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A 75-year-old Chinese American man presented to behavioral neurology clinic for a second opinion of dementia with Lewy body disease (DLB). The clinical manifestations met the criteria for a probable DLB diagnosis. Yet, in-depth evaluation unveiled clinical history, family history, and neuroimaging evidences that suggested a diagnosis of behavioral variant frontotemporal dementia (FTD). A heterozygous pathogenic variant in the microtubule-associated protein Tau ( MAPT ) was identified through genetic testing and confirmed the diagnosis of autosomal dominant MAPT -related FTD. This case is the first reported instance of MAPT -related FTD presenting with well-formed visual hallucinations in an elderly Chinese American.

Importance: Subjective cognitive decline (SCD) is recognized to be in the Alzheimer disease (AD) cognitive continuum. The SCD Initiative International Working Group recently proposed SCD-plus (SCD+) features that increase risk for future objective cognitive decline but that have not been assessed in a large community-based setting., Objective: To assess SCD risk for mild cognitive impairment (MCI), AD, and all-cause dementia, using SCD+ criteria among cognitively normal adults., Design, Setting, and Participants: The Framingham Heart Study, a community-based prospective cohort study, assessed SCD between 2005 and 2019, with up to 12 years of follow-up. Participants 60 years and older with normal cognition at analytic baseline were included. Cox proportional hazards (CPH) models were adjusted for baseline age, sex, education, APOE ε4 status, and tertiles of AD polygenic risk score (PRS), excluding the APOE region. Data were analyzed from May 2021 to November 2023., Exposure: SCD was assessed longitudinally using a single question and considered present if endorsed at the last cognitively normal visit. It was treated as a time-varying variable, beginning at the first of consecutive, cognitively normal visits, including the last, at which it was endorsed., Main Outcomes and Measures: Consensus-diagnosed MCI, AD, and all-cause dementia., Results: This study included 3585 participants (mean [SD] baseline age, 68.0 [7.7] years; 1975 female [55.1%]). A total of 1596 participants (44.5%) had SCD, and 770 (21.5%) were carriers of APOE ε4. APOE ε4 and tertiles of AD PRS status did not significantly differ between the SCD and non-SCD groups. MCI, AD, and all-cause dementia were diagnosed in 236 participants (6.6%), 73 participants (2.0%), and 89 participants (2.5%), respectively, during follow-up. On average, SCD preceded MCI by 4.4 years, AD by 6.8 years, and all-cause dementia by 6.9 years. SCD was significantly associated with survival time to MCI (hazard ratio [HR], 1.57; 95% CI, 1.22-2.03; P <.001), AD (HR, 2.98; 95% CI, 1.89-4.70; P <.001), and all-cause dementia (HR, 2.14; 95% CI, 1.44-3.18; P <.001). After adjustment for APOE and AD PRS, the hazards of SCD were largely unchanged., Conclusions and Relevance: Results of this cohort study suggest that in a community setting, SCD reflecting SCD+ features was associated with an increased risk of future MCI, AD, and all-cause dementia with similar hazards estimated in clinic-based settings. SCD may be an independent risk factor for AD and other dementias beyond the risk incurred by APOE ε4 and AD PRS.

Objective: This study examined the utility of the Chinese-language translations of the word list memory test (Philadelphia Verbal Learning Test) and story memory test (Logical Memory subtest of the Wechsler Memory Scale) for differentiating cognitive diagnosis in older U.S. Chinese immigrants., Method: Participants were ≥ 60 years old, with Chinese language proficiency to complete a diagnostic workup at the Mount Sinai's Alzheimer's Disease Research Center. The workup included an evaluation by a geriatric psychiatrist and cognitive testing with a psychometrician. Diagnosis of normal, mild cognitive impairment (MCI), and dementia was made independent of the cognitive tests at consensus led by a dementia expert physician. Multivariable logistic regression models were used to assess the sensitivity of story and word list memory tests for distinguishing between groups. Receiver operating characteristic (ROC area/area under the curve [AUC]) was used to compare the predictive accuracy of the two tests., Results: The sample included 71 participants with normal cognition, 42 with MCI, and 24 with dementia. The MCI group was older and less educated than normal controls but younger and more educated than the dementia group. Delayed recall of both memory tests, but not immediate recall of either test, predicted diagnosis. While composite memory score of word list (AUC = 0.90) predicted diagnosis slightly better than that of stories (AUC = 0.85), the difference was not significant in this small sample ( p = .14)., Conclusions: Chinese-language translations of verbal memory tests, in particular delayed recall scores, were equally sensitive for classifying cognitive diagnosis in older U.S. Chinese immigrants. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Clinical neuropsychology has been a valuable asset to neurologic surgery, contributing to lateralization and localization of pathologic brain tissue, identification of eloquent cortex, and evaluation of postoperative neuropsychological functioning. Moreover, neuropsychologists provide empirically driven interventions aimed at supporting preparation and/or recovery of neurosurgery patients. Nonetheless, several challenges may limit the reliability, validity, and generalizability of the assessment data obtained and reduce the usefulness of other neuropsychological services provided. Specifically, linguistic, cultural, educational, and other biases associated with demographic characteristics can lead to a narrowed view of an individual's life experiences, which must be confronted to fulfill the mission of ensuring that all patients have access to care that is appropriate to their needs. Instead of perceiving these challenges as insurmountable barriers, such issues can be viewed as opportunities to catalyze change and foster innovation for the future of neuropsychological care in neurosurgical settings. In addition to reviewing the possible mechanisms of these obstacles, the current article offers tangible solutions at both a macro level (e.g., discipline-wide transformations) and micro level (e.g., individualized patient-centric approaches). Outlined are practical techniques to potentially improve consensus and standardization of methods, advance and globalize research, expand representativeness of measures and practices to serve diverse individuals, and increase treatment adherence through engagement of patients and their families., (Copyright © 2022. Published by Elsevier Inc.)

Over the last century, collaboration between clinical neuropsychologists and neurosurgeons has advanced the state of the science in both disciplines. These advances have provided the field of neuropsychology with many opportunities for innovation in the care of patients prior to, during, and following neurosurgical intervention. Beyond giving a general overview of how present-day advances in technology are being applied in the practice of neuropsychology within a neurological surgery department, this article outlines new developments that are currently unfolding. Improvements in remote platform, computer interface, "real-time" analytics, mobile devices, and immersive virtual reality have the capacity to increase the customization, precision, and accessibility of neuropsychological services. In doing so, such innovations have the potential to improve outcomes and ameliorate health care disparities., (Copyright © 2022. Published by Elsevier Inc.)

The disciplines of neuropsychology and neurosurgery have a history of partnership that has improved prognoses for patients with neurologic diagnoses that once had poor outcomes. This article outlines the evolution of this relationship and describes the current role that clinical neuropsychology has within a department of neurological surgery across the preoperative, intraoperative, and postoperative stages of treatment. Understanding the foundations of collaboration between neuropsychology and neurosurgery contextualizes present challenges and future innovations for advancing excellence along the continuum of care for all neurosurgical patients., (Copyright © 2022. Published by Elsevier Inc.)

Background: Few studies have examined the prevalence of alcohol and drug use in individuals with multiple sclerosis (MS). The current study sought to examine the prevalence and associated demographic, disease-related, and psychological correlates of substance use in an East Coast United States outpatient MS sample., Methods: 157 individuals with MS completed questionnaires prior to, during or after their visit with an MS neurologist. These questionnaires included: the Alcohol Use Disorders Identification Test-Consumption (AUDIT-C), CAGE, CAGE-Adapted to Include Drugs (CAGE-AID), Patient Health Questionnaire-9 item (PHQ-9), Beck Depression Inventory-Second Edition (BDI-II) and Hospital Anxiety and Depression Scale-Anxiety (HADS-A)., Results: On the AUDIT-C, 40% of individuals with MS met or exceeded the cutoff for excessive alcohol use. They were more highly educated and younger than non-drinkers. Utilizing the CAGE, 6% of the sample met criteria for a lifetime history of excessive alcohol use and men endorsed higher rates of alcohol use than women. Only a small portion of the sample endorsed a history of drug use (CAGE-AID, 4%). Drug use was associated with greater disability and depression symptoms, but lower self-reported anxiety., Conclusions: Current alcohol use was prevalent in this sample, and excessive use was associated with men, younger age, and more education. Reported drug use was minimal and associated with greater disability, more self-reported depression, but fewer anxiety symptoms., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Abnormal neurodevelopment has been widely reported in combined methylmalonic aciduria (MMA) and homocystinuria, cblC type (cblC disease), but neurodevelopmental phenotypes in cblC have not previously been systematically studied. We sought to further characterize developmental neurology in children with molecularly-confirmed cblC. Thirteen children at our center with cblC, born since implementation of expanded newborn screening in New York State, undertook standard-of-care evaluations with a pediatric neurologist and pediatric ophthalmologist. At most recent follow-up (mean age 50 months, range 9-84 months), of twelve children with early-onset cblC, three (25%) had a history of clinical seizures and two (17%) meet criteria for microcephaly. A majority of children had hypotonia and nystagmus. Twelve out of thirteen (92%) underwent neurodevelopmental evaluation (mean age 41 months; range 9-76 months), each child tested with standardized parental interviews and, where possible, age- and disability-appropriate neuropsychological batteries. All patients showed evidence of developmental delay with the exception of one patient with a genotype predictive of attenuated disease and near-normal biochemical parameters. Neurodevelopmental deficits were noted most prominently in motor skills, with relative preservation of socialization and communication skills. Nine children with early-onset cblC underwent magnetic resonance imaging and spectroscopy (MRI/MRS) at mean age of 47 months (range 6-81 months); common abnormalities included callosal thinning, craniocaudally short pons, and increased T2 FLAIR signal in periventricular and periatrial white matter. Our study further characterizes variable neurodevelopmental phenotypes in treated cblC, and provides insights into the etiopathogenesis of disordered neurodevelopment frequently encountered in cblC. Plasma homocysteine and MMA, routinely measured at clinical follow-up, may be poor predictors for neurodevelopmental outcomes. Additional data from large, prospective, multi-center natural history studies are required to more accurately define the role of these metabolites and others, as well as that of other genetic and environmental factors in the etiopathogenesis of the neurologic components of this disorder., (© 2013.)

Background: Compromised learning and cognitive fatigue are critical clinical features in multiple sclerosis., Objectives: This study was designed to determine the effect of repeated exposures within and across study visits on performance measures of learning and cognitive fatigue in relapsing-remitting multiple sclerosis (RRMS)., Methods: Thirty patients with RRMS and 30 controls were recruited. Using a burst measurement design (i.e. repeated assessments within and across study visits) the oral version of the Symbol Digit Modalities Test (SDMT) was administered three times during the baseline and two consecutive monthly follow-up visits for a total of nine test administrations. Learning was assessed within and across study visits whereas cognitive fatigue was assessed during the course of each test administration that was divided into three 30-second intervals., Results: Linear mixed-effect models revealed compromised learning within (95% CI: 2.6355 to 3.9867) and across (95% CI: 1.3250 to 3.1861) visits and worse cognitive fatigue (95% CI: -2.1761 to -0.1720) in patients with RRMS compared with controls. Among patients with RRMS, worse self-rated cognitive dysfunction predicted poor learning within (95% CI: -0.1112 to -0.0020) and across (95% CI: -0.0724 to -0.0106) visits., Conclusions: Burst design is optimal to study learning and cognitive fatigue. This methodology, using the SDMT or other time-efficient tests as outcome measures, can be successfully implemented in longitudinal studies and clinical trials.