Impact of Von Willebrand Factor-platelet Aggregates in Patients With Type 2B Disease

NCT ID: NCT05015244

Last Updated: 2022-08-24

Basic Information

• Recruitment Status: UNKNOWN
• Clinical Phase: NA
• Total Enrollment: 5 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2019-06-01
• Study Completion Date: 2023-12-31

Brief Summary

The working hypothesis is that patients with Von Willebrand Disease-type 2B may have neurological symptoms due to the chronic formation of platelets/Von Willebrand Factor aggregates. Interestingly, several patients present nonspecific neurological symptoms (nystagmus, headaches, memory disorder, …) which may be associated with cerebral microangiopathy and chronic microvessel inflammation secondary to Von Willebrand Disease-type 2B due to chronic exposure to Von Willebrand Factor-platelets complexes.

Detailed Description

Investigator will use an extensive series of neuropsychological tests that cover virtually all cognitive domains. A measurement of global cognitive function will be assessed by the Mini Mental State Examination (MMSE). The verbal memory function will be assessed by the Rey Auditory Verbal Learning Test (RAVLT). Visuospatial memory will be administered by the Rey's Complex Figure Test (RCFT). To evaluate speed of mental processes, Stroop test (three subtasks), the Paper and Pencil Memory Scanning Task (four subtasks), the Symbol-Digit Substitution Task will be used. To evaluate attention, the verbal series attention test (VSAT) will be used. To register subjective cognitive failures investigator will administer the Cognitive failures questionnaire (CFQ). The neurological evaluation will be completed with 3T (Tesla) Magnetic Resonance Imaging (MRI) with different MRI sequences including T1 3D SPACE, FLAIR 3D , 3D SWI (Susceptibility weighted imaging) and 2D EPI (Echo Planar Imaging) to address the presence of cerebral small vessel disease. The presence of each of the four MRI markers for cerebral small vessel disease (white matter hyperintensities, lacunes, cerebral microbleeds and perivascular spaces will be addressed.

Conditions

Von Willebrand Disease, Type 2B

Study Design

• Allocation Method: NA
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: BASIC_SCIENCE
• Blinding Strategy: NONE

Study Groups

Von Willebrand Disease Type 2B patient with neurological symptoms

evaluation of patients using 3T (Tesla) Magnetic Resonance Imaging (MRI) and series of neuropsychological tests that cover virtually all cognitive domains.

Group Type: EXPERIMENTAL

3T (Tesla) Magnetic Resonance Imaging (MRI)

Intervention Type: PROCEDURE

Neurological evaluation using MRI and neuropsychological tests

Interventions

3T (Tesla) Magnetic Resonance Imaging (MRI)

Neurological evaluation using MRI and neuropsychological tests

Intervention Type: PROCEDURE

Other Intervention Names

Series of neuropsychological tests that cover virtually all cognitive domains

Eligibility Criteria

Inclusion Criteria

• patients with type 2B disease
• neurological signs
• signature of inform consent

Exclusion Criteria

• contraindication for MRI
• Pregnant women
• other hemorrhagic disease

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 70 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

University Hospital, Caen

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Locations

University Hospital

Caen, Calvados, France

Site Status: RECRUITING

Countries

France

Facility Contacts

Frederic VARNIER

Role: primary

directiongenerale@chu-caen.fr

0231063106 ext. +33

Other Identifiers

2018-A03005-50

Identifier Type: -

Identifier Source: org_study_id