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Updated: May 7, 2026
Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
Published on: December 6, 2016
[Amyloid β-related angiitis (ABRA) with dural contrast enhancement]
Daichi Imamura1, Junko Ishii1, Satoshi Hosoki1
1Department of Neurology, Kobe City Medical Center General Hospital.
Abstract:
An 82-year-old woman presented with a rapidly progressive decline in cognitive function, which had previously been worsening over several months. Brain MRI revealed a lesion predominantly involving the leptomeninges of the right parietal lobe. Based on the presence of leptomeningeal-predominant lesions, cerebral microbleeds, and superficial siderosis, amyloid β-related angiitis (ABRA) was suspected. However, due to atypical findings such as dural enhancement, we performed a brain biopsy, leading to a definitive diagnosis of ABRA. Steroid therapy resulted in improvements in both clinical symptoms and imaging findings. Imaging findings in this case suggest that inflammation originating in the leptomeninges extended bidirectionally into both the brain parenchyma and the dura mater. This progression pattern is of particular interest for understanding the pathophysiology of ABRA and may offer a future possibility of avoiding brain biopsy by identifying characteristic imaging features.
Insights
Amyloid β-related angiitis (ABRA) can cause cognitive decline. This case highlights inflammation spreading from leptomeninges to brain and dura, potentially aiding diagnosis without biopsy.
Area of Science:
- Neurology
- Neuroradiology
- Pathology
Background:
- Amyloid β-related angiitis (ABRA) is an inflammatory condition affecting cerebral blood vessels.
- It can lead to cognitive decline and neurological deficits.
- Leptomeningeal involvement is a key feature, but atypical presentations can occur.
Purpose of the Study:
- To report a case of ABRA with unusual dural enhancement.
- To investigate the imaging characteristics and inflammatory spread in ABRA.
- To explore potential non-invasive diagnostic markers for ABRA.
Main Methods:
- Clinical presentation of an 82-year-old woman with rapidly progressive cognitive decline.
- Brain MRI demonstrating leptomeningeal lesions, microbleeds, and superficial siderosis.
- Brain biopsy for definitive diagnosis and histopathological confirmation of ABRA.
- Assessment of treatment response to steroid therapy.
Main Results:
- The patient presented with cognitive decline and MRI findings suggestive of ABRA.
- Atypical dural enhancement prompted a brain biopsy, confirming ABRA.
- Steroid therapy led to significant clinical and radiological improvement.
- Imaging suggested bidirectional inflammation from leptomeninges to parenchyma and dura.
Conclusions:
- This case illustrates an unusual pattern of inflammation in ABRA extending into the dura.
- Understanding this progression may help identify characteristic imaging features.
- Future research could focus on non-invasive methods to diagnose ABRA, potentially avoiding brain biopsy.
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