Moya –Moya disease revealed by a subarachnoid hemorrhage: A case report

Abstract

Background: Hemorrhage is a major category of the clinical symptoms of moyamoya disease (MMD). Intracerebral and intraventricular hemorrhages are the most common hemorrhages in MMD, and subarachnoid hemorrhages (SAHs) are notably rare. Case report: A 43 year old female patient, with no previous pathological history, admitted with a frank meningeal syndrome followed by a disorder of consciousness.She was admitted with a GCS at 15, which was subsequently reduced to 10. She was hemodynamically and respiratory stable with a BP of 12/8 cm/Hg, a respiratory rate of 16 cycles/min and a heart rate of 92 beats/min. Initial imaging showed subarachnoid hemorrhage Fisher IV. A cerebral angiography-CT showed vascular thrombosis of the internal carotid artery whitout aneurysm and arteriovenous malformation and dural fistulas. An EVD (external ventricular drain) was placed. After three days of hospitalization in the intensive care unit, a cerebral arteriogram was performed showing vascular arrest in the right internal carotid artery in its pre-cavernous portion with absence of visualization of the right sylvian artery and the presence of several perforating cerebral artery and external carotid artery bypass systems suggestive of Moya Moya. Conclusion: Moya Moya disease is a rare entity of unknown origin. Subarachnoid hemorrhage is a rare manifestation. It’s mainly occurs in a young adult. Cerebral angiography remains the gold standard for diagnosis.