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(Stroke. 1999;30:457-459.)
© 1999 American Heart Association, Inc.

Short Communication

Brain Stem MRI Signal Abnormalities in CADASIL

H. Chabriat, MD; R. Mrissa, MD; C. Levy, MD; K. Vahedi, MD; H. Taillia, MD; M. T. Iba-Zizen, MD; A. Joutel, MD, PhD; E. Tournier-Lasserve, MD M.-G. Bousser, MD

From the Service de Neurologie, Hôpital Lariboisière (H.C., R.M., K.V., H.T., M.-G.B.); the Service de Radiologie, Hôpital Saint-Antoine (C.L.), and the Service de Neuroradiologie, Hôpital des XV-XX (M.T.I.), INSERM U25 (A.J., E.T.-L.), Faculté de Médecine Necker, Paris, France.

Correspondence to Pr Bousser M.G., Service de Neurologie, Hôpital Lariboisière, 2 rue Ambroise Paré, Paris, France.

Abstract

Background—We recently showed that the severity of MRI signal abnormalities increases with age in CADASIL, an arteriopathy due to mutations of notch 3 gene on chromosome 19. Previous results also suggest that the various hemispheric subcortical areas have a different vulnerability to ischemia in this disease. The distribution of the lesions at the brain stem level has not yet been reported.

Case Descriptions—We reviewed the MRIs of 68 affected patients having signal abnormalities in the hemispheric white matter to assess the distribution and clinical consequences of brain stem signal abnormalities in CADASIL. We found hypersignals on T2-weighted images in the brain stem in 45% of the subjects. The pons was more frequently involved (100%) than the mesencephalon (69%) and the medulla (35%). Hyposignals on T1-weighted images, at the brain stem level, were observed only in two thirds of these subjects. The lack of signal abnormalities reaching the brain stem surface and the absence of cerebellar lesions were noteworthy.

Conclusions—Brain stem signal abnormalities observed in CADASIL are found in regions irrigated only by perforating arteries. These results support parallel observations made for CADASIL-associated signal abnormalities in the cerebral hemispheres and emphasize the importance of the angioarchitecture of the cerebral vasculature to explain why a condition characterized by a systemic vessel wall pathology is manifested only as a brain disease.

Key Words: brain stem • CADASIL • cerebral arteries • hypersignals • magnetic resonance imaging

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