Von Recklinghausen's Disease associated with Occlusion of Bilateral Middle Cerebral Artery, Moyamoya Phenomenon, and an Anterior Communicating Artery Aneurysm

Abstract

Histopathological examination of cerebrovascular occlusive lesions with von Recklinghausen's disease has rarely been reported. The authors are presenting an autopsy case of von Recklinghausen's disease with the occlusion of bilateral proximal middle cerebral arteries (M1), basal moyamoya, and an anterior communicating artery aneurysm. A 60-year-old female suffered a subarachnoid hemorrhage with drowsiness and nuchal rigidity. Although the neck-clipping was performed without any trouble, the patient died of pneumonia 26 days after the operation. Histopathological examination revealed hypoplasia of bilateral M1 and extensive arteriosclerotic changes of the circle of Willis. The authors concluded that the primary hypoplasia of bilateral M1 induced basal moyamoya as collateral circulation and hemodynamic stresses facilitated the formation of the anterior communicating artery aneurysm. It is not clear whether there is any etiological relationship between hypoplasia of the bilateral M1 and von Recklinghausen's disease.