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Journal of Neurology

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01-07-2019 | CADASIL | Original Communication

Primary involvement of neurovascular coupling in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Authors: Andrea Jokumsen-Cabral, Ana Aires, Susana Ferreira, Elsa Azevedo, Pedro Castro

Published in: Journal of Neurology | Issue 7/2019

Abstract

Background

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most frequent monogenic cause of cerebral ischemia, but reliable biomarkers to monitor the disease are lacking.

Aims and objectives

To evaluate cerebral autoregulation (CA), vasoreactivity (VR), and neurovascular coupling (NVC) in CADASIL patients through a battery of dynamic transcranial Doppler tests.

Methods

We screened our database for all pre-dementia CADASIL cases. We monitored cerebral blood flow velocity (CBFV) with transcranial Doppler, blood pressure, and expiratory carbon dioxide (CO₂) non-invasively. CA was assessed by transfer function from the spontaneous oscillations of blood pressure to CBFV, VR with inhalation of CO₂ at 5%, and hyperventilation and NVC by the CBFV response to visual stimulation.

Results

We included 27 CADASIL patients and 20 healthy controls with similar age and sexes. CA and VR were similar between groups. However, NVC was significantly affected in CADASIL patients, with lower magnitudes of CBFV upsurge (overshoot 19 ± 5 vs 26 ± 6%, p = 0.013; gain 12 ± 7 vs 17 ± 5%, p = 0.003) and altered time behavior during visual stimulation (natural frequency 0.18 ± 0.06 vs 0.24 ± 0.06 Hz, p = 0.005; rate time 0.7 ± 1.7 vs 2.7 ± 3.5 s, p = 0.025).

Conclusion

Our results express a primary and selective involvement of the neurovascular unit in CADASIL rather than a generalized cerebral vasomotor disturbance. Functional cerebrovascular testing could be useful in patient evaluation and monitoring.

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Title: Primary involvement of neurovascular coupling in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy
Authors: Andrea Jokumsen-Cabral
Ana Aires
Susana Ferreira
Elsa Azevedo
Pedro Castro
Publication date: 01-07-2019
Publisher: Springer Berlin Heidelberg
Keyword: CADASIL
Published in: Journal of Neurology / Issue 7/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09331-y

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Primary involvement of neurovascular coupling in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Abstract

Background

Aims and objectives

Methods

Results

Conclusion

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Aims and objectives

Methods

Results

Conclusion

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