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Cerebellar hemorrhages in patients with Dutch-type hereditary cerebral amyloid angiopathy
BACKGROUND: Recent studies suggest that superficially located cerebellar intracerebral hemorrhage (ICH) and microbleeds might point towards sporadic cerebral amyloid angiopathy (CAA). AIMS: We investigated the proportion of cerebellar ICH and asymptomatic macro- and microbleeds in Dutch-type heredit...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9260473/ https://www.ncbi.nlm.nih.gov/pubmed/34427476 http://dx.doi.org/10.1177/17474930211043663 |
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author | Voigt, S de Kruijff, PC Koemans, EA Rasing, I van Etten, ES Terwindt, GM van Osch, MJP van Buchem, MA van Walderveen, MAA Wermer, MJH |
author_facet | Voigt, S de Kruijff, PC Koemans, EA Rasing, I van Etten, ES Terwindt, GM van Osch, MJP van Buchem, MA van Walderveen, MAA Wermer, MJH |
author_sort | Voigt, S |
collection | PubMed |
description | BACKGROUND: Recent studies suggest that superficially located cerebellar intracerebral hemorrhage (ICH) and microbleeds might point towards sporadic cerebral amyloid angiopathy (CAA). AIMS: We investigated the proportion of cerebellar ICH and asymptomatic macro- and microbleeds in Dutch-type hereditary CAA (D-CAA), a severe and essentially pure form of CAA. METHODS: Symptomatic patients with D-CAA (defined as ≥1 symptomatic ICH) and presymptomatic D-CAA mutation-carriers were included. We assessed magnetic resonance imaging scans for symptomatic (cerebellar) ICH and asymptomatic cerebellar macro- and microbleeds according to the STRIVE-criteria. Location was assessed as superficial-cerebellar (cortex, vermis or juxta-cortical) or deep-cerebellar (white matter, pedunculi cerebelli and gray nuclei). RESULTS: We included 63 participants (mean age 58 years, 60% women, 42 symptomatic). In total, the 42 symptomatic patients with D-CAA had 107 symptomatic ICH (range 1–7). None of these ICH were located in the cerebellum. Six of 42 (14%, 95%CI 4–25%) symptomatic patients and none of the 21 (0%, 95%CI 0–0%) presymptomatic carriers had ≥ 1 asymptomatic cerebellar macrobleed(s). All macrobleeds were superficially located. Cerebellar microbleeds were found in 40 of 63 (64%, 95%CI 52–76) participants (median 1.0, range 0–159), 81% in symptomatic patients and 29% in presymptomatic carriers. All microbleeds were strictly or predominantly superficially (ratio superficial versus deep 15:1) located. CONCLUSIONS: Superficially located asymptomatic cerebellar macrobleeds and microbleeds are common in D-CAA. Cerebellar microbleeds are already present in the presymptomatic stage. Despite the high frequency of cerebellar micro and macrobleeds, CAA pathology did not result in symptomatic cerebellar ICH in patients with D-CAA. |
format | Online Article Text |
id | pubmed-9260473 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-92604732022-07-08 Cerebellar hemorrhages in patients with Dutch-type hereditary cerebral amyloid angiopathy Voigt, S de Kruijff, PC Koemans, EA Rasing, I van Etten, ES Terwindt, GM van Osch, MJP van Buchem, MA van Walderveen, MAA Wermer, MJH Int J Stroke Research BACKGROUND: Recent studies suggest that superficially located cerebellar intracerebral hemorrhage (ICH) and microbleeds might point towards sporadic cerebral amyloid angiopathy (CAA). AIMS: We investigated the proportion of cerebellar ICH and asymptomatic macro- and microbleeds in Dutch-type hereditary CAA (D-CAA), a severe and essentially pure form of CAA. METHODS: Symptomatic patients with D-CAA (defined as ≥1 symptomatic ICH) and presymptomatic D-CAA mutation-carriers were included. We assessed magnetic resonance imaging scans for symptomatic (cerebellar) ICH and asymptomatic cerebellar macro- and microbleeds according to the STRIVE-criteria. Location was assessed as superficial-cerebellar (cortex, vermis or juxta-cortical) or deep-cerebellar (white matter, pedunculi cerebelli and gray nuclei). RESULTS: We included 63 participants (mean age 58 years, 60% women, 42 symptomatic). In total, the 42 symptomatic patients with D-CAA had 107 symptomatic ICH (range 1–7). None of these ICH were located in the cerebellum. Six of 42 (14%, 95%CI 4–25%) symptomatic patients and none of the 21 (0%, 95%CI 0–0%) presymptomatic carriers had ≥ 1 asymptomatic cerebellar macrobleed(s). All macrobleeds were superficially located. Cerebellar microbleeds were found in 40 of 63 (64%, 95%CI 52–76) participants (median 1.0, range 0–159), 81% in symptomatic patients and 29% in presymptomatic carriers. All microbleeds were strictly or predominantly superficially (ratio superficial versus deep 15:1) located. CONCLUSIONS: Superficially located asymptomatic cerebellar macrobleeds and microbleeds are common in D-CAA. Cerebellar microbleeds are already present in the presymptomatic stage. Despite the high frequency of cerebellar micro and macrobleeds, CAA pathology did not result in symptomatic cerebellar ICH in patients with D-CAA. SAGE Publications 2021-09-10 2022-07 /pmc/articles/PMC9260473/ /pubmed/34427476 http://dx.doi.org/10.1177/17474930211043663 Text en © 2021 World Stroke Organization https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Research Voigt, S de Kruijff, PC Koemans, EA Rasing, I van Etten, ES Terwindt, GM van Osch, MJP van Buchem, MA van Walderveen, MAA Wermer, MJH Cerebellar hemorrhages in patients with Dutch-type hereditary cerebral amyloid angiopathy |
title | Cerebellar hemorrhages in patients with Dutch-type hereditary
cerebral amyloid angiopathy |
title_full | Cerebellar hemorrhages in patients with Dutch-type hereditary
cerebral amyloid angiopathy |
title_fullStr | Cerebellar hemorrhages in patients with Dutch-type hereditary
cerebral amyloid angiopathy |
title_full_unstemmed | Cerebellar hemorrhages in patients with Dutch-type hereditary
cerebral amyloid angiopathy |
title_short | Cerebellar hemorrhages in patients with Dutch-type hereditary
cerebral amyloid angiopathy |
title_sort | cerebellar hemorrhages in patients with dutch-type hereditary
cerebral amyloid angiopathy |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9260473/ https://www.ncbi.nlm.nih.gov/pubmed/34427476 http://dx.doi.org/10.1177/17474930211043663 |
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