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We aimed to characterize CNS manifestations and assess associations with clinical variables and plasma neurofilament light chain (NfL), using history of transient focal neurological episodes (TFNEs) as the main outcome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a cross-sectional study at Unidade Corino de Andrade in Porto (January 2021\u2013April 2023), including patients with ATTRv amyloidosis caused by the V30M mutation. History of TFNEs, stroke, seizures, headaches and symptoms\/signs of cranial nerve dysfunction were assessed through patient interview and neurological examination. Plasma NfL was analyzed cross-sectionally and longitudinally (0\u20136\u201312\u00a0months).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      151 patients were included (56.3% male, median age 48 [IQR 44\u201353], with a median disease duration of 15\u00a0years [IQR 11\u201320]. Most had early-onset disease (94.7%). TFNE history occurred in 29.1%, headaches in 23.8%, seizures in 6.6% and stroke in 1.3%. Patients with past TFNEs had longer disease duration than those without. No other robust differences were identified. Longitudinally, pNfL levels showed an overall effect of time (\n                      <jats:italic>p<\/jats:italic>\n                      =0.013), driven by a decrease between 6 and 12\u00a0months, with no significant differences between baseline and either follow-up timepoint. There were no differences in pNfL trajectories according to TFNEs history (time-by-TFNE interaction,\n                      <jats:italic>p<\/jats:italic>\n                      =0.416).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The high frequency of CNS symptoms in this sample supports including CNS assessment in routine follow-up of patients with ATTRv amyloidosis with longstanding disease. Further longitudinal studies are needed to clarify determinants of CNS involvement.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-026-14146-9","type":"journal-article","created":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T13:55:07Z","timestamp":1789912507000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Central nervous system manifestations in hereditary transthyretin amyloidosis: a cross-sectional study with 151 patients"],"prefix":"10.1007","volume":"273","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1106-7166","authenticated-orcid":false,"given":"Lu\u00edsa","family":"Sousa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Coelho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joana","family":"Fernandes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1rcio","family":"Cardoso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Alves","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Martins","family":"da Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Paula","family":"Sousa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel Mendon\u00e7a","family":"Pinto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael Mani\u00e9s","family":"Pereira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu\u00eds","family":"Ruano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Lemos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00eas","family":"Baldeiras","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Reis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo","family":"Taipa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,20]]},"reference":[{"key":"14146_CR1","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1136\/jnnp-2021-327909","volume":"93","author":"A Carroll","year":"2022","unstructured":"Carroll A, Dyck PJ, de Carvalho M et al (2022) Novel approaches to diagnosis and management of hereditary transthyretin amyloidosis. 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Sousa received honoraria to provide scientific lectures from Alnylam (2024, 2025) and Sobi (2022, 2023) and funding from Pfizer to cover scientific meeting expenses (2022); T. Coelho served on the scientific advisory board of Pfizer and received funding from Pfizer, Sobi and Alnylam for scientific meeting expenses; M. Cardoso received support from Pfizer, Sobi, Alnylam and AstraZeneca to participate in scientific meetings and to present scientific sessions; C. Alves received honoraria or financial support from Pfizer, Alnylam and Akcea to participate in scientific meetings and to present scientific sessions; L. Ruano received honoraria to provide scientific consulting and\/or lectures from Biogen, Merck, Novartis, Sanofi and Roche; and funding from Biogen, Sanofi and Roche to cover scientific meeting expenses; A.M. Silva currently works at Alexion AstraZeneca Rare Disease; R. Taipa received support from Alnylam to participate in scientific meetings and to present scientific sessions; M.M. Pinto, R.M. Pereira, C. Lemos, A.P. Sousa, I. Baldeiras and C. Reis report no disclosures.","order":1,"name":"Ethics","label":"Conflicts of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Written consent was obtained from all participants. The study was approved by the Ethics Committee of Centro Hospitalar Universit\u00e1rio de Santo Ant\u00f3nio, in accordance with the Declaration of Helsinki.","order":2,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"611"}}