Moyamoya disease associated with antiphospholipid syndrome

Abstract

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.

Keywords

• Moyamoya, antiphospholipid syndrome, hemiparesis, convulsion

Moyamoya hastalığı ve antifosfolipid sendrom birlikteliği

Abstract

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.

Keywords

• Moyamoya, antifosfolipid sendrom, hemiparezi, konvülzyon

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