Moyamoya disease is a chronic cerebrovascular disease characterized by progressive stenosis and/or occlusion of the intracranial internal carotid and its proximal branches with a created abnormal collateral network of small arteries. If the vascular occlusion is not bilateral and the patient has been diagnosed with a specific underlying illness, we refer to this condition as Moyamoya syndrome.
The presentation of Moyamoya disease or syndrome in the form of a non-aneurysmal subarachnoid hemorrhage is rare. The 62-year-old woman was admitted for 24 hours lasting pain in the area of the neck, forehead, and temples.
She was somnolent, vomited repeatedly, and had double vision. The brain computed tomography discovered subarachnoid hemorrhage.
The brain CT angiography revealed an occlusion of the left middle cerebral artery and the digital subtraction angiography, moreover, showed a network of tiny tortuous moyamoya vessels in the left middle cerebral artery territory. The brain perfusion was not impaired.
The patient was treated with corticosteroids and anti-platelet drugs. A diagnosis of Moyamoya syndrome was determined.
She was dismissed without neurological deficit or subjective problems. After six years, the patient's general condition deteriorated because of sepsis, and even after getting maximum antibiotic therapy, she died.
Moyamoya disease or syndrome should be considered in the differential diagnosis as the cause of subarachnoid hemorrhage in the absence of other clear vascular pathology as its potential source.