Abstract
A 36-year-old housewife was admitted to the hospital, because of consciousness disturbance. She had no particular past history with the exception of measles at the age of four years.
On admission, she was comatose and neurological examinations revealed left hemiplegia with decerebrate rigidity. Her blood pressure was 130/80 mmHg. Physical examinations were otherwise unremarkable. The CT scan demonstrated massive cerebral hemorrhage in the right hemisphere. A right common carotid angiogram showed occlusion of the internal carotid artery associate with abnormal vascular network at the base of the brain and occlusion of the external cerebral artery (Fig. 1 A B). She died on the third hospital day.
The main autopsy findings of the case were as follows :
1) Massive hemorrhage was observed in the right hemisphere. No particular histological change was obtained except that there were acute changes due to cerebral hemorrhage.
2) Cerebral vessels : The numerous abnormal small vessels were observed at the base of the brain, predominantly in the right. Bilateral internal carotid arteries were occluded macroscopically from their origins to terminal portions. The terminal portion looked like strings (Fig. 3). The superficial temporal artery and middle meningeal artery were also occluded. Histological examinations disclosed marked fibrous intimal thickening with fragmented internal elastic lamina and thinning of the media (Fig. 4 A B). No atheromatous deposits nor inflamatory cells were observed within those vessels. The anterior cerebral artery (A1) was stenosed due to intimal thickening and the internal elastic lamina showed wave-like bending. The cortical and intraparenchymal vessels did not show significant change except for the abnormal small vessels in the base of the temporal lobe (Fig. 2).
3) Systemic arterial system : Relatively severe atheromatous changes for her age were observed especially in the arch and thoracic aorta. A localized ossification with the formation of bone marrow was observed in the lower part of the thoracic aorta (Fig. 6). The common histological features of the aorta and its branches were fibrous intimal thickening with atheromatous deposits, severe thinning of the media, fibrosis of the adventitia and marked concentric intimal thickening of he vasa vasorum (Fig. 5). The pulmonary arteries were also affected.
4) Visceral organs : Histological findings of the other organs were unremarkable except for scattered fibrosis in the myocardium.
