[Involvement of the large vessels supplying the brain in sickle cell disease: a possible cause of Moya-moya (author's transl)].
In two children (1 girl and 1 boy aged 6 years) with an SS haemoglobinopathy, a neurological deficit affecting one half of the body led to arteriograms being performed. These revealed stenoses of the major vessels (above all anterior and middle cerebral with development of a collateral circulation giving an appearance suggestive of Moya-moya syndrome). Various aetiologies have been suggested for this syndrome: congenital disease, oral contraceptives, atheroma. Sickle cell disease is a new cause. The mechanism would appear to be fibrosis proliferating secondary to sub-endothelial anoxia caused by sludging in the vasa vasorum.
D000755:Anemia, Sickle Cell / Q000150:complications*; D001157:Arterial Occlusive Diseases / Q000209:etiology*; D002539:Cerebral Arterial Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D002648:Child; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000209:etiology*; D011859:Radiography
L'atteinte des gros vaisseaux à destinée cérébrale au cours des drépanocytoses: une cause possible de Moya-moya.