Donate Help Contact The AHA Sign In Home
American Heart Association
Stroke
Search: search_blue_button Advanced Search
« Previous Article | Table of Contents | Next Article »
Stroke. 1993;24:122-125

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Baudrimont, M.
Right arrow Articles by Bousser, M. G.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Baudrimont, M.
Right arrow Articles by Bousser, M. G.
Right arrowPubmed/NCBI databases
*OMIM

Stroke, Vol 24, 122-125, Copyright © 1993 by American Heart Association

ARTICLES

Autosomal dominant leukoencephalopathy and subcortical ischemic stroke. A clinicopathological study

M Baudrimont, F Dubas, A Joutel, E Tournier-Lasserve and MG Bousser
Service de Neurologie A, INSERM U 298, Centre Hospitalier Universitaire, Angers, France.

BACKGROUND AND PURPOSE: We recently described an autosomal dominant syndrome characterized mainly by recurrent strokes and neuroimaging evidence of leukoencephalopathy. We now report the pathological findings in one of the affected subjects. CASE DESCRIPTION: A 40-year- old woman experienced her first grand mal seizure in 1971. From 1983 on she suffered recurrent strokes, seizures, and psychiatric disturbances with depressions, manic episodes, and dementia. In 1988, after her fourth stroke, she became tetraplegic with a severe pseudobulbar palsy, and she died in 1990. Pathological examination disclosed a recent capsulolenticular hematoma, multiple small deep infarcts, a diffuse myelin loss and pallor of the hemispheric white matter, and a widespread vasculopathy of the small arteries penetrating the white matter. The arterial wall was markedly thickened with an extensive nonamyloid eosinophilic deposit in the media and reduplication of the internal elastic lamella. CONCLUSIONS: The underlying lesion of this hereditary disorder is located in the small arteries and is of unknown etiology. It differs from arteriosclerotic and amyloid angiopathies but is similar to that described in some cases of hereditary multi-infarct dementia.


This article has been cited by other articles:


Home page
 BrainHome page
S. Tikka, K. Mykkanen, M.-M. Ruchoux, R. Bergholm, M. Junna, M. Poyhonen, H. Yki-Jarvinen, A. Joutel, M. Viitanen, M. Baumann, et al.
Congruence between NOTCH3 mutations and GOM in 131 CADASIL patients
Brain, April 1, 2009; 132(4): 933 - 939.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M. Monet, V. Domenga, B. Lemaire, C. Souilhol, F. Langa, C. Babinet, T. Gridley, E. Tournier-Lasserve, M. Cohen-Tannoudji, and A. Joutel
The archetypal R90C CADASIL-NOTCH3 mutation retains NOTCH3 function in vivo
Hum. Mol. Genet., April 15, 2007; 16(8): 982 - 992.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
A. Viswanathan, F. Gray, M.-G. Bousser, M. Baudrimont, and H. Chabriat
Cortical Neuronal Apoptosis in CADASIL
Stroke, November 1, 2006; 37(11): 2690 - 2695.
[Abstract] [Full Text] [PDF]

Home page
 DevelopmentHome page
L. S. Tang, H. M. Alger, and F. A. Pereira
COUP-TFI controls Notch regulation of hair cell and support cell differentiation
Development, September 15, 2006; 133(18): 3683 - 3693.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
A Ragoschke-Schumm, H Axer, O W Witte, S Isenmann, C Fitzek, M Dichgans, N Peters, and J Mueller-Hoecker
Intracerebral haemorrhage in CADASIL
J. Neurol. Neurosurg. Psychiatry, November 1, 2005; 76(11): 1606 - 1607.
[Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
A V MacLean, R Woods, L M Alderson, S P Salloway, S Correia, S Cortez, and E G Stopa
Spontaneous lobar haemorrhage in CADASIL
J. Neurol. Neurosurg. Psychiatry, March 1, 2005; 76(3): 456 - 457.
[Full Text] [PDF]

Home page
 StrokeHome page
C. Dubroca, P. Lacombe, V. Domenga, J. Maciazek, B. Levy, E. Tournier-Lasserve, A. Joutel, and D. Henrion
Impaired Vascular Mechanotransduction in a Transgenic Mouse Model of CADASIL Arteriopathy
Stroke, January 1, 2005; 36(1): 113 - 117.
[Abstract] [Full Text] [PDF]

Home page
 RadiologyHome page
R. van den Boom, S. A. J. Lesnik Oberstein, M. D. Ferrari, J. Haan, and M. A. van Buchem
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy: MR Imaging Findings at Different Ages--3rd-6th Decades
Radiology, December 1, 2003; 229(3): 683 - 690.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
V. Parisi, F. Pierelli, F. Fattapposta, F. Bianco, L. Parisi, R. Restuccia, A. Malandrini, M. Ferrari, and P. Carrera
Early visual function impairment in CADASIL
Neurology, June 24, 2003; 60(12): 2008 - 2010.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
V Thijs, W Robberecht, R De Vos, and R Sciot
Coexistence of CADASIL and Alzheimer's disease
J. Neurol. Neurosurg. Psychiatry, June 1, 2003; 74(6): 790 - 792.
[Abstract] [Full Text] [PDF]

Home page
 Arterioscler. Thromb. Vasc. Bio.Home page
T. Iso, Y. Hamamori, and L. Kedes
Notch Signaling in Vascular Development
Arterioscler Thromb Vasc Biol, April 1, 2003; 23(4): 543 - 553.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
M. M. Ruchoux, V. Domenga, P. Brulin, J. Maciazek, S. Limol, E. Tournier-Lasserve, and A. Joutel
Transgenic Mice Expressing Mutant Notch3 Develop Vascular Alterations Characteristic of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Am. J. Pathol., January 1, 2003; 162(1): 329 - 342.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
N. Molko, S. Pappata, J.-F. Mangin, F. Poupon, D. LeBihan, M.-G. Bousser, and H. Chabriat
Monitoring Disease Progression in CADASIL With Diffusion Magnetic Resonance Imaging: A Study With Whole Brain Histogram Analysis
Stroke, December 1, 2002; 33(12): 2902 - 2908.
[Abstract] [Full Text] [PDF]

Home page
 RadiologyHome page
R. van den Boom, S. A. J. Lesnik Oberstein, S. G. van Duinen, M. Bornebroek, M. D. Ferrari, J. Haan, and M. A. van Buchem
Subcortical Lacunar Lesions: An MR Imaging Finding in Patients with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Radiology, September 1, 2002; 224(3): 791 - 796.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
M. Dichgans, M. Holtmannspotter, J. Herzog, N. Peters, M. Bergmann, and T. A. Yousry
Cerebral Microbleeds in CADASIL: A Gradient-Echo Magnetic Resonance Imaging and Autopsy Study
Stroke, January 1, 2002; 33(1): 67 - 71.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
S. A. J. Lesnik Oberstein, R. van den Boom, M. A. van Buchem, H. C. van Houwelingen, E. Bakker, E. Vollebregt, M. D. Ferrari, M. H. Breuning, and J. Haan
Cerebral microbleeds in CADASIL
Neurology, September 25, 2001; 57(6): 1066 - 1070.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
N. Molko, S. Pappata, J.F. Mangin, C. Poupon, K. Vahedi, A. Jobert, D. LeBihan, M.G. Bousser, and H. Chabriat
Diffusion Tensor Imaging Study of Subcortical Gray Matter in CADASIL
Stroke, September 1, 2001; 32(9): 2049 - 2054.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
D. P. Auer, T. Schirmer, J. O. Heidenreich, J. Herzog, B. Putz, and M. Dichgans
Altered white and gray matter metabolism in CADASIL: A proton MR spectroscopy and 1H-MRSI study
Neurology, March 13, 2001; 56(5): 635 - 642.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
M-G BOUSSER and E TOURNIER-LASSERVE
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: from stroke to vessel wall physiology
J. Neurol. Neurosurg. Psychiatry, March 1, 2001; 70(3): 285 - 287.
[Full Text] [PDF]

Home page
 RadiologyHome page
D. P. Auer, B. Pütz, C. Gössl, G.-K. Elbel, T. Gasser, and M. Dichgans
Differential Lesion Patterns in CADASIL and Sporadic Subcortical Arteriosclerotic Encephalopathy: MR Imaging Study with Statistical Parametric Group Comparison
Radiology, February 1, 2001; 218(2): 443 - 451.
[Abstract] [Full Text]

Home page
 StrokeHome page
K. J. Fryxell, M. Soderlund, T. V. Jordan, J. M. Verdi, and C. J. Kubu
An Animal Model for the Molecular Genetics of CADASIL
Stroke, January 1, 2001; 32(1): 6 - 11.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
A. Hassan and H. S. Markus
Genetics and ischaemic stroke
Brain, September 1, 2000; 123(9): 1784 - 1812.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
H. Chabriat, S. Pappata, L. Ostergaard, C. A. Clark, M. Pachot-Clouard, K. Vahedi, A. Jobert, D. Le Bihan, and M. G. Bousser
Cerebral Hemodynamics in CADASIL Before and After Acetazolamide Challenge Assessed With MRI Bolus Tracking
Stroke, August 1, 2000; 31(8): 1904 - 1912.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
J. G. Merino and V. Hachinski
Leukoaraiosis: Reifying Rarefaction
Arch Neurol, July 1, 2000; 57(7): 925 - 926.
[Full Text] [PDF]

Home page
 StrokeHome page
H. Chabriat, S. Pappata, C. Poupon, C. A. Clark, K. Vahedi, F. Poupon, J. F. Mangin, M. Pachot-Clouard, A. Jobert, D. Le Bihan, et al.
Clinical Severity in CADASIL Related to Ultrastructural Damage in White Matter : In Vivo Study With Diffusion Tensor MRI
Stroke, December 1, 1999; 30(12): 2637 - 2643.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
A. J. Larner, D. Kidd, P. Elkington, P. Rudge, and F. Scaravilli
Spatz-Lindenberg Disease : A Rare Cause of Vascular Dementia
Stroke, March 1, 1999; 30(3): 687 - 689.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
D. K. Jones, D. Lythgoe, M. A. Horsfield, A. Simmons, S. C. R. Williams, and H. S. Markus
Characterization of White Matter Damage in Ischemic Leukoaraiosis with Diffusion Tensor MRI
Stroke, February 1, 1999; 30(2): 393 - 397.
[Abstract] [Full Text] [PDF]

Home page
 StrokeHome page
H. Chabriat, R. Mrissa, C. Levy, K. Vahedi, H. Taillia, M. T. Iba-Zizen, A. Joutel, E. Tournier-Lasserve, and M.-G. Bousser
Brain Stem MRI Signal Abnormalities in CADASIL
Stroke, February 1, 1999; 30(2): 457 - 459.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Neuroradiol.Home page
T. A. Yousry, K. Seelos, M. Mayer, R. Brüning, I. Uttner, M. Dichgans, S. Mammi, A. Straube, N. Mai, and M. Filippi
Characteristic MR Lesion Pattern and Correlation of T1 and T2 Lesion Volume with Neurologic and Neuropsychological Findings in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
AJNR Am. J. Neuroradiol., January 1, 1999; 20(1): 91 - 100.
[Abstract] [Full Text]

Home page
 StrokeHome page
L. Pantoni and J. H. Garcia
Pathogenesis of Leukoaraiosis : A Review
Stroke, March 1, 1997; 28(3): 652 - 659.
[Abstract] [Full Text]

Home page
 StrokeHome page
G. C. Roman
From UBOs to Binswanger's Disease: Impact of Magnetic Resonance Imaging on Vascular Dementia Research
Stroke, August 1, 1996; 27(8): 1269 - 1273.
[Full Text]

Home page
 Arch NeurolHome page
R. W. Baloh and H. V. Vinters
White Matter Lesions and Disequilibrium in Older People: II. Clinicopathologic Correlation
Arch Neurol, October 1, 1995; 52(10): 975 - 981.
[Abstract] [PDF]

Home page
 StrokeHome page
L. Pantoni and J. H. Garcia
The Significance of Cerebral White Matter Abnormalities 100 Years After Binswanger's Report : A Review
Stroke, July 1, 1995; 26(7): 1293 - 1301.
[Abstract] [Full Text]


Stroke Home | Subscriptions | Archives | Feedback | Authors | Help | AHA Journals Home | Search
Copyright © 1993 American Heart Association, Inc. All rights reserved. Unauthorized use prohibited.