Primary Angiitis of the Central Nervous System: A Report of Three Cases from a Single Colombian Center

Coronel-Restrepo, Nicolás; Bonilla-Abadía, Fabio; Cortes, Omar A.; Izquierdo, Jorge Hernán; Shinchi, A. Masaru; Bravo, Juan Carlos; Tobón, Gabriel J.; Cañas, Carlos A.

doi:10.1155/2013/940438

Cited by 10 publications

(11 citation statements)

References 11 publications

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“…Unlike our case, segmental granulomatous changes typical of granulomatous angiitis of the CNS (GACNS), was not observed [5,6]. The case by Ho et al reported presence of granulomatous findings supporting histopathological criteria for PACNS/GACNS [3].…”

Section: Accepted Manuscriptsupporting

confidence: 49%

Unihemispheric cerebral vasculitis: Case report and review of literature

Johnson

Jakubek

Hawley³

2016

Journal of the Neurological Sciences

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Section: Accepted Manuscriptsupporting

confidence: 49%

Unihemispheric cerebral vasculitis: Case report and review of literature

Johnson

Jakubek

Hawley³

2016

Journal of the Neurological Sciences

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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%

“…Salvarani et al report a median dose of 1 g and Oon et al a median dose of 800–1000 mg/mo 11 , 35 Oral cyclophosphamide was administered in 9 of 30 studies and was given daily with a median starting dose of 150 mg/day and a median treatment duration of 7 months, with a median starting dose of 100 mg/day and a median treatment duration of 10 months, or with 2 mg/kg/day and a treatment duration of 6 months. 11 , 25 , 34 , 43 , 49 , 52 Five studies did not report whether glucocorticoids or cyclophosphamide were given orally or IV. 3 , 37 , 38 , 47 , 48 Other induction treatments include cyclophosphamide alone or combinations of glucocorticoids with azathioprine, mycophenolate mofetil, rituximab, or methotrexate.…”

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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“…The symptomatology in those with predominant involvement of small vessels includes seizures, dyskinesia, and altered consciousness, while the medium/large vessel variant commonly generates focal symptoms 8 . Sartiet al 3 Angiographic MRI abnormalities are present in 90% of cases, mainly ischemic ones in 59% 9,10 . Multiple cortical and subcortical infarcts (63%) are often detected bilaterally (83%) 1,11 , as in this report in which there were lesions in both cerebellar hemispheres.…”

Section: Discussionmentioning

confidence: 98%

Diagnostic Challenge in Middle-Aged Woman With Recurrent Ischemic Strokes: A Case of Primary Central Nervous System Vasculitis and Literature Review

Raposo,

Augsten,

Lopes

2024

Rev. Contemp.

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Primary vasculitides of the central nervous system are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the central nervous system. Its spectrum extends from primary angiitis of the central nervous system to cerebral amyloid angiopathy. This case portrays a 46-year-old woman with a history of multiple strokes. History of multiple comorbidities such as systemic arterial hypertension, prediabetes, and smoking. During follow-up after the second ischemic event, a patent foramen ovale was detected with positive microbubbles stand. Therefore, she was using oral anticoagulants. Transthoracic echocardiogram and magnetic resonance angiography without relevant changes. However, she resorted to the neurological deficit three more times. Cerebrospinal fluid (CSF) analysis was done twice; both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. Discrimination of angiitis and reversible cerebral vasoconstriction syndrome was challenging and based on the demographic and clinical features of the patient. The characteristics of this case that corroborate the hypothesis of angiitis are the patient's age, which is in the median age range (40-60 years), chronic headache, and focal symptoms not temporally associated with the headache. Intravenous methylprednisolone pulse therapy (one gram/day) was performed for five days. Evolved with progressive improvement of the motor deficit. On hospital discharge, it was prescribed prednisone, and after ten days, Azathioprine 2.5mg/Kg/day was started. Diagnosis of primary central nervous system vasculitis requires high clinical suspicion due to the low specificity of the clinical features. This report reinforces the importance of investigating CNS vasculitis in young patients with a history of recurrent stroke.

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Primary Angiitis of the Central Nervous System: A Report of Three Cases from a Single Colombian Center

Cited by 10 publications

References 11 publications

Unihemispheric cerebral vasculitis: Case report and review of literature

Unihemispheric cerebral vasculitis: Case report and review of literature

Primary Angiitis of the CNS

Diagnostic Challenge in Middle-Aged Woman With Recurrent Ischemic Strokes: A Case of Primary Central Nervous System Vasculitis and Literature Review

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