Moyamoya Disease
Conditions
Brief summary
Adult patients with moyamoya disease (MMD) are reported to suffer from considerable impairment of executive function/attention. Although reduced cerebrovascular reserve (CVR) in frontal areas has been detected by perfusion MRI and then confirmed to be associated with executive dysfunction in adult MMD, the structural and functional changes is still unclear with progression of executive dysfunction. Furthermore, it is very important to study the association between the neurocognitive and radiological improvement after surgical revascularization, so as to help detecting cerebral regions which are involved in executive deterioration or improvement after surgery. Then the investigators can determine whether these regions can be used as indicators to decide rational therapeutic schedule and timing of adult MMD with executive dysfunction. Thus the aim of this study is to primarily find out the neuropsychological and radiological correlates in adult MMD, and then to quantitatively evaluate the effectiveness of surgical revascularization in prevention of executive dysfunction in adult MMD.
Detailed description
Previous studies of adult moyamoya disease (MMD) have revealed that vascular cognitive impairment (VCI) is the consequence of ischemic damage to dynamic factors such as cerebral hypoperfusion, rather than to cerebral gray matter. However, it is still unclear which regions are affected by MMD and how these regions respond to the progressive cognitive decline. In other way, the investigators need to detect spatial patterns in the brain activity of MMD in order to understand its pathophysiological nature. Surgical revascularization has been accepted as the only effective form of treatment in preventing future ischemic episodes. However, its effectiveness in cognitive protection is still unknown. Thus, the investigators determine to quantitatively evaluate cognitive and radiological outcomes in adult MMD postoperatively and during follow-ups.
Interventions
OTHERConservative treatment
Patients will be medically treated with antiplatelets, antiepileptics, antihypertensives and vasodilators depending on the presentation.
PROCEDURESurgical Revascularization
Most patients in this group will be performed combined procedures of superficial temporal to middle cerebral artery bypass (STA-MCA) and encephalo-duro-myo-synangiosis (EDMS). Patients not suitable for combined procedures will be performed EDMS.
Sponsors
Huashan Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
SINGLE (Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
Yes
Inclusion criteria
* Right-handed Chinese people aged over 18 years * No evidence of recent or remote infarct in the cerebral cortical, basal ganglia, brainstem or cerebellum * No evidence of recent or remote intracerebral hemorrhage * Diagnosis confirmed by digital subtraction angiography (DSA) according to Suzuki scale * No surgical intervention before recruitment * Physically capable of cognitive testing * Geographically accessible and reliable for follow-up.
Exclusion criteria
* Significant neurological diseases or psychiatric disorders that could affect cognition * Other cerebrovascular diseases (such as atherosclerosis or vasculitides) likely to cause focal cerebral ischemia * Concomitant cerebrovascular diseases (such as aneurysms or arteriovenous malformation) that need surgical intervention * Severe systemic diseases * Pregnant or perinatal stage women * Any diseases likely to death within 2 yeas * Taking drugs such as benzodiazepine clonazepam * Any contraindications or allergy to aspirin * Allergy to iodine or radiographic contrast media * Past history of surgical revascularization * Concurrent participation in any other experimental treatment trial * Any condition that in the surgeon's judgment suggests the patient an unsuitable surgical candidate.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Neurocognitive outcomes at 3/6/12/24-month follow-up after surgical revascularization or conservative treatment. | 2 years | Measure neurocognitive outcomes with a battery of neuropsychological tests covering global cognition, executive, memory, language, and visuospatial functions. It involves the Mini-mental state examination (MMSE), the Memory and Executive Screening test (MES), the Trail Making Test (TMT), the Auditory Verbal Learning Test (AVLT), the verbal fluency test (VFT), the Rey-Osterrieth complex figure test (CFT), etc. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| The number of participants who suffer from all kinds of adverse events related to surgery. | 30 days | — |
| Postoperative neurocognitive outcomes before discharge. | average of 1 week after surgery | Measure neurocognitive outcomes about 1 week after surgery according to participants' physical condition, using the tests mentioned in the primary outcome measurement. |
| Postoperative radiological outcomes before discharge. | average of 1 week after surgery | Measure cerebral perfusion simultaneously with neurocognitive testing, using the single photon emission computed tomography (SPECT). |
| The number of participants who suffer from all stroke or death during 30 days to 24 months | 24 months | — |
| The changes from baseline in single photon emission computed tomography (SPECT). | 2 years | — |
| The changes from baseline in blood oxygen level-dependent functional magnetic resonance imaging (BOLD fMRI) during rest. | 2 years | Data analysis techniques include amplitude of frequency fluctuation (ALFF), regional homogeneity (ReHo), independent component analysis (ICA), voxel-based morphometry analysis (VBM), etc. |
| The changes from baseline in modified Rankin scale (mRS) and national institutes of health stroke scale (NIHSS). | at 7 days, 30 days, 3/6/12/24 months | — |
Countries
China
Contacts
Primary ContactYu Lei, MD
Backup ContactYuxiang Gu, MD,PhD
Outcome results
None listed