Left: MIP reconstructed MR angiography of a 11-year-old girl with<unk> .Right: healthy patient, for comparison. Cerebral angiography is the gold standard of diagnosing<unk>  and its progression. According to Suzuki's system, it can be classified into six stages: * Stage 1 Narrowing of carotid fork * Stage 2 Initiation of the<unk>  and dilatation of intracranial main arteries * Stage 3 Intensification of the<unk>  and defects of the anterior cerebral artery and middle cerebral artery * Stage 4 Minimization of the<unk>  and defects of the posterior cerebral artery * Stage 5 Reduction of the<unk>  and development of external carotid artery collaterals * Stage 6 Disappearance of the<unk>  and circulation only via external cerebral artery and vertebral artery Magnetic resonance angiography (MRA) is also useful in diagnosing the<unk>  with good correlation with Suzuki's grading system. Proliferation of smooth muscle cells in the walls of the<unk> -affected arteries has been found to be representative of the<unk> . A study of six autopsies of six patients who died from<unk>  lead to the finding that there is evidence that supports the theory that there is a thickening, or proliferation, of the innermost layer of the vessels affected by<unk> . These vessels are the ACA (anterior cerebral artery), MCA (middle cerebral artery), and ICA (internal carotid artery). The occlusion of the ICA results in concomitant diminution of the "puff-of-smoke" collaterals, as they are supplied by the ICA. Often nuclear medicine studies such as SPECT (single photon emission computerized tomography) are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with<unk> . Conventional angiography provides the conclusive diagnosis of<unk>  in most cases and should be performed before any surgical considerations. Darren B. Orbach explains how the<unk>  progresses as well as the role angiography plays in detecting the progression of<unk>  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<unk> , 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 paper's "graphic novels of the year" in 2019 and The Observer newspaper's graphic novel of the month in November 2019. Associated biomarkers Smith (2015) conducted a study that looked into specific biological markers that correlate to<unk> . Some of the categories of these biomarkers include phenotypes - conditions commonly related to<unk> , radiographical markers for the diagnosis of<unk> , and proteins as well as cellular changes that occur in cases of<unk> . Similar to<unk> , there are conditions that are closely associated with<unk> . Some of the more common medical conditions that are closely associated with<unk>  include trisomy 21 (Down Syndrome), sickle cell<unk> , and neurofibromatosis type 1. There is also evidence that identifies hyperthyroidism and congenital dwarfing syndromes as two of the more loosely associated syndromes that correlate with the possibility of being diagnosed with<unk>  later in life. There is also research that has shown that certain radiographic biomarkers that lead to the diagnosis of<unk>  have been identified. The specific radiographic markers are now considered an acceptable key component to<unk>  and have been added to the International Classification of<unk> s (ICD). These biomarkers of<unk>  are "stenosis of the distal ICA's up to and including the bifurcation, along with segments of the proximal ACA and MCA...dilated basal collateral vessels must be present" Some other common findings that have not been added to the classification index of those with<unk>  which are found using radiography involve very distinct changes in the vessels of the brain. These changes include newly formed vessels made to compensate for another change noted, ischemia and cerebrovascular reserve, both found on MRI. Functional changes include evidence of ischemia in vessels of the brain (ICA, ACA, MCA, specifically). It is important to also note that the radiographic biomarkers, in order to be classified as<unk> , all findings must be bilateral. If this is not the case and the findings are unilateral, it is diagnosed as<unk>  syndrome. There are also several protein biomarkers that have been linked to the<unk>  diagnosis. Although the sample size of the studies performed are small due to the rarity of the<unk> , the findings are indicative of a correlation between the<unk>  and several specific protein biomarkers. Other studies have confirmed the correlation of<unk>  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<unk> .
Moyamoya disease
Vein of Galen aneurysmal malformations
Cerebral amyloid angiopathy
Cerebral arteriovenous malformation
Meningism
Giant cell arteritis
Hypertensive leukoencephalopathy
Chiari malformation