Primary Angiitis of the Central Nervous System

Powers, William J.

doi:10.1016/j.ncl.2014.12.004

Cited by 39 publications

(32 citation statements)

References 77 publications

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“…Angiography revealed very mild focal callosal abnormalities and histological analysis casted doubts about the possibility of a dimorphic fungus, as there were no signs of vessel wall damage. 7 Considering the catastrophic clinical worsening, amphotericin was started, steroids were tapered and a fast and dramatic clinical and radiological improvement ensued, and this argued against a possible spontaneous remission or a delayed steroid effect. 4,8 Electron microscopy confirmed the presence of corpora amylacea-like bodies, described by Susac et al 9 and these were associated with antifungal antibodies.…”

Section: Discussionmentioning

confidence: 99%

Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

Gil‐Gouveia¹,

Marto²,

Vilela³

et al. 2019

Acta Med Port

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Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

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Section: Discussionmentioning

confidence: 99%

Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

Gil‐Gouveia¹,

Marto²,

Vilela³

et al. 2019

Acta Med Port

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“…Conventional angiography is better than MRA or CT angiography9 but with a sensitivity of 60%–70% and specificity of 30% for CNS vasculitis 10. In 59 patients, only 19% with angiographic vasculitis were confirmed to have it 11. Therefore, angiography alone is insufficient for diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis

et al. 2019

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A 68-year-old man, with a history of non-Hodgkin’s lymphoma in remission, was admitted for homonymous hemianopsia, headaches and subacute progressive cognitive decline. Imaging revealed brain infarcts and angiography suggested vasculitis. A brain biopsy, however, revealed an intravascular large B-cell lymphoma (IVLBL). Central nervous system (CNS) vasculitis and IVLBL of the brain are extremely rare diseases that can have an almost identical clinical presentation. Angiographic findings are very similar but usually are reported as compatible with vasculitis. Brain biopsy or a random skin biopsy are crucial in diagnosing IVLBL as the accuracy of angiographic findings for CNS vasculitis is low.

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“…Antiphospholipid syndrome (APL) was diagnosed based on the revised international criteria for definite antiphospholipid syndrome [10]. The diagnosis of CNS sarcoidosis, primary CNS angiitis was confirmed by positive biopsy [11, 12]. …”

Section: Methodsmentioning

confidence: 99%

Cerebrospinal fluid humoral immunity in the differential diagnosis of multiple sclerosis

et al. 2017

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BackgroundThe diagnostic accuracy of cerebrospinal fluid oligoclonal bands (CSF-OCB) detected by isoelectric focusing (IEF) in patients with multiple sclerosis (MS) was evaluated in our study.MethodsThree hundred and twenty-one patients with MS and other central nervous system (CNS) immune mediated disorders were assessed (CIMD). Cerebrospinal fluid and matched serum samples were examined for the presence of OCB by IEF-IB (isoelectric focusing with immunoblotting).ResultsIsolated oligoclonal bands (ISO-OCB) were the only predictor of MS diagnosis independent of age, gender and CSF-OCB. ISO-OCB ≥ 3.5 detected by IEF yielded a sensitivity of 98% and specificity of 87% in distinguishing MS from MS mimickers.ConclusionsFor the neurologist, a score of ≥ 4 ISO-OCB supports the diagnosis of MS. On the other hand, ISO-OCB ≤3 favors CIMD. Further studies with larger population samples are warranted to confirm these findings.

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Primary Angiitis of the Central Nervous System

Cited by 39 publications

References 77 publications

Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis

Cerebrospinal fluid humoral immunity in the differential diagnosis of multiple sclerosis

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