A case with isolated angiopathy of the central nervous system progressing to anterior and middle cerebral artery occlusion

We report the case of a 24-year-old man with isolated angiopathy of the central nervous system (IACNS) which progressed to large-vessel arterial occlusion. At two months after experiencing difficulty in walking, the patient suddenly developed severe headache and progressive paralysis of the left extremities and was admitted to hospital. A brain CT scan disclosed cerebral infarction. His left hemiparesis rapidly improved without treatment. One month later, however, left hemiparesis recurred. He was then transferred to Kinki Medical University Hospital for evaluation. Physical examination on adminission whowed left hemiparesis with left facial paresis. The results of general laboratory work-up were unremarkable. No abnormalities were detected in serological and immunological tests. The patients cardiac echogram and ECG were normal Brain MRI demonstrated multiple infarctions in the area o the right internal capsule, caudate and parietal lobe. Cerebral angiography revealed multiple segmental stenosis, irregularity and narrowing of the right anterior callosomarginal artery and anterior cerebral artery. These data suggested IACNS. Leptomeningeal biopsy was negative. The patient was treated with high-dose steroids, but complete left hemiplegia progressed during the next two months. Brain MRI at that time showed high-intensity signals within the areas of the anterior cerebral and middle cerebral arteries. On cerebral angiography, the right anterior and middle cerebral arteries were found to be completely obstructed. The typical angiographic abnormalities of IACNS include multiple localized areas of narrowing, and occlusions of vessels, reflecting the predominance of small-vessel inflammation. Our case was unusual in its progression to large-vessel arterial occlusion.