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Current Pain and Headache Reports
Published in: Current Pain and Headache Reports 10/2016

01-10-2016 | Imaging (L Mechtler, Section Editor)

CADASIL: Imaging Characteristics and Clinical Correlation

Authors: Shuhan Zhu, Stephanie J. Nahas

Published in: Current Pain and Headache Reports | Issue 10/2016

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Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene located on chromosome 19p13. CADASIL causes a clinical syndrome of migraines (frequently with aura), progressive strokes, and cognitive decline in adults leading to severe functional impairment by the seventh decade of life. Genetic testing is the gold standard for diagnosing this condition, but the syndrome can be suspected clinically based on history and a characteristic pattern of confluent subcortical white matter disease in the anterior temporal poles and external capsule. Additional abnormalities include cerebral microbleeds and large vessel stenosis, particularly in Asian populations. Familiarity with radiologic findings in CADASIL is essential to the correct diagnosis and subsequent management of this disease.
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Metadata
Title
CADASIL: Imaging Characteristics and Clinical Correlation
Authors
Shuhan Zhu
Stephanie J. Nahas
Publication date
01-10-2016
Publisher
Springer US
Published in
Current Pain and Headache Reports / Issue 10/2016
Print ISSN: 1531-3433
Electronic ISSN: 1534-3081
DOI
https://doi.org/10.1007/s11916-016-0584-6