Pseudotumoral presentation of cerebral amyloid angiopathy–related inflammation

Abstract

The identification of one or several nonenhancing space-occupying lesions, especially in elderly patients presenting with cognitive impairment, should raise suspicion for the pseudotumoral presentation of CAA-I and lead to T2*-GRE sequences. Perfusion MRI and MRS appear to be useful techniques for the differential diagnosis of this entity.

Cerebral amyloid angiopathy-related inflammation with epilepsy mimicking a presentation of brain tumor: A case report and review of the literature

“…CAA is generally associated with intracerebral hemorrhage, dementia, and small cerebral infarctions in the elderly population, but in a small proportion of cases is related to inflammatory responses to vascular deposits of Aβ, as so-called CAA-ri [ [1] , [2] , [3] , [4] , 11 ]. CAA-ri generally presents with subacute cognitive decline, mild headaches and new-onset seizures, rather than the chronic types of dementia or hemorrhagic strokes associated with CAA.…”

“…Microscopically, the histopathological characteristics of CAA-ri demonstrate amyloid deposition within vessel walls and perivascular, transmural, or intramural inflammatory changes with or without granuloma formation [ 3 , 4 , 8 , 13 ]. These findings are basically consistent with CAA and the details are as follows.…”

“…In the present case, we used DFS staining to detect amyloid protein in addition to Congo red staining. Moreover, the diagnosis of CAA-ri requires clear histological findings of angiitis of the central nervous system in addition to the findings mentioned above [ [2] , [3] , [4] , 16 ]. Characteristically, perivascular inflammatory cells mostly comprise monocyte/microglial cells (positive staining for CD68).…”

“…Cerebral amyloid angiopathy (CAA), a common small vessel disease of the brain, is characterized by progressive deposition of β-amyloid (Aβ) protein in the walls of small to medium-sized arteries and capillaries in the cerebral cortex and overlying leptomeninges [ [1] , [2] , [3] , [4] ]. The typical presentation of CAA is spontaneous lobar intracerebral hemorrhage in an elderly patient [ 2 , 4 ]. However, CAA can also manifest as subacute cognitive impairment, dementia, transient neurological symptoms, or epilepsy [ 1 ].…”

“…Cognitive and behavioral changes are the most common symptoms of CAA-ri, followed by focal neurological signs, headache, and seizures [ 3 , 5 ]. In addition to this, CAA-ri can also mimic brain tumor from clinical and radiological point of view [ 3 , 4 , 8 , 9 ]. In this paper, we report an elder case of nonconvulsive status epilepticus (NCSE) due to CAA-ri in the right occipital lobe.…”