Moyamoya hastalığı ve antifosfolipid sendrom birlikteliği
Moyamoya hastalığı (MMH) çoğunlukla ön serebral dolaşıma ait vasküler yapıları özellikle de ön ve orta serebral arterlerin proksimal segmentlerini tutan bir hastalıktır. Etiyolojisi net olarak bilinmemektedir. Hastalık sıklıkla serebral iskemi, nadiren de serebral hemoraji ile ortaya çıkmaktadır. Serebral anjiografideki patolojik bulguların menenjit, nörofibromatozis, neoplazmlar, Down sendromu ve polikistik böbrek hastalığı gibi durumlarla bir arada bulunması halinde Moyamoya sendromu (MMS) olarak isimlendirilmektedir. Graves hastalığı, Behçet hastalığı ve Antifosfolipid sendrom (AFS) gibi otoimmün hastalıklar da MS gelişmesine neden olabilmektedirler. Kliniğimizde Antifosfolipid sendrom ile beraber MMS tanısı alan ve çocukluk çağı akut serebral infarkt nedenleri arasında oldukça ender görülen bu hastalık ilginç bulunarak sunuldu.
Moyamoya disease associated with antiphospholipid syndrome
Moyamoya (MMD) is a disease that often involves the vascular structures of anterior cerebral circulation, particularly the proximal segments of anterior and middle cerebral arteries. The etiology of the disease is unknown. MMD often presents with cerebral ischemia and rarely with cerebral hemorrhage. The pathology is termed Moyamoya syndrome (MMS) when the pathological cerebral angiography findings are accompanied by meningitis, neurofibromatosis, neoplasm, Down syndrome or polycystic kidney disease. Autoimmune diseases including Graves’ disease, Behcet’s disease and antiphospholipid syndrome might also lead to the development of MMS. In this manuscript, we presented an interesting case of MMD associated with antiphospholipid syndrome, which is quite a rare cause of acute cerebral infarction in childhood.
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