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1.  The Epidemiology of Frontotemporal Dementia 
Frontotemporal dementia, a heterogeneous neurodegenerative disorder, is a common cause of young-onset dementia (i.e., dementia developing in midlife or earlier). The estimated point prevalence is 15–22/100,000, and incidence 2.7–4.1/100,000. Some 25% are late-life onset cases. Population studies show nearly equal distribution by gender, which contrasts with myriad clinical and neuropathology reports. FTD is frequently familial and hereditary; five genetic loci for causal mutations have been identified, all showing 100% penetrance. Non-genetic risk factors for are yet to be identified. FTD shows poor life expectancy but with survival comparable to that of Alzheimer disease. Recent progress includes the formulation of up-to-date diagnostic criteria for the behavioral and language variants, and the development of new and urgently needed instruments for monitoring and staging the illness. There is still need for descriptive populations studies, to fill gaps in our knowledge about minority groups and developing regions. More pressing, however, is the need for reliable physiologic markers for disease. There is a present imperative to develop a translational science to form the conduit for transferring neurobiological discoveries and insights from bench to bedside.
PMCID: PMC3932112  PMID: 23611343
2.  Hippocampal sclerosis dementia 
Dementia & neuropsychologia  2013;7(1):83-87.
To describe characteristics of hippocampal sclerosis dementia.
Convenience sample of Hippocampal sclerosis dementia (HSD) recruited from the Johns Hopkins University Brain Resource Center. Twenty-four cases with post-mortem pathological diagnosis of hippocampal sclerosis dementia were reviewed for clinical characterization.
The cases showed atrophy and neuronal loss localized to the hippocampus, amygdala and entorrhinal cortex. The majority (79.2%) had amnesia at illness onset, and many (54.2%) showed abnormal conduct and psychiatric disorder. Nearly 42% presented with an amnesic state, and 37.5% presented with amnesia plus abnormal conduct and psychiatric disorder. All eventually developed a behavioral or psychiatric disorder. Disorientation, executive dysfunction, aphasia, agnosia and apraxia were uncommon at onset. Alzheimer disease (AD) was the initial clinical diagnosis in 89% and the final clinical diagnosis in 75%. Diagnosis of frontotemporal dementia (FTD) was uncommon (seen in 8%).
HSD shows pathological characteristics of FTD and clinical features that mimic AD and overlap with FTD. The findings, placed in the context of earlier work, support the proposition that HSD belongs to the FTD family, where it may be identified as an amnesic variant.
PMCID: PMC3867983  PMID: 24363834
dementia classification; hippocampal sclerosis; frontotemporal dementia; neuropsychiatry
3.  Estimating severity of illness and disability in Frontotemporal Dementia: Preliminary analysis of the Dementia Disability Rating (DDR) 
Current measures of severity and disability do not stage or track the progression of disability in frontotemporal dementia (FTD) well. We investigated the reliability of the newly developed Dementia Disability Rating (DDR) in the measurement and staging of illness severity in FTD and dementia of the Alzheimer type (DAT).
Material/ Methods
We studied 48 consecutive patients of the Johns Hopkins FTD and Young-Onset Dementias Clinic, with diagnoses of DAT, FTD, vascular dementia and “other” cognitive disorder (CDNOS). Cases were scored on the CDR and DDR by three trained raters, based on neuropsychiatric examinations performed at first visit and other assessments performed within the preceding year. Consensus ratings were assigned in conference.
Inter-rater correlations of DDR sum of ranks scores for DAT ranged from 0.88 to 0.91, for FTD 0.89–0.96 and for CDNOS 0.85–0.97. Similar correlations were observed of the CDR sum of rank scores for DAT and FTD. Correlations of DDR summary scores for DAT were 0.67–0.91 and for FTD 0.79–0.91, as compared to CDR data: 0.87–0.92 (p<0.0001) and 0.80–0.93 (p<0.0001) for DAT and FTD respectively. In DAT patients the correlation between CDR and DDR summary scores was higher than in FTD patients, whereas correlations based on sum of ranks scores were high in both groups.
These preliminary data indicate the DDR measures disability in DAT and FTD, with reliability comparable to the CDR. Convergent validity was demonstrated for the DDR.
PMCID: PMC3903383  PMID: 24478794
4.  What Is the Life Expectancy in Frontotemporal Lobar Degeneration? 
Neuroepidemiology  2011;37(3-4):166-167.
PMCID: PMC3953732  PMID: 22056969
5.  Childless Elders in Assisted Living: Findings from the Maryland Assisted Living Study 
Journal of housing for the elderly  2013;27(1-2):206-220.
We compared data drawn from a random sample of 399 current assisted living (AL) residents and a subsample of 222 newly admitted residents for two groups: childless AL residents and AL residents with children. The percentage of childless AL residents (26%) in our study was slightly higher than US population estimates of childless persons age 65 and over (20%). In the overall sample, the two groups differed significantly by age, race and women’s years of education. The childless group was slightly younger, had a higher percentage of African American residents, and had more years of education than the group with children. In the subsample, we looked at demographic, functional, financial and social characteristics and found that compared to residents with children, fewer childless residents had a dementia diagnosis, received visits from a relative while more paid less money per month for AL and reported having private insurance. As childlessness among older adults continues to increase, it will become increasingly important to understand how child status affects the need for and experience of long-term care.
PMCID: PMC3977594  PMID: 24729653
childless; elderly; assisted living; long term care
6.  Management of Frontotemporal Dementia in Mental Health and Multidisciplinary Settings 
Diagnosis of frontotemporal dementia (FTD) in the mental health setting and issues pertaining to longitudinal care of this population in a specialty clinic are reviewed. FTD is often misdiagnosed as a psychiatric disorder, most commonly as a mood disorder. FTD has features that overlap with those of major depression, mania, obsessive-compulsive disorder and schizophrenia. We describe these features and how to differentiate FTD from these psychiatric disorders. This paper also describes practical issues in the management of FTD, specifically the issues that clinicians, patients and their families face in managing this disease. Areas of clinical care along the continuum are explored; FTD care involves collaborative management of symptoms and disability, and assisting patients and families in adapting to the disease.
PMCID: PMC3929950  PMID: 23611352
7.  Frontotemporal dementia and psychiatry 
PMCID: PMC3947598  PMID: 23611342
8.  12-month incidence, prevalence, persistence, and treatment of mental disorders among individuals recently admitted to assisted living facilities in Maryland 
To estimate the 12-month incidence, prevalence, and persistence of mental disorders among recently admitted assisted living (AL) residents and to describe the recognition and treatment of these disorders.
Two hundred recently admitted AL residents in 21 randomly selected AL facilities in Maryland received comprehensive physician-based cognitive and neuropsychiatric evaluations at baseline and 12 months later. An expert consensus panel adjudicated psychiatric diagnoses (using DSM-IV-TR criteria) and completeness of workup and treatment. Incidence, prevalence, and persistence were derived from the panel's assessment. Family and direct care staff recognition of mental disorders was also assessed.
At baseline, three-quarters suffered from a cognitive disorder (56% dementia, 19% Cognitive Disorders Not Otherwise Specified) and 15% from an active non-cognitive mental disorder. Twelve-month incidence rates for dementia and non-cognitive psychiatric disorders were 17% and 3% respectively, and persistence rates were 89% and 41% respectively. Staff recognition rates for persistent dementias increased over the 12-month period but 25% of cases were still unrecognized at 12 months. Treatment was complete at 12 months for 71% of persistent dementia cases and 43% of persistent non-cognitive psychiatric disorder cases.
Individuals recently admitted to AL are at high risk for having or developing mental disorders and a high proportion of cases, both persistent and incident, go unrecognized or untreated. Routine dementia and psychiatric screening and reassessment should be considered a standard care practice. Further study is needed to determine the longitudinal impact of psychiatric care on resident outcomes and use of facility resources.
PMCID: PMC3648853  PMID: 23290818
incidence; dementia; psychiatric disorder; treatment; recognition
9.  Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia 
Brain  2011;134(9):2456-2477.
Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, ‘possible’ behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). ‘Probable’ behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia ‘with definite frontotemporal lobar degeneration’ requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer’s disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86%) met ‘possible’ criteria, and 104 (76%) met criteria for ‘probable’ behavioural variant frontotemporal dementia. In contrast, 72 cases (53%) met previously established criteria for the syndrome (P < 0.001 for comparison with ‘possible’ and ‘probable’ criteria). Patients who failed to meet revised criteria were significantly older and most had atypical presentations with marked memory impairment. In conclusion, the revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotemporal lobar degeneration. Greater sensitivity of the proposed criteria may reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations. Future studies will be needed to establish the reliability and specificity of these revised diagnostic guidelines.
PMCID: PMC3170532  PMID: 21810890
behavioural variant frontotemporal dementia; diagnostic criteria; frontotemporal lobar degeneration; FTD; pathology
10.  The physical environment influences neuropsychiatric symptoms and other outcomes in assisted living residents 
Although the number of elderly residents living in assisted living (AL) facilities is rising, few studies have examined the AL physical environment and its impact on resident well-being. We sought to quantify the relationship of AL physical environment with resident outcomes including neuropsychiatric symptoms (NPS), quality of life (QOL), and fall risk, and to compare the effects for demented and non-demented residents.
Prospective cohort study of a stratified random sample of 326 AL residents living in 21 AL facilities. Measures included the Therapeutic Environmental Screening Scale for Nursing Homes and Residential Care (TESS-NH/RC) to rate facilities and in-person assessment of residents for diagnosis (and assessment of treatment) of dementia, ratings on standardized clinical, cognitive, and QOL measures. Regression models compared environmental measures with outcomes. TESS-NH/RC is modified into a scale for rating the AL physical environment AL-EQS.
The AL Environmental Quality Score (AL-EQS) was strongly negatively associated with Neuropsychiatric Inventory (NPI) total score (p <0.001), positively associated with Alzheimer Disease Related Quality of Life (ADRQL) score (p = 0.010), and negatively correlated with fall risk (p = 0.042). Factor analysis revealed an excellent two-factor solution, Dignity and Sensory. Both were strongly associated with NPI and associated with ADRQL.
The physical environment of AL facilities likely affects NPS and QOL in AL residents, and the effect may be stronger for residents without dementia than for residents with dementia. Environmental manipulations that increase resident privacy, as well as implementing call buttons and telephones, may improve resident well-being.
PMCID: PMC3143504  PMID: 20077498
physical environment; neuropsychiatric symptoms; assisted living; behavior; dementia
11.  Correlates of Functional Dependence among Recently-Admitted Assisted Living Residents with and without Dementia 
To describe and contrast functional dependency (FD) levels among recently-admitted AL residents with and without dementia and to assess the differential contribution of cognitive, behavioral, medical, and social factors on FD within each group.
A cross-sectional study.
A random sample of 28 AL facilities in the Central Maryland region.
Two hundred and sixty-two AL residents assessed <1 year after admission.
Participants were given comprehensive in-person dementia assessments. Cognitive, behavioral, medical, and social factors were also assessed. FD was operationalized as impairment in activities of daily living.
The fifty-nine percent of residents with dementia had higher levels of FD (p<0.001) and were more likely to require assistance in all assessed task-specific ADL domains (p<0.001) except mobility (p=0.653). In multivariate models, global cognition, medical health status, and presence of diabetes explained 43% of the variance in FD in the dementia group. Twenty-five percent of the variance in FD was explained by depression, neuropsychiatric symptoms, and global cognition in those without dementia.
Recently-admitted AL residents have substantial levels of FD. FD is higher among residents with dementia compared to those without and the association of cognitive, mental health, and medical variables with FD differ as a function of dementia status. Future research should examine how these dimensions affect FD longitudinally and whether they may serve as targets for interventions and quality of care improvement initiatives.
PMCID: PMC2746023  PMID: 19497544
disability; cognitive impairment; long-term care; assisted living
12.  Personality disorder traits as predictors of subsequent first-onset panic disorder or agoraphobia 
Comprehensive psychiatry  2008;50(3):209-214.
Determining how personality disorder traits and panic disorder and/or agoraphobia relate longitudinally is an important step in developing a comprehensive understanding of the etiology of panic/agoraphobia. In 1981, a probabilistic sample of adult (≥ 18 years old) residents of east Baltimore were assessed for Axis I symptoms and disorders using the Diagnostic Interview Schedule (DIS); psychiatrists re-evaluated a sub-sample of these participants and made Axis I diagnoses, as well as ratings of individual DSM-III personality disorder traits. Of the participants psychiatrists examined in 1981, 432 were assessed again in 1993–1996 using the DIS. Excluding participants who had baseline panic attacks or panic-like spells from the risk groups, baseline timidity (avoidant, dependent, and related traits) predicted first-onset DIS panic disorder or agoraphobia over the follow-up period. These results suggest that avoidant and dependent personality traits are predisposing factors, or at least markers of risk, for panic disorder and agoraphobia - not simply epiphenomena.
PMCID: PMC2691589  PMID: 19374963
15.  The Association between Activity Participation and Time to Discharge in the Assisted Living Setting 
Given the recent evidence that activity participation may reduce cognitive or functional decline, the effect of activity on residents’ ability to remain in assisted living (AL) is of interest. We examined the relationship between participation in activities and the length of time residents remain in AL.
The data reported here were gathered in the initial phase of the Maryland Assisted Living Study (MDAL), an epidemiologic study of dementia and other psychiatric disorders in AL. A stratified, random sample of 198 residents of 22 AL facilities in central Maryland were evaluated using a number of cognitive, behavioral, general health, and functional assessments. The total amount of time each resident spent in group and solitary activity in the prior month was recorded. The dependent variable, time to discharge (TTD), was the length of time between the resident’s evaluation by the study team and the time at which the resident died, or was discharged or administratively censored.
Levels of activity participation at baseline were associated with longer TTD in an univariate Cox proportional hazards model. After adjusting for MMSE, GMHR, and the PGDRS mobility score in the multivariate model, activity participation remained associated with longer TTD, albeit with marginal statistical significance (p=0.057).
Higher activity participation was associated with longer TTD in AL. This suggests that engagement in activities may delay functional decline or the perception of decline observed by caregivers in AL. Further research is needed to replicate and understand this finding.
PMCID: PMC2650476  PMID: 18044814
Activity participation; assisted living; psychiatric disorders; dementia and activities

Results 1-15 (15)