ArticleJournal of neurology2018
Age-dependent cognitive dysfunction in untreated hereditary transthyretin amyloidosis.
Article in Journal of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- Cognitive manifestations and brain integrity in hereditary transthyretin amyloidosis: a systematic review.Journal of neurology · 2025Pooled it
- Central Nervous System Manifestations Associated with Hereditary Transthyretin Amyloidosis: a Narrative Review.Arquivos de neuro-psiquiatria · 2026Review
- Early Detection of Central Nervous System Involvement in ATTRv Using Multimodal MRI and Cognitive Assessments.European journal of neurology · 2026Article
- Central nervous system involvement in cardiac amyloidosis: Redefining the heart-brain axis.European journal of clinical investigation · 2026Review
- Exploring cognitive functions and brain structure in Hereditary Transthyretin amyloidosis using brain MRI and neuropsychological assessment.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Tetrameric Transthyretin as a Protective Factor Against Alzheimer's Disease.Molecular neurobiology · 2025Review
- Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.Advances in therapy · 2024Review
- Tafamidis concentration required for transthyretin stabilisation in cerebrospinal fluid.Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis · 2023Article
- Novel approaches to diagnosis and management of hereditary transthyretin amyloidosis.Journal of neurology, neurosurgery, and psychiatry · 2022Review
- A circulating, disease-specific, mechanism-linked biomarker for ATTR polyneuropathy diagnosis and response to therapy prediction.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Does protein aggregation drive postmitotic tissue degeneration?Science translational medicine · 2021Review
- The frequency of central nervous system complications in the Cypriot cohort of ATTRV30M neuropathy transplanted patients.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Article
- Cerebrospinal fluid and vitreous body exposure to orally administered tafamidis in hereditary ATTRV30M (p.TTRV50M) amyloidosis patients.Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis · 2018Article
Corrections and comments
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Central nervous system (CNS) involvement in hereditary transthyretin (TTR) amyloidosis has been described in patients whose disease course was modified by liver transplant. However, cognitive dysfunction has yet to be investigated in those patients. Moreover, CNS involvement in untreated patients or asymptomatic mutation carriers remains to be studied. A series of 340 carriers of the TTRVal30Met mutation (180 symptomatic and 160 asymptomatic) underwent a neuropsychological assessment, which included the Dementia Rating Scale-2 (DRS-2), auditory verbal learning test, semantic fluency, phonemic fluency, and trail making test. Cognitive deficits were identified at the individual level, after adjusting the neuropsychological test scores for demographic characteristics (sex, age, and education), based on large national normative data. The presence of cognitive dysfunction was determined by deficit in DRS-2 and/or multiple cognitive domains. Participants were also screened for depression based on a self-report questionnaire. The frequency of cognitive dysfunction was higher (p = 0.003) in symptomatic (9%) than in asymptomatic (2%) carriers. Among older carriers (≥ 50 years), the frequency of cognitive dysfunction was higher (p < 0.001) in symptomatic (36%) than asymptomatic (4%) individuals. Among younger participants (< 50 years), the frequency of cognitive dysfunction was not different (p = 0.631) between symptomatic patients (2%) and asymptomatic (1%) carriers. This cross-sectional study shows that cognitive dysfunction is part of the broad spectrum of clinical manifestations in older hereditary TTR amyloidosis patients with peripheral polyneuropathy, even in the early stages of the disease.
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