Cerebral amyloid angiopathy-related inflammation: current status and future implications.
10.1097/CM9.0000000000001427
- VernacularTitle:Cerebral amyloid angiopathy-related inflammation: current status and future implications
- Author:
Juan-Juan WU
1
;
Ming YAO
;
Jun NI
Author Information
1. Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.
- Publication Type:Review
- MeSH:
Amyloid beta-Peptides;
Cerebral Amyloid Angiopathy;
Cerebral Hemorrhage;
Humans;
Inflammation;
Magnetic Resonance Imaging;
Vasculitis
- From:
Chinese Medical Journal
2021;134(6):646-654
- CountryChina
- Language:English
-
Abstract:
Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.
