Conventional angiography provides the conclusive diagnosis of moyamoya disease in most cases and should be performed before any surgical considerations.Darren B. Orbach explains how the disease progresses as well as the role angiography plays in detecting the progression of moyamoya in a short video. In 2019, author and artist Sarah Lippett published a graphic novel about her decade-long struggle to get a diagnosis and treatment for moyamoya disease, called A Puff of Smoke (published with Jonathan Cape). The book was praised in the newspaper The Guardian as a "wonderfully drawn memoir of a serious childhood illness." It was one of the papers "graphic novels of the year" in 2019 and The Observer newspapers graphic novel of the month in November 2019. Associated biomarkers
Smith (2015) conducted a study that looked into specific biological markers that correlate to moyamoya disease. Some of the categories of these biomarkers include phenotypes - conditions commonly related to moyamoya, radiographical markers for the diagnosis of moyamoya, and proteins as well as cellular changes that occur in cases of moyamoya.Similar to moyamoya disease, there are conditions that are closely associated with moyamoya disease. Some of the more common medical conditions that are closely associated with moyamoya disease include trisomy 21 (Down Syndrome), sickle cell disease, and neurofibromatosis type 1.
Although the sample size of the studies performed are small due to the rarity of the disease, the findings are indicative of a correlation between the disease and several specific protein biomarkers. Other studies have confirmed the correlation of moyamoya and adhesion molecule 1 (ICAM-1) being increased as compared to normal vascular function counterparts. Furthermore, it has been concluded that the localization of inflammatory cells suggests that the inflammation stimulus itself may be responsible for the proliferation and occlusion in the ICA, ACA, and MCA found in those with moyamoya disease. Treatment
Drugs such as antiplatelet agents (including aspirin) are usually given to prevent clots, but surgery is usually recommended. Because moyamoya tends to affect only the internal carotid artery and nearby sections of the adjacent anterior and middle cerebral arteries, surgeons can direct other arteries, such as the external carotid artery or the superficial temporal artery to replace its circulation. The arteries are either sewn directly into the brain circulation, or placed on the surface of the brain to reestablish new circulation after a few weeks.There are many operations that have been developed for the condition, but currently the most favored are the in-direct procedures EDAS, EMS, and multiple burr holes and the direct procedure STA-MCA. Combined revascularisation procedure, which includes both the direct superficial temporal artery (STA) to middle cerebral artery (MCA) bypass performed with a combination of in-direct procedures, is considered the treatment of choice.