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

Abstract

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.

Conclusion: The 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.

Keywords: Cerebral amyloid angiopathy; Neoplasm; Pseudotumoral; Tumor-like.

Figure 1.

Left anterior temporal lesion with mass effect shows high signal in T2 W (A) and FLAIR (B) and hypointensity in T1W, with lack of contrast enhancement (C). High signal in DWI sequence (D) and ADC map (not shown) is due to vasogenic edema. Scattered intralesional foci of microhemorrhages can be seen in SWI (E). C-PiB PET: Extensive and bilateral cortical deposits of Beta amyloid (F). MRI: magnetic resonance imaging, DWI: diffusion-weighted magnetic resonance imaging, FLAIR: T2-weighted-Fluid-Attenuated Inversion Recovery, ADC map: apparent diffusion coefficient, C-PiB PET: C-Pittsburgh compound B Positron Emission Tomography.

Figure 2.

Brain biopsy, hematoxylin-eosin stain where it is observed. A: Homogeneous eosinophilic thickening of the vascular wall. B: Eosinophilic thickening of the parietal wall with peri adventitial hemosiderin deposits (old microhemorrhage). C: Positive immune staining of β amyloid in the vascular walls (black arrow) and in plaques within the parenchyma.