{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T03:50:22Z","timestamp":1774929022037,"version":"3.50.1"},"reference-count":9,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,12,1]],"date-time":"2019-12-01T00:00:00Z","timestamp":1575158400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2019,12,30]],"date-time":"2019-12-30T00:00:00Z","timestamp":1577664000000},"content-version":"vor","delay-in-days":29,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Neurol"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Introduction<\/jats:title>\n<jats:p>Charles Bonnet syndrome is characterized by simple or complex visual hallucinations (VH) due to damage along the visual pathways. We report a functional MRI study of brain correlates of VH in the context of a severe optic atrophy in a patient with Leber\u2019s Hereditary Optic Neuropathy (LHON).<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case report<\/jats:title>\n<jats:p>A 62-year-old man was diagnosed with LHON (11778\/ND4 mtDNA mutation) after subacute visual loss in left eye (right eye was amblyopic). One month later, he experienced VH of a few seconds consisting in \u201cmoving red and blue miniature cartoons\u201d. One year later VH content changed in colored mosaic (10\u201315\u2009s duration), usually stress-related, and blue and white flashes (2\u20135\u2009s), triggered by unexpected auditory stimuli. Audiometry revealed mild sensorineural hearing loss. Three block design functional MRI paradigms were administrated: 1) random \u201cclap\u201d, 2) \u201ccheckerboard\u201d and 3) non-random \u201cbeep\u201d. After random \u201cclaps\u201d simple flashes were evoked with bilateral activation of primary and secondary visual cortex, cuneus, precuneus and insula. Neither hallucinations nor cortex activation were registered after \u201ccheckerboard\u201d stimulation, due to the severe visual impairment. Primary and secondary auditory cortices were \u201cbeep\u201d-activated, without eliciting VH by non-random \u201cbeep\u201d.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>The peculiarity of our case is that VH were triggered by random auditory stimuli, possibly due to a cross-modal plasticity between visual and auditory networks, likely\u00a0influenced by the sensorineural deafness. Functional alterations of both networks in resting conditions have been demonstrated in LHON patients, even without an auditory deficit. Finally, the absence of VH triggered by expected stimuli is consistent with the \u201cexpectation suppression theory\u201d, based on increased neural activations after unexpected but not by predicted events.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12883-019-1579-9","type":"journal-article","created":{"date-parts":[[2019,12,30]],"date-time":"2019-12-30T15:02:37Z","timestamp":1577718157000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Functional MRI study in a case of Charles Bonnet syndrome related to LHON"],"prefix":"10.1186","volume":"19","author":[{"given":"V.","family":"Vacchiano","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Tonon","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Mitolo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Evangelisti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Carbonelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Liguori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Lodi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"Carelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4639-8929","authenticated-orcid":false,"given":"C.","family":"La Morgia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2019,12,30]]},"reference":[{"issue":"9004","key":"1579_CR1","doi-asserted-by":"publisher","first-page":"794","DOI":"10.1016\/S0140-6736(96)90869-7","volume":"347","author":"RJ Teunisse","year":"1996","unstructured":"Teunisse RJ, Cruysberg JR, Hoefnagels WH, Verbeek AL, Zitman FG. Visual hallucinations in psychologically normal people: Charles Bonnet's syndrome. Lancet. 1996;347(9004):794\u20137.","journal-title":"Lancet."},{"issue":"8","key":"1579_CR2","doi-asserted-by":"publisher","first-page":"738","DOI":"10.1038\/3738","volume":"1","author":"DH Ffytche","year":"1998","unstructured":"Ffytche DH, Howard RJ, Brammer MJ, David A, Woodruff P, Williams S. The anatomy of conscious vision: an fMRI study of visual hallucinations. Nat Neurosci. 1998;1(8):738\u201342.","journal-title":"Nat Neurosci"},{"issue":"3","key":"1579_CR3","doi-asserted-by":"publisher","first-page":"777","DOI":"10.1007\/s00415-019-09205-3","volume":"266","author":"H Kolarova","year":"2019","unstructured":"Kolarova H, Catarino CB, Priglinger C, Klopstock T. Charles bonnet syndrome in Leber's hereditary optic neuropathy. 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He is also founded for research program by Stealth Pharmaceuticals, and his research is supported by grants from the Italian Ministry of Health, Telethon, the Emilia Romagna Region, the patient\u2019s organization MITOCON and by private donations.Dr. C La Morgia is involved in clinical trials with Santhera Pharmaceuticals (Raxone in Leber\u2019s Hereditary Optic Neuropathy) and GenSight Biologics (gene therapy with GS10 in Leber\u2019s Hereditary Optic Neuropathy) and received speaker honoraria for educational courses and travel reimbursements from Santhera Pharmaceuticals. Dr. La Morgia is funded by the Ministry of Health grant GR-2013-02358026 and is co-PI for the Ministry of Health grant GR-2016-02361449.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"350"}}