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In Silico Clinical Trials for Cardiovascular Disease
Published on: May 27, 2022
Neuropsychological correlates of moyamoya disease: A clinical case study
Shelby B Hughes1, Anastasia Matchanova2, Lisa Delano-Wood2,3
1San Diego State University/University of California, San Diego Joint Doctoral Program in Clinical Psychology, San Diego, CA, USA.
Objective:
Moyamoya disease (MMD) is a rare, progressive occlusive cerebrovascular disorder characterized by stenosis of the internal carotid arteries and compensatory collateral vessel formation, often leading to ischemic or hemorrhagic events. We present the case of a 50-year-old bilingual Filipina woman with a neuroimaging-confirmed diagnosis of MMD (Suzuki grade III) who underwent staged bilateral surgical revascularization procedures (right intracranial bypass and left encephaloduroarteriosynangiosis [EDAS]), with her left EDAS complicated by an acute left anterior cerebral artery (ACA)/middle cerebral artery (MCA) border zone infarct. Post-operatively, she exhibited expressive and receptive aphasia, dysarthria, right hemiparesis, and functional decline requiring extensive rehabilitation.
Methods:
A comprehensive medical record review in conjunction with a clinical interview and administration and interpretation of a comprehensive neuropsychological battery was completed approximately 18-months post-surgical intervention.
Results:
Neuropsychological evaluation revealed global impairments in memory, executive functioning, processing speed, and visuospatial abilities, with particularly severe language deficits, including a language shift from English to Tagalog, further compounding cognitive and functional decline.
Conclusion:
This case report adds to the small body of literature identifying neuropsychological patterns of impairment in MMD. Additionally, in conjunction with prior research, findings emphasize the importance of early detection and timely surgical intervention in order to optimize cognitive and functional outcomes.
Insights
Moyamoya disease (MMD) can cause significant cognitive decline, including language deficits, even after surgical revascularization. Early detection and intervention are crucial for optimizing outcomes in MMD patients.
Area of Science:
- Neuroscience
- Cerebrovascular Medicine
- Neuropsychology
Background:
- Moyamoya disease (MMD) is a rare, progressive cerebrovascular disorder.
- It involves internal carotid artery stenosis and collateral vessel formation, leading to ischemic or hemorrhagic events.
Purpose of the Study:
- To present a case study of a bilingual Filipina woman with MMD.
- To detail her neuroimaging findings, surgical interventions, and post-operative neuropsychological outcomes.
Main Methods:
- Neuroimaging confirmed Moyamoya disease (Suzuki grade III).
- The patient underwent staged bilateral surgical revascularization (intracranial bypass and encephaloduroarteriosynangiosis).
- A comprehensive neuropsychological battery was administered 18 months post-surgery.
Main Results:
- The patient experienced an acute infarct following surgery.
- Neuropsychological evaluation revealed global cognitive impairments (memory, executive function, processing speed, visuospatial abilities).
- Severe language deficits were noted, including a language shift from English to Tagalog.
Conclusions:
- This case highlights specific neuropsychological patterns in MMD.
- Findings underscore the importance of early detection and timely surgical intervention for MMD.
- Optimizing cognitive and functional outcomes in MMD requires prompt management.
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