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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown aetiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery (ICA) and an abnormal vascular network at the base of the brain. Familial occurrence is observed in about 20%. It occurs in both children and adults. It probably represents the end point for several vasculopathies. Surgical revascularization is the preferred treatment for this entity.
Stroke and Transient Ischemic Attacks of the Brain and Eye
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Moyamoya disease is commonly found in individuals of Asian heritage, and there is no known cause. Genetic factors appear to play a major role in moyamoya. The proportion of patients who have affected first-degree relatives is 10% in Japan. Less common associations, which may be causal, include sickle cell disease, NF-1, cranial therapeutic irradiation, Down's syndrome, and rarely, congenital cardiac anomaly, renal artery stenosis, giant cervicofacial hemangiomas, and hyperthyroidism.
Test Paper 1
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic, progressive vasculo-occlusive disease involving the circle of Willis, typically the supraclinoid internal carotid arteries. It has a bimodal age distribution, affecting children and adults. In children, ischaemic strokes are most pronounced, whereas in adults haemorrhage from the abnormal vessels is more common.
Moyamoya syndrome in a male pseudohermaphrodite patient with congenital adrenal hyperplasia – a rare association. Case report and review of literature
Published in British Journal of Neurosurgery, 2023
Remesh Chirayil Vasudevan, Reshma Vachali Madayi, Rohit Ravindranath Nambiar
Moyamoya occurs twice as often in females as in males.8,9 Though genetics of moyamoya disease is not well understood, recent data have shown a link between mutations of RNF213 gene and pathogenesis of moyamoya syndrome, particularly among Asians. The RNF213 gene is located on chromosome 17 (17q25.3), and the RNF213 protein coded by this gene is involved in normal development of blood vessels and hence, mutations in the RNF213 gene may contribute to narrowing of blood vessels and their proliferation, which is characteristic of moyamoya. At least 24 such mutations have been associated with moyamoya disease, the most common of which involves base-pair substitution resulting in replacement of arginine by lysine at protein position 4810. Mutations of TIMP-2, ACTA2 and mutations on chromosome 3p, 6, 8 (8q23) and 17(near NF1 gene) have also been implicated in the origin and development of moyamoya. Interestingly, the gene coding for 11- beta-hydroxylase (CYP11B1) the deficiency of which causes CAH, is also located on chromosome 8 (8q24).
Occipital artery-anterior cerebral artery bypass with posterior auricular artery-middle cerebral artery bypass for stenosis of the internal carotid artery bifurcation
Published in British Journal of Neurosurgery, 2021
Ryoko Niwa, Toshikazu Kimura, Shunsuke Ichi
Superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis is the most commonly used procedure to augment cerebral blood flow. It is performed as a primary treatment in various clinical circumstances involving cerebral ischaemia, including Moyamoya disease,1 and severe vascular stenosis due to atherosclerosis.2,3 It is also used occasionally as a supplementary approach to restoring ischaemic blood flow during brain tumour resection or intracranial aneurysm surgery.4,5 Although it is performed less frequently, STA-anterior cerebral artery (ACA) anastomosis is also considered suitable for revascularisation of the ACA territory;6 however, the STA is not appropriate to use as a donor artery in select cases, including congenital aplasia or hypoplasia, post-traumatic change, previous surgical incision, or previous extracranial-intracranial bypass surgery. In such cases, several alternative procedures can be considered, including interposition graft bypass,7–9 occipital artery (OA)-MCA bypass 10–14 and posterior auricular artery (PAA)-MCA bypass.15–18 Here we report on a patient with severe stenosis of the internal carotid artery (ICA) bifurcation treated by OA-ACA bypass together with PAA-MCA bypass.
Black-blood magnetic resonance imaging suggesting central nervous system vasculitis in moyamoya syndrome associated with systemic lupus erythematosus
Published in Immunological Medicine, 2021
Keiichiro Kadoba, Keisuke Nishimura, Daisuke Waki, Tsutomu Okada, Takao Kumazawa, Rintaro Saito, Hiroyuki Murabe, Toshihiko Yokota
Moyamoya disease (MMD) is an idiopathic disorder characterized by progressive stenosis and occlusion of the distal internal carotid arteries and their major intracerebral branches. The vascular stenosis/occlusion is accompanied by the growth of small collateral vessels in the cerebral perforating vessels, hence the name came ‘moyamoya’ (which describes the angiographic view of collateral vessels as a puff of smoke in Japanese). The etiology of moyamoya disease is unknown. However, these angiographic findings are not specific to the disease. Moyamoya-like vasculopathy might develop in patients with other well-characterized diseases or syndromes, including SLE. In these patients, the condition is called moyamoya syndrome (MMS) rather than MMD. Both conditions predispose patients to brain ischemia (i.e., stroke, transient ischemic attacks, and seizures) [1,2].