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Neurology. 2017 Mar 14;88(11):1077-1080. doi: 10.1212/WNL.0000000000003717. Epub 2017 Feb 15.

CADASIL accelerated by acute hypotension: Arterial and venous contribution to leukoaraiosis.

Author information

1
From the Northern Medical Program and Division of Neurology (J.A.P.), Department of Medicine, University of British Columbia, Vancouver; Departments of Anatomic Pathology (J.K.) and Medicine (Neurology Division) (S.E.B.), Sunnybrook Health Sciences Centre, University of Toronto; Hurwitz Brain Sciences Program (F.G., S.E.B.), Canadian Partnership for Stroke Recovery (F.G., S.E.B.), and LC Campbell Cognitive Neurology Unit (F.G., S.E.B.), Sunnybrook Research Institute, University of Toronto; and Stroke Prevention & Atherosclerosis Research Centre (J.S.D.), Robarts Research Institute, Western University, London, Canada. pettersj@unbc.ca.
2
From the Northern Medical Program and Division of Neurology (J.A.P.), Department of Medicine, University of British Columbia, Vancouver; Departments of Anatomic Pathology (J.K.) and Medicine (Neurology Division) (S.E.B.), Sunnybrook Health Sciences Centre, University of Toronto; Hurwitz Brain Sciences Program (F.G., S.E.B.), Canadian Partnership for Stroke Recovery (F.G., S.E.B.), and LC Campbell Cognitive Neurology Unit (F.G., S.E.B.), Sunnybrook Research Institute, University of Toronto; and Stroke Prevention & Atherosclerosis Research Centre (J.S.D.), Robarts Research Institute, Western University, London, Canada.

Abstract

OBJECTIVE:

To underline the importance of blood pressure regulation in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and to describe changes that occur in the veins in this condition, specifically venous collagenosis associated with leukoaraiosis.

METHODS:

Case report with neuroimaging and pathologic data.

RESULTS:

A 61-year-old man with genetically confirmed CADASIL was initially lucid following a motor vehicle accident but subsequently became hypotensive (60/40 mm Hg) due to an open femur fracture and required intubation. Multiple new white matter infarcts appeared on brain imaging. A second hypotensive episode days later was associated with new coin-sized infarcts in the bilateral corona radiata and cerebellar peduncles, and resulted in quadriplegia. No embolic source was found on cardiac or vascular imaging. He died 5 weeks post trauma. Autopsy revealed extensive subcortical and periventricular leukoencephalopathy and multiple cavitations involving deep subcortical gray and white matter. Small arteries had thickened walls, disruption of the muscularis, and intimal periodic acid-Schiff (PAS)-positive material. Both larger periventricular and small caliber veins had thickened walls that were PAS-negative and trichrome-positive, consistent with venous collagenosis. There was no pathologic evidence of global hypoxia or diffuse axonal injury.

CONCLUSIONS:

The findings suggest rapid acceleration of CADASIL pathology from acute hypotension in the setting of impaired vasoreactivity. In addition, collagenosis of veins in the affected white matter regions suggests that the veins may play an important, though largely overlooked, role in maintaining white matter integrity.

PMID:
28202707
PMCID:
PMC5384836
DOI:
10.1212/WNL.0000000000003717
[Indexed for MEDLINE]
Free PMC Article

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