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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| surgical revascularization | Experimental | Patients will be assigned to either surgical or conservative treatment depending on their clinical symptoms and radiological assessment. |
|
| conservative treatment | Experimental | Normal conservative treatment without surgical intervention. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Surgical Revascularization | Procedure | 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. |
| Measure | Description | Time Frame |
|---|---|---|
| Neurocognitive outcomes at 3/6/12/24-month follow-up after surgical revascularization or conservative treatment. | 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. | 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| The number of participants who suffer from all stroke or death during 30 days to 24 months | 24 months | |
| The number of participants who suffer from all kinds of adverse events related to surgery. | 30 days |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Yu Lei, MD | Contact | +86 021 52889999 | piliyouxia_lei@126.com | |
| Yuxiang Gu, MD,PhD | Contact | guyuxiang1972@126.com |
| Name | Affiliation | Role |
|---|---|---|
| Yuxiang Gu, MD,PhD | Department of Neurosurgery, Huashan Hospital, Fudan University | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Neurosurgery, Huashan Hospital | Recruiting | Shanghai | Shanghai Municipality | 200040 | China |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 30688272 | Derived | Su JB, Xi SD, Zhou SY, Zhang X, Jiang SH, Xu B, Chen L, Lei Y, Gao C, Gu YX. Microstructural damage pattern of vascular cognitive impairment: a comparison between moyamoya disease and cerebrovascular atherosclerotic disease. Neural Regen Res. 2019 May;14(5):858-867. doi: 10.4103/1673-5374.249234. |
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| ID | Term |
|---|---|
| D009072 | Moyamoya Disease |
| ID | Term |
|---|---|
| D002340 | Carotid Artery Diseases |
| D002561 | Cerebrovascular Disorders |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
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| ID | Term |
|---|---|
| D000072700 | Conservative Treatment |
| ID | Term |
|---|---|
| D013812 | Therapeutics |
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|
| Conservative treatment | Other | Patients will be medically treated with antiplatelets, antiepileptics, antihypertensives and vasodilators depending on the presentation. |
|
| Postoperative neurocognitive outcomes before discharge. | Measure neurocognitive outcomes about 1 week after surgery according to participants' physical condition, using the tests mentioned in the primary outcome measurement. | average of 1 week after surgery |
| Postoperative radiological outcomes before discharge. | Measure cerebral perfusion simultaneously with neurocognitive testing, using the single photon emission computed tomography (SPECT). | average of 1 week after surgery |
| 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 |
| 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. | Data analysis techniques include amplitude of frequency fluctuation (ALFF), regional homogeneity (ReHo), independent component analysis (ICA), voxel-based morphometry analysis (VBM), etc. | 2 years |
| D009422 | Nervous System Diseases |
| D002539 | Cerebral Arterial Diseases |
| D020765 | Intracranial Arterial Diseases |
| D001157 | Arterial Occlusive Diseases |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |