CADASIL management or what to do when there is little one can do

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare disease that leads to migraine, mood disorders, recurrent lacunar strokes and early vascular dementia. This autosomal-dominant condition is caused by mutations in the NOTCH3 gene and is cha...

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Published inExpert review of neurotherapeutics Vol. 9; no. 2; pp. 197 - 210
Main Authors del Río-Espínola, Alberto, Mendióroz, Maite, Domingues-Montanari, Sophie, Pozo-Rosich, Patricia, Solé, Esther, Fernández-Morales, Jessica, Fernández-Cadenas, Israel, Montaner, Joan
Format Journal Article
LanguageEnglish
Published England Taylor & Francis 01.02.2009
Informa Healthcare
Subjects
CADASIL
CADASIL - diagnosis
CADASIL - pathology
CADASIL - therapy
Degeneration
Headache
Humans
Leukoencephalopathy
Migraine
Mood
Mutation
notch3
Notch3 protein
reactive oxygen species
Reviews
sirtuin
Smooth muscle
Stroke
vascular dementia
vascular smooth muscle cell
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Summary:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare disease that leads to migraine, mood disorders, recurrent lacunar strokes and early vascular dementia. This autosomal-dominant condition is caused by mutations in the NOTCH3 gene and is characterized by degeneration of vascular smooth muscle cells. At present, no evidence-based treatment for CADASIL is available and only relief of symptoms can be offered to patients. This review focuses on an update of CADASIL management, based on the recent clinical and basic evidence, and discusses possible new treatment targets for CADASIL.
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ISSN:1473-7175
1744-8360
DOI:10.1586/14737175.9.2.197