• 1
    Chabriat H, Joutel A, Dichgans M, Tournier-Lasserve E, Bousser M-G. CADASIL. Lancet Neurol 2009; 8: 643653
  • 2
    Buffon F, Porcher R, Hernandez K, Kurtz A, Pointeau S, Vahedi K, Bousser MG, Chabriat H. Cognitive profile in CADASIL. J Neurol Neurosurg Psychiatry 2006; 77: 175180
  • 3
    Viswanathan A, Gschwendtner A, Guichard JP, Buffon F, Cumurciuc R, O'Sullivan M, Holtmannspötter M, Pachai C, Bousser MG, Dichgans M, Chabriat H. Lacunar lesions are independently associated with disability and cognitive impairment in CADASIL. Neurology 2007; 69: 172179
  • 4
    O'Sullivan M, Jarosz JM, Martin RJ, Deasy N, Powell JF, Markus HS. MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL. Neurology 2001; 56: 628634
  • 5
    O'Sullivan M, Singhal S, Charlton R, Markus HS. Diffusion tensor imaging of thalamus correlates with cognition in CADASIL without dementia. Neurology 2004; 62: 702707
  • 6
    Chabriat H, Pappata S, Poupon C, Clark CA, Vahedi K, Poupon F, Mangin JF, Pachot-Clouard M, Jobert A, Le Bihan D, Bousser M. Clinical severity in CADASIL related to ultrastructural damage in white matter: in vivo study with diffusion tensor MRI. Stroke 1999; 30: 26372643
  • 7
    Holtmannspotter M, Peters N, Opherk C, Martin D, Herzog J, Bruckmann H, Samann P, Gschwendtner A, Dichgans M. Diffusion magnetic resonance histograms as a surrogate marker and predictor of disease progression in CADASIL: a two-year follow-up study. Stroke 2005; 36: 25592565
  • 8
    Yamamoto Y, Ihara M, Tham C, Low WC, Slade JY, Moss T, Oakley AE, Polvikoski T, Kalaria RN. Neuropathological correlates of temporal pole white matter hyperintensities in CADASIL. Stroke 2009; 40: 20042011
  • 9
    Allan LM, Rowan EN, Firbank MJ, Thomas AJ, Parry SW, Polvikoski TM, O'Brien JT, Kalaria RN. Long term incidence of dementia, predictors of mortality and pathological diagnosis in older stroke survivors. Brain 2011; 134: 37163727
  • 10
    Craggs LJ, Hagel C, Kuhlenbaeumer G, Borjesson-Hanson A, Andersen O, Viitanen M, Kalimo H, McLean CA, Slade JY, Hall RA, Oakley AE, Yamamoto Y, Deramecourt V, Kalaria RN. Quantitative vascular pathology and phenotyping familial and sporadic cerebral small vessel diseases. Brain Pathol 2013. doi:10.1111/bpa.12041
  • 11
    Perry RH, Oakley AE. Newcastle Brain Map. London: Wolfe, 1993
  • 12
    Kalaria RN, Kenny RA, Ballard CG, Perry R, Ince P, Polvikoski T. Towards defining the neuropathological substrates of vascular dementia. J Neurol Sci 2004; 226: 7580
  • 13
    Deramecourt V, Slade JY, Oakley AE, Perry RH, Ince PG, Maurage CA, Kalaria RN. Staging and natural history of cerebrovascular pathology in dementia. Neurology 2012; 78: 10431050
  • 14
    Hof PR, Nimchinsky EA, Morrison JH. Neurochemical phenotype of corticocortical connections in the macaque monkey: quantitative analysis of a subset of neurofilament protein-immunoreactive projection neurons in frontal, parietal, temporal, and cingulate cortices. J Comp Neurol 1995; 362: 109133
  • 15
    Lindner M, Fokuhl J, Linsmeier F, Trebst C, Stangel M. Chronic toxic demyelination in the central nervous system leads to axonal damage despite remyelination. Neurosci Lett 2009; 453: 120125
  • 16
    Voelker CC, Garin N, Taylor JS, Gahwiler BH, Hornung JP, Molnar Z. Selective neurofilament (SMI-32, FNP-7 and N200) expression in subpopulations of layer V pyramidal neurons in vivo and in vitro. Cereb Cortex 2004; 14: 12761286
  • 17
    Yamamoto Y. Density of Microvascular Components in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. Newcastle upon Tyne: Medical Sciences, Newcastle University, 2006
  • 18
    Lammie GA, Brannan F, Slattery J, Warlow C. Nonhypertensive cerebral small-vessel disease: an autopsy study. Stroke 1997; 28: 22222229
  • 19
    Coulthard A, Blank SC, Bushby K, Kalaria RN, Burn DJ. Distribution of cranial MRI abnormalities in patients with symptomatic and subclinical CADASIL. Br J Radiol 2000; 73: 256265
  • 20
    Manganelli F, Ragno M, Cacchiò G, Iodice V, Trojano L, Silvaggio F, Scarcella M, Grazioli M, Santoro L, Perretti A. Motor cortex cholinergic dysfunction in CADASIL: a transcranial magnetic demonstration. Clin Neurophysiol 2008; 119: 351355
  • 21
    Miao Q, Paloneva T, Tuisku S, Roine S, Poyhonen M, Viitanen M, Kalimo H. Arterioles of the lenticular nucleus in CADASIL. Stroke 2006; 37: 22422247
  • 22
    Miao Q, Paloneva T, Tuominen S, Poyhonen M, Tuisku S, Viitanen M, Kalimo H. 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. Brain Pathol 2004; 14: 358364
  • 23
    Peters N, Opherk C, Danek A, Ballard C, Herzog J, Dichgans M. The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia. Am J Psychiatry 2005; 162: 20782085
  • 24
    O'Sullivan M, Barrick TR, Morris RG, Clark CA, Markus HS. Damage within a network of white matter regions underlies executive dysfunction in CADASIL. Neurology 2005; 65: 15841590
  • 25
    Fernando MS, Simpson JE, Matthews F, Brayne C, Lewis CE, Barber R, Kalaria RN, Forster G, Esteves F, Wharton SB, Shaw PJ, O'Brien J, Ince PG. White matter lesions in an unselected cohort of the elderly: molecular pathology suggests origin from chronic hypoperfusion injury. Stroke 2006; 37: 13911398
  • 26
    Ihara M, Polvikoski TM, Hall R, Slade JY, Perry RH, Oakley AE, Englund E, O'Brien JT, Ince PG, Kalaria RN. Quantification of myelin loss in frontal lobe white matter in vascular dementia, Alzheimer's disease, and dementia with Lewy bodies. Acta Neuropathol 2010; 119: 579589
  • 27
    Catani M, Ffytche DH. The rises and falls of disconnection syndromes. Brain 2005; 128: 22242239
  • 28
    Tatsch K, Koch W, Linke R, Poepperl G, Peters N, Holtmannspoetter M, Dichgans M. Cortical hypometabolism and crossed cerebellar diaschisis suggest subcortically induced disconnection in CADASIL: an 18F-FDG PET study. J Nucl Med 2003; 44: 862869
  • 29
    O'Sullivan M, Ngo E, Viswanathan A, Jouvent E, Gschwendtner A, Saemann PG, Duering M, Pachai C, Bousser M-G, Chabriat H, Dichgans M. Hippocampal volume is an independent predictor of cognitive performance in CADASIL. Neurobiol Aging 2009; 30: 890897
  • 30
    Gemmell E, Bosomworth H, Allan L, Hall R, Khundakar A, Oakley AE, Deramecourt V, Polvikoski TM, O'Brien JT, Kalaria RN. Hippocampal neuronal atrophy and cognitive function in delayed poststroke and aging-related dementias. Stroke 2012; 43: 808814