Impact of Von Willebrand Factor-platelet Aggregates in Patients With Type 2B Disease
试验速览
- 阶段
- 不适用
- 入组人数
- 5
- 试验地点
- 2
- 主要终点
- Rate of cerebral lesions in patients with type 2B disease
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Basic Science
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 70 Years(Adult, Older Adult)
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •patients with type 2B disease
- •neurological signs
- •signature of inform consent
排除标准
- •contraindication for MRI
- •Pregnant women
- •other hemorrhagic disease
结局指标
主要结局
Rate of cerebral lesions in patients with type 2B disease
时间窗: at the inclusion
Analysis of MRI sequences and % of patients with cerebral lesions
次要结局
- Verbal memory function evaluation(at the inclusion)
- Attention evaluation(at the inclusion)
- Subjective cognitive failures evaluation(at the inclusion)
- Visuospatial memory evaluation(at the inclusion)
- Global cognitive function evaluation(at the inclusion)
- Speed of mental processes evaluation(at the inclusion)
