scholarly journals Spatial and temporal intracerebral hemorrhage patterns in Dutch-type hereditary cerebral amyloid angiopathy

2021 ◽  
pp. 174749302110570
Author(s):  
Sabine Voigt ◽  
Siham Amlal ◽  
Emma A Koemans ◽  
Ingeborg Rasing ◽  
Ellis S van Etten ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Temporal Pattern ◽  
Occipital Lobe ◽  
Time Interval ◽  
Amyloid Angiopathy ◽  
Degree Relative ◽  
Amyloid Load ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid ◽  
Histopathological Studies

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.

scholarly journals Hereditary cerebral amyloid angiopathy, Piedmont-type mutation

2020 ◽  
Vol 6 (2) ◽  
pp. e411 ◽  
Author(s):  
Mariel G. Kozberg ◽  
Susanne J. van Veluw ◽  
Matthew P. Frosch ◽  
Steven M. Greenberg
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Ex Vivo ◽  
Amyloid Plaques ◽  
Amyloid Angiopathy ◽  
Pathologic Examination ◽  
Potential Association ◽  
History Of ◽  
Intracerebral Hemorrhages ◽  
Β Amyloid ◽  
Cerebral Amyloid

ObjectiveWe present here a case report of a patient with a family history of intracerebral hemorrhages (ICHs) who presented with multiple large lobar hemorrhages in rapid succession, with cognitive sparing, who was found to have a mutation in the β-amyloid coding sequence of amyloid precursor protein (Leu705Val), termed the Piedmont-type mutation, the second ever reported case of this form of hereditary cerebral amyloid angiopathy (CAA).MethodsTargeted pathologic examination was performed aided by the use of ex vivo MRI.ResultsSevere CAA was observed mainly involving the leptomeningeal vessels and, to a far lesser extent, cortical vessels, with no amyloid plaques or neurofibrillary tangles.ConclusionsThis leptomeningeal pattern of β-amyloid deposition coupled with multiple large hemorrhages demonstrates unique pathophysiologic characteristics of CAA associated with the Piedmont-type mutation, suggesting a potential association between leptomeningeal CAA and larger ICHs.

scholarly journals Predictors of localization, outcome, and etiology of spontaneous intracerebral hemorrhages: focus on cerebral amyloid angiopathy

2020 ◽  
Vol 127 (6) ◽  
pp. 963-972 ◽  
Author(s):  
Bernadett Fakan ◽  
Zita Reisz ◽  
Denes Zadori ◽  
Laszlo Vecsei ◽  
Peter Klivenyi ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid

Cerebral amyloid angiopathy as a cause of multiple intracerebral hemorrhages

Neurology ◽  
1984 ◽  
Vol 34 (6) ◽  
pp. 730-730 ◽  
Author(s):  
C. Gilles ◽  
J. M. Brucher ◽  
P. Khoubesserian ◽  
J. J. Vanderhaeghen
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid

scholarly journals Original article The association between cerebral amyloid angiopathy and atherosclerosis in patients with intracerebral hemorrhages

2013 ◽  
Vol 3 ◽  
pp. 243-249
Author(s):  
Tadeusz Andrzej Mendel ◽  
Teresa Wierzba-Bobrowicz ◽  
Tomasz Stępień ◽  
Grażyna Maria Szpak
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid

Hereditary dementia with intracerebral hemorrhages and cerebral amyloid angiopathy

Neurology ◽  
2004 ◽  
Vol 63 (2) ◽  
pp. 234-240 ◽  
Author(s):  
A. M. Remes ◽  
S. Finnila ◽  
H. Mononen ◽  
H. Tuominen ◽  
R. Takalo ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid

scholarly journals Correction to: Predictors of localization, outcome, and etiology of spontaneous intracerebral hemorrhages: focus on cerebral amyloid angiopathy

2020 ◽  
Vol 127 (6) ◽  
pp. 973-975
Author(s):  
Bernadett Fakan ◽  
Zita Reisz ◽  
Denes Zadori ◽  
Laszlo Vecsei ◽  
Peter Klivenyi ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid

scholarly journals Current Management and Therapeutic Strategies for Cerebral Amyloid Angiopathy

2021 ◽  
Vol 22 (8) ◽  
pp. 3869
Author(s):  
Yasuteru Inoue ◽  
Yukio Ando ◽  
Yohei Misumi ◽  
Mitsuharu Ueda
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Β ◽  
Future Research ◽  
Amyloid Angiopathy ◽  
Research Directions ◽  
Isolation Techniques ◽  
Intracerebral Hemorrhages ◽  
Cortical Capillaries ◽  
Future Research Directions ◽  
Cerebral Amyloid

Cerebral amyloid angiopathy (CAA) is characterized by accumulation of amyloid β (Aβ) in walls of leptomeningeal vessels and cortical capillaries in the brain. The loss of integrity of these vessels caused by cerebrovascular Aβ deposits results in fragile vessels and lobar intracerebral hemorrhages. CAA also manifests with progressive cognitive impairment or transient focal neurological symptoms. Although development of therapeutics for CAA is urgently needed, the pathogenesis of CAA remains to be fully elucidated. In this review, we summarize the epidemiology, pathology, clinical and radiological features, and perspectives for future research directions in CAA therapeutics. Recent advances in mass spectrometric methodology combined with vascular isolation techniques have aided understanding of the cerebrovascular proteome. In this paper, we describe several potential key CAA-associated molecules that have been identified by proteomic analyses (apolipoprotein E, clusterin, SRPX1 (sushi repeat-containing protein X-linked 1), TIMP3 (tissue inhibitor of metalloproteinases 3), and HTRA1 (HtrA serine peptidase 1)), and their pivotal roles in Aβ cytotoxicity, Aβ fibril formation, and vessel wall remodeling. Understanding the interactions between cerebrovascular Aβ deposits and molecules that accumulate with Aβ may lead to discovery of effective CAA therapeutics and to the identification of biomarkers for early diagnosis.

Cerebral amyloid angiopathy and dementia in patients with intracerebral hemorrhages

2009 ◽  
Vol 283 (1-2) ◽  
pp. 260
Author(s):  
T. Mendel ◽  
G.M. Szpak ◽  
E. Bertrand ◽  
T. Wierzba-Bobrowicz ◽  
E. Lewandowska ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid
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