Repeated recurrent intracerebral hemorrhage may be involved in cerebral amyloid angiopathy-related inflammation: An autopsy case

We report an autopsy case of repeated recurrent intracerebral hemorrhage (ICH) diagnosed as having cerebral amyloid angiopathy-related inflammation (CAA-ri) in a 65-year-old woman. She had no history of hypertension or other risk factors for stroke. She had a history of lobar ICH in the right parietal lobe 30 months prior. Follow-up magnetic resonance imaging (MRI) performed 29 months after the initial ICH revealed superficial siderosis in the left frontal lobe. She initially presented with severe headache and dysarthria. An initial computed tomography (CT) revealed a subarachnoid hemorrhage on the left frontal lobe. One hour later, an epileptic seizure occurred, and another CT performed at the time revealed lobar ICH in the frontal lobe. Emergency surgical hematoma evacuation was performed. The cortical artery was removed from the surgical specimen. Hematoxylin and eosin (HE) staining revealed lymphocytic perivascular cuffs, and immunohistochemical staining revealed abundant amyloid-β (Aβ) deposits. The patient was diagnosed as having CAA-ri. On the next day, and 19, 46, 55, 78, 79, and 85 days after admission, ICH recurred. We did not administer immunosuppressive drugs, such as high-dose corticosteroids or cyclophosphamide, because of the patient's condition. Following the last ICH, the patient died on the 90th day after symptom onset. An autopsy was performed. Histologically, abundant Aβ deposits were observed within the leptomeningeal and cortical arteries. Further dense-cored amyloid plaques were observed in the cortical samples. Our findings that ICH occurred initially three times in the left hemispheres and later five times in the right hemispheres were considered immune-mediated effects on vascular function. Further, superficial hemosiderosis in the left frontal lobe occurred before the first three ICH events, and hyperintensity of the white matter on MRI was identified in the right parietal lobe, a typical finding of CAA-ri. We hypothesize that the inflammatory process in CAA-ri may be related to recurrent ICH.