PMID- 10227618
OWN - NLM
STAT- MEDLINE
DCOM- 19990517
LR  - 20190514
IS  - 0028-3878 (Print)
IS  - 0028-3878 (Linking)
VI  - 52
IP  - 7
DP  - 1999 Apr 22
TI  - Quantitative MRI in CADASIL: correlation with disability and cognitive 
      performance.
PG  - 1361-7
AB  - OBJECTIVE: To study correlations between total lesion load on brain MRI and 
      clinical features, and to evaluate the influence of demographic variables on 
      quantitative MRI variables in cerebral autosomal dominant arteriopathy with 
      subcortical infarcts and leukoencephalopathy (CADASIL). BACKGROUND: CADASIL is a 
      hereditary form of small-vessel disease caused by mutations within the Notch3 
      gene. MRI abnormalities have been found both in asymptomatic and symptomatic 
      CADASIL individuals. METHODS: Quantitative measurements on cerebral MRI were 
      performed in 64 CADASIL individuals. MRI lesions were quantified using a 
      semi-automated segmentation technique based on local thresholds. RESULTS: MRI 
      total lesion volume correlated significantly with disability (Rankin Scale) on 
      both T1- and proton density (PD)-weighted images. There was a significant inverse 
      correlation between total lesion volume and overall cognitive performance as 
      determined by the Mini-Mental State Examination. Age but not sex was correlated 
      with lesion load both on T1- and PD-weighted images. There was no detectable 
      influence of the Notch3 genotype on quantitative MRI variables. CONCLUSIONS: This 
      study demonstrates correlations between MRI lesion volume and clinical 
      characteristics in CADASIL. Longitudinal studies are now warranted to investigate 
      whether quantitative MRI could be used as an adjunct outcome measure in future 
      therapeutic trials in CADASIL.
FAU - Dichgans, M
AU  - Dichgans M
AD  - Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians-University, 
      Munich, Germany. mdichgans@brain.nefo.med.uni-muenchen.de
FAU - Filippi, M
AU  - Filippi M
FAU - Bruning, R
AU  - Bruning R
FAU - Iannucci, G
AU  - Iannucci G
FAU - Berchtenbreiter, C
AU  - Berchtenbreiter C
FAU - Minicucci, L
AU  - Minicucci L
FAU - Uttner, I
AU  - Uttner I
FAU - Crispin, A
AU  - Crispin A
FAU - Ludwig, H
AU  - Ludwig H
FAU - Gasser, T
AU  - Gasser T
FAU - Yousry, T A
AU  - Yousry TA
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
SB  - IM
MH  - Adult
MH  - Aged
MH  - Brain/pathology/physiopathology
MH  - Cerebral Arterial Diseases/*pathology/psychology
MH  - Cerebral Infarction/*pathology/psychology
MH  - Cognition/physiology
MH  - Disability Evaluation
MH  - Female
MH  - Humans
MH  - Leukoencephalopathy, Progressive Multifocal/*pathology/psychology
MH  - Magnetic Resonance Imaging/*methods
MH  - Male
MH  - Middle Aged
EDAT- 1999/05/05 00:00
MHDA- 1999/05/05 00:01
CRDT- 1999/05/05 00:00
PHST- 1999/05/05 00:00 [pubmed]
PHST- 1999/05/05 00:01 [medline]
PHST- 1999/05/05 00:00 [entrez]
AID - 10.1212/wnl.52.7.1361 [doi]
PST - ppublish
SO  - Neurology. 1999 Apr 22;52(7):1361-7. doi: 10.1212/wnl.52.7.1361.