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Published on: March 7, 2019
Spatial and temporal intracerebral hemorrhage patterns in Dutch-type hereditary cerebral amyloid angiopathy
Sabine Voigt1, Siham Amlal1, Emma A Koemans1
1Department of Neurology, Leiden University Medical Center, the Netherlands.
Insights
Intracerebral hemorrhages in Dutch-type Cerebral Amyloid Angiopathy (D-CAA) preferentially occur in the occipital lobe. Recurrent hemorrhages did not show accelerated timing or increased volume.
Area of Science:
- Neurology
- Genetics
- Pathology
Background:
- Cerebral Amyloid Angiopathy (CAA) is a progressive neurodegenerative disease characterized by the deposition of amyloid-beta protein in the walls of cerebral arteries.
- Dutch-type CAA (D-CAA) is a hereditary form associated with specific genetic mutations, leading to an increased risk of intracerebral hemorrhages (ICH).
- Understanding the topographical and temporal patterns of ICH in D-CAA is crucial for elucidating disease mechanisms and improving patient management.
Purpose of the Study:
- To investigate the topographical and temporal patterns of initial (index) and subsequent (recurrent) intracerebral hemorrhages (ICH) in patients with Dutch-type hereditary Cerebral Amyloid Angiopathy (D-CAA).
- To enhance the understanding of the development and progression of CAA-related ICH.
Main Methods:
- A cohort of patients with confirmed D-CAA or a relevant family history and at least one lobar ICH was included.
- Topographical analysis involved mapping ICH locations (frontal, parietal, temporal, occipital; infra/supratentorial) and calculating occurrence ratios relative to lobe volume.
- Temporal analysis examined the time intervals between recurrent ICH events using medical records.
Main Results:
- 72 patients with D-CAA experienced a total of 214 ICH events, all lobar and supratentorial.
- Index ICHs showed a significant preference for the occipital lobe (34%) compared to other lobes, with higher occurrence ratios relative to lobe volume.
- While 34% of patients with multiple ICH had their second event in the same lobe, there was no significant acceleration in time or increase in hematoma volume between subsequent ICHs.
Conclusions:
- Index and recurrent ICHs in D-CAA exhibit a topographical preference for the occipital lobe and are least common in the frontal lobe.
- Contrary to expectations, subsequent ICHs in D-CAA do not demonstrate accelerated timing or a gradual increase in hematoma volume.
Aim:
To investigate whether there is a topographical and temporal pattern of index and recurrent intracerebral hemorrhages (ICH) in Dutch-type hereditary Cerebral Amyloid Angiopathy (D-CAA) to increase our understanding on CAA-related ICH development.
Methods:
We included patients with DNA confirmed D-CAA or a history with ≥1 lobar ICH and ≥1 first-degree relative with D-CAA. Topographical pattern was studied by location (proportion frontal/parietal/temporal/occipital; infra/supratentorial and occurrence ratios relative to lobe volume) and volume of index and recurrent ICHs were determined on CT. Temporal pattern was examined by time between recurrent ICHs was retrieved from medical records.
Results:
We included 72 patients with D-CAA (mean age at index ICH 55 years) with in total 214 ICH. The median follow-up time was 7 years (range 0.8 to 28 years). All ICH were lobar and supratentorial. The index ICH was most frequently located in the occipital lobe (34% vs. 22% in the other three lobes; with index ICH occurrence ratios relative to lobe volume of 1.9 for occipital, 1.0 for temporal, 1.2 for parietal, and 0.5 for frontal, p = 0.001). In 16/47 (34%) patients with multiple ICH, the second ICH was located in the same lobe as the index ICH. The median time-interval between subsequent ICH was #1-2 ICH 27 months, #2-3 ICH 14 months, and #3-4 ICH 7 months (p = 0.6) There was no difference in volume between index and recurrent ICHs.
Conclusions:
We found that index and recurrent ICHs in D-CAA have a preference for the occipital lobe and are least frequent in the frontal lobe, which adds to the existing knowledge of histopathological studies on amyloid load in CAA. Surprisingly, there was no acceleration in time nor gradual increase of hematoma volume between subsequent ICHs.
