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Titolo:
Clinical severity in CADASIL related to ultrastructural damage in white matter - In vivo study with diffusion tensor MRI
Autore:
Chabriat, H; Pappata, S; Poupon, C; Clark, CA; Vahedi, K; Poupon, F; Mangin, JF; Pachot-Clouard, M; Jobert, A; Le Bihan, D; Bousser, MG;
Indirizzi:
Univ Paris 07, CHU Lariboisiere, Dept Neurol, Orsay, France Univ Paris 07Orsay France CHU Lariboisiere, Dept Neurol, Orsay, France CEA, Serv Hosp Frederic Joliot, DRM, F-91406 Orsay, France CEA Orsay France F-91406 osp Frederic Joliot, DRM, F-91406 Orsay, France INSERM U334, CEA, SHFJ, F-91406 Orsay, France INSERM U334 Orsay France F-91406 U334, CEA, SHFJ, F-91406 Orsay, France
Titolo Testata:
STROKE
fascicolo: 12, volume: 30, anno: 1999,
pagine: 2637 - 2643
SICI:
0039-2499(199912)30:12<2637:CSICRT>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
AUTOSOMAL-DOMINANT ARTERIOPATHY; STATE-EXAMINATION MMSE; SUBCORTICAL INFARCTS; ANISOTROPIC DIFFUSION; CEREBRAL-ISCHEMIA; NOTCH3 MUTATIONS; WATER DIFFUSION; LEUKOENCEPHALOPATHY; DEMENTIA; STROKE;
Keywords:
dementia; diffusion; leukoencephalopathy; magnetic resonance imaging; white matter;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
49
Recensione:
Indirizzi per estratti:
Indirizzo: Chabriat, H Hop Lariboisiere, Neurol Serv, 2 Rue Ambroise Pare, F-75010 Paris, France Hop Lariboisiere 2 Rue Ambroise Pare Paris France F-75010 nce
Citazione:
H. Chabriat et al., "Clinical severity in CADASIL related to ultrastructural damage in white matter - In vivo study with diffusion tensor MRI", STROKE, 30(12), 1999, pp. 2637-2643

Abstract

Background and Purpose-CADASIL is a newly recognized cause of subcortical ischemic strokes that progressively leads to dementia associated with pseudobulbar palsy and severe motor disability. This deleterious progression andthe severity of clinical presentation are widely variable among affected subjects. The exact role played by MRI white-matter abnormalities, a hallmark of the disease, in the severity of the clinical phenotype remains poorly understood,Methods-To address this issue, we used diffusion tensor imaging (DTI), a new MRI technique highly sensitive to white-matter microstructural changes, in 16 symptomatic patients and 10 age-matched controls. Mean diffusivity and anisotropy of diffusion were measured within hyperintensities identified on T2-weighted images (T2WI) and outside these lesions on 4 slices at the level of centrum semiovale. Results-We found a 60% increase of water mean diffusivity and a parallel loss of diffusion anisotropy in hyperintensities identified on T2WI, The same pattern of diffusion changes, but of lesser intensity, was found in the normal-appearing white matter on T2WI, Mean diffusivity in regions with increased signal on T2WI was higher in patients with severe clinical disabilitycompared with those with no or mild deficit (1.33+/-0.11 versus 1.13t0.11 10(-3) mm(2)/s, P<0.01), Furthermore, diffusion measured within T2 hyperintensities correlated with both the Mini-Mental State Examination and Rankin scale scores. In patients with a severe clinical status, the increase of water diffusion in these regions exceeded 70% in comparison with values obtained in the normal white matter in control subjects. Conclusions-These results indicate that DTI is able to detect important ultrastructural changes in regions with increased signal on T2WI and within the normal-appearing white matter in CADASIL, The diffusion changes might berelated to both neuronal loss and demyelination, The degree of the underlying ultrastructural alterations is related to the severity of the clinical status with a possible threshold level of white-matter damage above which severe neurological impairment may occur in this disease. DTI appears to be a promising technique for monitoring disease progression in CADASIL.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/09/20 alle ore 00:54:17