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Visualisation of amyloid deposition within the brain of long-term hereditary transthyretin amyloidosis survivors by 18F-flutemetamol positron emission tomography
Umeå University, Faculty of Medicine, Department of Pharmacology and Clinical Neuroscience.
Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Geriatric Medicine.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.ORCID iD: 0000-0002-1175-2369
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.
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2019 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 26, no S1, p. 287-287, article id EPR3027Article in journal, Meeting abstract (Other academic) Published
Abstract [en]

Background and aims: Hereditary transthyretin amyloid (ATTRv) amyloidosis caused by the transthyretin (TTR) Val30Met (p.V50M) mutation is characterised by peripheral neuropathy, and central nervous (CNS) complications has rarely been reported. However, liver transplantation has prolonged the patients’ survival, and CNS complications attributed to amyloid angiopathy caused by CNS synthesis of variant TTR have been reported. The aim of the study was to ascertain CNS amyloid deposition in long-term ATTRv survivors.

Methods: 20 ATTR Val30Met patients with symptoms from the CNS and a median disease duration of 16 years (9-25 years) together with five Alzheimer (AD) patients, who served as positive controls were included in the study. Amyloid CNS deposits were assessed by 18F- flutemetamol PET/CT examination utilising relative z scores with pons as reference.

Results: Expectedly, all Alzheimer patients had an clearly increased global composite z score above 2.0 compared with 55% of the ATTRv patients. There was an increased local uptake corresponding to cerebellum in 12 ATTRv patients compared to only one in the AD group (fig 1). Four of these ATTRv patients had a global composite z score within the normal range. No correlation between duration after 9 years and amyloid CNS deposition was noted.

Conclusion: Amyloid deposition within the brain after long-standing ATTRv amyloidosis is increased and is often noted in the cerebellum. However, not all patient display amyloid CNS deposition, thus, additional causes for CNS complications should always be considered.

Place, publisher, year, edition, pages
John Wiley & Sons, 2019. Vol. 26, no S1, p. 287-287, article id EPR3027
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Neurology
Identifiers
URN: urn:nbn:se:umu:diva-161913DOI: 10.1111/ene.14018ISI: 000474481001092OAI: oai:DiVA.org:umu-161913DiVA, id: diva2:1341827
Conference
5th Congress of the European Academy of Neurology (EAN), June 29 – July 2, 2019, Oslo, Norway
Available from: 2019-08-12 Created: 2019-08-12 Last updated: 2019-08-12Bibliographically approved

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Unéus, EricaWilhelmsson, ChristerSuhr, OleAnan, IntissarWixner, JonasPilebro, BjörnÅhlström Riklund, KatrineSundström, Torbjörn

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Unéus, EricaWilhelmsson, ChristerSuhr, OleAnan, IntissarWixner, JonasPilebro, BjörnÅhlström Riklund, KatrineSundström, Torbjörn
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Department of Pharmacology and Clinical NeuroscienceGeriatric MedicineDepartment of Public Health and Clinical MedicineCardiologyDiagnostic Radiology
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