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. 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.

Inflammatory Cerebral Amyloid Angiopathy: A Broad Clinical Spectrum

“…Intrathecal production of these antibodies was demonstrated, and antibody titers have been shown to decrease after steroid therapy. 11 59 60 61 62 Piazza et al 60 found that antibody titers tend to be correlated with clinical and imaging findings. These autoantibodies could potentially serve as biomarkers for iCAA in the future, which would discriminate this condition from noninflammatory CAA or from other neuroinflammatory disorders and even help in monitoring the treatment response, although further studies are needed to confirm this.…”

“… 36 Three-quarters of patients presented with acute or subacute encephalopathy with or without focal neurological signs (46%–58%) and headache (22%–41%), with seizures in one-third. 8 11 14 23 24 27 29 30 31 36 37 39 54 80 83 Constitutional symptoms are often absent, although patients may complain of dizziness or nausea. 3 Immune-mediated vascular function changes are believed to be responsible for iCAA symptoms, and induce relatively rapid cognitive and/or functional decline over weeks to months, which are factors in the differential diagnosis for rapidly progressing dementia syndrome.…”

“… 3 Relevant focal deficits identified included visual symptoms (14%), aphasia (14%–26%), ataxia (7%), and hemiparesis (16%–20%). 11 22 27 37 47 The presentation of ICH is not typical, unlike in noninflammatory CAA. 8 23 39 …”

“… 37 A mass lesion (namely amyloidoma) is seen in about 15% of patients with iCAA, and biopsy is often required to differentiate the inflammatory process from a tumor. 8 11 21 29 81 94 A mass effect is prominent, but there is typically no leptomeningeal enhancement. 21 As in other types of iCAA, the colocalization of CMBs and white-matter signal changes contribute to making the correct diagnosis, which must be considered to avoid unnecessary surgical procedures.…”

“…Aβ was first identified in the brain in 1909, and this eosinophilic, insoluble, extracellular protein is also present in the senile plaques of Alzheimer’s disease (AD); indeed, pathological evidence of CAA is present in 80%–94% of brains with concomitant AD. 1 4 5 6 7 8 9 10 11 12 Loss of vessel wall elasticity and increased fragility due to amyloid deposition leads to vessel rupture. 1 13 Typically considered to produce cerebral microbleeds (CMBs) and larger lobar intracerebral hemorrhages (ICHs), the clinical spectrum of CAA has recently expanded to include nonhemorrhagic manifestations including transient focal neurological episodes, chronic cognitive decline and dementia, small cortical infarcts, cortical atrophy, and leukoencephalopathy.…”