Cerebellar Superficial Siderosis in Cerebral Amyloid Angiopathy (CSS)

Cerebellar Superficial Siderosis in Cerebral Amyloid Angiopathy on 1.5T T2*-Weighted Imaging

Cerebellar superficial siderosis (SS) has been recently reported to be present in about 10% of both hereditary (n=50) and sporadic (n=46) cerebral amyloid angiopathy (CAA) patients on 3T MRI using susceptibility-weighted imaging (SWI) in the majority of patients. In that study, cerebellar SS was associated with a higher number of supratentorial lobar and superficial cerebellar macrobleeds (although cerebellar SS was not directly located adjacent to these cerebellar macrobleeds). It is unclear if cerebellar SS is caused by in situ leakage of cerebellar leptomeningeal vessels or rather represents hemorrhagic diffusion from cerebellar parenchymal micro/macrobleeds or from supratentorial bleeding sources via the tentorium cerebelli (TC).

Study Overview

• Status: Completed
• Conditions: Cerebral Amyloid Angiopathy; Siderosis
• Intervention / Treatment: Other: None, pure observational study

• Study Type: Observational
• Enrollment (Actual): 111

Contacts and Locations

Study Locations

• France
  • Nîmes, France, 30029
    • CHU de Nimes

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Sporadic probable CAA patients (according to the modified Boston criteria) presenting with acute symptoms related to acute ICH, acute subarachnoid haemorrhage, or cortical SS, between September 2009 and January 2022 in Nîmes University Hospital, France

Description

Inclusion Criteria:

• CAA patients (according to the modified Boston criteria)
• with acute symptoms related to acute ICH, acute subarachnoid haemorrhage, or cortical SS

Exclusion Criteria:

• Patients with recent trauma,
• Patients with anticoagulation treatment,
• Patients with pathological blood coagulation tests (activated partial thromboplastin time [aPTT] ratio=patient's aPTT/normal control aPTT] >1.2; or partial thromboplastin time [PTT] <75%) or platelet count (<100 x 109/L)
• Patients with inflammatory CAA

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
cerebral amyloid angiopathy (CAA); Clinical and 1.5T MRI T2*-weighted imaging characteristics analyses from sporadic probable CAA patients (according to the modified Boston criteria) presenting with acute symptoms related to acute ICH, acute subarachnoid haemorrhage, or cortical SS,	Other: None, pure observational study; None, pure observational study

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
MRI analyse : presence of cerebellar SS	presence of cerebellar SS (Yes/No)	baseline
MRI analyse : Location of cerebellar SS	Location of cerebellar SS (vermis, anterior lobe, posterior lobe)	baseline
MRI analyse : characteristics of cerebellar SS	number of cerebellar sulci involved	baseline
MRI analyse : characteristics of cerebellar SS	spatial relationship with cerebellar micro- and macrobleeds	baseline
MRI analyse : Presence of cerebellar macrobleeds	Presence of cerebellar macrobleeds (Yes/No)	baseline
MRI analyse : Presence of cerebral macrobleeds	Presence of cerebral macrobleeds (Yes/No)	baseline
MRI analyse : Characteristics of cerebral macrobleeds	spatial relationship of cerebral macrobleeds with the TC [adjacent vs. non-adjacent to TC]	baseline
MRI analyse : Characteristics of cortical SS	spatial relationship with TC (adjacent vs. non-adjacent to TC)	baseline
MRI analyse : hemosiderin	presence of hemosiderin deposition along the TC.	baseline

Collaborators and Investigators

• Sponsor: Centre Hospitalier Universitaire de Nīmes

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2022
• Primary Completion (Actual): August 31, 2022
• Study Completion (Actual): August 31, 2022

Study Registration Dates

• First Submitted: May 24, 2022
• First Submitted That Met QC Criteria: May 24, 2022
• First Posted (Actual): May 27, 2022

Study Record Updates

• Last Update Posted (Estimate): December 6, 2022
• Last Update Submitted That Met QC Criteria: December 5, 2022
• Last Verified: December 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Metabolic Diseases; Cerebrovascular Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Lung Diseases; Genetic Diseases, Inborn; Occupational Diseases; Proteostasis Deficiencies; Metabolism, Inborn Errors; Brain Diseases, Metabolic; Pneumoconiosis; Lung Diseases, Interstitial; Lung Injury; Brain Diseases, Metabolic, Inborn; Cerebral Arterial Diseases; Intracranial Arterial Diseases; Cerebral Small Vessel Diseases; Amyloidosis, Familial; Amyloidosis; Cerebral Amyloid Angiopathy; Cerebral Amyloid Angiopathy, Familial; Siderosis
• Other Study ID Numbers: Local/2022/Dr-02

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No