{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T00:09:52Z","timestamp":1790467792853,"version":"4.1.0"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2022,2,28]],"date-time":"2022-02-28T00:00:00Z","timestamp":1646006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2022,2,28]],"date-time":"2022-02-28T00:00:00Z","timestamp":1646006400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2022,7]]},"DOI":"10.1007\/s00415-022-11036-8","type":"journal-article","created":{"date-parts":[[2022,2,28]],"date-time":"2022-02-28T18:06:37Z","timestamp":1646071597000},"page":"3926-3930","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Familial British dementia: a clinical and multi-modal imaging case study"],"prefix":"10.1007","volume":"269","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5785-4830","authenticated-orcid":false,"given":"Matthew J.","family":"Harris","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher A.","family":"Lane","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Coath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian B.","family":"Malone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Cash","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josephine","family":"Barnes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederik","family":"Barkhof","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan M.","family":"Schott","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,2,28]]},"reference":[{"issue":"5","key":"11036_CR1","first-page":"838","volume":"22","author":"PA Freeborough","year":"1998","unstructured":"Freeborough PA, Fox NC (1998) Modeling brain deformations in Alzheimer disease by fluid registration of serial 3D MR images. JCAT 22(5):838\u2013843","journal-title":"JCAT"},{"issue":"5","key":"11036_CR2","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1109\/42.640753","volume":"16","author":"PA Freeborough","year":"1997","unstructured":"Freeborough PA, Fox NC (1997) The boundary shift integral: an accurate and robust measure of cerebral volume changes from registered repeat MRI. IEEE Trans Med Imaging 16(5):623\u2013629","journal-title":"IEEE Trans Med Imaging"},{"issue":"5","key":"11036_CR3","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1093\/brain\/123.5.975","volume":"123","author":"S Mead","year":"2000","unstructured":"Mead S, James-Galton M, Revesz T et al (2000) Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings. Brain 123(5):975\u2013991","journal-title":"Brain"},{"issue":"3","key":"11036_CR4","doi-asserted-by":"publisher","first-page":"721","DOI":"10.1093\/brain\/113.3.721","volume":"113","author":"GT Plant","year":"1990","unstructured":"Plant GT, R\u00e9v\u00e9sz T, Barnard RO et al (1990) Familial cerebral amyloid angiopathy with nonneuritic amyloid plaque formation. Brain 113(3):721\u2013747","journal-title":"Brain"},{"issue":"6738","key":"11036_CR5","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1038\/21637","volume":"399","author":"R Vidal","year":"1999","unstructured":"Vidal R, Frangione B, Rostagno A et al (1999) A stop-codon mutation in the BRI gene associated with familial British dementia. Nature 399(6738):776\u2013781","journal-title":"Nature"},{"key":"11036_CR6","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1111\/j.1749-6632.2000.tb06908.x","volume":"920","author":"J Ghiso","year":"2000","unstructured":"Ghiso J, Vidal R, Rostagno A et al (2000) Amyloidogenesis in familial British dementia is associated with a genetic defect on chromosome 13. Ann N Y Acad Sci 920:84\u201392","journal-title":"Ann N Y Acad Sci"},{"issue":"3","key":"11036_CR7","doi-asserted-by":"publisher","first-page":"481","DOI":"10.3233\/JAD-131364","volume":"40","author":"M Del Campo","year":"2014","unstructured":"Del Campo M, Teunissen CE (2014) Role of BRI2 in dementia. J Alzheimers Dis 40(3):481\u2013494","journal-title":"J Alzheimers Dis"},{"issue":"9","key":"11036_CR8","doi-asserted-by":"publisher","first-page":"885","DOI":"10.1093\/jnen\/62.9.885","volume":"62","author":"T Revesz","year":"2003","unstructured":"Revesz T, Ghiso J, Lashley T et al (2003) Cerebral amyloid angiopathies: a pathologic, biochemical, and genetic view. J Neuropathol Exp Neurol 62(9):885\u2013898","journal-title":"J Neuropathol Exp Neurol"},{"issue":"4","key":"11036_CR9","doi-asserted-by":"publisher","first-page":"E7","DOI":"10.3171\/2012.1.FOCUS11370","volume":"32","author":"P Mehndiratta","year":"2012","unstructured":"Mehndiratta P, Manjila S, Ostergard T et al (2012) Cerebral amyloid angiopathy-associated intracerebral hemorrhage: pathology and management. Neurosurg Focus 32(4):E7","journal-title":"Neurosurg Focus"},{"issue":"3","key":"11036_CR10","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1093\/brain\/63.3.237","volume":"63","author":"C Worster-Drought","year":"1940","unstructured":"Worster-Drought C, Greenfield JG, McMenemey WH (1940) A form of familial presenile dementia with spastic paralysis: including the pathological examination of a case. Brain 63(3):237\u2013254","journal-title":"Brain"},{"key":"11036_CR11","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1136\/jnnp.2006.096792","volume":"78","author":"H Jokinen","year":"2007","unstructured":"Jokinen H, Ryberg C, Kalska H et al (2007) Corpus callosum atrophy is associated with mental slowing and executive deficits in patients with age-related white matter hyperintensities: the LADIS Study. J Neurol Neurosurg Psychiatry 78:491\u2013496","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"6","key":"11036_CR12","doi-asserted-by":"publisher","first-page":"e66556","DOI":"10.1371\/journal.pone.0066556","volume":"8","author":"AM Tuladhar","year":"2013","unstructured":"Tuladhar AM, Snaphaan L, Shumskaya E et al (2013) Default mode network connectivity in stroke patients. PLoS ONE 8(6):e66556","journal-title":"PLoS ONE"},{"issue":"6","key":"11036_CR13","doi-asserted-by":"publisher","first-page":"1700","DOI":"10.1161\/STROKEAHA.111.632448","volume":"43","author":"J Zhang","year":"2012","unstructured":"Zhang J, Zhang Y, Xing S et al (2012) Secondary neurodegeneration in remote regions after focal cerebral infarction: a new target for stroke management? Stroke 43(6):1700\u20131705","journal-title":"Stroke"},{"issue":"7","key":"11036_CR14","doi-asserted-by":"publisher","first-page":"2432","DOI":"10.1002\/hbm.22782","volume":"36","author":"P Schaapsmeerders","year":"2015","unstructured":"Schaapsmeerders P, van Uden IW, Tuladhar AM et al (2015) Ipsilateral hippocampal atrophy is associated with long-term memory dysfunction after ischemic stroke in young adults. Hum Brain Mapp 36(7):2432\u20132442","journal-title":"Hum Brain Mapp"},{"issue":"8","key":"11036_CR15","doi-asserted-by":"publisher","first-page":"811","DOI":"10.1016\/S1474-4422(16)30030-8","volume":"15","author":"P Fotiadis","year":"2016","unstructured":"Fotiadis P, van Rooden S, van der Grond J et al (2016) Cortical atrophy in patients with cerebral amyloid angiopathy: a case-control study. Lancet Neurol 15(8):811\u2013819","journal-title":"Lancet Neurol"},{"issue":"7","key":"11036_CR16","doi-asserted-by":"publisher","first-page":"1736","DOI":"10.1016\/j.jstrokecerebrovasdis.2016.03.041","volume":"25","author":"T Doden","year":"2016","unstructured":"Doden T, Sato H, Sasahara E et al (2016) Clinico-radiological characteristics and pathological diagnosis of cerebral amyloid angiopathy-related intracerebral haemorrhage. J Stroke Cerebrovasc Dis 25(7):1736\u20131745","journal-title":"J Stroke Cerebrovasc Dis"},{"issue":"9","key":"11036_CR17","doi-asserted-by":"publisher","first-page":"2324","DOI":"10.1161\/STROKEAHA.112.657759","volume":"43","author":"A Charidimou","year":"2012","unstructured":"Charidimou A, Peeters A, Fox Z et al (2012) Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre magnetic resonance imaging cohort study and meta-analysis. Stroke 43(9):2324\u20132330","journal-title":"Stroke"},{"issue":"1","key":"11036_CR18","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1159\/000347114","volume":"3","author":"RW Paterson","year":"2013","unstructured":"Paterson RW, Uchino K, Emsley HC et al (2013) Recurrent stereotyped episodes in cerebral amyloid angiopathy: response to migraine prophylaxis in two patients. Cerebrovasc Dis Extra 3(1):81\u201384","journal-title":"Cerebrovasc Dis Extra"},{"key":"11036_CR19","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1159\/000088665","volume":"20","author":"JA Roch","year":"2005","unstructured":"Roch JA, Nighoghossian N, Hermier M et al (2005) Transient neurologic symptoms related to cerebral amyloid angiopathy: usefulness of T2*-weighted imaging. Cerebrovasc Dis 20:412\u2013414","journal-title":"Cerebrovasc Dis"},{"issue":"2","key":"11036_CR20","doi-asserted-by":"publisher","first-page":"423","DOI":"10.3233\/JAD-2010-1241","volume":"19","author":"VL Villemagne","year":"2010","unstructured":"Villemagne VL, Pike K, Pejoska S et al (2010) 11C-PiB PET ABri imaging in worster-drought syndrome (familial british dementia): a case report. J Alzheimers Dis 19(2):423\u2013428","journal-title":"J Alzheimers Dis"},{"issue":"2","key":"11036_CR21","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1016\/S0002-9440(10)63993-4","volume":"158","author":"JL Holton","year":"2001","unstructured":"Holton JL, Ghiso J, Lashley T et al (2001) Regional distribution of amyloid-Bri deposition and its association with neurofibrillary degeneration in familial British dementia. Am J Pathol 158(2):515\u2013526","journal-title":"Am J Pathol"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-022-11036-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-022-11036-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-022-11036-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T04:38:07Z","timestamp":1655872687000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-022-11036-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,28]]},"references-count":21,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2022,7]]}},"alternative-id":["11036"],"URL":"https:\/\/doi.org\/10.1007\/s00415-022-11036-8","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,28]]},"assertion":[{"value":"16 December 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 February 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 February 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 February 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"CL is now a full-time employee of Roche Products Lts, and a shareholder in F.Hoffmann-La Roche. There are no other relevant financial or non-financial interests to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"The participant has consented to the submission of the case report to the journal and for publication of the supplementary videos which identify the patient.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to publish"}},{"value":"Written informed consent was obtained from the participant to publish the case report and the supplementary videos which identify the participant.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Statements"}}]}}