2003
DOI: 10.1046/j.1440-1789.2003.00519.x
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Cerebral arterial pathology of CADASIL and CARASIL (Maeda syndrome)

Abstract: Two familial cerebro-vascular diseases characterized by different cerebral arterial pathologies and presenting in non-hypertensive young and middle-aged adults are described. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by the deposition of smudged periodic acid-Schiff (PAS)-positive granules known as granular osmiophilic materials (GOM) in the media of small arteries and arterioles. The medial smooth muscle cells are completely lost, and… Show more

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Cited by 58 publications
(58 citation statements)
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“…8,12,22,23 In the cerebral small arteries, smooth muscle cells were extensively lost, even in arteries without sclerotic changes. Sclerotic changes were mild and infrequent; most of the arteries were enlarged rather than exhibiting luminal stenosis.…”
Section: Cerebral Small Vessel Pathology In Carasilmentioning
confidence: 99%
“…8,12,22,23 In the cerebral small arteries, smooth muscle cells were extensively lost, even in arteries without sclerotic changes. Sclerotic changes were mild and infrequent; most of the arteries were enlarged rather than exhibiting luminal stenosis.…”
Section: Cerebral Small Vessel Pathology In Carasilmentioning
confidence: 99%
“…These features include fibrous proliferation of the intima, hyaline degeneration of the media, loss of vascular smooth muscle cells, and thickening and fragmentation of the internal elastic lamina [6]. There is dilatation of arterial lumen rather than luminal stenosis [7]; the ischemic brain insults are thought to be due to the disturbance of autoregulatory mechanisms for cerebral blood flow [8].…”
Section: Case Discussionmentioning
confidence: 99%
“…For CARASIL patients, the main pathology falls on the small penetrating arteries, with lack of vascular smooth muscle cells (VSMCs) in the media and intense adventitial fibrosis (2). Loss of VSMCs is the primary feature of CARASIL, followed by deposition of the granular material in the media and fibrosis of the arterial wall (2, 3).…”
mentioning
confidence: 99%
“…Loss of VSMCs is the primary feature of CARASIL, followed by deposition of the granular material in the media and fibrosis of the arterial wall (2, 3). Arterial fibrosis results in the deficiency of contraction of the arteries, leading to ischemic stroke and other clinical symptoms of CARASIL (2,3). TGF-β promotes fibrosis by stimulating the proliferation and activation of FBs (4).…”
mentioning
confidence: 99%