Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome

Abstract

In the absence of histological data, early recognition of the clinical symptoms and typical radiologic features of CAA-related inflammation is essential to enable timely establishment of proper treatment.

The Inflammatory Form of Cerebral Amyloid Angiopathy or “Cerebral Amyloid Angiopathy-Related Inflammation” (CAARI)

“…Martucci and colleagues [20] reported 10 patients with CAARI diagnosed by the Chung criteria. In their report, 7/ 10 patients were women, and the average age was 75 years.…”

Sporadic cerebral amyloid angiopathy as a cause of relapsing lobar hemorrhage, convexal subarachnoid hemorrhage and cortical superficial siderosis

“…The better lesion/tissue contrast achieved by the suppression of the signal intensity of cerebrospinal fluid on the FLAIR sequence, not only in the subarachnoid space, but also in the cerebral parenchyma, can be especially useful for the evaluation of CAA-related white matter lesions and SAH. In addition to these sequences, DW sequence, with apparent diffusion coefficient (ADC) maps can be useful to distinguish CAA-related silent infarctions from other white matter lesions, including vasogenic edema and leukoaraiosis [2][3][4][5]. In our patient, FLAIR sequence showed a new SAH recurred in the right rolandic cortical sulcus that would have gone hardly recognized without the execution of that sequence.…”

“…Microinfarcts are predominantly lobar (cortical-subcortical), usually in patients with severe CAA. One possible mechanism for these ischemic lesions is occlusion or reduced perfusion in amyloid laden cortical vessels affected by CAA [3,4]. CAA-related intracerebral hemorrhages (ICH) increased with age.…”

“…Amyloid deposits have been also observed in the capillaries of central nervous system, parenchyma and leptomeninges [2]. CAA mostly occurs in the sporadic form in the elderly, while rare familial forms occur in younger patients and are generally lead to more severe clinical manifestations [3][4][5]. CAA is considered the third most common cause of spontaneous intracerebral hemorrhage after hypertension and subarachnoid aneurysmal hemorrhage [1][2][3].…”

“…CAA is considered the third most common cause of spontaneous intracerebral hemorrhage after hypertension and subarachnoid aneurysmal hemorrhage [1][2][3]. The formation and deposition of insoluble oligomeric structures, trigger a cascade of events including release of inflammatory components, activation of the complement system, oxidative stress, alteration of the blood-brain barrier permeability, and cell toxicity [3,4]. At the initial stage, the vessel wall structure is intact, but as the disease progresses, there is pan-mural amyloid accumulation and loss of smooth muscle cells.…”