The rare association of moyamoya disease and cerebral arteriovenous malformations: a case report

Abstract

A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow-up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.

Fig. 1

Moyamoya disease and concurrent cerebral arteriovenous malformation in 36-year-man. A. Composite anteroposterior (AP) view of bilateral carotid angiogram shows right temporal cerebral rteriovenous malformation. B, C. Stereotactic MRI with dose plan show that 17.5 Gy (yellow isodose line) is prescribed to perihphery of cerebral arteriovenous malformation nidus (magenta line). Supraclinoid segment of right internal carotid arteries and proximal middle cerebral artery received dose of 6.25 Gy (green isodose line). D, E. Nine months (D) and 34 months (E) after gamma-knife surgery. Time-of-light (TOF) MR angiograms show progressive focal stenosis at bilateral middle cerebral artery. F. At 51 months after gamma-knife radiosurgery, bilateral carotid angiogram confirms cure of cerebral arteriovenous malformation and occlusion of bilateral supraclinoid internal carotid arteries, proximal middle cerebral artery and anterior cerebral artery with moyamoya collateral vessels.