CADASIL

[1]  E. Vicaut,et al.  Characteristic Features of in vivo Skin Microvascular Reactivity in CADASIL , 2007, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[2]  F. Walker Huntington's disease , 2007, The Lancet.

[3]  Brian Levine,et al.  Cognitive rehabilitation in the elderly: A randomized trial to evaluate a new protocol , 2006, Journal of the International Neuropsychological Society.

[4]  Ji-Kang Park,et al.  Intracerebral hemorrhages in CADASIL , 2006, Neurology.

[5]  A. Członkowska,et al.  Ultrastructural picture of blood vessels in muscle and skin biopsy in CADASIL. , 2006, Folia neuropathologica.

[6]  J. D. De Bleecker,et al.  Intracerebral haemorrhage in CADASIL. A case report. , 2006, Acta neurologica Belgica.

[7]  S. McFadden,et al.  Auditory processing disorder in children diagnosed with nonverbal learning disability. , 2006, American journal of audiology.

[8]  M. Dichgans,et al.  Heritability of MRI Lesion Volume in CADASIL: Evidence for Genetic Modifiers , 2006 .

[9]  H. Chabriat,et al.  Cortical Neuronal Apoptosis in CADASIL , 2006, Stroke.

[10]  H. Chabriat,et al.  Multiple Simultaneous Cerebral Infarctions in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy , 2006, Cerebrovascular Diseases.

[11]  H. Chabriat,et al.  Formes pauci symptomatiques de CADASIL après 60 ans , 2006 .

[12]  M. Viitanen,et al.  Arterioles of the Lenticular Nucleus in CADASIL , 2006, Stroke.

[13]  Martin Dichgans,et al.  Blood pressure and haemoglobin A1c are associated with microhaemorrhage in CADASIL: a two-centre cohort study. , 2006, Brain : a journal of neurology.

[14]  M. Viitanen,et al.  Ophthalmologic findings in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: a cross-sectional study. , 2006, Ophthalmology.

[15]  M. Viitanen,et al.  Cerebral arteriolar pathology in a 32-year-old patient with CADASIL. , 2006, Neuropathology and applied neurobiology.

[16]  E. Nagata,et al.  Notch3 ectodomain is a major component of granular osmiophilic material (GOM) in CADASIL , 2006, Acta Neuropathologica.

[17]  T. Tabira,et al.  CADASIL-causing mutations do not alter Notch3 receptor processing and activation , 2006, Neuroreport.

[18]  Yaakov Stern,et al.  Cognitive reserve and Alzheimer disease. , 2006, Alzheimer disease and associated disorders.

[19]  V. Álvarez,et al.  A new de novo Notch3 mutation causing CADASIL , 2006, European journal of neurology.

[20]  I. Bone,et al.  Single gene disorders causing ischaemic stroke , 2006, Journal of Neurology.

[21]  J. S. Kim,et al.  Characteristics of CADASIL in Korea , 2006, Neurology.

[22]  M. Dichgans,et al.  Brain volume changes in CADASIL , 2006, Neurology.

[23]  A. Federico,et al.  Physiology and pathology of notch signalling system , 2006, Journal of cellular physiology.

[24]  J. Sundberg,et al.  Role of COL4A1 in small-vessel disease and hemorrhagic stroke. , 2006, The New England journal of medicine.

[25]  E. Vicaut,et al.  Electrocardiogram in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Patients Without Any Clinical Evidence of Coronary Artery Disease: A Case-Control Study , 2006, Stroke.

[26]  H. Chabriat,et al.  A novel hereditary small vessel disease of the brain , 2006, Annals of neurology.

[27]  H. Chabriat,et al.  Dilation of Virchow‐Robin spaces in CADASIL , 2006, European journal of neurology.

[28]  R. Porcher,et al.  Cognitive profile in CADASIL , 2006, Journal of Neurology, Neurosurgery & Psychiatry.

[29]  L. Pantoni,et al.  Cerebellar arteriovenous malformation and vertebral artery aneurysm in a CADASIL patient , 2006, Acta neurologica Scandinavica.

[30]  H. Chabriat,et al.  Cerebral microhemorrhage. Commentary , 2006 .

[31]  L. Murray,et al.  Treating attention in mild aphasia: evaluation of attention process training-II. , 2006, Journal of communication disorders.

[32]  A. Federico,et al.  Systemic Blood Pressure Profile in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy , 2005, Stroke.

[33]  Nils Peters,et al.  Diffusion Magnetic Resonance Histograms as a Surrogate Marker and Predictor of Disease Progression in CADASIL: A Two-Year Follow-Up Study , 2005, Stroke.

[34]  Hugues Chabriat,et al.  Diffusion histograms in CADASIL. , 2005, Stroke.

[35]  M. O’Sullivan,et al.  Damage within a network of white matter regions underlies executive dysfunction in CADASIL , 2005, Neurology.

[36]  A. Danek,et al.  The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia. , 2005, The American journal of psychiatry.

[37]  C. Sudlow,et al.  Comparing risks of death and recurrent vascular events between lacunar and non-lacunar infarction. , 2005, Brain : a journal of neurology.

[38]  Jean-François Mangin,et al.  Shape and Volume of Lacunar Infarcts: A 3D MRI Study in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy , 2005, Stroke.

[39]  H. Markus,et al.  The spatial distribution of MR imaging abnormalities in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy and their relationship to age and clinical features. , 2005, AJNR. American journal of neuroradiology.

[40]  S. Salloway,et al.  Donepezil in Vascular Dementia: Combined Analysis of Two Large-Scale Clinical Trials , 2005, Dementia and Geriatric Cognitive Disorders.

[41]  C. Choi,et al.  Large cerebral artery involvement in CADASIL , 2005, Neurology.

[42]  O. Witte,et al.  Intracerebral haemorrhage in CADASIL , 2005, Journal of Neurology Neurosurgery & Psychiatry.

[43]  D. St. Clair,et al.  Functional analysis of a recurrent missense mutation in Notch3 in CADASIL , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[44]  M. Dichgans,et al.  Spectrum of mutations in biopsy-proven CADASIL: implications for diagnostic strategies. , 2005, Archives of neurology.

[45]  T. Tabira,et al.  Functional redundancy of the Notch gene family during mouse embryogenesis: analysis of Notch gene expression in Notch3-deficient mice. , 2005, Biochemical and biophysical research communications.

[46]  P. Libby,et al.  CADASIL mutations impair Notch3 glycosylation by Fringe. , 2005, Human molecular genetics.

[47]  A. Federico,et al.  The spectrum of mutations for CADASIL diagnosis , 2005, Neurological Sciences.

[48]  H. Wiendl,et al.  CADASIL: underdiagnosed in psychiatric patients? , 2005, Acta psychiatrica Scandinavica.

[49]  Raphaël Porcher,et al.  Longitudinal Diffusion Changes in Cerebral Hemispheres after MCA Infarcts , 2005, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[50]  A. Joutel,et al.  Impaired Cerebral Vasoreactivity in a Transgenic Mouse Model of Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Arteriopathy , 2005, Stroke.

[51]  I. Bone,et al.  The prevalence of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) in the west of Scotland , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[52]  A. Federico,et al.  Peripheral neuropathy in CADASIL , 2005, Journal of Neurology.

[53]  L. Pantoni,et al.  The spectrum of Notch3 mutations in 28 Italian CADASIL families , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[54]  B. Singhal Leukodystrophies: Indian scenario , 2005, Indian journal of pediatrics.

[55]  S. Salloway,et al.  Spontaneous lobar haemorrhage in CADASIL , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[56]  D. Dodick,et al.  Migraine and Cerebral White Matter Lesions: When to Suspect Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) , 2005, The neurologist.

[57]  M. Viitanen,et al.  Mice carrying a R142C Notch 3 knock–in mutation do not develop a CADASIL‐like phenotype , 2005, Genesis.

[58]  B. Lévy,et al.  Impaired Vascular Mechanotransduction in a Transgenic Mouse Model of CADASIL Arteriopathy , 2005, Stroke.

[59]  M. Mascalchi,et al.  Slowly progressive familial dementia with recurrent strokes and white matter hypodensities on CT scan , 1992, The Italian Journal of Neurological Sciences.

[60]  M. Viitanen,et al.  The pathogenesis of CADASIL: an update , 2004, Journal of the Neurological Sciences.

[61]  B. Klonjkowski,et al.  Notch3 is required for arterial identity and maturation of vascular smooth muscle cells. , 2004, Genes & development.

[62]  M. Viitanen,et al.  Scanning Laser Doppler Flowmetry Shows Reduced Retinal Capillary Blood Flow in CADASIL , 2004, Stroke.

[63]  M. Dichgans,et al.  Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients. , 2004, Brain : a journal of neurology.

[64]  M. Viitanen,et al.  Fibrosis and Stenosis of the Long Penetrating Cerebral Arteries: the Cause of the White Matter Pathology in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy , 2004, Brain pathology.

[65]  M. Dichgans,et al.  CADASIL-associated Notch3 mutations have differential effects both on ligand binding and ligand-induced Notch3 receptor signaling through RBP-Jk. , 2004, Experimental cell research.

[66]  M. Viitanen,et al.  Detection of the founder effect in Finnish CADASIL families , 2004, European Journal of Human Genetics.

[67]  H. Markus,et al.  The influence of genetic and cardiovascular risk factors on the CADASIL phenotype. , 2004, Brain : a journal of neurology.

[68]  M. Dichgans,et al.  Histopathological abnormalities in ocular blood vessels of CADASIL patients. , 2004, American journal of ophthalmology.

[69]  A. Federico,et al.  Hemodynamic evaluation of the optic nerve head in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. , 2004, Archives of neurology.

[70]  H. Chabriat,et al.  Migraine with aura and brain magnetic resonance imaging abnormalities in patients with CADASIL. , 2004, Archives of neurology.

[71]  V. Mok,et al.  Effect of White Matter Changes on Cognitive Impairment in Patients With Lacunar Infarcts , 2004, Stroke.

[72]  M. Dichgans,et al.  A Two-Year Clinical Follow-Up Study in 80 CADASIL Subjects: Progression Patterns and Implications for Clinical Trials , 2004, Stroke.

[73]  M. Viitanen,et al.  Insidious Cognitive Decline in CADASIL , 2004, Stroke.

[74]  M. Golomb,et al.  Recurrent hemiplegia, normal MRI, and NOTCH3 mutation in a 14-year-old: Is this early CADASIL? , 2004, Neurology.

[75]  P. Massin,et al.  Retinal abnormalities in CADASIL: a retrospective study of 18 patients , 2004, Journal of Neurology, Neurosurgery & Psychiatry.

[76]  A. Federico,et al.  A novel NOTCH3 frameshift deletion and mitochondrial abnormalities in a patient with CADASIL. , 2004, Archives of neurology.

[77]  Timo Kurki,et al.  Positron Emission Tomography Examination of Cerebral Blood Flow and Glucose Metabolism in Young CADASIL Patients , 2004, Stroke.

[78]  M. Dichgans,et al.  Retinal vascular abnormalities in CADASIL , 2004, Neurology.

[79]  H. Markus,et al.  Abnormal Vasoconstrictor Responses to Angiotensin II and Noradrenaline in Isolated Small Arteries From Patients With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) , 2004, Stroke.

[80]  M. O’Sullivan,et al.  Diffusion tensor imaging of thalamus correlates with cognition in CADASIL without dementia , 2004, Neurology.

[81]  H. Kwiecinski,et al.  CADASIL or CADVaSIL? , 2004, Neuropathology : official journal of the Japanese Society of Neuropathology.

[82]  F. Schweisguth,et al.  Regulation of Notch Signaling Activity , 2004, Current Biology.

[83]  A. Joutel,et al.  Pathogenic mutations associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy differently affect Jagged1 binding and Notch3 activity via the RBP/JK signaling Pathway. , 2004, American journal of human genetics.

[84]  R. Tiozzo,et al.  A new missense mutation (Cys297→Phe) of the low density lipoprotein receptor in Italian patients with familial hypercholesterolemia (FHTrieste) , 1994, Human Genetics.

[85]  O. Andersen,et al.  The microvascular changes in cases of hereditary multi-infarct disease of the brain , 1994, Acta Neuropathologica.

[86]  P. Sourander,et al.  Hereditary multi-infarct dementia , 1977, Acta Neuropathologica.

[87]  K. Kosik,et al.  Distribution pattern of Notch3 mutations suggests a gain-of-function mechanism for CADASIL. , 2004, Genomics.

[88]  D. Leys,et al.  Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy , 2004, Acta Neuropathologica.

[89]  B. Sellhaus,et al.  Identification of the characteristic vascular changes in a sural nerve biopsy of a case with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) , 2004, Acta Neuropathologica.

[90]  G. Amer,et al.  Small arterial granular degeneration in familial Binswanger's syndrome , 2004, Acta Neuropathologica.

[91]  M. Ferrari,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: MR imaging findings at different ages--3rd-6th decades. , 2003, Radiology.

[92]  U. Lendahl,et al.  Characterization of Notch3‐deficient mice: Normal embryonic development and absence of genetic interactions with a Notch1 mutation , 2003, Genesis.

[93]  E. Barbeau,et al.  A case of late-onset CADASIL with interhemispheric disconnection features , 2003, Journal of Neurology.

[94]  H. Kwiecinski,et al.  CADASIL: new cases and new questions , 2003, Acta Neuropathologica.

[95]  McKay Moore Sohlberg,et al.  Practice guidelines for direct attention training , 2003 .

[96]  J. Ahlskog,et al.  Progressive supranuclear palsy phenotype secondary to CADASIL. , 2003, Parkinsonism & related disorders.

[97]  J. Hauw,et al.  Glycohistochemical characterization of vascular muscle cell destruction in CADASIL subjects by lectins, neoglycoconjugates and galectin‐specific antibodies , 2003, Neuropathology and applied neurobiology.

[98]  Y. Stern The Concept of Cognitive Reserve: A Catalyst for Research , 2003, Journal of clinical and experimental neuropsychology.

[99]  Hugh S Markus,et al.  Frequency of subclinical lacunar infarcts in ischemic leukoaraiosis and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. , 2003, AJNR. American journal of neuroradiology.

[100]  L. McCullough,et al.  Estrogen and ischemic neuroprotection: an integrated view , 2003, Trends in Endocrinology & Metabolism.

[101]  G. Román,et al.  Vascular Dementia: A Historical Background , 2003, International Psychogeriatrics.

[102]  P. Macfarlane,et al.  Myocardial Infarction in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) , 2003, Medicine.

[103]  Nils Peters,et al.  Cortical hypometabolism and crossed cerebellar diaschisis suggest subcortically induced disconnection in CADASIL: an 18F-FDG PET study. , 2003, Journal of nuclear medicine : official publication, Society of Nuclear Medicine.

[104]  M. Ferrari,et al.  Evaluation of diagnostic NOTCH3 immunostaining in CADASIL , 2003, Acta Neuropathologica.

[105]  Faiza Behloul,et al.  Cerebral Hemodynamics and White Matter Hyperintensities in CADASIL , 2003, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[106]  M. Mesulam,et al.  Cholinergic denervation in a pure multi-infarct state , 2003, Neurology.

[107]  T. Gridley Notch signaling and inherited disease syndromes. , 2003, Human molecular genetics.

[108]  C. Duyckaerts,et al.  The vascular lesions in vascular and mixed dementia: the weight of functional neuroanatomy , 2003, Neurobiology of Aging.

[109]  Silent Infarcts Demonstrated by Diffusion-Weighted MRI in CADASIL , 2003, European Neurology.

[110]  H. Markus,et al.  “CADASIL coma”: an underdiagnosed acute encephalopathy , 2003, Journal of neurology, neurosurgery, and psychiatry.

[111]  P. Massin,et al.  Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy , 2003, Neurology.

[112]  H. Markus,et al.  Yield of Screening for CADASIL Mutations in Lacunar Stroke and Leukoaraiosis , 2003, Stroke.

[113]  A. Joutel,et al.  Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. , 2003, The American journal of pathology.

[114]  U. Lendahl,et al.  A CADASIL-mutated Notch 3 receptor exhibits impaired intracellular trafficking and maturation but normal ligand-induced signaling , 2002, Proceedings of the National Academy of Sciences of the United States of America.

[115]  Jean-François Mangin,et al.  Monitoring Disease Progression in CADASIL With Diffusion Magnetic Resonance Imaging: A Study With Whole Brain Histogram Analysis , 2002, Stroke.

[116]  M. Dichgans Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Phenotypic and mutational spectrum , 2002, Journal of the Neurological Sciences.

[117]  J. Brillault,et al.  Lessons from CADASIL , 2002, Annals of the New York Academy of Sciences.

[118]  R. Barber,et al.  Pathologies and Pathological Mechanisms for White Matter Hyperintensities in Depression , 2002, Annals of the New York Academy of Sciences.

[119]  K. Arima,et al.  Arterial Changes in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Relation to Pathogenesis of Diffuse Myelin Loss of Cerebral White Matter: Examination of Cerebral Medullary Arteries by Reconstruction of Serial Sections of an Autopsy Case , 2002, Stroke.

[120]  H. Chui,et al.  Subcortical ischaemic vascular dementia , 2002, The Lancet Neurology.

[121]  M. Simpson,et al.  Diagnostic strategies in CADASIL , 2002, Neurology.

[122]  I. Le Ber,et al.  Unusual presentation of CADASIL with reversible coma and confusion. , 2002, Neurology.

[123]  S. O'Riordan,et al.  CADASIL imitating multiple sclerosis: the importance of MRI markers , 2002, Multiple sclerosis.

[124]  M. Ferrari,et al.  Subcortical lacunar lesions: an MR imaging finding in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. , 2002, Radiology.

[125]  A. Malandrini,et al.  Asymptomatic cores and paracrystalline mitochondrial inclusions in CADASIL , 2002, Neurology.

[126]  K. Krishnan,et al.  Investigation of Notch3 as a candidate gene for bipolar disorder using brain hyperintensities as an endophenotype. , 2002, American journal of medical genetics.

[127]  U. Lendahl,et al.  Mouse Notch 3 expression in the pre- and postnatal brain: relationship to the stroke and dementia syndrome CADASIL. , 2002, Experimental cell research.

[128]  C. Godfraind,et al.  Morphometric analysis of ultrastructural vascular changes in CADASIL: analysis of 50 skin biopsy specimens and pathogenic implications , 2002, Acta Neuropathologica.

[129]  M. Viitanen,et al.  CADASIL: a Common Form of Hereditary Arteriopathy Causing Brain Infarcts and Dementia , 2002, Brain pathology.

[130]  Wenli Wang,et al.  Notch3 Signaling in Vascular Smooth Muscle Cells Induces c-FLIP Expression via ERK/MAPK Activation , 2002, The Journal of Biological Chemistry.

[131]  Y. Itoyama,et al.  Silent Cerebral Microbleeds on T2*-Weighted MRI: Correlation with Stroke Subtype, Stroke Recurrence, and Leukoaraiosis , 2002, Stroke.

[132]  T. Mathews,et al.  Cadasil: Presenting as a Mood Disorder , 2002, Scottish medical journal.

[133]  J. Boulter,et al.  CADASIL Notch3 Mutant Proteins Localize to the Cell Surface and Bind Ligand , 2002, Circulation research.

[134]  M. Dichgans CADASIL: A Monogenic Condition Causing Stroke and Subcortical Vascular Dementia , 2002, Cerebrovascular Diseases.

[135]  Y. Stern What is cognitive reserve? Theory and research application of the reserve concept , 2002, Journal of the International Neuropsychological Society.

[136]  Keith D. Cicerone,et al.  Remediation of 'working attention' in mild traumatic brain injury , 2002, Brain injury.

[137]  Nils Peters,et al.  Cerebral Microbleeds in CADASIL: A Gradient-Echo Magnetic Resonance Imaging and Autopsy Study , 2002, Stroke.

[138]  F. Feuerhake,et al.  Reversible coma with raised intracranial pressure: an unusual clinical manifestation of CADASIL , 2002, Acta Neuropathologica.

[139]  H. Chabriat,et al.  Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis , 2001, The Lancet.

[140]  W. Bradley,et al.  Acetazolamide for the treatment of migraine with aura in CADASIL , 2001, Neurology.

[141]  M. Dichgans,et al.  NOTCH3 mutation involving three cysteine residues in a family with typical CADASIL , 2001, Neurology.

[142]  M. Grandis,et al.  Very High Prevalence of Right-to-Left Shunt on Transcranial Doppler in an Italian Family with Cerebral Autosomal Dominant Angiopathy with Subcortical Infarcts and Leukoencephalopathy , 2001, European Neurology.

[143]  J. Molina,et al.  Mitochondrial dysfunction associated with a mutation in the Notch3 gene in a CADASIL family , 2001, Neurology.

[144]  K. Abe,et al.  Two cases of Japanese CADASIL with corpus callosum lesion. , 2001, The Tohoku journal of experimental medicine.

[145]  G. Weinmaster,et al.  Vascular expression of Notch pathway receptors and ligands is restricted to arterial vessels , 2001, Mechanisms of Development.

[146]  D. LeBihan,et al.  Diffusion Tensor Imaging Study of Subcortical Gray Matter in CADASIL , 2001, Stroke.

[147]  M. Viitanen,et al.  Phenotype of a Homozygous CADASIL Patient in Comparison to 9 Age-Matched Heterozygous Patients With the Same R133C Notch3 Mutation , 2001, Stroke.

[148]  R. Kalaria Advances in molecular genetics and pathology of cerebrovascular disorders , 2001, Trends in Neurosciences.

[149]  Paul Scherz,et al.  Functional analysis of secreted and transmembrane proteins critical to mouse development , 2001, Nature Genetics.

[150]  M. Filippi,et al.  A magnetic resonance imaging study of the cervical cord of patients with CADASIL , 2001, Neurology.

[151]  D. Le Bihan,et al.  Diffusion tensor imaging: Concepts and applications , 2001, Journal of magnetic resonance imaging : JMRI.

[152]  H S Markus,et al.  MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL , 2001, Neurology.

[153]  D. Auer,et al.  Altered white and gray matter metabolism in CADASIL , 2001, Neurology.

[154]  M Rovaris,et al.  Correlations Between Clinical Findings and Magnetization Transfer Imaging Metrics of Tissue Damage in Individuals With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy , 2001, Stroke.

[155]  M. Bousser,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: from stroke to vessel wall physiology , 2001 .

[156]  C Gössl,et al.  Differential lesion patterns in CADASIL and sporadic subcortical arteriosclerotic encephalopathy: MR imaging study with statistical parametric group comparison. , 2001, Radiology.

[157]  K. J. Fryxell,et al.  An Animal Model for the Molecular Genetics of CADASIL , 2001, Stroke.

[158]  M. Dichgans,et al.  Reduced Cerebrovascular CO2 Reactivity in CADASIL: A Transcranial Doppler Sonography Study , 2001, Stroke.

[159]  Nick C Fox,et al.  Description of a simple test for CADASIL disease and determination of mutation frequencies in sporadic ischaemic stroke and dementia patients , 2000, Journal of neurology, neurosurgery, and psychiatry.

[160]  H. Markus,et al.  Genetics and ischaemic stroke. , 2000, Brain : a journal of neurology.

[161]  D. Le Bihan,et al.  Cerebral hemodynamics in CADASIL before and after acetazolamide challenge assessed with MRI bolus tracking. , 2000, Stroke.

[162]  H. Chabriat,et al.  Splice site mutation causing a seven amino acid Notch3 in-frame deletion in CADASIL , 2000, Neurology.

[163]  M. Dichgans,et al.  Small in-frame deletions and missense mutations in CADASIL: 3D models predict misfolding of Notch3v EGF-like repeat domains , 2000, European Journal of Human Genetics.

[164]  D. Clair,et al.  Detection of complement factor B in the cerebrospinal fluid of patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy disease using two-dimensional gel electrophoresis and mass spectrometry , 2000, Neuroscience Letters.

[165]  D. Burn,et al.  Distribution of cranial MRI abnormalities in patients with symptomatic and subclinical CADASIL. , 2000, The British journal of radiology.

[166]  J. Parisi,et al.  De novo mutation in the Notch3 gene causing CADASIL , 2000, Annals of neurology.

[167]  F. Chapon,et al.  The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients. , 2000, The Journal of clinical investigation.

[168]  J Duncan,et al.  Rehabilitation of executive functioning: An experimental–clinical validation of Goal Management Training , 2000, Journal of the International Neuropsychological Society.

[169]  A. Hofman,et al.  Cerebral white matter lesions and cognitive function: The Rotterdam scan study , 2000, Annals of neurology.

[170]  H. Chabriat,et al.  [CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy): clinical features and neuroimaging]. , 2000, Bulletin de l'Academie nationale de medecine.

[171]  M. Nomura,et al.  Placenta Growth Factor and Vascular Endothelial Growth Factor B and C Expression in Microvascular Endothelial Cells and Pericytes , 1999, The Journal of Biological Chemistry.

[172]  D. Le Bihan,et al.  Clinical severity in CADASIL related to ultrastructural damage in white matter: in vivo study with diffusion tensor MRI. , 1999, Stroke.

[173]  B. Winblad,et al.  CADASIL: hereditary disease of arteries causing brain infarcts and dementia , 1999, Neuropathology and applied neurobiology.

[174]  M. Dichgans,et al.  Cerebrospinal fluid findings in CADASIL , 1999, Neurology.

[175]  J. Haan,et al.  Diagnostic Notch3 sequence analysis in CADASIL: Three new mutations in Dutch patients , 1999, Neurology.

[176]  J. Mohr,et al.  The natural history of CADASIL: a pooled analysis of previously published cases. , 1999, Stroke.

[177]  S. Artavanis-Tsakonas,et al.  Notch Signaling : Cell Fate Control and Signal Integration in Development , 1999 .

[178]  M. Filippi,et al.  Quantitative MRI in CADASIL , 1999, Neurology.

[179]  H. Chabriat,et al.  Brain stem MRI signal abnormalities in CADASIL. , 1999, Stroke.

[180]  K. Hewan-Lowe,et al.  Vasculopathic changes of CADASIL can be focal in skin biopsies. , 1999, Ultrastructural pathology.

[181]  D. Zelterman,et al.  Neutropenic infections in 100 patients with non-Hodgkin’s lymphoma or Hodgkin’s disease treated with high-dose BEAM chemotherapy and peripheral blood progenitor cell transplant: out-patient treatment is a viable option , 1999, Bone Marrow Transplantation.

[182]  A. Joutel,et al.  Notch signalling pathway and human diseases. , 1998, Seminars in cell & developmental biology.

[183]  J. Mohr CADASIL and white matter syndromes , 1998, Annals of neurology.

[184]  M. Dichgans,et al.  The phenotypic spectrum of CADASIL: Clinical findings in 102 cases , 1998, Annals of neurology.

[185]  A. Joutel,et al.  Differential diagnosis of a vascular leukoencephalopathy within a CADASIL family: use of skin biopsy electron microscopy study and direct genotypic screening , 1998, Journal of Neurology.

[186]  J. Mohr,et al.  CADASIL in a North American family , 1998, Neurology.

[187]  H. Chabriat,et al.  Patterns of MRI lesions in CADASIL , 1998, Neurology.

[188]  M. Moskowitz,et al.  Stroke protection by 3-hydroxy-3-methylglutaryl (HMG)-CoA reductase inhibitors mediated by endothelial nitric oxide synthase. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[189]  A. Israël,et al.  The Notch1 receptor is cleaved constitutively by a furin-like convertase. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[190]  H. Chabriat,et al.  SPECT study of a German CADASIL family , 1998, Neurology.

[191]  J. Dichgans,et al.  Acetazolamide-responsive migraine in CADASIL , 1998, Neurology.

[192]  M. Vérin,et al.  Cognitive Alterations in Non-Demented CADASIL Patients , 1998, Cerebrovascular Diseases.

[193]  Ruchoux,et al.  Endothelial changes in muscle and skin biopsies in patients with CADASIL , 1998, Neuropathology and applied neurobiology.

[194]  J. Weissenbach,et al.  Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients , 1997, The Lancet.

[195]  S. Nelson,et al.  Hereditary endotheliopathy with retinopathy, nephropathy, and stroke(HERNS) , 1997, Neurology.

[196]  J. Weissenbach,et al.  Notch3 Mutations in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), a Mendelian Condition Causing Stroke and Vascular Dementia , 1997, Annals of the New York Academy of Sciences.

[197]  C. Maurage,et al.  CADASIL: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy , 1997, Journal of neuropathology and experimental neurology.

[198]  G. Mahr,et al.  CADASIL presenting as bipolar disorder. , 1997, Psychosomatics.

[199]  H. Chabriat,et al.  [CADASIS. Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalophathy]. , 1997, Revue neurologique.

[200]  S. Greenberg,et al.  Diagnosis of cerebral amyloid angiopathy. Sensitivity and specificity of cortical biopsy. , 1997, Stroke.

[201]  M. Dichgans,et al.  CADASIL: skin biopsy allows diagnosis in early stages , 1997, Acta neurologica Scandinavica.

[202]  G. Guazzi,et al.  Unusual clinical features and early brain MRI lesions in a family with cerebral autosomal dominant arteriopathy , 1997, Neurology.

[203]  M. Dichgans,et al.  Angiographic complications in CADASIL , 1997, The Lancet.

[204]  P. Hedera,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: Study of two American families with predominant dementia , 1997, Journal of the Neurological Sciences.

[205]  I. Greenwald,et al.  Assessment of normal and mutant human presenilin function in Caenorhabditis elegans. , 1996, Proceedings of the National Academy of Sciences of the United States of America.

[206]  H. Chabriat,et al.  An additional monogenic disorder that masquerades as multiple sclerosis. , 1996, American journal of medical genetics.

[207]  Dennis E Bulman,et al.  Familial Hemiplegic Migraine and Episodic Ataxia Type-2 Are Caused by Mutations in the Ca2+ Channel Gene CACNL1A4 , 1996, Cell.

[208]  J. Weissenbach,et al.  Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia , 1996, Nature.

[209]  W. Brück,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): a morphological study of a German family , 1996, Acta Neuropathologica.

[210]  G. Hynd,et al.  The Staggered Spondaic Word TestPerformance of Children With Attention-Deficit Hyperactivity Disorder , 1996 .

[211]  I. Campbell,et al.  Solution Structure of a Pair of Calcium-Binding Epidermal Growth Factor-like Domains: Implications for the Marfan Syndrome and Other Genetic Disorders , 1996, Cell.

[212]  W. Weiner,et al.  Improvement of both tardive dystonia and akathisia after botulinum toxin injection. , 1996, Neurology.

[213]  A. Straube,et al.  Identification of a key recombinant narrows the CADASIL gene region to 8 cM and argues against allelism of CADASIL and familial hemiplegic migraine. , 1996, Genomics.

[214]  B. Dubois,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, genetic homogeneity, and mapping of the locus within a 2-cM interval. , 1996, American journal of human genetics.

[215]  M. Vérin,et al.  New phenotype of the cerebral autosomal dominant arteriopathy mapped to chromosome 19: migraine as the prominent clinical feature. , 1995, Journal of neurology, neurosurgery, and psychiatry.

[216]  M. Hutchinson,et al.  Familial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL) , 1995, Annals of neurology.

[217]  P. Davous,et al.  [Cadasil--a new model for subcortical dementia]. , 1995, Revue neurologique.

[218]  B. Dubois,et al.  Clinical spectrum of CADASIL: a study of 7 families , 1995, The Lancet.

[219]  Iva Greenwald,et al.  Facilitation of lin-12-mediated signalling by sel-12, a Caenorhabditis elegans S182 Alzheimer's disease gene , 1995, Nature.

[220]  S. Pappatà,et al.  Cytokines and acute-phase response in acute stroke. , 1995 .

[221]  F. Salvi,et al.  An italian kindred with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) , 1995, Annals of neurology.

[222]  M. Rossor,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)--confirmation by cerebral biopsy in 2 cases. , 1995, Clinical neuropathology.

[223]  D. Pollen,et al.  Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease , 1995, Nature.

[224]  D. Leys,et al.  Autosomal dominant migraine with MRI white‐matter abnormalities mapping to the CADASIL locus , 1995, Neurology.

[225]  J. Pruvo,et al.  Cadasil: une nouvelle affection familiale responsable d'infarctus cérébraux et de démence , 1995 .

[226]  F. Munschauer,et al.  Pontine ischemic rarefaction , 1995, Annals of neurology.

[227]  P. van Bogaert,et al.  A gene for hereditary paroxysmal cerebellar ataxia maps to chromosome 19p , 1995, Annals of neurology.

[228]  M. Gomori,et al.  Hereditary leukoencephalopathy and palmoplantar keratoderma , 1995, Neurology.

[229]  M. Manfredi,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL). Clinical, neuroimaging, pathological and genetic study of a large Italian family. , 1995, Brain : a journal of neurology.

[230]  D. Clair,et al.  Hereditary multi-infarct dementia unlinked to chromosome 19q12 in a large Scottish pedigree: evidence of probable locus heterogeneity. , 1995, Journal of medical genetics.

[231]  K. Hirayama,et al.  Familial young-adult-onset arteriosclerotic leukoencephalopathy with alopecia and lumbago without arterial hypertension. , 1995, European neurology.

[232]  Y. Olsson In memory of Patrick Sourander (1917-1993). , 2008, Brain pathology.

[233]  H. Chabriat,et al.  Presence of ultrastructural arterial lesions in muscle and skin vessels of patients with CADASIL. , 1994, Stroke.

[234]  L. Wilkins Guidelines for the molecular genetics predictive test in Huntington's disease , 1994, Neurology.

[235]  C. Marsden,et al.  The behavioural and motor consequences of focal lesions of the basal ganglia in man. , 1994, Brain : a journal of neurology.

[236]  Y. Olsson,et al.  Structural and vasoactive factors influencing intracerebral arterioles in cases of vascular dementia and other cerebrovascular disease: a review. Immunohistochemical studies on expression of collagens, basal lamina components and endothelin-1. , 1994, Dementia.

[237]  M. Bousser,et al.  Summary of the proceedings of the First International Workshop on CADASIL. Paris, May 19-21, 1993. , 1994, Stroke.

[238]  C. Fallet-Bianco,et al.  Autosomal dominant arteriopathic leuko‐encephalopathy and Alzheimer's disease * , 1994, Neuropathology and applied neurobiology.

[239]  A. J. Kumar,et al.  Basal ganglia volumes and white matter hyperintensities in patients with bipolar disorder. , 1994, The American journal of psychiatry.

[240]  U. Francke,et al.  Missense mutations impair intracellular processing of fibrillin and microfibril assembly in Marfan syndrome. , 1993, Human molecular genetics.

[241]  S. Finklestein,et al.  The clinical spectrum of cerebral amyloid angiopathy , 1993, Neurology.

[242]  J. Weissenbach,et al.  A gene for familial hemiplegic migraine maps to chromosome 19 , 1993, Nature Genetics.

[243]  J. Weissenbach,et al.  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy maps to chromosome 19q12 , 1993, Nature Genetics.

[244]  A. Joutel,et al.  Autosomal Dominant Leukoencephalopathy and Subcortical Ischemic Stroke: A Clinicopathological Study , 1993, Stroke.

[245]  J. Mas,et al.  A familial disorder with subcortical ischemic strokes, dementia, and leukoencephalopathy , 1992, Neurology.

[246]  M. Iba-Zizen,et al.  Autosomal Dominant Syndrome With Strokelike Episodes and Leukoencephalopathy , 1991, Stroke.

[247]  C. Fallet-Bianco,et al.  [Familial subcortical dementia with arteriopathic leukoencephalopathy. A clinico-pathological case]. , 1991, Revue neurologique.

[248]  S. Artavanis-Tsakonas,et al.  The notch gene product is a glycoprotein expressed on the cell surface of both epidermal and neuronal precursor cells during Drosophila development , 1989, The Journal of cell biology.

[249]  M. Tuszynski,et al.  Risk factors and clinical manifestations of pathologically verified lacunar infarctions. , 1989, Stroke.

[250]  J. Atkinson,et al.  Cerebroretinal vasculopathy. A new hereditary syndrome. , 1988, Ophthalmology.

[251]  S. Artavanis-Tsakonas,et al.  Nucleotide sequence from the neurogenic locus Notch implies a gene product that shares homology with proteins containing EGF-like repeats , 1985, Cell.

[252]  J. Selhorst,et al.  Microangiopathy of the brain and retina , 1979, Neurology.

[253]  B. Brownell,et al.  CHRONIC FAMILIAL VASCULAR ENCEPHALOPATHY , 1977, The Lancet.

[254]  W. Kirchner Age differences in short-term retention of rapidly changing information. , 1958, Journal of experimental psychology.