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Published on: May 23, 2016
CADASIL accelerated by acute hypotension: Arterial and venous contribution to leukoaraiosis
Jacqueline A Pettersen1, Julia Keith2, Fuqiang Gao2
1From 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.
Insights
Acute hypotension accelerated cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) pathology. Vein collagenosis in affected white matter suggests veins play a key role in maintaining brain integrity.
Area of Science:
- Neurology
- Pathology
- Vascular Biology
Background:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic condition affecting small blood vessels in the brain.
- Blood pressure regulation is critical in managing CADASIL, but the role of veins remains understudied.
Observation:
- A case report details a patient with confirmed CADASIL who experienced rapid neurological decline following hypotensive episodes after trauma.
- Neuroimaging revealed new infarcts and leukoencephalopathy, correlating with periods of low blood pressure.
- Autopsy showed characteristic arterial changes and, notably, venous collagenosis in affected white matter regions.
Findings:
- Hypotension significantly exacerbated CADASIL pathology, leading to new infarcts and leukoaraiosis.
- Venous collagenosis, characterized by thickened, collagen-rich vein walls, was observed in the affected white matter.
- The patient's presentation and autopsy findings suggest impaired vasoreactivity contributed to the accelerated disease progression.
Implications:
- This case highlights the critical role of maintaining stable blood pressure in CADASIL patients, especially those with impaired vasoreactivity.
- The observed venous collagenosis suggests that veins may be more significantly involved in CADASIL pathogenesis than previously recognized.
- Further research into the role of venous changes in white matter integrity is warranted for a comprehensive understanding of CADASIL.
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.
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