"The term "inflammatory cerebral amyloid angiopathy" can be used as an umbrella term encompassing two subtypes: cerebral amyloid angiopathy-related inflammation (CAA-ri) and amyloid β-related angiitis (ABRA) 2,6. However, many authors interchange the terms "cerebral amyloid angiopathy-related inflammation" and "inflammatory cerebral amyloid angiopathy," either encompassing of amyloid β-related angiitis 8 or in distinction to it 3. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10 or amyloid β-related angiitis alone 7 without mention of the other."
"Clinical presentation "
"The term "inflammatory cerebral amyloid angiopathy" can be used as an umbrella term encompassing two subtypes: cerebral amyloid angiopathy-related inflammation (CAA-ri) and amyloid β-related angiitis (ABRA) 2,6. However, many authors interchange the terms "cerebral amyloid angiopathy-related inflammation" and "inflammatory cerebral amyloid angiopathy," either encompassing of amyloid β-related angiitis 8 or in distinction to it 3. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10 or amyloid β-related angiitis alone 7 without mention of the other."
"The term "inflammatory cerebral amyloid angiopathy" can be used as an umbrella term encompassing two subtypes: cerebral amyloid angiopathy-related inflammation (CAA-ri) and amyloid β-related angiitis (ABRA) 2,6. However, many authors interchange the terms "cerebral amyloid angiopathy-related inflammation" and "inflammatory cerebral amyloid angiopathy," either encompassing of amyloid β-related angiitis 8 or in distinction to it 3. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10 or amyloid β-related angiitis alone 7 without mention of the other."
"Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous system, cerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9."
"Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous system, cerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9."
"Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous system, cerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9."
"Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous system, cerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9."
"Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous system, cerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9."
"The term "inflammatory cerebral amyloid angiopathy" can be used as an umbrella term encompassing two subtypes: cerebral amyloid angiopathy-related inflammation (CAA-ri) and amyloid β-related angiitis (ABRA) 2,6. However, many authors interchange the terms "cerebral amyloid angiopathy-related inflammation" and "inflammatory cerebral amyloid angiopathy," either encompassing of amyloid β-related angiitis 8 or in distinction to it 3. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10 or amyloid β-related angiitis alone 7 without mention of the other."
Expected headings
"Possible"
"Probable"
"Proposed changes"
"Clinical presentation "
"White matter lesions"
"Haemorrhagic lesions"
"Leptomeningeal/vascular involvement"
"MRI with white matter hyperintensities (unifocal or multifocal, corticosubcortical or deep) that extend to the immediately subcortical white matter"
"Classically, CT and MRI demonstrate an area of vasogenic oedema involving the subcortical white matter 1. Lesions are usually unifocal but multifocal involvement is occasionally present at the time of diagnosis (~30%) 1. It should be noted, however, that inflammatory cerebral amyloid angiopathy also involves leptomeningeal vessels and in some cases the majority of radiographic changes may be leptomeningeal rather than parenchymal 16."
"A significant proportion of patients respond readily to treatment with corticosteroids, with or without another immunosuppressive medication (e.g. cyclophosphamide) 15, with improvement evident within a week or two of commencement of treatment. In fact, in a subgroup of patients, spontaneous remission is encountered 1. In the remainder, which accounts for 60% of all affected individuals, even with treatment, severe disability or death are encountered 2. Even with immunosuppression, approximately 25% of patients die within one year 15."
"Inflammatory cerebral amyloid angiopathy is nearly always seen in the context of sporadic cerebral amyloid angiopathy, but has rarely been reported in iatrogenic cerebral amyloid angiopathy as well 20,22.Brain biopsy remains the definitive diagnostic tool, revealing perivascular and intramural inflammatory infiltrates, fibrinoid deposits, and granulomatous changes affecting both leptomeninges and cortical vessels 3,4.Histopathology is further characterised by positive Congo red staining and beta-amyloid immunoreactivity, confirming amyloid deposition 3,4."