Neurocognitive Performance After Cerebral Revascularization in Adult Moyamoya Disease.

BACKGROUND AND PURPOSE: Cerebral revascularization using EC-IC bypass is widely used to treat moyamoya disease, but the effects of surgery on cognition are unknown. We compared performance on formal neurocognitive testing in adults with moyamoya disease before and after undergoing direct EC-IC bypass. METHODS: We performed a structured battery of 13 neurocognitive tests on 84 adults with moyamoya disease before and 6 months after EC-IC bypass. The results were analyzed using reliable change indices for each test, to minimize test-retest variability and practice effects. RESULTS: Twelve patients (14%) showed significant decline postoperatively, 9 patients (11%) improved, and 63 patients (75%) were unchanged. Similar results were obtained when the analysis was confined to those who underwent unilateral (33) or bilateral (51) revascularization. CONCLUSIONS: The majority of patients showed neither significant decline nor improvement in neurocognitive performance after EC-IC bypass surgery. Uncomplicated EC-IC bypass seems not to be a risk factor for cognitive decline in this patient population.

Neurocognitive impairment in adults with moyamoya disease without stroke.

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.

Comparison of the frontal systems behavior scale and neuropsychological tests of executive functioning in predicting instrumental activities of daily living.

Both neuropsychological tests of executive functioning and the Frontal Systems Behavior Scale (FrSBe) consistently predict instrumental activity-of-daily-living capacity. However, the nature of the predictive relationship between the FrSBe and neuropsychological tests of executive functioning has received limited attention. The current study was designed to assess the incremental validity of the FrSBe in predicting instrumental activity-of-daily-living functioning when added to comprehensive testing of executive functioning in a sample of 100 adult general neuropsychological referrals. A composite measure of executive test performance was calculated, and a family member completed the FrSBe and an instrumental activity-of-daily-living measure. Stepwise multiple regression analysis using the executive composite measure and the FrSBe accounted for 44% of the variance in instrumental activity capacity, and the addition of the FrSBe increased predictive ability by approximately 50%. The current results also indicate that FrSBe Scale E is more important as a predictor of instrumental activity capacity than the two self-regulation measures, Scale A and Scale D.

Effect of moyamoya disease on neuropsychological functioning in adults.

OBJECTIVE: Moyamoya disease is a cerebrovascular disorder characterized by progressive occlusion of vessels comprising the circle of Willis, resulting in formation of collaterals that have a cloudy appearance on angiography. Neuropsychological research on the cognitive effects of the disorder in adults has been limited in scope and generalizability; only a few case studies have been published. The current study was intended to more comprehensively document the nature of cognitive impairment in moyamoya disease by assessing a large number of adult cases with a neuropsychological assessment test battery. METHODS: Thirty-six adult patients with neurodiagnostically confirmed moyamoya disease were given presurgical neuropsychological assessments. RESULTS: Mean group performances were within normal limits for all measures assessed. The highest rate of impairment was for measures of executive functioning. The lowest rates occurred with memory and perception measures. Cognitive impairment was present in 11 (31%) of the patients; it was judged to be moderate to severe in four patients (11%). Five patients reported a mild level of depression, and two patients reported a moderate level. CONCLUSION: The present findings suggest that moyamoya disease diagnosed in adults can impair cognition but that the effect is not as severe as in pediatric cases. Executive functioning is most affected. Memory and, to a large extent, intellect are spared. The current pattern of results suggests brain region-behavior correlations that deserve further study.

Measurement of Depression in Epilepsy.

The current study was designed to evaluate the accuracy of the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) and the Beck Depression Inventory (BDI) in determining the presence of depression in epileptic patients. A psychiatric diagnostic interview was used as an external criterion for the presence of depression. The participants were 61 patients undergoing presurgical evaluation for treatment of epilepsy at the epilepsy treatment center of a research medical center. The results of the present study indicate that the accuracy of assessment of depression in epilepsy with psychological tests varies with the depression scale or subscale used. The BDI performed in general more effectively as a diagnostic instrument than the MMPI-2. The results suggest that the latter test was negatively impacted by somatic content present in some scales and subscales.