Mainly subarachnoid amyloid angiopathy with pseudotumoral course

Franco-Macías, Emilio; Cerdá-Fuertes, Nuria; Rivas‐Infante, E.; Roldán-Lora, Florinda; Avila-Polo, R.; Moniche, Francisco

doi:10.1016/j.clineuro.2015.12.006

Cited by 2 publications

(2 citation statements)

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“…This tumefactive mass-like aspect has been described in the literature in 15% of CAA-RI and 14% of CAA without inflammation [ 1 ]. Most of these latter are probably inflammatory forms of CAA, but a patchy distribution of perivascular lymphocytic infiltrates [ 7 ], or the possible disappearance of inflammatory infiltrates due to onset of steroid treatment before the performance of cerebral biopsy, could lead to misdiagnosis of CAA-RI as without inflammation [ 11 ]. In a more recent review of CAA-RI, Danve et al [ 9 ] reported that up to 26% of CAA-RI cases had mass-like lesions that were usually asymmetrical and either non-enhancing or minimally enhancing.…”

Section: Resultsmentioning

confidence: 99%

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature

Roca

Gonzalez

Bala

et al. 2021

Psychiatry Investig

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Objective Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare and potentially treatable encephalopathy that usually affects people older than 50 years old and has an acute or subacute clinical presentation characterized by rapidly evolving cognitive decline, focal deficits and seizures. In a small subset of patients the disease can adopt a pseudotumoral form in the neuroimages that represents a very difficult diagnostic challenge. Methods Here in we report a patient with a tumour-like presentation of histopathologically confirmed CAA-RI. Results We also conducted a search and reviewed the clinical and radiological features of 41 cases of pseudotumoral CAA-RI previously reported in the literature in order to identify those characteristics that should raise diagnostic suspicions of the disease, there by avoiding unnecessary surgical treatments. ConclusionThe therapy of CAA-RI with steroids is usually effective and clinical and radiological remission can be achieved in the first month in approximately 70% of cases.

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Section: Resultsmentioning

confidence: 99%

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature

Roca

Gonzalez

Bala

et al. 2021

Psychiatry Investig

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“… 37 42 However, early steroid treatment may obscure evidence of an inflammatory response in subsequent biopsy specimens, which may have happened to our patient 1. 96 Moreover, a negative biopsy does not exclude an iCAA diagnosis due to the segmental distribution of pathological changes.…”

Section: Diagnosismentioning

confidence: 99%

Inflammatory Cerebral Amyloid Angiopathy: A Broad Clinical Spectrum

Souza

Tasker

2023

J Clin Neurol

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Cerebral amyloid angiopathy (CAA) is a common central nervous system (CNS) vasculopathy, which in some cases is associated with subacute encephalopathy, seizures, headaches, or strokes due to vascular inflammation directed against vascular amyloid accumulation. The pathological subtypes of inflammatory CAA include CAA-related inflammation (CAAri) with mostly perivascular lymphocytic infiltrates, or amyloid-beta (Aβ)-related angiitis (ABRA) with transmural granulomatous inflammation. CAAri and ABRA probably represent part of the spectrum of CNS vasculopathies, intermediate between CAA and primary CNS vasculitis, and they are closely related to Aβ-related imaging abnormalities and other manifestations of an inflammatory response directed against Aβ in the leptomeninges and cerebral parenchyma. As treatment strategies in Alzheimer's disease shift toward potentially effective antiamyloid immunotherapy, the incidence rate of inflammatory CAA (which is probably an underrecognized condition) is likely to increase. Its clinical features are varied and include subacute encephalopathy, behavioral symptoms, headaches, seizures, and focal neurological deficits, which necessitate a high degree of suspicion for this disorder that often responds to treatment. The recent definition of the typical clinical and radiological syndrome has increased its recognition and may eliminate the need for invasive histological sampling in at least some affected patients. Here we review the pathophysiology, clinical spectrum, and approach to diagnosis, and discuss illustrative cases that highlight the wide range of clinical presentations.

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Mainly subarachnoid amyloid angiopathy with pseudotumoral course

Cited by 2 publications

References 5 publications

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature

Inflammatory Cerebral Amyloid Angiopathy: A Broad Clinical Spectrum

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