Cerebral amyloid angiopathy associated with inflammation: A systematic review of clinical and imaging features and outcome

Abstract

Background Cerebral amyloid angiopathy associated with inflammation is an increasingly recognized condition, characterized by an inflammatory response to the vascular deposits of β-amyloid within the brain that are the hallmark of cerebral amyloid angiopathy. Two main patterns of this inflammatory response have been identified to date: one involving a perivascular inflammatory cell infiltrate (cerebral amyloid angiopathy-related inflammation); the other a transmural vasculitic process (A-beta related angiitis). Unlike cerebral amyloid angiopathy itself, which predisposes to intracerebral hemorrhage and has no known treatment, cerebral amyloid angiopathy associated with inflammation typically presents in diverse ways and diagnosis may be challenging and delayed. Aims We sought to summarize the clinical features, imaging appearances and available data on outcome and treatment responses, using information derived from a systematic review of pathologically proven cases of cerebral amyloid angiopathy associated with inflammation. Summary of review We identified 213 distinct pathologically proven cases of cerebral amyloid angiopathy-related inflammation/A-beta related angiitis, from 104 publications. The clinical presentation, imaging features, pathology, treatment, and outcomes of cerebral amyloid angiopathy associated with inflammation are described. Conclusions Cerebral amyloid angiopathy associated with inflammation is an important and increasingly recognized clinical condition, which affects the older patient population and presents most commonly with cognitive decline, seizures, and headaches. Future research is required to develop and validate diagnostic criteria and determine optimum treatment strategies.

Early onset cerebral amyloid angiopathy following childhood exposure to cadaveric dura

“…This latter imaging finding is similar to changes observed in patients with the inflammatory variant of CAA (CAArelated inflammation), 11,12 which is thought to occur as a consequence of spontaneously generated anti-Aβ autoantibodies 13 and bears a close resemblance to the amyloidrelated imaging abnormalities (ARIA) that can occur after treatment with anti-Aβ immunotherapy. This latter imaging finding is similar to changes observed in patients with the inflammatory variant of CAA (CAArelated inflammation), 11,12 which is thought to occur as a consequence of spontaneously generated anti-Aβ autoantibodies 13 and bears a close resemblance to the amyloidrelated imaging abnormalities (ARIA) that can occur after treatment with anti-Aβ immunotherapy.…”

“…The characteristic clinicoradiological presentation should help guide noninvasive diagnosis in at-risk individuals; all patients had prominent recurrent generalized tonic-clonic seizures at or shortly after presentation, and 2 of 3 had abnormal cortical and sulcal signal changeboth atypical features in sporadic late onset CAA. This latter imaging finding is similar to changes observed in patients with the inflammatory variant of CAA (CAArelated inflammation), 11,12 which is thought to occur as a consequence of spontaneously generated anti-Aβ autoantibodies 13 and bears a close resemblance to the amyloidrelated imaging abnormalities (ARIA) that can occur after treatment with anti-Aβ immunotherapy. 14 Both ARIA and CAA-related inflammation can be either mildly symptomatic or asymptomatic, [15][16][17] leading to speculation that this might represent physiological attempts at Aβ clearance.…”

Nationwide survey on cerebral amyloid angiopathy in Japan

“…The average age of disease onset indicated in our study was similar to that reported in previous studies . The most common features were cognitive decline, seizure and headache . In this study, cognitive impairment and headache were the most frequent initial manifestations.…”

“…The predilection of CAA-related ICH, especially symptomatic macrohemorrhage, could not be explained by the distribution of CAA, whereas cortical microhemorrhages are compatible with the severity of CAA, suggesting that CAA-related The average age of disease onset indicated in our study was similar to that reported in previous studies [21]. The most common features were cognitive decline, seizure and headache [6,21]. In this study, cognitive impairment and headache were the most frequent initial manifestations.…”

Clinical Reasoning: Rapid progression of reversible cognitive impairment in an 80-year-old man

“…Interestingly, small asymptomatic DWI ischemic lesions have been described recently in patients with CAA over the course of the disease. 3,4…”