{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T00:48:44Z","timestamp":1786495724733,"version":"build-2736575974"},"reference-count":9,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2025,9,10]],"date-time":"2025-09-10T00:00:00Z","timestamp":1757462400000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Y69H (p.Y89H) variant hereditary transthyretin (ATTRv) amyloidosis causes meningeal amyloidosis, with mutant TTR deposits localized to the leptomeninges and vitreous body.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The effect of tafamidis meglumine on neurological disorders, such as the frequency of transient focal neurological episodes (TFNEs), magnetic resonance imaging (MRI) findings, and TTR levels in cerebrospinal fluid, was investigated in two patients diagnosed with Y69H ATTRv mutation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The initial symptoms in both patients were TFNEs, such as aphasia, sensory disturbance, motor paralysis, ataxia, and drop attacks. Neither epileptic drugs nor antiplatelet therapy decreased the frequency of attacks. The patients exhibited diffuse leptomeningeal enhancement on brain and spinal MRI. Tafamidis meglumine was initiated at a dose of 20\u00a0mg\/day and was found to be partially effective. The TFNEs nearly resolved, and meningeal enhancement on brain MRI improved; however, the neurological deficits progressed over the following 2 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Tafamidis had a partial effect on TFNEs and meningeal contrast enhancement on MRI; however, cerebellar ataxia and cognitive decline continued to progress.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/brb3.70856","type":"journal-article","created":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T05:35:47Z","timestamp":1757568947000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Effects of Tafamidis Meglumine on Transient Focal Neurological Episodes and Meningeal Contrast Enhancement in Hereditary Transthyretin\u2010Related Meningeal Amyloidosis: Report of Two Patients Carrying the c.265T&gt;C (p.Y89H) Variant"],"prefix":"10.1002","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-6096-1889","authenticated-orcid":false,"given":"Natsumi","family":"Saito","sequence":"first","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuko","family":"Kuroha","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayaka","family":"Ishiyama","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiro","family":"Wakasugi","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayoshi","family":"Tokutake","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arika","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsuya","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Nishiniigata Chuo Hospital Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,9,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awaf028"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(12)61333\u20106"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000001054"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2018.1479249"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000178983.20975.af"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/NRL.0000000000000337"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.5692\/clinicalneurol.cn\u2010001852"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2023.2167595"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478\u2010015\u20100216\u20100"}],"container-title":["Brain 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