Cerebral Amyloid Angiopathy-Related Inflammation
Year: 2025
Type: article
Study design: Case Report
Abstract: A 73-year-old man presented with acute cognitive decline, visual hallucination, and recurrent attacks of right limb weakness for several days. He was unable to read newspapers and forgot what just happened. He reported seeing worms on the ground or plastic bags floating in the air. Two days before admission, he noticed right limb weakness which lasted for 1 h. The symptom recurred the next day, along with dizziness and slurred speech, and he was sent to our emergency department. On arrival, he was disoriented and had incoherent speech and impaired recent memory. Right hemianopia and right hemiparesis (Medical Research Council scale: 4 out of 5) were noted. Brain magnetic resonance imaging (MRI) with contrast demonstrated asymmetric white matter hyperintensities (WMHs) at the left parietal lobe [Figure 1] and multiple microbleeds compatible with cortical superficial siderosis [Figure 2]. Cerebral amyloid angiopathy-related inflammation (CAA-ri) was suspected and angiography was done to exclude other vascular causes, particularly isolated cortical vein thrombosis. He had significant improvement quickly and his wife declined steroid treatment. Brain MRI 3 months later showed resolution of the WMHs. According to his wife, he had complete recovery except being easier to get angry than before.Figure 1: Axial fluid-attenuated inversion recovery brain magnetic resonance imaging showed a confluent hyperintensity lesion extending from the left posterior periventricular area to left parietal lobe.Figure 2: Axial susceptibility weighted imaging brain magnetic resonance imaging revealed multiple microbleeds scattered predominantly at the left temporal and parietal lobes, compatible with cortical superficial siderosis.CAA-ri is recognized as a rare subtype of CAA. In a recent meta-analysis of 378 patients with CAA-ri, cognitive decline was the most common clinical feature (70%), followed by focal neurological deficits (55%), encephalopathy (54%), seizures (37%) and headache (31%).[1] Typical imaging findings were WMHs (98%), lobar cerebral microbleeds (96%), gadolinium-enhancing lesions (54%), cortical superficial siderosis (51%), and lobar macrohemorrhage (40%). The treatment is usually high dose intravenous corticosteroid pulse therapy followed by a gradual oral taper for several months. In a multicenter prospective study, most of the 113 patients had clinical (84.1%) and radiologic (74.1%) recovery after 12 months.[2] Ethics statement This study was conducted in accordance with the ethical principles outlined in the Declaration of Helsinki and its amendments. The authors certify that they have obtained all appropriate patient consent form. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Data availability statement Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Acta Neurologica Taiwanica
Authors: Ting-Yi Tseng, Kuei-Hong Kuo, Tzu-Hsien Lai
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4408920370