Neuropathological correlates of temporal pole white matter hyperintensities in CADASIL
Open accessAbstract
Background and Purpose— White matter (WM) hyperintensities on MRI or leukoaraiosis is characteristic of stroke syndromes. Increased MRI signals in the anterior temporal pole are suggested to be diagnostic for cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), with 90% sensitivity and 100% specificity. The structural correlates of these specific WM hyperintensities seen on T2-weighted and FLAIR sequences in the temporal pole of CADASIL are unclear. We assessed pathological changes in postmortem tissue from the temporal pole to reveal the cause of CADASIL-specific WM hyperintensities. Methods— A combination of tinctorial and immunostaining approaches and in vitro imaging methods were used to quantify the extent of perivascular space (PVS), arteriosclerosis determined as the sclerotic index, WM myelination as the myelin index, and damage within the WM as accumulated degraded myelin basic protein in samples of the anterior temporal pole from 9 CADASIL and 8 sporadic subcortical ischemic vascular dementia cases, and 5 similarly aged (young) and 5 older controls. Luxol fast blue-stained serial sections from a CADASIL case were also used to reconstruct the temporal pole, which was then compared to the MR images. Results— Luxol fast blue sections used to reconstruct the temporal pole revealed an abundance of enlarged PVS in the WM that topographically appeared as indistinct opaque regions. The mean and total areas of the PVS per WM area (%PVS) were significantly greater in CADASIL compared to the controls. The myelin index was severely reduced in CADASIL in relation to the subcortical ischemic vascular dementia and control sample that was consistent with increased immunoreactivity of degraded myelin basic protein, indicating myelin degeneration. Cerebral microvessels associated with the PVS exhibited a 4.5-fold greater number of basophilic (hyalinized) vessels and a 57% increase in the sclerotic index values in CADASIL subjects compared to young controls. A significant correlation between the quantity of hyalinized vessels and sclerotic index values was also apparent ( P <0.05). Conclusions— Our findings suggest that MRI hyperintensities in the temporal pole of CADASIL patients are explained by enlarged PVS and degeneration of myelin accompanied by lack of drainage of the interstitial fluid rather than lacunar infarcts. Consistent with the lack of MR hypersignals in the temporal pole of older subcortical ischemic vascular dementia subjects, our observations imply greater progression of pathological changes in CADASIL patients.
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Yamamoto et al. (2009) proposed that MRI temporal pole hyperintensities in CADASIL reflect enlarged perivascular spaces together with myelin depletion and axonal damage.
“They proposed that MRI temporal pole hyperintensities in CADASIL patients reflect enlarged PVS, together with myelin depletion and axonal damage.”
From Clinical and research applications of magnetic resonance imaging in the study of CADASIL (2019) · cites this paper as [74]
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“Neuropathologically, CADASIL-specific WM hyperintensities observed on T2-weighted and FLAIR sequences in the temporal pole are best related to the enlarged perivascular space and degeneration of myelin accompanied by lack of drainage of the interstitial fluid rather than lacunar infarcts.(34) These results suggest tha…”
BackgroundDilated perivascular spaces in small-vessel disease: a study in CADASIL
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“One study on patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) demonstrated that WMH in the temporal pole could be explained by significantly enlarged PVS in the same area.(29) The phenomenon that WMH may preferentially grow along PVS had been mentioned…”
BackgroundQuantitative Vascular Pathology and Phenotyping Familial and Sporadic Cerebral Small Vessel Diseases
Craggs, L., Hagel, C., Kuhlenbäeumer, G., Börjesson‐Hanson, A., Andersen, O., Viitanen, M. H., Kalimo, H., McLean, C., Slade, J. Y., Hall, R., et al. (2013) · Brain Pathology · doi:10.1111/bpa.12041 · Cited by 64 · see citing works
Self-citationMorphometry of the hippocampal microvasculature in post‐stroke and age‐related dementias
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“All images were also analysed using Image Pro analysis (Yamamoto et al., 2009; Craggs et al., 2013).”
MethodSelf-citationCADASIL: A NOTCH3-associated cerebral small vessel disease
Yuan, L., Chen, X., Jankovic, J. J., & Deng, H. (2024) · Journal of Advanced Research · doi:10.1016/j.jare.2024.01.001 · Cited by 70 · see citing works
Hippocampal perivascular spaces are related to aging and blood pressure but not to cognition
Yao, M., Zhu, Y., Soumaré, A., Dufouil, C., Mazoyer, B. M., Tzourio, C., & Chabriat, H. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.03.021 · Cited by 61 · see citing works
“In Cerebral Autosomal-Dominant Arteriopathy with Subcortial Infarcts and Leukoencephalopathy, an hereditary ischemic SVD, white-matter edema was found strongly related to the accumulation of dPVS in temporal lobes (Yamamoto et al., 2009) and possibly involved in the increase of brain volume with accumulation of WMH (Y…”
BackgroundCognat, E., Cléophax, S., Domenga, V., & Joutel, A. (2014) · Acta Neuropathologica Communications · doi:10.1186/2051-5960-2-49 · Cited by 57 · see citing works
“This approach has clear advantages over other widely used qualitative or semi-quantitative approaches [19,41].”
BackgroundBlood brain barrier leakage is not a consistent feature of white matter lesions in CADASIL
Rajani, R. M., Ratelade, J., Domenga, V., Hase, Y., Kalimo, H., Kalaria, R. N., & Joutel, A. (2019) · Acta Neuropathologica Communications · doi:10.1186/s40478-019-0844-x · Cited by 56 · see citing works
Self-citationSevere white matter astrocytopathy in CADASIL
Hase, Y., Chen, A., Bates, L. L., Craggs, L., Yamamoto, Y., Gemmell, E., Oakley, A. E., Korolchuk, V. I., & Kalaria, R. N. (2018) · Brain Pathology · doi:10.1111/bpa.12621 · Cited by 56 · see citing works
Self-citationYamamoto, Y., Liao, Y., Lee, Y., Ihara, M., & Choi, J. C. (2023) · Journal of Clinical Neurology · doi:10.3988/jcn.2023.19.1.12 · Cited by 58 · see citing works
Self-citationMicroangiopathy in the cerebellum of patients with mitochondrial DNA disease
Lax, N. Z., Pienaar, I. S., Reeve, A. K., Hepplewhite, P. D., Jaros, E., Taylor, R. W., Kalaria, R. N., & Turnbull, D. M. (2012) · Brain · doi:10.1093/brain/aws110 · Cited by 54 · see citing works
“All arterioles with a diameter 450 mm were quantified by measuring the internal and external diameter of the vessel wall which was immune-positive for collagen IV or alpha-smooth muscle actin (modified from Yamamoto et al., 2009).”
MethodSelf-citationHase, Y., Polvikoski, T. M., Firbank, M., Craggs, L., Hawthorne, E., Platten, C., Stevenson, W., Deramecourt, V., Ballard, C. G., Kenny, R. A., et al. (2019) · Brain Pathology · doi:10.1111/bpa.12769 · Cited by 51 · see citing works
“© 2019 International Society of Neuropathology 196 vessels, essentially as described previously (8, 56).”
BackgroundMethodSelf-citationCraggs, L., Fenwick, R., Oakley, A. E., Ihara, M., & Kalaria, R. N. (2014) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12188 · Cited by 48 · see citing works
Self-citationCraggs, L., Yamamoto, Y., Ihara, M., Fenwick, R., Burke, M., Oakley, A. E., Roeber, S., Duering, M., Kretzschmar, H. A., & Kalaria, R. N. (2013) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12073 · Cited by 48 · see citing works
Self-citationUemura, M. T., Ihara, M., Maki, T., Nakagomi, T., Kaji, S., Uemura, K., Matsuyama, T., Kalaria, R. N., Kinoshita, A., & Takahashi, R. (2017) · Brain Pathology · doi:10.1111/bpa.12523 · Cited by 45 · see citing works
Self-citationDifferent types of white matter hyperintensities in CADASIL: Insights from 7-Tesla MRI
Guio, F. D., Vignaud, A., Chabriat, H., & Jouvent, É. (2017) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1177/0271678x17690164 · Cited by 44 · see citing works
Heterogeneous blood‐brain barrier dysfunction in cerebral small vessel diseases
Ying, Y., Li, Y., Yao, T., Shao, X., Tang, W., Montagne, A., Chabriat, H., Wang, D. J. J., Wang, C., Yang, Q., et al. (2024) · Alzheimer s & Dementia · doi:10.1002/alz.13874 · Cited by 42 · see citing works
“Some pathological studies already showed the displacement of AQP4 31 and enlarged PVS in CADASIL, 32,33 which could be hypothesized as a source of the k w decrease.”
BackgroundLoos, C., Klarenbeek, P., van Oostenbrugge, R. J., & Staals, J. (2015) · PLoS ONE · doi:10.1371/journal.pone.0137323 · Cited by 42 · see citing works
Cognat, E., Baron‐Menguy, C., Domenga, V., Cléophax, S., Fouillade, C., Monet, M., Dewerchin, M. A., & Joutel, A. (2014) · Stroke · doi:10.1161/strokeaha.113.003339 · Cited by 42 · see citing works
“First, we did not assess whether eliminating wild-type NOTCH3 in the TgPAC-Notch3R169C mice exacerbates cerebrovascular dysfunction, which has been recognized as one of the earliest manifestations in mouse models of CADASIL.21 The TgPAC-Notch3R169C mice and the Notch3−/− mice exhibit profound vascular dysfunction, inc…”
BackgroundInfluentialEvaluation of Long-Term Cryostorage of Brain Tissue Sections for Quantitative Histochemistry
Estrada, L. I., Robinson, A. A., Amaral, A. C. S., Giannaris, E. L., Heyworth, N. C., Mortazavi, F., Ngwenya, L. B., Roberts, D. E., Cabral, H., Killiany, R., et al. (2017) · Journal of Histochemistry & Cytochemistry · doi:10.1369/0022155416686934 · Cited by 42 · see citing works
“The intensity measure used is analogous to methods used by Yamamoto et al.(16) and Ihara et al.”
MethodYu, X., Yin, X., Hong, H., Wang, S., Jiaerken, Y., Zhang, F., Pasternak, O., Zhang, R., Yang, L., Lou, M., et al. (2021) · Fluids and Barriers of the CNS · doi:10.1186/s12987-021-00264-1 · Cited by 42 · see citing works
“Abbreviations AD: Axial diffusivity; ADt: Tissue compartment AD; AFD: Apparent fiber density; ANTs: Advanced Normalization Tools; BBB: Blood–brain barrier; CADASIL: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CSD: Constrained spherical deconvolution; CSF: Cerebrospinal f…”
BackgroundResultInfluentialYao, M., Jouvent, E., During, M., Godin, O., Hervé, D., Guichard, J. P., Zhu, Y.-C., Gschwendtner, A., Opherk, C., Dichgans, M., et al. (2012) · Stroke · doi:10.1161/strokeaha.112.664854 · Cited by 42 · see citing works
“This highly specific aspect is presumably related to the accumulation of dilated Virchow-Robin spaces.(7) Postmortem data recently showed that dilated Virchow-Robin spaces and degeneration of myelin are the 2 main pathological lesions underlying WMH in the temporal lobes.”
BackgroundInfluentialClinical and research applications of magnetic resonance imaging in the study of CADASIL
Schoemaker, D., Quiroz, Y. T., Torrico-Teave, H., & Arboleda-Velasquez, J. F. (2019) · Neuroscience Letters · doi:10.1016/j.neulet.2019.01.014 · Cited by 36 · see citing works
McAleese, K. E., Graham, S., Dey, M., Walker, L., Erskine, D., Johnson, M. A., Johnston, E., Thomas, A. J., McKeith, I. G., DeCarli, C. S., et al. (2018) · Brain Pathology · doi:10.1111/bpa.12685 · Cited by 37 · see citing works
Gravesteijn, G., Munting, L. P., Overzier, M., Mulder, A. A., Hegeman, I. M., Derieppe, M. P. P., Koster, A. J., van Duinen, S. G., Meijer, O. C., Aartsma-Rus, A. M., et al. (2019) · Translational Stroke Research · doi:10.1007/s12975-019-00742-7 · Cited by 34 · see citing works
Humphreys, C. A., Smith, C., & Wardlaw, J. M. (2021) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12737 · Cited by 33 · see citing works
White matter myelin loss in the brains of aged dogs.
Chambers, J., Uchida, K., & Nakayama, H. (2012) · Experimental Gerontology · doi:10.1016/j.exger.2011.12.003 · Cited by 43 · see citing works
“The myelin density of the WM was assessed using the method described by Yamamoto et al. with some minor modifications (Ihara et al., 2010; Yamamoto et al., 2009).”
MethodJones, O., Cutsforth-Gregory, J., Chen, J., Bhatti, M. T., Huston, J., & Brinjikji, W. (2021) · American Journal of Neuroradiology · doi:10.3174/ajnr.a7326 · Cited by 33 · see citing works
Reduced binding of Pittsburgh Compound-B in areas of white matter hyperintensities
Goodheart, A. E., Tamburo, E. L., Minhas, D. S., Aizenstein, H. J., McDade, E., Snitz, B. E., Price, J. C., Mathis, C. A., López, O. L., Klunk, W. E., et al. (2015) · NeuroImage Clinical · doi:10.1016/j.nicl.2015.09.009 · Cited by 31 · see citing works
“, 2011), these results suggest that PiB may be binding to myelin and that decreased binding in areas of WMH adds to the previous evidence of myelin damage in WMH (Murray et al., 2012; Yamamoto et al., 2009).”
ResultTaoka, T., Ito, R., Nakamichi, R., Nakane, T., Kawai, H., & Naganawa, S. (2022) · Magnetic Resonance in Medical Sciences · doi:10.2463/mrms.rev.2022-0012 · Cited by 30 · see citing works
“99”
Baker, M. L., Wang, J. J., Liew, G., Hand, P. J., Silva, D. A. D., Lindley, R. I., Mitchell, P. L. R., Wong, M. C., Rochtchina, E., Wong, T. Y., et al. (2010) · Stroke · doi:10.1161/strokeaha.110.594317 · Cited by 30 · see citing works
Fang, X.-J., Yu, M., Wu, Y., Zhang, Z.-H., Wang, W.-W., Wang, Z.-X., & Yuan, Y. (2017) · Chinese Medical Journal · doi:10.4103/0366-6999.204935 · Cited by 29 · see citing works
Development of a Multicomponent Intervention to Prevent Alzheimer's Disease
Saitô, S., Yamamoto, Y., & Ihara, M. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00490 · Cited by 30 · see citing works
“Morphological changes in the PVS were also reported in patients with hereditary arteriopathy including Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) (85) and Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CARASIL) (86).”
BackgroundSelf-citationAnimal models of monogenic migraine
Chen, S., Tolner, E. A., & Eikermann‐Haerter, K. (2016) · Cephalalgia · doi:10.1177/0333102416645933 · Cited by 26 · see citing works
Human iPS cell-derived mural cells as an in vitro model of hereditary cerebral small vessel disease
Yamamoto, Y., Kojima, K., Taura, D., Sone, M., Washida, K., Egawa, N., Kondo, T., Minakawa, E. N., Tsukita, K., Enami, T., et al. (2020) · Molecular Brain · doi:10.1186/s13041-020-00573-w · Cited by 25 · see citing works
Self-citationThe pericyte: A critical cell in the pathogenesis of CADASIL
Ruchoux, M., Kalaria, R. N., & Román, G. C. (2021) · Cerebral Circulation - Cognition and Behavior · doi:10.1016/j.cccb.2021.100031 · Cited by 26 · see citing works
Self-citationMicroglia and macrophages in the neuro-glia-vascular unit: From identity to functions
Chen, S., Li, J., Meng, S., He, T., Shi, Z., Wang, C., Wang, Y., Cao, H., Huang, Y., Zhang, Y., et al. (2023) · Neurobiology of Disease · doi:10.1016/j.nbd.2023.106066 · Cited by 24 · see citing works
“Impairment of the clearance of abundant granular osmiophilic material (GOM) deposits is also related to BBB damage, vessel wall thickening, and perivascular space enlargement[86; 87].”
Retinopathy and Lobar Intracerebral Hemorrhage
Baker, M. L. (2010) · Archives of Neurology · doi:10.1001/archneurol.2010.249 · Cited by 24 · see citing works
McAleese, K. E., Firbank, M., Hunter, D., Sun, L., Hall, R., Neal, J., Mann, D. M. A., Esiri, M. M., Jellinger, K. A., O'Brien, J. T., et al. (2012) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2012.01310.x · Cited by 24 · see citing works
Boston, G., Jobson, D., Mizuno, T., Ihara, M., & Kalaria, R. N. (2024) · Cerebral Circulation - Cognition and Behavior · doi:10.1016/j.cccb.2024.100227 · Cited by 25 · see citing works
“While the volumes could potentially be different between even p.R75P and p.R544C carriers, this opens the field to further exploration of differences in white matter pathologies in relation to different types of mutations [7,52,66].”
BackgroundSelf-citationGenetic Animal Models of Cerebral Vasculopathies
Lee, J. H., Bacskai, B. J., & Ayata, C. (2011) · Progress in molecular biology and translational science · doi:10.1016/b978-0-12-394596-9.00002-0 · Cited by 21 · see citing works
Chen, T., Lee, S.-H., Zheng, W.-R., Hsu, C.-C., Cho, K., Kuo, L., Chou, C. C. ‐., Chiu, M., Tee, B. L., & Cheng, T. (2022) · Particle and Fibre Toxicology · doi:10.1186/s12989-022-00485-8 · Cited by 20 · see citing works
ER stress induced immunopathology involving complement in CADASIL: implications for therapeutics
Panahi, M., Hase, Y., Gallart‐Palau, X., Mitra, S., Watanabe, A., Low, R. W. C., Yamamoto, Y., Falla, D. S., Hainsworth, A. H., Ihara, M., et al. (2023) · Acta Neuropathologica Communications · doi:10.1186/s40478-023-01558-1 · Cited by 20 · see citing works
“The diagnosis of CADASIL was confirmed by the presence of NOTCH3 gene mutations [64].”
Self-citationNovel Characteristics of Race-Specific Genetic Functions in Korean CADASIL
Kim, Y., & Lee, S. (2019) · Medicina · doi:10.3390/medicina55090521 · Cited by 20 · see citing works
“Because the AT pole has a unique rotatory structure and is vascularized by anterior temporal artery, AT hyperintensities are enlarged perivascular spaces that exhibit myelin degeneration and subsequent axonal disruption, and are not lacunar infarctions [11,16].”
BackgroundGlial Vascular Degeneration in CADASIL
Brennan-Krohn, T., Salloway, S., Correia, S., Dong, M., & de la Monte, S. M. (2010) · Journal of Alzheimer s Disease · doi:10.3233/jad-2010-100036 · Cited by 20 · see citing works
“…lacunar infarcts and extensive subcortical white matter hyper-intense signals on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the subcortical white matter [7], including the anterior temporal lobe, which has been shown to be a useful sign for the diagnosis of CADASIL [8].”
Ishiyama, H., Kim, H., Saitô, S., Takeda, S., Takegami, M., Yamamoto, Y., Abe, S., Nakazawa, S., Tanaka, T., Washida, K., et al. (2024) · Annals of Neurology · doi:10.1002/ana.26916 · Cited by 18 · see citing works
“48 A previous study estimated that p.R75P indirectly affects cysteine at codon 76, which is associated with the fi xation of the double hairpin in the EGFr of NOTCH3, leading to a disul fi de-independent conformational change.”
BackgroundSelf-citationDe novo mutation in the NOTCH3 gene causing CADASIL
Stojanov, D. A., Grozdanović, D., Petrović, S., Benedeto-Stojanov, D., Stefanović, I. B., Stojanović, N., & Ilić, D. N. (2014) · Biomolecules and Biomedicine · doi:10.17305/bjbms.2014.2297 · Cited by 18 · see citing works
McAleese, K. E., Walker, L., Erskine, D., Johnson, M. A., Koss, D. J., Thomas, A. J., & Attems, J. (2020) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12664 · Cited by 18 · see citing works
Neuronal densities and vascular pathology in the hippocampal formation in CADASIL
Yamamoto, Y., Hase, Y., Ihara, M., Khundakar, A. A., Roeber, S., Duering, M., & Kalaria, R. N. (2020) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2020.09.016 · Cited by 17 · see citing works
Self-citationKim, H., Lim, Y., Lee, E., Oh, Y. J., & Kim, K. (2020) · PLoS ONE · doi:10.1371/journal.pone.0234797 · Cited by 17 · see citing works
“The different tendencies of temporal pole involvement between CADASIL with cysteine-sparing mutations and that with cysteine-involving mutations might be explained by the temporal pole’s unique convolutional structure and vascularization [26].”
BackgroundConfluent Thalamic Hyperintensities in CADASIL
Camus-Jacqmin, D. M., Hervé, D., Viswanathan, A., Guichard, J., Duering, M., Dichgans, M., & Chabriat, H. (2010) · Cerebrovascular Diseases · doi:10.1159/000319607 · Cited by 17 · see citing works
“The observation of a strip-like aspect in some cases of CTH in relation to an ipsilateral lesion in the internal capsule are in support of this latter hypothesis. Other underlying processes cannot be excluded. Particularly, an increased number of dilated perivascular spaces, a frequent pathological feature of CADASIL,…”
ResultHuang, Y., ZHANG, W., Lin, L., Feng, J., Zhao, X. X., Guo, W. H., & Wei, W. (2009) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2009.01049.x · Cited by 16 · see citing works
Waller, R., Narramore, R., Simpson, J. E., Heath, P. R., Verma, N., Tinsley, M., Barnes, J. R., Haris, H. T., Henderson, F. E., Matthews, F. E., et al. (2020) · Brain Pathology · doi:10.1111/bpa.12928 · Cited by 16 · see citing works
“The SI score allows the quantification of arteriolosclerosis as a measure of SVD severity (33,34).”
BackgroundSelf-citationRoseborough, A. D., Rasheed, B., Jung, Y., Nishimura, K., Pinsky, W., Langdon, K. D., Hammond, R. R., Pasternak, S., KHAN, A. R., & Whitehead, S. N. (2021) · Brain Pathology · doi:10.1111/bpa.13017 · Cited by 13 · see citing works
“Stenosis and Perivascular Spaces: Sclerotic index (SI) and PVS volume were calculated using a previously described method [35].”
MethodAhmed‐Jushuf, F., Jiwa, N. S., Arwani, A. S., Foot, P., Bridges, L., Kalaria, R. N., Esiri, M. M., & Hainsworth, A. H. (2016) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2016.03.002 · Cited by 14 · see citing works
“CADASIL cases were genetically confirmed for NOTCH3 mutations, as detailed previously (Yamamoto et al., 2009).”
Self-citationJia, X., Li, Y., Ying, Y., Jia, X., Tang, W., Bian, Y., Zhang, J., Wang, D. J. J., Cheng, X., & Yang, Q. (2023) · NeuroImage Clinical · doi:10.1016/j.nicl.2023.103485 · Cited by 13 · see citing works
“Early involvement of white matter hyperintensities in the anterior temporal poles has been identified as highly suggestive of CADASIL (O’Sulivan et al., 2001; Yamamoto et al., 2009).”
BackgroundDisability independent of cerebral white matter demyelination in progressive multiple sclerosis
Singh, V., Zheng, Y., Ontaneda, D., Mahajan, K. R., Holloman, J. P., Fox, R. J., Nakamura, K., & Trapp, B. D. (2024) · Acta Neuropathologica · doi:10.1007/s00401-024-02796-w · Cited by 13 · see citing works
“Additional 10 µm-thick paraffin hemispheric sections were stained with Hematoxylin and Eosin (H&E) and analyzed for periventricular small vessel disease (SVD) using standard protocols for perivascular space area and vessel sclerotic index [30, 47].”
MethodOkada, T., Washida, K., Irie, K., Saitô, S., Noguchi, M., Tomita, T., Koga, M., Toyoda, Ḱ., Okazaki, S., Koizumi, T., et al. (2020) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2020.00130 · Cited by 13 · see citing works
Self-citationCao, Y., Zhang, D., Han, F., Jiang, N., Yao, M., & Zhu, Y. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25168796 · Cited by 13 · see citing works
“These unique anatomical and physiological characteristics of the temporal pole could explain the differential impact observed in CADASIL phenotypes depending on the type of mutation [50].”
BackgroundGrey matter volume alterations in CADASIL: a voxel-based morphometry study
Rossi‐Espagnet, M. C., Romano, A., Carducci, F., Calabria, L. F., Fiorillo, M., Orzi, F., & Bozzao, A. (2012) · The Journal of Headache and Pain · doi:10.1007/s10194-012-0418-9 · Cited by 11 · see citing works
“[9] confirmed, in a post-mortem analysis,”
Insulin-Independent and Dependent Glucose Transporters in Brain Mural Cells in CADASIL
Panahi, M., Rodriguez, P. R., Fereshtehnejad, S.-M., Arafa, D., Bogdanovic, N., Winblad, B., Cedazo-Minguez, A., Rinne, J., Darreh-Shori, T., Hase, Y., et al. (2020) · Frontiers in Genetics · doi:10.3389/fgene.2020.01022 · Cited by 9 · see citing works
“The diagnosis of CADASIL was confirmed by the presence of NOTCH3 gene mutations or the presence of GOM in arteries within skin biopsies (Yamamoto et al., 2009).”
BackgroundSelf-citationDetection of early neuronal damage in CADASIL patients by q-space MR imaging
Yamada, K., Sakai, K., Akazawa, K., Sugimoto, N., Nakagawa, M., & Mizuno, T. (2012) · Neuroradiology · doi:10.1007/s00234-012-1105-x · Cited by 10 · see citing works
“There are a couple of conceivable causes, which include enlarged extracellular space due to loss of myelin/neurons and widening of perivascular space, which is known to be a unique microscopic feature of CADASIL [25].”
BackgroundKim, H., Lim, Y., Oh, Y. J., Lee, E., & Kim, K. (2018) · PLoS ONE · doi:10.1371/journal.pone.0208620 · Cited by 10 · see citing works
Dziewulska, D., Sulejczak, D., & Wężyk, M. (2017) · Folia Neuropathologica · doi:10.5114/fn.2017.72387 · Cited by 9 · see citing works
“space and degeneration of myelin accompanied by lack of drainage of the interstitial fluid [20].”
BackgroundDuan, Q., Lyu, J., Cheng, K., Wang, X., Meng, Z., Wu, X., Chen, W., Wang, G., Niu, Q., Li, X., et al. (2023) · Journal of Magnetic Resonance Imaging · doi:10.1002/jmri.28768 · Cited by 9 · see citing works
“31 The descriptions of perivascular spaces and their fl uid drainage functions are not completely clear and remain controversial.”
BackgroundNOTCH3 Mutation Causes Glymphatic Impairment and Promotes Brain Senescence in CADASIL
Li, C., Li, H., Men, X., Wang, Y., Kang, X., Hu, M., Su, X., Wang, S., Lu, D., Shen, S., et al. (2025) · CNS Neuroscience & Therapeutics · doi:10.1111/cns.70140 · Cited by 8 · see citing works
White matter lesions in myotonic dystrophy type 1 co-locate with dilated perivascular spaces
Renard, D., & Taïeb, G. (2014) · Clinical Neurology and Neurosurgery · doi:10.1016/j.clineuro.2014.08.016 · Cited by 8 · see citing works
Jouvent, É., Alili, N., Hervé, D., & Chabriat, H. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-201086 · Cited by 9 · see citing works
“Whitematterlesionsinthe temporallobesaremainlyrelatedtotheaccumulation ofdilatedperivascularspaceswithmajorstructural alterationsofastrocytesinCADASIL[12].”
Nehme, A., Gioia, L. C., Jacquin, G., Poppe, A. Y., Guillon, L. L., & Odier, C. (2020) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/cjn.2020.57 · Cited by 7 · see citing works
“Certain hereditary forms of CSVD show a predilection for specific brain regions, such as the anterior temporal lobe in CADASIL and the pons in pontine autosomal dominant microangiopathy and leukoencephalopathy.(4,5) With aging, posterior brain arteries have thinner walls and less elastin than their anterior counterpar…”
BackgroundNOTCH3 mutations in a cohort of Portuguese patients within CADASIL spectrum phenotype
Almeida, M. R., Elias, I., Fernandes, C., Machado, R., Galego, O., & Santo, G. (2021) · Neurogenetics · doi:10.1007/s10048-021-00679-w · Cited by 8 · see citing works
- Authors
- Yamamoto, Y., Ihara, M., Tham, C., Low, R. W. C., Slade, J. Y., Moss, T., Oakley, A. E., Polvikoski, T., & Kalaria, R. N.
- Affiliations
- From Institute for Ageing and Health, Newcastle General Hospital, Westgate Road, Newcastle-upon-Tyne, United Kingdom.
- Newcastle Hospitals - Campus for Ageing and Vitality (GB)
- Newcastle upon Tyne Hospitals NHS Foundation Trust (GB)
- Corresponding author
- Kalaria, Raj N.
- Venue
- Stroke
- Published
- 2009-06
- Volume
- 40
- Issue
- 6
- Pages
- 2004-2011
- ISSN
- 0039-2499 (ISSN-L)
- 1524-4628
- PMC
- PMC2724668
- OpenAlex
- W2150076690
- Cited by
- 155
- References
- 44
- Citations by year
- 2026: 4 · 2025: 14 · 2024: 10 · 2023: 7 · 2022: 5 · 2021: 14 · 2020: 13 · 2019: 7 · 2018: 6 · 2017: 13 · 2016: 7 · 2015: 7 · 2014: 14 · 2013: 7 · 2012: 7
- FWCI
- 5.53
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Stroke (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Newcastle University ePrints (Newcastle Univesity)
- Funders
- Medical Research Council
- National Institute of Neurological Disorders and Stroke (R21NS054047)
- Topics
- Health Sciences › Medicine › Neurology › Cerebrovascular and genetic disorders
- Life Sciences › Neuroscience › Neurology › Neurological Disease Mechanisms and Treatments
- Health Sciences › Medicine › Neurology › Intracerebral and Subarachnoid Hemorrhage Research
- Keywords
- CADASIL, white matter hyperintensities, temporal pole, enlarged perivascular spaces, myelin degeneration, myelin basic protein, arteriosclerosis, subcortical vascular dementia, cerebral microvasculature, interstitial fluid drainage, neuropathology, postmortem brain, Luxol fast blue
- MeSH terms
- Adult, Aged, Aging, Algorithms, Capillaries, Female, Humans, Image Processing, Computer-Assisted, Immunohistochemistry, Magnetic Resonance Imaging, Male, Middle Aged, Myelin Sheath, Risk Factors, Sclerosis, Temporal Lobe, Dementia, Vascular, Paraffin Embedding, CADASIL
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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