Dataset Information

Spontaneous coronary artery dissection in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy syndrome: a case report.

ABSTRACT:

Background

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) syndrome is a genetically inherited condition most notably affecting the central nervous system in young adults. There is limited knowledge on its association with coronary arteries, and its association with spontaneous coronary artery dissection (SCAD) has not been previously reported.

Case summary

A 61-year-old woman who is known to have CADASIL syndrome presented with anterior ST-segment myocardial infarction and underwent emergency angiography. This showed appearance consistent with SCAD in the mid left anterior descending artery with tubular stenosis.

Discussion

The association between CADASIL syndrome and SCAD has not been previously reported. The similarity in the underlying pathophysiology of these two conditions makes this case intriguing.

SUBMITTER: Tsanaxidis N

PROVIDER: S-EPMC6764544 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Publications

Spontaneous coronary artery dissection in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy syndrome: a case report.

Tsanaxidis Nikolaos N Elshafie Sally S Munir Shahzad S

European heart journal. Case reports 20190826 3

<h4>Background</h4>Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) syndrome is a genetically inherited condition most notably affecting the central nervous system in young adults. There is limited knowledge on its association with coronary arteries, and its association with spontaneous coronary artery dissection (SCAD) has not been previously reported.<h4>Case summary</h4>A 61-year-old woman who is known to have CADASIL syndrome presented with ...[more]

PMID: 31660501

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Project description:Arteries in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) are susceptible to smooth muscle loss and fibrosis, but the molecular components underlying these dramatic vascular changes are not well characterized. The purpose of this study was to investigate the distribution of collagen isoforms in the cerebral vessels of North American CADASIL patients with classical NOTCH3 mutations. Expression of types I-VI collagen in brains obtained at autopsy from six CADASIL patients with cysteine-altering mutations in NOTCH3 was compared to control brain expression. We identified a consistent increase of types I, III, IV, and VI collagen in CADASIL brains. Strong accumulation of types I, III, IV and VI collagen was noted in all calibers of vessels, including small and medium-sized leptomeningeal arteries, small penetrating white matter arteries, and capillaries. Within leptomeningeal arteries, where we could define the three tunicae of each vessel, we found distinct collagen subtype distribution patterns in CADASIL. Types I and III collagen were largely found in either adventitial/medial or transmural locations. Type IV collagen was strictly intimal/medial. Type VI collagen was adventitial or adventitial/medial. Within the thickened penetrating arteries of CADASIL patients, all four collagens extended through most of the arterial wall. We observed increased staining of capillaries in CADASIL for types I, IV, and VI collagen. In conclusion, brain vascular collagen subtypes are increased in CADASIL in multiple layers of all sizes of arteries, with disease-specific changes most prominent in the tunica media and thickened small penetrating vessels. In diseased arteries, types I, III, and VI collagen spreads from an external location (adventitia) into the vascular media, while type IV collagen accumulates in an internal pattern (intima and media). These observations are consistent with a pathological role for collagen accumulation in the vascular media in CADASIL.

Dataset Information

Spontaneous coronary artery dissection in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy syndrome: a case report.

Background

Case summary

Discussion

Publications

Spontaneous coronary artery dissection in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy syndrome: a case report.

Similar Datasets

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