Abstract
Cerebral amyloid angiopathy related inflammation (CAA-RI) is a rare form of CAA characterized by subacute encephalitic symptoms (cognitive decline, seizures, focal deficits) associated with extensive and confluent white matter lesions co-localizing with lobar microbleeds on brain MRI. We report two cases of unusual CAA-RI mimicking meningoencephalitis but without typical brain lesions on FLAIR and T2* sequences. These 2 cases may extend the clinical spectrum of CAA-RI by suggesting the possible occurrence of quite purely meningeal forms of CAA-RI.
Highlights
Cerebral amyloid angiopathy related inflammation (CAA-RI) is a very rare disorder resulting from vascular and/or perivascular inflammation in the close vicinity of Aβ deposits
We report two cases of CAA-RI with prominent meningeal involvement and no parenchymal lesion on brain MRI, whose diagnosis was long delayed
In the present two cases, the combination of progressive headache associated with fever and confusion, in the total absence of visible parenchymal abnormality on CTscan, but with pleiocytosis in the cerebrospinal fluid led to suspect initially an infectious meningoencephalitis
Summary
Cerebral amyloid angiopathy related inflammation (CAA-RI) is a very rare disorder resulting from vascular and/or perivascular inflammation in the close vicinity of Aβ deposits. The clinical presentation of CAA-RI is characterized by acute to subacute encephalitic symptoms (confusion, rapidly cognitive decline, focal neurological deficits, seizures, and/or headaches) [1]. The MRI pattern is stereotyped with extensive, confluent, more or less symmetric white matter lesions that most often co-localize with cortical microbleeds. A leptomeningeal or parenchymal contrast enhancement may occasionally occur [2, 3]. We report two cases of CAA-RI with prominent meningeal involvement and no parenchymal lesion on brain MRI, whose diagnosis was long delayed
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