Impact of Von Willebrand Factor-platelet Aggregates in Patients With Type 2B Disease (Von2B)

  • STATUS
    Recruiting
  • End date
    Dec 14, 2023
  • participants needed
    5
  • sponsor
    University Hospital, Caen
Updated on 4 October 2022

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.

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.

Details
Condition Von Willebrand Disease, Type 2B
Treatment 3T (Tesla) Magnetic Resonance Imaging (MRI)
Clinical Study IdentifierNCT05015244
SponsorUniversity Hospital, Caen
Last Modified on4 October 2022

Eligibility

Yes No Not Sure

Inclusion Criteria

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

Exclusion Criteria

contraindication for MRI
Pregnant women
other hemorrhagic disease
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