Résumé
Arterial dissections usually arise from an intimal tear and can allow the development of an intramural hematoma. Dissection occurs frequently in the aorta or its branches and also occurs commonly in the internal carotid or vertebro-basilar arteries. The condition is rare in the intracranial cerebral arteries, especially above the supraclinoid segment of the internal carotid artery. The diagnosis of arterial dissection used to be made by conventional cerebral angiography. But recently, magnetic resonance imaging (MRI) is helpful to diagnose arterial dissection. We report two cases of middle cerebral arterial dissection using MRI.
Sujets)
Aorte , Artères , Artère carotide interne , Angiographie cérébrale , Artères cérébrales , Diagnostic , Hématome , Imagerie par résonance magnétique , Artère cérébrale moyenneRésumé
Primary merosin (laminin alpha2 chain)-deficient congenital muscular dystrophy (CMD) is a uncommon and severe form of CMD, which is caused by the mutations in the laminin alpha2 chain gene. It is an autosomal recessively inherited form of muscular dystrophy that is associated with severe neonatal hypotonia, a high serum creatine kinase level, and abnormal brain imaging without intellectual dysfunction. We report a case of merosin-deficient CMD confirmed by the immunocytochemical analysis of the frozen muscle biopsy. This is the first case of merosin-deficient CMD in Korea.