ISSN:
1432-0533
Keywords:
Mitochondrial encephalomyopathy (MELAS)
;
Mitochondrial angiopathy
;
Smooth muscle and endothelial cells
;
Pial arteriole and small artery
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary We studied cerebral blood vessels of two autopsied patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). All the main cerebral arteries in the proximal portion at the brain base and more distal portion at the cortical surface, as well as within the brain parenchyma were examined by electron microscopy. There was a striking increase in number of mitochondria in the smooth muscle and endothelial cells, which were most prominent in the pial arterioles and small arteries up to 250 μm in diameter and less frequent and severe in the larger pial arteries and intracerebral arterioles and small arteries. These vascular changes have not hitherto been described in MELAS, or in other disorders affecting blood vessels of the brain and other organs. It is suggested that the vascular changes are caused by primary mitochondrial dysfunction in the vascular smooth muscle and endothelial cells of the brain and that they constitute the pathogenic base of the brain lesions and their unusual distribution pattern in MELAS.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00688185
