Monitoring Disease Progression in CADASIL With Diffusion Magnetic Resonance Imaging

Abstract

Background and Purpose-In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a large increase in water diffusion has been found both inside and outside the cerebral lesions as detected on conventional MRI. The aim of the present study was to assess the sensitivity of diffusion tensor imaging for monitoring the progression of cerebral tissue damage during the course of CADASIL. Methods-With the use of diffusion tensor imaging, whole brain trace of the diffusion tensor [Trace(D)] histograms were obtained in 22 CADASIL patients and 12 age-matched controls at baseline, in 14 patients after a mean delay of 21 months, and in 5 controls after a mean delay of 29 months. Parameters derived from these histograms (mean value, peak height, and peak location) were analyzed at baseline and during the follow-up. Results-At baseline, all the histogram parameters differed between patients and controls and were found to be significantly correlated with both the Mini-Mental State Examination score and Rankin Scale score in the patient group. The follow-up study showed a decrease in the peak height associated with an increase in the mean value of whole brain Trace(D) histograms in the 14 CADASIL patients scanned twice. The diffusion changes appeared larger in the patients whose Rankin score increased during the study period. Conclusions-These results suggest that the measurement of water diffusion over time is a sensitive marker for the progression of tissue damage in the brain. Thus, quantitative diffusion MRI can be used to monitor disease progression in CADASIL and possibly in other types of small-vessel brain disorders.

Longitudinal Diffusion Changes in Cerebral Hemispheres after MCA Infarcts

“…Despite this discrepancy, we think that the reduction in anisotropy detected in the contralateral hemisphere at M6 is not linked to artifacts for different reasons. First, the histogram method used in the present study offers many advantages compared with other techniques of imaging analysis and has been validated in various conditions (Molko et al, 2002;Nusbaum et al, 2000). Particularly, this highly reproducible method does not require operatordependant delineation of regions of interest or algorithms for registration (Molko et al, 2002;Sormani et al, 2000).…”

“…First, the histogram method used in the present study offers many advantages compared with other techniques of imaging analysis and has been validated in various conditions (Molko et al, 2002;Nusbaum et al, 2000). Particularly, this highly reproducible method does not require operatordependant delineation of regions of interest or algorithms for registration (Molko et al, 2002;Sormani et al, 2000). Second, the results obtained using this technique at the global and regional levels in the infarcted hemisphere provided coherent data and confirmed results already obtained by different authors (Werring et al, 2000;Zelaya et al, 1999).…”

CADASIL: pathogenesis, clinical and radiological findings and treatment

“…In the future, neurobiological markers of early injury will probably be used in studies trying to influence the onset and progression of clinical changes; this should help reducing the number of patients needed in therapeutic studies. The search for these markers in MR studies involving analysis of the patterns of appearance and progression of atrophy and accumulation of white matter lesions and other typical structural changes is under way, as well as analyses of diffusion histograms and metabolic relations as assessed by spectroscopy 89,[132][133][134][135] . The main marker of disease progression is, unfortunately, increasing age, which can be seen as a non modifiable risk factor.…”

Predictors of Clinical Worsening in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

“…Hence, counseling mutation carriers and selecting patients for clinical trials 5 have been difficult. Given the paucity of prognostic data, [6][7][8][9][10] we initiated a prospective longitudinal study to identify the predictors of clinical progression.…”