Peter Karzmark

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.

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.

Validity of the serial seven procedure

Serial subtraction by seven (Serial Seven Test, SST) is frequently used in mental status evaluation for dementia as a measure of concentration. Validation research on the SST has been limited. The literature to date raises significant concern about the nature of the procedure. The purpose of the present study was to evaluate the nature and validity of the SST. The participants were 80 consecutive outpatient referrals to the neuropsychology consultation service of two large general medical hospitals. All subjects were administered a comprehensive neuropsychological assessment battery, including the Mini Mental State Examination version of the SST. Multiple regression analysis indicated that calculation skill is at least as important as concentration, in predicting SST performance. Measures of overall level of cognitive dysfunction, education, and psychopathology were unrelated to SST performance. The results suggest that SST performance is heavily influenced by basic arithmetic skill and that the procedure should be used with caution as a measure of concentration or other nonacademic mental abilities. Copyright

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.

Neuroradiologic Correlates of Cognitive Impairment in Adult Moyamoya Disease

BACKGROUND AND PURPOSE: MMD has been shown to result in impairment of executive functioning in adults. The purpose of this study was to correlate presurgical neuropsychological assessments with the severity of primary MMD as measured by CBF and CVR and with secondary damage from MMD as estimated by cortical stroke and WMD. MATERIALS AND METHODS: A retrospective analysis of 31 adult patients with MMD was performed. Xe-CT was used to obtain CBF and CVR, and MRI was reviewed to grade cortical stroke and WMD. Two tests of executive functioning (FAS and TMT-B) were correlated with imaging findings. A multiple regression analysis was performed. RESULTS: There was a significant overall positive relationship between mean CBF and FAS (P = .038) and TMT-B scores (P = .014). A significant negative relationship was present between the WMD score and the FAS (P = .009) and TMT-B scores (P = .015). Per-region analysis demonstrated that FAS and TMT-B scores were significantly decreased by the presence of a posterior stroke (P < .0001 and P = .001) or WMD (P = .006 and P = .004). All patients with posterior parieto-occipital WMD or stroke also had secondary disease in the anterior regions. CONCLUSIONS: Impaired executive functioning in adults with MMD is most strongly associated secondary damage in the form of WMD or cortical stroke. The effect is most profound with parieto-occipital lobe involvement, likely a reflection of overall disease severity. Increasing global WMD burden may be a better indicator of cognitive decline than cortical infarction. Patients with higher baseline CBF seem to have better cognitive functioning.

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.

The Effect of Cognitive, Personality, and Background Factors on the WAIS-III Arithmetic Subtest

In the Wechsler system the Arithmetic subtest has been viewed as a measure of concentration, working memory, or freedom from distractibility. However, a wide range of other influences on Arithmetic performance has been proposed. The current study was intended to examine these to further characterize what is measured by the Arithmetic subtest. Participants were 118 adults referred for neuropsychological assessment. The results indicate a strong association between WAIS-III Arithmetic and the other WMI (Working Memory Index) subtests. Arithmetic also showed a high association with Arithmetic skill and verbal memory. Moderate contributions to Arithmetic performance were found for most other cognitive measures. Measures of anxiety and of background factors, such as perceived difficulty learning Arithmetic, were weakly related to Arithmetic scores. These results suggest that although Arithmetic may be considered a measure of concentration or working memory, many other factors influence it and its specificity as a concentration measure is limited.

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.

Accuracy statistics in predicting Independent Activities of Daily Living (IADL) capacity with comprehensive and brief neuropsychological test batteries

ABSTRACT This investigation was designed to determine the predictive accuracy of a comprehensive neuropsychological and brief neuropsychological test battery with regard to the capacity to perform instrumental activities of daily living (IADLs). Accuracy statistics that included measures of sensitivity, specificity, positive and negative predicted power and positive likelihood ratio were calculated for both types of batteries. The sample was drawn from a general neurological group of adults (n = 117) that included a number of older participants (age >55; n = 38). Standardized neuropsychological assessments were administered to all participants and were comprised of the Halstead Reitan Battery and portions of the Wechsler Adult Intelligence Scale-III. A comprehensive test battery yielded a moderate increase over base-rate in predictive accuracy that generalized to older individuals. There was only limited support for using a brief battery, for although sensitivity was high, specificity was low. We found that a comprehensive neuropsychological test battery provided good classification accuracy for predicting IADL capacity.

Predictors of activities of daily living in older adults: The Frontal Systems Behavior Scale (FrSBe) and neuropsychological test performance

famous name and face retrieval in relation to performance on the FCSRT and to PiB retention (DVR, cerebellar reference) as a continuous variable in the precuneus, co-varying for age. Results: Retrieval of famous names (t(24)1⁄41.14, p 1⁄4 .267) and occupations (t(24)1⁄41.55, p 1⁄4 .135) was equivalent for MCI and CN subjects. Across the entire sample, retrieval of the names of famous individuals was positively related to both free recall (r1⁄4.723, p1⁄4.001) and cued recall on the FCSRT (r1⁄4.629, p1⁄4.003). Similarly, retrieval of occupations of famous individuals was positively related to free recall (r1⁄4.579, p1⁄4.007) and cued recall (r1⁄4.566, p1⁄4.009) on the FCSRT. Within the PiB-PET subgroup, increasing Abeta deposition was related to worse performance retrieving occupations (r1⁄4.555, p1⁄40.01) with a trend for retrieving famous names (r1⁄4.366, p1⁄4.074). Conclusions: Results suggest that semantic memory decline co-occurs with episodic memory decline and is furthermore associated with underlying Alzheimer’s pathology. Semantic memory decline may be a useful early marker in MCI and preclinical AD.