Long-term hemodynamic changes in cerebral proliferative angiopathy presenting with intracranial hemorrhage: illustrative case

Abstract

Background: Cerebral proliferative angiopathy (CPA) is a rare vascular proliferative disease; however, long-term follow-up reports are scarce. The authors report a rare case and document a patient's medical history over 20 years.

Observations: A 5-year-old girl developed left frontal lobe hemorrhage, presenting with headache. At 8 years of age, angiography showed diffuse capillary ectasia without an arteriovenous shunt. Single-photon emission computed tomography (SPECT) showed normal cerebral blood flow (CBF). She had normal growth without systemic disease. At 25 years of age, an intraventricular hemorrhage occurred, presenting with sudden headache. Angiography revealed vascular lesion enlargement, increased feeding arteries, dural supply to the nidus and peri-nidal lesion, and flow-related aneurysm. SPECT showed remarkable decreases in CBF in the nidus and peri-nidal lesion. Cerebral proliferative angiopathy (CPA) was diagnosed, and the aneurysm arising at the lateral posterior choroidal artery caused the hemorrhage. Coil embolization of the aneurysm was performed with a flow-guide catheter and extremely soft platinum coils. New aneurysms were not noted 1.5 years after the procedure.

Lessons: This is the first report to demonstrate hemodynamic changes in CPA on angiography and SPECT over 17 years. The development of endovascular devices has enabled the embolization of ruptured aneurysms at the peripheral cerebral artery.

Keywords: coil embolization; endovascular treatment; flow-related aneurysm; proliferative angiopathy.

FIG. 1.

Imaging examinations at initial hemorrhage. A: Gadolinium-enhanced T1-weighted MR imaging shows a left frontal shaggy enhanced lesion and scarring from the hemorrhage in the left frontal lobe at the age of 5 years. B: SPECT shows normal CBF in the left frontal lesion and in the left cerebral hemisphere at the age of 8 years. C: The arterial phase of the left common carotid angiogram, anteroposterior (AP; upper) and lateral (lower) views shows diffuse capillary ectasias in the left frontal lobe. D: The capillary phase of the left common carotid angiogram, AP (upper) and lateral (lower) views shows diffuse ectasias without an arteriovenous shunt. E: Right common carotid angiogram, AP view. F: Left vertebral angiogram, oblique view, shows no vessel anomaly. Angiography was performed at the age of 8 years.

FIG. 2.

Serial T2-weighted MR images at 15 years (A), 19 years (B), and 24 years (C) show gradual enlargement of the diffuse ectasic lesion and intermingled brain tissues from the left frontal lobe to the parietal lobe. D: MR angiography at 24 years shows no flow-related aneurysms at the lateral posterior choroidal artery.

FIG. 3.

Images of the second hemorrhage at 25 years of age. A: After the sudden onset of headache at 25 years old, plain computed tomography (CT) shows an IVH with a high-density spot at the left lateral ventricle (arrow). B: MR angiography shows an aneurysm at the left lateral posterior choroidal artery (asterisk), indicative of a flow-related aneurysm. C: SPECT shows a remarkable decrease in CBF in the nidus and peri-nidal lesion.

FIG. 4.

Angiograms after the second hemorrhage, AP view (upper) and lateral view (lower). Left internal carotid angiogram, arterial phase (A) and capillary phase (B), shows stenosis of the left M1 segment of the MCA and A1 segment of the ACA. Enlargement of the peripheral MCA is observed. An AV shunt is not observed. Left external carotid angiography (C) shows enlarged MMAs and collateral flow from the MMA to the CPA lesion and peri-lesion. A right common carotid angiogram (D) shows that the left ACA feeds the CPA via the anterior communicating artery. The right MMA is enlarged, and the left frontal CPA is supplied by the right anterior falcine artery. A left vertebral angiogram (E) shows collateral flow to the CPA via the lateral posterior choroidal artery, and a flow-related aneurysm appears (arrows).

FIG. 5.

Endovascular embolization of the flow-related aneurysm of the left lateral posterior choroidal artery. A: Preprocedural angiogram showing the flow-related aneurysm (arrow). B: Three-dimensional (3D) left vertebral angiogram demonstrating an aneurysm (arrow). C: The aneurysm is embolized with extremely soft platinum coils through a flow-guide microcatheter. A balloon catheter stabilizes the microcatheter during coil embolization. D: Postprocedural angiogram demonstrating an embolized aneurysm (arrow) with a slight neck remnant. E: Postprocedural 3D left vertebral angiogram shows an embolized aneurysm with coils. F: MR angiography 1.5 years after the procedure shows obliteration of the flow-related aneurysm.