Learning objectives
- To know and recognize the clinical presentation of cerebral amyloid angiopathy-related inflammation (CAA-ri).
- To describe the CT and MRI imaging findings of CAA-ri.
- To make an adequate differential diagnosis in order to provide proper therapy that may improve the clinical outcome.
Background
Cerebral amyloid angiopathy (CAA) is a common condition,
usually sporadic,
present in 10-40% of elderly brains and its prevalence can be higher than 80% in Alzheimer disease (AD).
CAA is responsible for 10-20% of lobar intracerebral haemorrhages (ICH) and it is considered the most frequent non-traumatic cause of ICH in non-hypertensive patients [1].
CAA is a cerebrovascular disorder caused by deposition of amyloid-β protein in the tunica media and adventitia of cerebral blood vessels,
especially in both medium- and small-sized.
This leads to a vascular...
Findings and procedure details
The pathological substrate of CAA-ri is the presence of an inflammatory response induced by the deposition of amyloid-β protein.
It can be categorized into two pathologic subtypes: a non-destructive inflammatory infiltration called CAA-related inflammation (CAA-ri) and a transmural inflammatory infiltrate with or without granuloma called amyloid β-related angiitis (ABRA) [8].
However,
both entities have similar clinical and radiologic features and cannot be distinguished on neuroimaging [9].
The main radiological feature of CAA-ri is the onset of vasogenic oedema arising on previous haemorrhagic foci (Fig. 2)...
Conclusion
CAA-ri is a recently described inflammatory form of CAA characterized by the onset of areas of vasogenic oedema associated to previous cortical or cortical-subcortical bleeds.
It has a poor prognosis,
if not identified early and treated appropriately.
Early radiological pattern recognition is extremely important to achieve an imaging-based diagnosis,
avoid brain biopsy and establish proper treatment that improves the outcome of the patient.
Personal information
Dr.
Luis Quintana Barriga
Resident in training.Department of Radiology,
Hospital Universitario Virgen del Rocío.
Manuel Siurot,
s/n,
41013 Sevilla,
Spain.
email:
[email protected]
Dr.
Rafael F.
Ocete Pérez
Neuroradiology Section.
Department of Radiology,
Hospital Universitario Virgen del Rocío.
Manuel Siurot,
s/n,
41013 Sevilla,
Spain.
email:
[email protected]
References
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Yamada M.
Cerebral Amyloid Angiopathy: Emerging Concepts.
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2015;17(1):17.
van Rooden S,
van der Grond J,
van den Boom R,
Haan J,
Linn J,
Greenberg SM,
et al.
Descriptive analysis of the Boston criteria applied to a Dutch-type cerebral amyloid angiopathy population.
Stroke.
2009;40(9):3022–7.
Martucci M,
Sarria S,
Toledo M,
Coscojuela P,
Vert C,
Siurana S,
et al.
Cerebral amyloid angiopathy-related inflammation: Imaging findings and clinical outcome.
Neuroradiology....