Vocalization in Dementia: A Case Report and Review of the Literature

Background: Vocalizations are part of the spectrum of the ‘negative' behavioral and psychological symptoms of dementia (BPSD). We describe a patient with moderate-stage mixed dementia of Alzheimer's disease and cerebrovascular disease and a left orbitofrontal lesion exhibiting vocalization. The use of ‘redirection' has been demonstrated to be an effective nonpharmacological means of controlling BPSD, while reducing caregiver distress. Case Report: A 78-year-old right-handed African-American female presented with complaints of worsening memory and BPSD, causing significant caregiver distress. Throughout the evaluation, she constantly vocalized her son's name and made a continuous grunting noise, correlating with increased anxiety/agitation. We utilized a redirection technique, which achieved the immediate reduction of the vocalization symptoms. Caregiver psychoeducation was provided allowing them to use the redirection technique at home. Conclusions: In patients with dementia exhibiting negative symptoms of BPSD, using nonpharmacologic techniques (i.e. redirection) may be indicated. Psychotropic medications rarely address negative BPSD symptoms, while simultaneously decreasing patient's quality of life. Nonpharmacologic approaches are beneficial as first-line therapy for negative BPSD

Differences in the Experience of Caregiving Between Spouse and Adult Child Caregivers in Dementia With Lewy Bodies.

Background and objectivesDementia caregiving has been associated with increased burden, depression, grief, a decreased sense of well-being and quality of life, and a weakening of social support. Little is known about the experience of caregiving in Dementia with Lewy Bodies (DLB). The present study examines differences in the caregiving experience of spouse versus adult child caregivers of individuals with DLB.Research design and methodsIn this cross-sectional analytic study of spouses (n = 255) and adult children (n = 160) caregivers of individuals with DLB, participants completed an online survey of burden, grief, depression, well-being, quality of life, and social support.ResultsAdult child caregivers were more likely to care for women (p < .001) and see the care recipient less often (p < .001) than spouses. Adult child caregivers reported lower quality of life (p < .001) and more caregiver burden (p < .009), but also greater social support (p < .001) than spouses. Between group analyses of caregiver type by disease severity demonstrated that spousal caregivers experience greater grief with advancing disease (p = .005), while adult child caregivers increase social support with advancing disease (p < .001).Discussion and implicationsSpouses and adult children experience DLB caregiving differently. This was explained by the younger age of the adult child caregiver, frequency of contact with the care recipient, and differences in the care recipient's characteristics, frequency of neuropsychiatric symptoms, and disease severity. DLB caregiver support for this population should target psychoeducation for complicated neuropsychiatric symptoms in the care recipient. Screening all DLB caregivers for burden, grief, and depression is suggested to identify those that may benefit most from intervention. Spouses specifically may benefit from interventions that target increasing social support, while adult child caregivers may benefit from interventions aimed at mitigating burden and improving quality of life

Verbal and visuospatial deficits in dementia with Lewy bodies

This is the publisher's version, also available electronically from http://www.neurology.org/content/65/8/1232.Objective: To investigate the cognitive decline in dementia with Lewy bodies (DLBs) and characterize the contribution of Lewy bodies (LBs) to cognitive impairment in the presence of concurrent Alzheimer disease (AD). Methods: Cognitive deficits and rates of progression attributable to DLB and AD neuropathology were investigated in three groups of participants from the longitudinal cohort of the Alzheimer Disease Research Center at Washington University with autopsy-confirmed diagnoses of pure DLB (n = 9), mixed DLB/AD (n = 57), and pure AD (n = 66). Factor analysis was used to recover latent constructs in a comprehensive psychometric test battery, analysis of variance was used to test group differences on the observed dimensions, and random effects models were used to test longitudinal rates of cognitive decline. Results: Patients with AD pathology performed worse on the verbal memory dimension. Patients with LB pathology performed worse on the visuospatial dimension. Combined pathology affected visuospatial performance but not verbal memory. The rate of cognitive decline in the DLB, DLB/AD combined, and the pure AD groups was equivalent. Conclusions: The comorbid presence of DLB and AD alters the cognitive presentation of visuospatial deficits in dementia but does not alter dementia progression. Both visuospatial and verbal abilities declined at similar rates across the three patient groups. DLB diagnosis may be improved, particularly when there is comorbid AD, by using domain-specific testing

Cortical–subcortical interactions in hypersomnia disorders: Mechanisms underlying cognitive and behavioral aspects of the sleep–wake cycle

Subcortical circuits mediating sleep–wake functions have been well characterized in animal models, and corroborated by more recent human studies. Disruptions in these circuits have been identified in hypersomnia disorders (HDs) such as narcolepsy and Kleine–Levin Syndrome, as well as in neurodegenerative disorders expressing excessive daytime sleepiness. However, the behavioral expression of sleep–wake functions is not a simple on-or-off state determined by subcortical circuits, but encompasses a complex range of behaviors determined by the interaction between cortical networks and subcortical circuits. While conceived as disorders of sleep, HDs are equally disorders of wake, representing a fundamental instability in neural state characterized by lapses of alertness during wake. These episodic lapses in alertness and wakefulness are also frequently seen in neurodegenerative disorders where electroencephalogram demonstrates abnormal function in cortical regions associated with cognitive fluctuations (CFs). Moreover, functional connectivity MRI shows instability of cortical networks in individuals with CFs. We propose that the inability to stabilize neural state due to disruptions in the sleep–wake control networks is common to the sleep and cognitive dysfunctions seen in hypersomnia and neurodegenerative disorders

Comparison of the Caregiving Experience of Grief, Burden, and Quality of Life in Dementia with Lewy Bodies, Alzheimer’s Disease, and Parkinson’s Disease Dementia

Background: Caregivers of persons living with Alzheimer’s disease (AD), dementia with Lewy bodies (DLB), and Parkinson’s disease dementia (PDD) are faced with numerous challenges. However, little is known about the caregiving experience across different dementias. Objective: The aims of this cross-sectional study were to examine the differences in the caregiver experience between DLB, PDD, and AD. Methods: Respondents were caregivers (N = 515; 384 DLB, 69 AD, 62 PDD) who completed a 230-question survey including sociodemographics, disease severity, neuropsychiatric symptoms, and measures of grief, burden, depression, quality of life, social support, well-being, care confidence, and mastery/self-efficacy. Results: There were no differences in caregiver age, sex, race, or education, or in the distribution of disease severity between diagnostic groups. Constructs were highly intercorrelated with positive attributes (caregiver QoL, care recipient QoL, social support, well-being, mastery and care confidence) being inversely correlated with negative attributes (burden, grief, and depression). Across dementia etiologies, no differences were reported for quality of life, social support, depression, well-being, psychological well-being, mastery, care confidence, burden or grief. Instead, we found that the caregiver’s experience was dependent on caregiver characteristics, person living with dementia characteristics and their most disturbing symptom, with behavior, personality changes, and sleep having the greatest effect on constructs. Conclusion: Caregiver ratings of psychosocial constructs may be more dependent on care recipient-caregiver dyad characteristics and the current symptoms than the underlying cause of those symptoms. Interventions to improve the caregiving experience should be developed to address specific psychosocial constructs rather than focusing on disease etiology or stage

Negotiating the Joint Career: Couples Adapting to Alzheimer's and Aging in Place

To understand the impact of memory loss on aging in place, this paper investigated dyads where one spouse had been diagnosed with memory loss. In-depth qualitative interviews were conducted with ten couples (N = 20). Grounded theory methods were used to collect, code, and analyze data into themes. Data revealed consensus among and between dyads that it was best to focus on living, rather than what had been or might someday be lost. Nonetheless, differences according to gender and cognitive status (e.g., diagnosed or spouse) were reported. Given population aging, identifying the impact of gender roles and social norms on the potential for aging in place with memory loss is critical. Community services and care practices must be sensitive to the ways that couples prioritized and organized their relationship prior to diagnosis in order to encourage positive patterns of care between couples, foster successful adaptation to changing needs, and support in-home arrangements as long as possible

Personality traits distinguishing dementia with Lewy bodies from Alzheimer disease

This is the publisher's version, also available electronically from http://www.neurology.org/content/68/22/1895.Objective: To identify personality traits that distinguish dementia with Lewy bodies (DLB) from Alzheimer disease (AD). Methods: We examined 290 participants enrolled in a longitudinal study (nondemented control = 34, DLB = 128, AD = 128) followed to autopsy. As part of the annual interview with the collateral source, the clinician asked about specific changes in personality, interests, and drives based on items from the Blessed Dementia Scale (BDS). Statistical analysis was performed using χ2 and Fisher exact tests. Factor analysis was performed to determine underlying structure and receiver operating characteristic curves assessed the ability for each of three derived factors to discriminate DLB from AD. Results: The sample was evaluated for a mean of 4.8 visits (range 1 to 14) with a mean age of 77.6 ± 9.9 years. The participants' cognitive status ranged from nondemented (Clinical Dementia Rating [CDR] 0) through all stages of dementia (CDR ≥ 0.5). Personality traits that distinguished DLB included diminished emotional responsiveness (p = 0.004), relinquishing hobbies (p = 0.01), growing apathy (p = 0.03), and purposeless hyperactivity (p = 0.003). Factor analyses of the BDS revealed a PASSIVE factor (diminished emotional responsiveness, relinquished hobbies, growing apathy, and purposeless hyperactivity) explaining 10.4% of variance and that DLB was more likely to manifest these personality traits than AD (p = 0.001). The PASSIVE factor discriminated DLB from AD (area under the curve = 0.61, 95% CI: 0.54 to 0.68, p = 0.006). Any change in personality is associated with the presence of visual hallucinations. Conclusions: Our results suggest that incorporating a brief, simple inventory of personality traits may improve the identification of individuals with dementia with Lewy bodies

Longitudinal Study of the Transition From Healthy Aging to Alzheimer Disease

BACKGROUND: Detection of the earliest cognitive changes signifying Alzheimer disease is difficult. OBJECTIVE: To model the cognitive decline in preclinical Alzheimer disease. DESIGN: Longitudinal archival study comparing individuals who became demented during follow-up and people who remained nondemented on each of 4 cognitive factors: global, verbal memory, visuospatial, and working memory. SETTING: Alzheimer Disease Research Center, Washington University School of Medicine, St Louis, Missouri. PARTICIPANTS: One hundred thirty-four individuals who became demented during follow-up and 310 who remained nondemented. MAIN OUTCOME MEASURES: Inflection point in longitudinal cognitive performance. RESULTS: The best-fitting model for each of the 4 factors in the stable group was linear, with a very slight downward trend on all but the Visuospatial factor. In contrast, a piecewise model with accelerated slope after a sharp inflection point provided the best fit for the group that progressed. The optimal inflection point for all 4 factors was prior to diagnosis of dementia: Global, 2 years; Verbal and Working Memory, 1 year; and Visuospatial, 3 years. These results were also obtained when data were limited to the subset (n = 44) with autopsy-confirmed Alzheimer disease. CONCLUSIONS: There is a sharp inflection point followed by accelerating decline in multiple domains of cognition, not just memory, in the preclinical period in Alzheimer disease when there is insufficient cognitive decline to warrant clinical diagnosis using conventional criteria. Early change was seen in tests of visuospatial ability, most of which were speeded. Research into early detection of cognitive disorders using only episodic memory tasks may not be sensitive to all of the early manifestations of disease

Overcoming the COVID-19 Pandemic for Dementia Research: Engaging Rural, Older, Racially and Ethnically Diverse Church Attendees in Remote Recruitment, Intervention and Assessment

Background: Access to cognitive screening in rural underserved communities is limited and was further diminished during the COVID-19 pandemic. We examined whether a telephone-based cognitive screening intervention would be effective in increasing ADRD knowledge, detecting the need for further cognitive evaluation, and making and tracking the results of referrals. Method: Using a dependent t-test design, older, largely African American and Afro-Caribbean participants completed a brief educational intervention, pre/post AD knowledge measure, and cognitive screening. Results: Sixty of 85 eligible individuals consented. Seventy-percent of the sample self-reported as African American, Haitian Creole, or Hispanic, and 75% were female, with an average age of 70. AD knowledge pre-post scores improved significantly (t (49) = −3.4, p \u3c .001). Of the 11 referred after positive cognitive screening, 72% completed follow-up with their provider. Five were newly diagnosed with dementia. Three reported no change in diagnosis or treatment. Ninety-percent consented to enrolling in a registry for future research. Conclusion: Remote engagement is feasible for recruiting, educating, and conducting cognitive screening with rural older adults during a pandemic

The muscle protein dysferlin accumulates in the Alzheimer brain

Dysferlin is a transmembrane protein that is highly expressed in muscle. Dysferlin mutations cause limb-girdle dystrophy type 2B, Miyoshi myopathy and distal anterior compartment myopathy. Dysferlin has also been described in neural tissue. We studied dysferlin distribution in the brains of patients with Alzheimer disease (AD) and controls. Twelve brains, staged using the Clinical Dementia Rating were examined: 9 AD cases (mean age: 85.9 years and mean disease duration: 8.9 years), and 3 age-matched controls (mean age: 87.5 years). Dysferlin is a cytoplasmic protein in the pyramidal neurons of normal and AD brains. In addition, there were dysferlin-positive dystrophic neurites within Aβ plaques in the AD brain, distinct from tau-positive neurites. Western blots of total brain protein (RIPA) and sequential extraction buffers (high salt, high salt/Triton X-100, SDS and formic acid) of increasing protein extraction strength were performed to examine solubility state. In RIPA fractions, dysferlin was seen as 230–272 kDa bands in normal and AD brains. In serial extractions, there was a shift of dysferlin from soluble phase in high salt/Triton X-100 to the more insoluble SDS fraction in AD. Dysferlin is a new protein described in the AD brain that accumulates in association with neuritic plaques. In muscle, dysferlin plays a role in the repair of muscle membrane damage. The accumulation of dysferlin in the AD brain may be related to the inability of neurons to repair damage due to Aβ deposits accumulating in the AD brain