Imaging spectrum of sporadic cerebral amyloid angiopathy: multifaceted features of a single pathological condition

Abstract

ObjectivesSporadic cerebral amyloid angiopathy (CAA) is common cause of cerebrovascular disorders that predominantly affect elderly patients. When symptomatic, cortical-subcortical intracerebral haemorrhage (ICH) in the elderly is the most well-known manifestation of CAA. Furthermore, the clinical presentation varies from a sudden neurological deficit to seizures, transient symptoms and acute progressive cognitive decline. Despite its clinical importance, this multifaceted nature poses a diagnostic challenge for radiologists. The aims of this study were to expound the characteristics of neuroimaging modalities, which cover a wide spectrum of CAA-related imaging findings, and to review the various abnormal findings for which CAA could be responsible.ConclusionsRadiologically, in addition to typical ICH, CAA leads to various types of abnormal findings, including microbleed, subarachnoid haemorrhage, superficial siderosis, microinfarction, reversible oedema, and irreversible leukoaraiosis. Taking into consideration the clinical importance of CAA-related disorders such as haemorrhagic risks and treatable oedema, it is necessary for radiologists to understand the wide spectrum of CAA-related imaging findings.Teaching Points• To describe the characteristics of imaging modalities and findings of CAA-related disorders.• MRI, especially gradient echo sequences, provides the useful information of CAA-related haemosiderin depositions.• To understand the wide spectrum of CAA-related neuroimaging and clinical features is important.

Delayed Identification of Cortical Superficial Siderosis in a Patient with Recurrent Transient Focal Neurological Symptoms: A Case Report

“…The clinical relevance of cSS is still being investigated, but its association with CAA and ICH risk is becoming better understood. A recent study demonstrated that cSS was found by MRI investigation in 44.1% of patients with probable CAA based on the modified Boston criteria ( Table 1) [5], that presented with cognitive impairment and transient focal neurological symptoms. Further, cSS progression was found to independently predict increased symptomatic ICH risk [6].…”

Clinical Neuropathological Analysis of 10 Cases of Cerebral Amyloid Angiopathy-Related Cerebral Lobar Hemorrhage

“…The distribution of CAA-related CLH indicated that certain characteristics of arteries, such as their branching patterns or susceptibility to arteriosclerosis, might affect their susceptibility to CAA-induced vascular bleeding disease 14) . The discrepancies between the CAA-related CLH distributions reported in in vivo MRI studies and at autopsy must be examined with extensive clinical and pathological studies 34) .…”

Cortical Superficial Siderosis of the Central Nervous System – An Overview

“…14 Focal seizures and migrainous auras may also be presenting symptoms. 15 Patients with AD associated to cSS performed worse in the minimental state examination than those without cSS. 16 In a memory clinic population the incidence of cSS is higher in patients with AD than in those with mild cognitive impairment and other dementia types.…”