Idiopathic primary intraventricular hemorrhage and cerebral small vessel disease

2021 ◽  
pp. 174749302110439
Author(s):  
Alvin S Das ◽  
Robert W Regenhardt ◽  
Elif Gokcal ◽  
Mitchell J Horn ◽  
Nader Daoud ◽  
...  

Background Although primary intraventricular hemorrhage is frequently due to trauma or vascular lesions, the etiology of idiopathic primary intraventricular hemorrhage (IP-IVH) is not defined. Aims Herein, we test the hypothesis that cerebral small vessel diseases (cSVD) including hypertensive cSVD (HTN-cSVD) and cerebral amyloid angiopathy are associated with IP-IVH. Methods Brain magnetic resonance imaging from consecutive patients (January 2011 to September 2019) with non-traumatic intracerebral hemorrhage from a single referral center were reviewed for the presence of HTN-cSVD (defined by strictly deep or mixed-location intracerebral hemorrhage/cerebral microbleeds) and cerebral amyloid angiopathy (applying modified Boston criteria). Results Forty-six (4%) out of 1276 patients were identified as having IP-IVH. Among these, the mean age was 74.4 ± 12.2 years and 18 (39%) were females. Forty (87%) had hypertension, and the mean initial blood pressure was 169.2 ± 40.4/88.8 ± 22.2 mmHg. Of the 35 (76%) patients who received a brain magnetic resonance imaging, two (6%) fulfilled the modified Boston criteria for possible cerebral amyloid angiopathy and 10 (29%) for probable cerebral amyloid angiopathy. Probable cerebral amyloid angiopathy was found at a similar frequency when comparing IP-IVH patients to the remaining patients with primary intraparenchymal hemorrhage (P-IPH) (27%, p = 0.85). Furthermore, imaging evidence for HTN-cSVD was found in 8 (24%) patients with IP-IVH compared to 209 (28%, p = 0.52) patients with P-IPH. Conclusions Among IP-IVH patients, cerebral amyloid angiopathy was found in approximately one-third of patients, whereas HTN-cSVD was detected in 23%—both similar rates to P-IPH patients. Our results suggest that both cSVD subtypes may be associated with IP-IVH.

Stroke ◽  
2021 ◽  
Author(s):  
Emma A. Koemans ◽  
Sabine Voigt ◽  
Ingeborg Rasing ◽  
Thijs W. van Harten ◽  
Wilmar M.T. Jolink ◽  
...  

Background and Purpose: Although evidence accumulates that the cerebellum is involved in cerebral amyloid angiopathy (CAA), cerebellar superficial siderosis is not considered to be a disease marker. The objective of this study is to investigate cerebellar superficial siderosis frequency and its relation to hemorrhagic magnetic resonance imaging markers in patients with sporadic and Dutch-type hereditary CAA and patients with deep perforating arteriopathy–related intracerebral hemorrhage. Methods: We recruited patients from 3 prospective 3 Tesla magnetic resonance imaging studies and scored siderosis and hemorrhages. Cerebellar siderosis was identified as hypointense linear signal loss (black) on susceptibility-weighted or T2*-weighted magnetic resonance imaging which follows at least one folia of the cerebellar cortex (including the vermis). Results: We included 50 subjects with Dutch-type hereditary CAA, (mean age 50 years), 45 with sporadic CAA (mean age 72 years), and 43 patients with deep perforating arteriopathy–related intracerebral hemorrhage (mean age 54 years). Cerebellar superficial siderosis was present in 5 out of 50 (10% [95% CI, 2–18]) patients with Dutch-type hereditary CAA, 4/45 (9% [95% CI, 1–17]) patients with sporadic CAA, and 0 out of 43 (0% [95% CI, 0–8]) patients with deep perforating arteriopathy–related intracerebral hemorrhage. Patients with cerebellar superficial siderosis had more supratentorial lobar (median number 9 versus 2, relative risk, 2.9 [95% CI, 2.5–3.4]) and superficial cerebellar macrobleeds (median number 2 versus 0, relative risk, 20.3 [95% CI, 8.6–47.6]) compared with patients without the marker. The frequency of cortical superficial siderosis and superficial cerebellar microbleeds was comparable. Conclusions: We conclude that cerebellar superficial siderosis might be a novel marker for CAA.


NeuroSci ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 115-120
Author(s):  
Jacques De Reuck ◽  
Florent Auger ◽  
Nicolas Durieux ◽  
Claude-Alain Maurage ◽  
Vincent Deramecourt ◽  
...  

Introduction and Purpose: Cerebral amyloid angiopathy (CAA) can be observed in patients with progressive supranuclear palsy (PSP), though to a lesser degree than in Alzheimer’s disease. The present post-mortem 7.0-tesla magnetic resonance imaging (MRI) evaluates whether CAA has an influence on the degree of hippocampal atrophy (HA) and on the incidence of associated micro-infarcts (HMIs) and cortical micro-bleeds (HMBs). Material and Methods: Eight brains with PSP-CAA were compared to 20 PSP brains without CAA. In addition to the neuropathological examination, the hippocampus was evaluated on the most representative coronal section with T2 and T2*-weighted MRI sequences. The average degree of HA was determined in both groups. The incidence of HMIs and HMBs was also compared as well as the frequency of cortical micro-infarcts (CoMIs) and cortical micro-bleeds (CoMBs) in the hemispheric neocortex. Results: The neuropathological examination showed a higher incidence of lacunar infarcts in the PSP-CAA brains compared to the PSP ones. With magnetic resonance imaging (MRI), the severity of HA and the incidence of HMIs and HMBs was similar between both groups. Additionally, the frequency of CoMIs and CoMBs in the neocortex was comparable. Conclusions: The association of CAA in PSP brains has no influence on the degree of HA and on the incidence of the small cerebrovascular lesions in the hippocampus as well as in the neocortex.


Stroke ◽  
2018 ◽  
Vol 49 (8) ◽  
pp. 1899-1905 ◽  
Author(s):  
Hilde van den Brink ◽  
Angela Zwiers ◽  
Aaron R. Switzer ◽  
Anna Charlton ◽  
Cheryl R. McCreary ◽  
...  

Stroke ◽  
2016 ◽  
Vol 47 (12) ◽  
pp. 3041-3044 ◽  
Author(s):  
Sanneke van Rooden ◽  
Anna M. van Opstal ◽  
Gerda Labadie ◽  
Gisela M. Terwindt ◽  
Marieke J.H. Wermer ◽  
...  

2018 ◽  
Vol 6 ◽  
pp. 2050313X1877717
Author(s):  
Vanessa Saliou ◽  
Douraied Ben Salem ◽  
Julien Ognard ◽  
Dewi Guellec ◽  
Pascale Marcorelles ◽  
...  

Background: Cerebral amyloid angiopathy–related inflammation is a rare condition with approximately 100 reported cases. Its clinical manifestations are varied. We report here a novel presentation of this disease. Case presentation: A 61-year-old Caucasian man presented with rapidly progressive paralysis of the IX, X, XI and XII right cranial nerves associated with right central facial nerve palsy. Brain computed tomography angiography and cerebral catheter angiography found a focal fusiform enlargement of the distal cervical portion of the right internal carotid artery, related to a pseudo-aneurysm suggesting an evolution of a dissection and intra-cranial vessel dysplasia. Brain magnetic resonance imaging showed multiple asymmetrical subcortical regions of hyperintensity on T2 fluid-attenuated inversion recovery sequences. Punctiform cortical hyposignals on T2-weighted gradient echo magnetic resonance imaging sequences were mostly congruent with the white matter hyperintensities. There was a decreased cerebral perfusion at the frontal hyperintense fluid-attenuated inversion recovery region. Spectrometry identified a lactate–lipid peak. A brain biopsy showed intravascular amyloid deposits. Corticosteroid therapy was initiated, leading to a dramatic improvement of both clinical condition and magnetic resonance imaging brain lesions. Conclusion: This case report suggests that extra-cranial vasculitis and dysplasia can exceptionally be found in patients satisfying cerebral amyloid angiopathy–related inflammation criteria.


Stroke ◽  
2021 ◽  
Vol 52 (5) ◽  
pp. 1851-1855
Author(s):  
Ingeborg Rasing ◽  
Sabine Voigt ◽  
Emma A. Koemans ◽  
Erik van Zwet ◽  
Paul C. de Kruijff ◽  
...  

Background and Purpose: Cortical calcifications have been reported in patients with cerebral amyloid angiopathy (CAA), although their prevalence and pathophysiology are unknown. We investigated the frequency of calcifications on computed tomography, their association with intracerebral hemorrhage (ICH) and their coexistence with a striped pattern of the occipital cortex reflecting microcalcifications on ultra-high-field 7T-magnetic resonance imaging in Dutch-type hereditary CAA (D-CAA) and sporadic CAA. Methods: We included D-CAA mutation carriers with a proven APP (amyloid precursor protein) mutation or ≥1 lobar ICH and ≥1 first-degree relative with D-CAA and sporadic CAA patients with probable CAA according to the modified Boston criteria. D-CAA carriers were regarded symptomatic when they had a history of symptomatic ICH. We assessed the presence, location, and progression of calcifications and their association with ICH and the striped occipital cortex. Results: We found cortical calcifications in 15/81 (19% [95% CI, 11–29]) D-CAA mutation carriers (15/69 symptomatic and 0/12 presymptomatic) and in 1/59 (2% [95% CI, 0–9]) sporadic CAA patients. Calcifications were all bilateral located in the occipital lobes. In 3/15 (20%) of the symptomatic D-CAA patients the calcifications progressed over a period up to 10 years. There was evidence of an association between cortical calcifications and new ICH development (hazard ratio, 7.1 [95% CI, 0.9–54.9], log-rank P =0.03). In 7/25 D-CAA symptomatic carriers in whom a 7T-magnetic resonance imaging was performed, a striped pattern of the occipital cortex was present; in 3/3 (100%) of those with calcifications on computed tomography and 4/22 (18%) of those without calcifications. Conclusions: Occipital cortical calcifications are frequent in D-CAA but seem to be rare in sporadic CAA. Their absence in presymptomatic carriers and their association with ICH might suggest that they are a marker for advanced CAA. Cortical calcifications on computed tomography seem to be associated with the striped occipital cortex on 7T-magnetic resonance imaging which may possibly represent an early stage of calcification.


2019 ◽  
Vol 47 (3-4) ◽  
pp. 121-126 ◽  
Author(s):  
Dimitri Renard ◽  
Teodora Parvu ◽  
Eric Thouvenot

Background: Recently, finger-like projections (FLP) and subarachnoid haemorrhage extension (SAHE) of lobar intracerebral haemorrhage (LH) on acute CT together with ApoE4 genotype have been used in a prediction model for histopathologically proven cerebral amyloid angiopathy (CAA). Our aim was to analyse FLP and SAHE on acute/early subacute magnetic resonance imaging (MRI) and to assess the association with probable CAA diagnosis according to modified Boston criteria. Methods: We retrospectively studied MRI scans (and CT if available) performed <7 days in a cohort of consecutive acute LH patients >55 years. Results: Forty-six patients (24 men and 22 women; mean age 73; 28 probable and 18 possible CAA patients) were analysed. Mean symptom onset-MRI delay was 1.3 days (including 26 patients with MRI <24 h). Both probable and possible CAA groups were comparable regarding age, sex, time MRI and CT performance, MRI field strength, and LH volume. On MRI, both FLP and SAHE were observed more frequently in probable than in possible CAA (FLP 43 vs. 6%, p = 0.0073; SAHE 79 vs. 44%, p = 0.027), and associated with larger LH volumes (FLP, p = 0.011; SAHE, p = 0.047). FLP was associated with earlier performed MRI (mean 0.3 vs. 1.75 days, p = 0.025). In the subgroup of 35 patients with available CT (performed a mean of 2.2 days before or after MRI), FLP presence on CT was observed more frequently in probable than in possible CAA (57 vs. 7%, p = 0.0039). Concordance of MRI and CT for FLP presence/absence was 89%. Conclusions: In acute LH patients, FLP and SAHE on acute/early subacute phase MRI are associated with probable CAA diagnosis. Larger LH volumes are associated with FLP and SAHE on MRI, and early performed MRI with FLP.


2021 ◽  
pp. 174749302199196
Author(s):  
EA Koemans ◽  
S Voigt ◽  
I Rasing ◽  
WMT Jolink ◽  
TW van Harten ◽  
...  

Background and aim To investigate whether a striped occipital cortex and intragyral hemorrhage, two markers recently detected on ultra-high-field 7-tesla-magnetic resonance imaging in hereditary cerebral amyloid angiopathy (CAA), also occur in sporadic CAA (sCAA) or non-sCAA intracerebral hemorrhage (ICH). Methods We performed 7-tesla-magnetic resonance imaging in patients with probable sCAA and patients with non-sCAA-ICH. Striped occipital cortex (linear hypointense stripes perpendicular to the cortex) and intragyral hemorrhage (hemorrhage restricted to the juxtacortical white matter of one gyrus) were scored on T2*-weighted magnetic resonance imaging. We assessed the association between the markers, other CAA-magnetic resonance imaging markers and clinical features. Results We included 33 patients with sCAA (median age 70 years) and 29 patients with non-sCAA-ICH (median age 58 years). Striped occipital cortex was detected in one (3%) patient with severe sCAA. Five intragyral hemorrhages were found in four (12%) sCAA patients. The markers were absent in the non-sCAA-ICH group. Patients with intragyral hemorrhages had more lobar ICHs (median count 6.5 vs. 1.0), lobar microbleeds (median count >50 vs. 15), and lower median cognitive scores (Mini Mental State Exam: 20 vs. 28, Montreal Cognitive Assessment: 18 vs. 24) compared with patients with sCAA without intragyral hemorrhage. In 12 (36%) patients, sCAA diagnosis was changed to mixed-type small vessel disease due to deep bleeds previously unobserved on lower field-magnetic resonance imaging. Conclusion Whereas a striped occipital cortex is rare in sCAA, 12% of patients with sCAA have intragyral hemorrhages. Intragyral hemorrhages seem to be related to advanced disease and their absence in patients with non-sCAA-ICH could suggest specificity for CAA.


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