Therefore, the use of AA-containing herbal remedies and the consumption of food contaminated by AAs still carry high risk. More strict precautions should be taken to protect the public from AA exposure.

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“…A total of 46 eligible articles (eFigure 1, links.lww.com/NXI/A629), comprising 911 unique patients diagnosed with PACNS, were included in the meta-analysis: 372 biopsy-confirmed, 394 angiogram-confirmed, and 145 patients who could not be assigned to a diagnostic procedure (eTable 1, links.lww.com/NXI/A628). 3,9-13,14-53 A majority of the included studies (n = 29, 63%) reported cases from Western Europe and the United States between 1988 and 2018. The median follow-up duration ranged from 0.75 to 61.2 months.…”

Section: Resultsmentioning

confidence: 99%

Primary Angiitis of the CNS

Beuker¹,

Strunk²,

Rawal³

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesTo facilitate and improve the diagnostic and therapeutic process by systematically reviewing studies on patients with primary angiitis of the CNS (PACNS).MethodsWe searched PubMed, looking at the period between 1988 and February 2020. Studies with adult patients with PACNS were included. We extracted and pooled proportions using fixed-effects models. Main outcomes were proportions of patients with certain clinical, imaging, and laboratory characteristics and neurologic outcomes.ResultsWe identified 46 cohort studies including a total of 911 patients (41% biopsy confirmed, 43% angiogram confirmed, and 16% without clear assignment to the diagnostic procedure). The most frequent onset symptoms were focal neurologic signs (63%), headache (51%), and cognitive impairment (41%). Biopsy- compared with angiogram-confirmed cases had higher occurrences of cognitive impairment (55% vs 39%) and seizures (36% vs 16%), whereas focal neurologic signs occurred less often (56% vs 95%). CSF abnormalities were present in 75% vs 65% and MRI abnormalities in 97% vs 98% of patients. Digital subtraction angiography was positive in 33% of biopsy confirmed, and biopsy was positive in 8% of angiogram-confirmed cases. In 2 large cohorts, mortality was 23% and 8%, and the relapse rate was 30% and 34%, during a median follow-up of 19 and 57 months, respectively. There are no randomized trials on the treatment of PACNS. The initial treatment usually includes glucocorticoids and cyclophosphamide.DiscussionPACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown.

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Primary angiitis of the central nervous system: 2 atypical cases

Cited by 11 publications

References 22 publications

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

Systematic Overview of Aristolochic Acids: Nephrotoxicity, Carcinogenicity, and Underlying Mechanisms

Primary Angiitis of the CNS

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