scholarly journals The Impact of Cerebral Amyloid Angiopathy in Various Neurodegenerative Dementia Syndromes: A Neuropathological Study

2019 ◽  
Vol 2019 ◽  
pp. 1-5 ◽  
Author(s):  
Jacques De Reuck
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Severe Form ◽  
Amyloid Angiopathy ◽  
Cerebrovascular Lesions ◽  
Mamillary Body ◽  
Microscopic Evaluation ◽  
Neurodegenerative Dementia ◽  
Cerebral Amyloid ◽  
Grade 3 ◽  
The Impact

Purpose. The Boston criteria for cerebral amyloid angiopathy (CAA) have to be confirmed by postmortem examination. The present study investigates the incidence and the cerebrovascular impact of the severity of CAA in various neurodegenerative dementia diseases.Material and Methods. 208 patients underwent an autopsy. They consisted of 92 brains with Alzheimer’s disease (AD), 46 with frontotemporal lobar degeneration (FTLD), 24 with progressive supranuclear palsy (PSP), 21 with Lewy body dementia (LBD), 5 with corticobasal degeneration (CBD), and 20 controls. In addition to the macroscopic examination, a whole coronal section of a cerebral hemisphere, at the level of the mamillary body, was taken for semiquantitative microscopic evaluation of the small cerebrovascular lesions.Results. CAA is present in 2/3% of the AD brains of which half of them have a severe form, grade 3. Only the latter displays more cerebrovascular lesions. CAA is present in 45% of the LBD brains. Cortical microinfarcts are only more frequent in the CAA grade 3 group. In LBD additional AD pathology is present in 41% of the CAA grade 0, 83% in grade 1-2, and 100% in grade 3. In PSP only 21% had CAA grade 1-2. In FTLD, CBD, and normal controls no CAA pathology is observed.Conclusions. The present study shows that CAA is most frequently associated to AD but that only the severe form displays more cerebrovascular lesions. LBD is the second most frequent disease associated to CAA with a clear correlation between the incidence of the associated AD features and the increasing severity of the CAA. In PSP only 21% display mild CAA features. PSP, tau-FTLD, and CBD are part of the Pick complex diseases, who are known to have a favourable vascular profile which can explain their low incidence of cerebrovascular lesions, in contrast to AD and LBD brains.

scholarly journals Successful electroconvulsive therapy for depression in a man with cerebral amyloid angiopathy

2021 ◽  
Vol 14 (2) ◽  
pp. e238922
Author(s):  
Geert Schurgers ◽  
Baer M G Arts ◽  
Alida A Postma ◽  
Anna de Kort
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Electroconvulsive Therapy ◽  
Severe Depression ◽  
Amyloid Angiopathy ◽  
Amyloid Beta Protein ◽  
Cerebral Blood Vessels ◽  
Safety Measures ◽  
Aβ Clearance ◽  
Cerebral Amyloid ◽  
The Impact

Cerebral amyloid angiopathy (CAA) is a condition characterised by accumulation of amyloid beta protein (Aβ) in the wall of cerebral blood vessels which increases the risk of intracranial haemorrhage and contributes to cognitive impairment. We describe the case of a man around the age of 70 with ‘probable’ CAA according to the modified Boston criteria and severe depression whose depression was treated successfully with electroconvulsive therapy (ECT). To the best of our knowledge, there are no earlier published reports of ECT in a patient with CAA. We briefly discuss possible safety measures for these patients, the impact of ECT on cognition in CAA and a possible influence of ECT on Aβ clearance.

scholarly journals Comparison of Post-mortem 7.0-Tesla Magnetic Resonance Imaging of the Brains of Alzheimer Patients with and without Cerebral Amyloid Angiopathy

2020 ◽  
Vol 4 (4) ◽  
Author(s):  
Jacques De Reuck ◽  
◽  
Florent Auger ◽  
Nicolas Durieux ◽  
Claude-Alain Maurage ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Post Mortem ◽  
Resonance Imaging ◽  
Cerebrovascular Lesions ◽  
Mri Sequences ◽  
Cerebral Amyloid ◽  
Magnetic Resonance Imaging Mri

Purpose: The influence of cerebral amyloid angiopathy (CAA) in Alzheimer’s disease (AD) remains unexplored. The present post-mortem study investigated possible differences in the degree of hippocampal atrophy (HA) between AD patients with and without CAA using 7.0-tesla magnetic resonance imaging (MRI). Also, the incidence of the hippocampal cortical micro-infarcts (HCoMIs) and hippocampal cortical micro-bleeds (HCoMBs) is compared to those in the neocortex. Methods: The examined post-mortem brains included 30 AD-CAA cases and 20 AD without CAA cases. The samples of the hippocampus were evaluated on the most representative coronal section with T2 and T2* MRI sequences. The average degree of HA was determined in both groups. The incidences of HCoMIs and HCoMBs, along with the frequency of CoMIs and CoMBs in the neocortex were compared in both groups: AD-with CAA and AD without CAA cases. Results: No significant differences were observed in the degree of HA and the incidence of hippocampal micro-infarcts (HMIs) and hippocampal micro-bleeds (HMBs) between the AD-CAA and the AD brains in contrast to the higher incidence of these cerebrovascular lesions in the neocortex of AD-CAA brains. The incidence of CoMIs and CoMBs in the neocortex showed similarity to that in the hippocampus of AD patients without CAA. Conclusions: CAA does not influence the degree of HA and the incidence of micro-infarcts (MIs) and micro-bleeds (MBs) in the hippocampus, in contrast to the high contribution of the latter with CAA in the neocortex. The hippocampus seems to be more spared from cerebrovascular involvement than the other parts of the brain.

scholarly journals PSEN1 Compound Heterozygous Mutations Associated with Cerebral Amyloid Angiopathy and Cognitive Decline Phenotype

2021 ◽  
Vol 22 (8) ◽  
pp. 3870
Author(s):  
Ilaria Palmieri ◽  
Marialuisa Valente ◽  
Lisa Maria Farina ◽  
Simone Gana ◽  
Brigida Minafra ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Cognitive Decline ◽  
Cerebral Amyloid Angiopathy ◽  
Presenilin 1 ◽  
Compound Heterozygous ◽  
Amyloid Angiopathy ◽  
First Case ◽  
Cerebral Amyloid ◽  
Compound Heterozygous Mutations ◽  
The Impact

Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by the deposition of amyloid beta-peptide (Aβ) aggregates. Aβ aggregates lead to vessel rupture and intracerebral hemorrhages, detected by magnetic resonance imaging (MRI). Presenile CAA is usually genetically determined by mutations in the amyloid precursor protein (APP) gene. However, mutations after codon 200 in the presenilin 1 (PSEN1) gene have been reported to facilitate CAA onset. Here, we analyzed the genetic bases in a patient of 55 years old affected by CAA and cognitive decline. DNA was isolated and genetic analysis was performed by Next-Generation Sequencing (NGS). RNA was extracted and retro-transcribed to perform segregation analysis by TOPO-TA cloning. WB analysis was carried out to check the impact of the mutations on protein. Two compound heterozygous mutations in PSEN1 exon 10, such as a novel stop-gain mutation (c.1070C > G) and a pathogenic splice variant (c.1129A > T), were found by NGS. Both mutations altered the presenilin 1 protein, truncating its C-terminal portion. This is the first case of CAA and cognitive decline caused by two compound mutations in PSEN1. With this report, we suggest extending the genetic analysis to PSEN1 when cerebral microbleeds are observed by MRI investigation in a patient affected by presenile cognitive decline.

scholarly journals Significance of Cortical Micro-Infarcts in the Human Brain

2021 ◽  
Vol 1 (1) ◽  
Author(s):  
Jacques L De Reuck
Keyword(s):  
Vascular Dementia ◽  
Cerebral Amyloid Angiopathy ◽  
Small Vessel Disease ◽  
Cerebral Small Vessel Disease ◽  
Small Vessel ◽  
Amyloid Angiopathy ◽  
White Matter Changes ◽  
Vessel Disease ◽  
Neurodegenerative Dementia ◽  
Cerebral Amyloid

Cortical micro-infarcts are due to cerebral small vessel disease. In contrast to the arteriosclerotic type of cerebral small vessel disease, cortical micro-infarcts are mainly related and due to cerebral amyloid angiopathy. Alzheimer’s disease is the most frequent neurodegenerative dementia disease associated to cerebral amyloid angiopathy and cortical micro-infarcts. Vascular dementia cannot only be due to lacunar infarcts and ischemic white matter changes, but can also be caused by cortical micro-infarcts. The latter are a frequent cause of vascular dementia and decrease globally the cerebral blood flow.

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