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Published on: December 16, 2022
Neurocognitive impairment in adults with moyamoya disease without stroke
Peter Karzmark1, Penelope D Zeifert, Teresa E Bell-Stephens
1Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, California 94305-5235, USA.
Background:
Adults with moyamoya disease (MMD) have been shown to manifest cognitive impairment, but it is unclear whether this is the result of ischemic stroke.
Objective:
To determine whether adults with MMD but without stroke have cognitive impairment.
Methods:
We performed detailed neuropsychological assessments in 30 adults with angiographically confirmed MMD without magnetic resonance imaging (MRI) evidence of stroke.
Results:
Twenty patients (67%) exhibited small T2 hyperintensities in the cerebral subcortical white matter on brain MRI but no evidence of gray matter damage. Significant cognitive impairment, defined as half of test scores ≥ 1 SD below the normal mean, was present in 7 patients (23%). Executive functioning, mental efficiency, and word finding were the ability areas most frequently impaired, whereas memory was relatively intact. Clinically significant emotional distress (depression and/or anxiety) was present in 11 patients (37%). Comparable cognitive findings were also observed in the subset of 10 patients (33%) with completely normal static brain MRI.
Conclusion:
Cognitive impairment in MMD can occur in the absence of ischemic stroke as manifested on MRI.
Insights
Cognitive impairment in moyamoya disease (MMD) can occur even without evidence of stroke on MRI scans. This study found that MMD patients without stroke still experienced significant deficits in executive function and mental processing.
Area of Science:
- Neurology
- Neuroscience
- Radiology
Background:
- Moyamoya disease (MMD) is known to cause cognitive impairment in adults.
- The exact cause of this cognitive decline, specifically whether it stems from ischemic stroke, remains unclear.
Purpose of the Study:
- To investigate cognitive function in adults diagnosed with MMD but without any MRI-confirmed evidence of stroke.
- To determine if cognitive impairment exists independently of ischemic events in MMD patients.
Main Methods:
- Neuropsychological assessments were conducted on 30 adult patients with angiographically confirmed MMD.
- Brain MRI was used to exclude evidence of stroke in the study participants.
Main Results:
- Despite the absence of stroke, 23% of MMD patients showed significant cognitive impairment.
- Impaired cognitive domains included executive functioning, mental efficiency, and word finding; memory was relatively preserved.
- Subtle white matter hyperintensities were observed in 67% of patients, but without gray matter damage.
Conclusions:
- Cognitive impairment in moyamoya disease can manifest independently of ischemic stroke.
- These findings suggest that MMD itself, or associated non-ischemic changes, contribute to cognitive deficits.
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