title: Confluent thalamic hyperintensities in CADASIL.
abstract: BACKGROUND
  CADASIL is responsible for diffuse hyperintensities in the white matter on FLAIR images. These lesions are often associated with focal lesions in the basal ganglia such as lacunar infarctions. The prevalence and significance of diffuse or confluent thalamic hyperintensities (CTH) remain unknown.
  
  
  METHODS
  The frequency of hyperintensities on FLAIR images in the thalamus was assessed in 147 CADASIL patients, and signal abnormalities on both FLAIR and T(1)-weighted images were categorized as focal/punctuate or diffuse/confluent by the same reader. The areas of increased diffusion were also analyzed on apparent diffusion coefficient maps. The association of CTH with vascular risk factors, the main clinical manifestations of the disease and MRI markers (brain parenchymal fraction, volume of white matter hyperintensities, volume of lacunar infarcts and number of microbleeds) was analyzed with generalized linear regression models.
  
  
  RESULTS
  CTH were detected in 12% of the CADASIL subjects in association with hypointensities on T(1)-weighted images. CTH corresponded to areas of increased diffusion on apparent diffusion coefficient maps. CTH were found significantly associated with age and independently related to the volume of white matter hyperintensities but not to that of lacunar infarctions or to cerebral atrophy after adjustment for age and sex. No significant association was found between CTH and global cognitive performances.
  
  
  CONCLUSIONS
  CTH are observed on FLAIR images in a sizeable proportion of CADASIL patients. They are mainly related to the extent of white matter hyperintensities and do not correlate with cognitive decline. Demyelination and/or loss of glial cells appear to be the most plausible cause of these confluent signal changes in the thalamus.
authors.affiliation: Service de Neurologie, Centre de référence pour les maladies rares des vaisseaux du cerveau et de l'oeil (CERVCO), INSERM U740, CHU Lariboisière, APHP, Paris, France.
authors.forename: Mathilde
authors.initials: M
authors.lastname: Jacqmin
authors.forename: Dominique
authors.initials: D
authors.lastname: Hervé
authors.forename: Anand
authors.initials: A
authors.lastname: Viswanathan
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Guichard
authors.forename: Marco
authors.initials: M
authors.lastname: During
authors.forename: Martin
authors.initials: M
authors.lastname: Dichgans
authors.forename: Hugues
authors.initials: H
authors.lastname: Chabriat
mesh_terms: D000328:Adult; D001479:Basal Ganglia / Q000473:pathology; D020520:Brain Infarction / Q000473:pathology; D046589:CADASIL / Q000473:pathology*; D002467:Cell Nucleus / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D013788:Thalamus / Q000473:pathology*