Late-Onset Alcohol Abuse as a Presenting Symptom of Neurodegenerative Diseases.

BACKGROUND: The association between lifetime alcohol abuse and a higher risk to develop dementia is well known. However, it is unknown whether older adults who begin abusing alcohol late in life have an underlying neurodegenerative disease. OBJECTIVE: Identify the frequency of lifelong alcohol abuse (L-AA), late-onset alcohol abuse (LO-AA), and alcohol abuse as a first symptom of dementia (AA-FS) in patients with neurodegenerative diseases. METHODS: Cross-sectional retrospective study of patients evaluated at an academic referral center with a clinical diagnosis of behavioral variant frontotemporal dementia (bvFTD), Alzheimer-type dementia (AD), and semantic variant primary progressive aphasia (svPPA) (n = 1,518). The presence of alcohol abuse was screened with the National Alzheimer's Coordinating Center questionnaire. L-AA was defined as onset < 40 years, LO-AA as onset ≥40 years, and AA-FS was defined when the abuse started within the first three years from symptom onset. RESULTS: The frequency of LO-AA was 2.2% (n = 33/1,518). LO-AA was significantly more frequent in patients with bvFTD than AD (7.5%, n = 13/173 versus 1.3%, n = 16/1,254, CI:1.0;11.4%), but not svPPA (4.4%, n = 4/91, CI: -4.4;10.7%). Similarly, AA-FS was more frequent in bvFTD patients than AD (5.7%, n = 10/173 versus 0.7%, n = 9/1,254, CI:0.5%;9.5%), but not svPPA (2.2%, n = 2/91, CI:-2.4;9.1%). CONCLUSION: LO-AA can be a presenting symptom of dementia, especially bvFTD. Alcohol abuse onset later in life should prompt a clinical investigation into the possibility of an underlying neurodegenerative process because delay in diagnosis and treatment may increase patient and caregiver burden. The results need to be interpreted with caution due to the limitations of the study.

Relationship Turmoil and Emotional Empathy in Frontotemporal Dementia.

BACKGROUND: Behavioral variant frontotemporal dementia (bvFTD) is characterized by marked deficits in empathy and social behavior; however, the effect of these symptoms on partner relationships has not been quantitatively measured. OBJECTIVE: We aimed to determine the effect of empathy loss and behavioral symptoms on partner and familial relationship status in bvFTD. We ascertained whether patients were currently in marriage/partner relationships or were separated/divorced, the timing and duration of these relationships, and whether the patients had relationship infidelity. We investigated the relationship status of 483 patients (156 with bvFTD, 38 with nonfluent variant primary progressive aphasia, 72 with semantic variant primary progressive aphasia, 49 with corticobasal syndrome, 45 with progressive supranuclear palsy syndrome, and 123 with Alzheimer disease) over the course of follow-up, and correlated relationship status with patients' first visit Interpersonal Reactivity Index and Neuropsychiatric Inventory. RESULTS: Relationship dissolution and infidelity were significantly more frequent among patients with bvFTD than in the other groups. Across all patients, empathy loss was associated with relationship dissolution. In the bvFTD group, patients who experienced relationship dissolution or infidelity had significantly lower empathy than those who did not. CONCLUSIONS: Changes in relationship status differed across dementia groups and were associated with empathy decline.

Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology.

Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

Psychiatric symptom burden in older people living with HIV with and without cognitive impairment: the UCSF HIV over 60 cohort study.

Psychiatric comorbidities are common in people living with HIV (PLWH) and adversely affect life satisfaction, treatment adherence and disease progression. There are few data to inform the burden of psychiatric symptoms in older PLWH, a rapidly growing demographic in the U.S. We performed a cross-sectional analysis to understand the degree to which symptom burden was associated with cognitive disorders in PLWH over age 60. Participants completed a standardized neuropsychological battery and were assigned cognitive diagnoses using Frascati criteria. We captured psychiatric symptom burden using the Geriatric Depression Scale (GDS) and proxy-informed Neuropsychiatric Inventory-Questionnaire (NPI-Q). Those diagnosed with HIV-associated neurocognitive disorders (HAND, n = 39) were similar to those without HAND (n = 35) by age (median = 67 years for each group, p = 0.696), education (mean = 16 years vs. 17 years, p = 0.096), CD4+ T-lymphocyte counts (mean = 520 vs. 579, p = 0.240), duration of HIV (median = 21 years for each group, p = 0.911) and sex (92% male in HAND vs. 97% in non-HAND, p = 0.617). Our findings showed similarities in HAND and non-HAND groups on both NPI-Q (items and clusters) and GDS scores. However, there was a greater overall symptom burden in HIV compared to healthy elder controls (n = 236, p < 0.05), with more frequent agitation, depression, anxiety, apathy, irritability and nighttime behavior disturbances (p < 0.05). Our findings demonstrate no differences in psychiatric comorbidity by HAND status in older HIV participants; but confirm a substantial neurobehavioral burden in this older HIV-infected population.

Distinct Subtypes of Behavioral Variant Frontotemporal Dementia Based on Patterns of Network Degeneration.

IMPORTANCE: Clearer delineation of the phenotypic heterogeneity within behavioral variant frontotemporal dementia (bvFTD) will help uncover underlying biological mechanisms and improve clinicians' ability to predict disease course and to design targeted management strategies. OBJECTIVE: To identify subtypes of bvFTD syndrome based on distinctive patterns of atrophy defined by selective vulnerability of specific functional networks targeted in bvFTD using statistical classification approaches. DESIGN, SETTING AND PARTICIPANTS: In this retrospective observational study, 90 patients meeting the Frontotemporal Dementia Consortium consensus criteria for bvFTD underwent evaluation at the Memory and Aging Center of the Department of Neurology at University of California, San Francisco. Patients underwent a multidisciplinary clinical evaluation, including clinical demographics, genetic testing, symptom evaluation, neurologic examination, neuropsychological bedside testing, and socioemotional assessments. All patients underwent structural magnetic resonance imaging at their earliest evaluation at the memory clinic. From each patient's structural imaging scans, the mean volumes of 18 regions of interest (ROI) constituting the functional networks specifically vulnerable in bvFTD, including the salience network (SN), with key nodes in the frontoinsula and pregenual anterior cingulate, and the semantic appraisal network (SAN), anchored in the anterior temporal lobe and subgenual cingulate, were estimated. Principal component and cluster analyses of ROI volumes were used to identify patient clusters with anatomically distinct atrophy patterns. Data were collected from from June 19, 2002, to January 13, 2015. MAIN OUTCOMES AND MEASURES: Evaluation of brain morphology and other clinical features, including presenting symptoms, neurologic examination signs, neuropsychological performance, rate of dementia progression, and socioemotional function, in each patient cluster. RESULTS: Ninety patients (54 men [60%]; 36 women [40%]; mean [SD] age at evaluation, 55.1 [9.7] years) were included in the analysis. Four subgroups of patients with bvFTD with distinct anatomic patterns of network degeneration were identified, including 2 salience network-predominant subgroups (frontal/temporal [SN-FT] and frontal [SN-F]), a semantic appraisal network-predominant group (SAN), and a subcortical-predominant group. Subgroups demonstrated distinct patterns of cognitive, socioemotional, and motor symptoms, as well as genetic compositions and estimated rates of disease progression. CONCLUSIONS AND RELEVANCE: Divergent patterns of vulnerability in specific functional network components make an important contribution to the clinical heterogeneity of bvFTD. The data-driven anatomic classification identifies biologically meaningful anatomic phenotypes and provides a replicable approach to disambiguate the bvFTD syndrome.

Non-pharmacological Management of Behavioral Symptoms in Frontotemporal and Other Dementias.

Worldwide prevalence of dementia is predicted to double every 20 years. The most common cause in individuals over 65 is Alzheimer's disease (AD), but in those under 65, frontotemporal dementia (FTD) is as frequent. The physical and cognitive decline that characterizes these diseases is commonly accompanied by troublesome behavioral symptoms. These behavioral symptoms contribute to significant morbidity and mortality among both patients and caregivers. Medications have been largely ineffective in managing these symptoms and carry significant adverse effects. Non-pharmacological interventions have been recommended to precede the utilization of pharmacological treatments. This article reviews the research about these interventions with special attention to the variations by etiology, especially FTD. The authors offer recommendations for improving utilization of these strategies and future research recommendations.

Non-pharmacological Management of Behavioral Symptoms in Frontotemporal and Other Dementias.

(Reprinted with permission from Current Neurology and Neuroscience Reports 2016; 16:14).

Neuropsychiatric Symptoms Predict Functional Status in Alzheimer's Disease.

BACKGROUND: Cognitive deficits are presumed to be the primary driver of functional impairment in Alzheimer's disease (AD); however, functional impairment is likely multifactorially determined. OBJECTIVE: Our objective was to determine the relative contribution of neuropsychiatric symptoms in predicting ratings of functional status. METHODS: A total of 223 patients received routine neurological and neuropsychological evaluations and met criteria of probable AD dementia based on the McKhann criteria. Demographic, cognitive, and neuropsychiatric variables were entered in a hierarchical linear regression analysis to predict functional status as measured by the Functional Activities Questionnaire (FAQ). RESULTS: The total model explained 29.7% of the variance (p <  0.001) in FAQ. Importantly, neuropsychiatric variables explained 12.7% of the unique variance, with apathy and sleep as significant contributors. CONCLUSION: Two neuropsychiatric variables, apathy and changes in sleep/nighttime behaviors, predicted ratings of functional status in AD patients independent of age, global cognition, memory and executive function measures, and depressive symptoms. These results highlight the importance of neuropsychiatric symptoms in understanding and potentially treating the functional limitations so prevalent in AD.

Verbal creativity in semantic variant primary progressive aphasia.

Emergence of visual and musical creativity in the setting of neurologic disease has been reported in patients with semantic variant primary progressive aphasia (svPPA), also called semantic dementia (SD). It is hypothesized that loss of left anterior frontotemporal function facilitates activity of the right posterior hemispheric structures, leading to de novo creativity observed in visual artistic representation. We describe creativity in the verbal domain, for the first time, in three patients with svPPA. Clinical presentations are carefully described in three svPPA patients exhibiting verbal creativity, including neuropsychology, neurologic exam, and structural magnetic resonance imaging (MRI). Voxel-based morphometry (VBM) was performed to quantify brain atrophy patterns in these patients against age-matched healthy controls. All three patients displayed new-onset creative writing behavior and produced extensive original work during the course of disease. Patient A developed interest in wordplay and generated a large volume of poetry. Patient B became fascinated with rhyming and punning. Patient C wrote and published a lifestyle guidebook. An overlap of their structural MR scans showed uniform sparing in the lateral portions of the language-dominant temporal lobe (superior and middle gyri) and atrophy in the medial temporal cortex (amygdala, limbic cortex). New-onset creativity in svPPA may represent a paradoxical functional facilitation. A similar drive for production is found in visually artistic and verbally creative patients. Mirroring the imaging findings in visually artistic patients, verbal preoccupation and creativity may be associated with medial atrophy in the language-dominant temporal lobe, but sparing of lateral dominant temporal and non-dominant posterior cortices.

Implementation of advanced practice nurse clinic for management of behavioral symptoms in dementia: a dyadic intervention (innovative practice).

Behavioral symptoms are common in all types of dementia and often result in significant caregiver stress and illness, institutionalization of the patient, and reduced quality of life for the patient and caregiver. Health care practitioners often lack the expertise or time to adequately assess behavioral symptoms or counsel caregivers about interventions. Our goal was to implement a specialty clinic managed by advanced practice nurses to assess and manage behavioral symptoms associated with dementia. The clinic evaluations consisted of an assessment of the patient by the Nurse Practitioner during the time that the family caregiver(s) was interviewed by the Clinical Nurse Specialist and focused on an assessment of the cognitive and functional abilities of the patient, identification of triggers for the problematic behaviors, and assessment of caregiver coping. We evaluated 66 dyads since implementation in February 2010. The patients were primarily female, Caucasian, 74.3 years of age with Alzheimer's disease. The majority of caregivers were spouses (n = 44) followed by adult children (n = 20) and then siblings (n = 2). Targeted interventions were developed and caregiver counseling, support, and education were an integral part of the consultation and included written information, video instruction, and internet resources. Evaluations indicated caregivers and referring providers found the appointment helpful in managing behavioral symptoms and caregiver stress.

Characterization of apathy in persons with frontotemporal dementia and the impact on family caregivers.

This study characterized daytime activity and apathy in patients with behavioral variant frontotemporal dementia (bvFTD) and semantic dementia (SD) and their family caregivers. Twenty-two patient-caregiver dyads were enrolled: 13 bvFTD and 9 SD. Data were collected on behavior and movement. Patients and caregivers wore Actiwatches for 2 weeks to record activity. We predicted that bvFTD patients would show greater caregiver report of apathy and less daytime activity compared with patients diagnosed with SD. Patients with bvFTD spent 25% of their day immobile, whereas patients with SD spent 16% of their day inactive. BvFTD caregivers spent 11% of their day immobile and SD caregivers were immobile 9% of their day. Apathy was present in all of the patients with bvFTD and in all but one patient with SD; the severity of apathy was greater in bvFTD compared with SD. Apathy correlated with caregiver emotional distress in both groups. In conclusion, apathy has been defined as a condition of diminished motivation that is difficult to operationalize. Among patients with frontotemporal dementia, apathy was associated with lower levels of activity, greater number of bouts of immobility, and longer immobility bout duration. Apathy and diminished daytime activity appeared to have an impact on the caregiver. Objective measures of behavioral output may help in formulation of a more precise definition of apathy.

Trajectories of behavioral disturbance in dementia.

Predicting the progression of dementia is a challenge for clinicians yet this information is highly valued by patients' families. An informally observed 4-stage model of dementia can be helpful in educating caregivers and preparing them for what lies ahead. In the behavioral variant of frontotemporal dementia (bvFTD), this model describes the evolution of behavioral disturbances and is characterized by an inflection point between stage 2 (progressively severe behavioral aberration) and stage 3 (increasing apathy and remission of behavior problems). In this study, we sought evidence for this model using a database of serial Neuropsychiatric Inventory (NPI) scores for 45 patients with FTD and 47 patients with Alzheimer's disease (AD). We transformed the NPI scores into a single variable for each participant that represented the yearly rate of change in total NPI score and used this as the dependent variable in a multivariate linear regression. Age at onset of dementia, NPI score at initial visit, and duration of illness at first NPI all contributed significantly to the regression model in the bvFTD group. Participants with an initial NPI acquired before 6 years of disease duration tended to have a more positive rate of change in NPI total score (representing worsening behavioral disturbances) than those with an initial NPI performed after 6 years. None of the aforementioned variables were significantly associated with yearly change in NPI total score in the AD group. These results support a crescendo-decrescendo trajectory of behavioral symptoms in bvFTD but do not suggest that there is a similar pattern in AD, and further longitudinal data collection is necessary.

Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion.

BACKGROUND: Some patients meeting behavioural variant frontotemporal dementia (bvFTD) diagnostic criteria progress slowly and plateau at mild symptom severity. Such patients have mild neuropsychological and functional impairments, lack characteristic bvFTD brain atrophy and have thus been referred to as bvFTD 'phenocopies' or slowly progressive (bvFTD-SP). The few patients with bvFTD-SP that have been studied at autopsy have demonstrated no evidence of FTD pathology, suggesting that bvFTD-SP is neuropathologically distinct from other forms of FTD. Here, two patients with bvFTD-SP with chromosome 9 open reading frame 72 (C9ORF72) hexanucleotide expansions are described. METHODS: 384 patients with an FTD clinical spectrum and Alzheimer's disease diagnoses were screened for C9ORF72 expansion. Two bvFTD-SP mutation carriers were identified. Neuropsychological and functional data, as well as brain atrophy patterns, assessed using voxel based morphometry (VBM), were compared with 44 patients with sporadic bvFTD and 85 healthy controls. RESULTS: Both patients were aged 48 years at baseline and met possible bvFTD criteria. In the first patient, VBM revealed thalamic and posterior insula atrophy. Over 7 years, his neuropsychological performance and brain atrophy remained stable. In the second patient, VBM revealed cortical atrophy with subtle frontal and insular volume loss. Over 2 years, her neuropsychological and functional scores as well as brain atrophy remained stable. CONCLUSIONS: C9ORF72 mutations can present with a bvFTD-SP phenotype. Some bvFTD-SP patients may have neurodegenerative pathology, and C9ORF72 mutations should be considered in patients with bvFTD-SP and a family history of dementia or motor neuron disease.

Comprehension of insincere communication in neurodegenerative disease: lies, sarcasm, and theory of mind.

Comprehension of insincere communication is an important aspect of social cognition requiring visual perspective taking, emotion reading, and understanding others' thoughts, opinions, and intentions. Someone who is lying intends to hide their insincerity from the listener, while a sarcastic speaker wants the listener to recognize they are speaking insincerely. We investigated whether face-to-face testing of comprehending insincere communication would effectively discriminate among neurodegenerative disease patients with different patterns of real-life social deficits. We examined ability to comprehend lies and sarcasm from a third-person perspective, using contextual cues, in 102 patients with one of four neurodegenerative diseases (behavioral variant frontotemporal dementia [bvFTD], Alzheimer's disease [AD], progressive supranuclear palsy [PSP], and vascular cognitive impairment) and 77 healthy older adults (normal controls--NCs). Participants answered questions about videos depicting social interactions involving deceptive, sarcastic, or sincere speech using The Awareness of Social Inference Test. All subjects equally understood sincere remarks, but bvFTD patients displayed impaired comprehension of lies and sarcasm compared with NCs. In other groups, impairment was not disease-specific but was proportionate to general cognitive impairment. Analysis of the task components revealed that only bvFTD patients were impaired on perspective taking and emotion reading elements and that both bvFTD and PSP patients had impaired ability to represent others' opinions and intentions (i.e., theory of mind). Test performance correlated with informants' ratings of subjects' empathy, perspective taking and neuropsychiatric symptoms in everyday life. Comprehending insincere communication is complex and requires multiple cognitive and emotional processes vulnerable across neurodegenerative diseases. However, bvFTD patients show uniquely focal and severe impairments at every level of theory of mind and emotion reading, leading to an inability to identify obvious examples of deception and sarcasm. This is consistent with studies suggesting this disease targets a specific neural network necessary for perceiving social salience and predicting negative social outcomes.

The experience of caregiving: differences between behavioral variant of frontotemporal dementia and Alzheimer disease.

OBJECTIVE: To examine caregiver strain, depression, perceived sense of control, and distress from patient neuropsychiatric symptoms in family caregivers of persons with Alzheimer disease (AD) and behavioral variant of frontotemporal dementia (bvFTD) and determine whether group differences exist. METHODS: Family caregivers were recruited from the Memory and Aging Center in San Francisco, California. Analyses of cross-sectional data on 53 family caregivers (AD = 31, bvFTD = 22) were performed. The Mann-Whitney U test was used to contrast groups. RESULTS: There were statistically significant differences between the AD and bvFTD caregivers in strain, distress, and perceived control but not in depression. On average, bvFTD caregivers experienced greater strain and distress, more depressive symptoms, and lower perceived control. CONCLUSIONS: Findings support that experiences of AD and bvFTD caregivers may differ. Further study is needed to identify possible explanatory factors for these group differences.

Neuropsychological correlates of dominance, warmth, and extraversion in neurodegenerative disease.

INTRODUCTION: Changes in personality differ qualitatively and quantitatively among patients with different neurodegenerative diseases, likely due to divergent patterns of regional neurodegeneration. Regional damage to circuits underlying various cognitive and emotional functions have been associated with interpersonal traits like dominance, extraversion, and warmth in patients with neurodegenerative diseases, suggesting that personality may in part be mediated by these more basic neuropsychological functions. In this study, we hypothesized that different combinations of cognitive, neuropsychiatric, and emotional measures would predict different interpersonal traits in patients with neurodegenerative diseases. METHODS: A battery of cognitive, neuropsychiatric, and emotional measures was administered to 286 patients with various neurodegenerative diseases such as Alzheimer's disease, behavioral variant frontotemporal dementia, semantic dementia, and progressive supranuclear palsy, and informants described patients' dominance, extraversion, and warmth using the Interpersonal Adjective Scales (IAS) personality questionnaire. Regression modeling was performed to identify which neuropsychological factors uniquely predicted current personality, controlling for age, gender, and premorbid personality. RESULTS: Social dominance covaried with patients' capacity for cognitive control and verbal fluency. Conversely, warmth did not rely on these executive or verbal skills, but covaried primarily with patients' capacity for emotional responsiveness. Extraversion, representing a blend of dominance and warmth, demonstrated an intermediate degree of relationship to both executive/verbal and emotional functions. CONCLUSIONS: These findings suggest that different personality traits are partly subserved by specific cognitive and emotional functions in neurodegenerative disease patients. While this study was performed in the context of brain damage, the results raise the question of whether individual differences in these neuropsychological abilities may also underlie variability in normal personality.

Let's inhibit our excitement: the relationships between Stroop, behavioral disinhibition, and the frontal lobes.

OBJECTIVE: The Stroop (Stroop, 1935) is a frequently used neuropsychological test, with poor performance typically interpreted as indicative of disinhibition and frontal lobe damage. This study tested those interpretations by examining relationships between Stroop performance, behavioral disinhibition, and frontal lobe atrophy. METHOD: Participants were 112 patients with mild cognitive impairment or dementia, recruited through UCSF's Memory and Aging Center. Participants received comprehensive dementia evaluations including structural MRI, neuropsychological testing, and informant interviews. Freesurfer, a semiautomated parcellation program, was used to analyze 1.5T MRI scans. Behavioral disinhibition was measured using the Neuropsychiatric Inventory (Cummings, 1997; Cummings et al., 1994) Disinhibition Scale. The sample (n = 112) mean age was 65.40 (SD = 8.60) years, education was 16.64 (SD = 2.54) years, and Mini-Mental State Examination (MMSE; Folstein et al., 1975) was 26.63 (SD = 3.32). Hierarchical linear regressions were used for data analysis. RESULTS: Controlling for age, MMSE, and color naming, Stroop performance was not significantly associated with disinhibition (ß = 0.01, ΔR² = 0.01, p = .29). Hierarchical regressions controlling for age, MMSE, color naming, intracranial volume, and temporal and parietal lobes, examined whether left or right hemisphere regions predict Stroop performance. Bilaterally, parietal lobe atrophy best predicted poorer Stroop (left: ß = 0.0004, ΔR² = 0.02, p = .002; right: ß = 0.0004, ΔR² = 0.02, p = .002). Of frontal regions, only dorsolateral prefrontal cortex atrophy predicted poorer Stroop (ß = 0.001, ΔR² = 0.01, p = .03); left and right anterior cingulate cortex atrophy predicted better Stroop (left: ß = -0.003, ΔR² = 0.01, p = .02; right: ß = -0.004, ΔR² = 0.01, p = .02). CONCLUSION: These findings suggest Stroop performance is a poor measure of behavioral disinhibition and frontal lobe atrophy even among a relatively high-risk population.

Interpersonal traits change as a function of disease type and severity in degenerative brain diseases.

BACKGROUND: Different degenerative brain diseases result in distinct personality changes as a result of divergent patterns of brain damage; however, little is known about the natural history of these personality changes throughout the course of each disease. OBJECTIVE: To investigate how interpersonal traits change as a function of degenerative brain disease type and severity. METHODS: Using the Interpersonal Adjective Scales, informant ratings of retrospective premorbid and current scores for dominance, extraversion, warmth and ingenuousness were collected annually for 1 to 4 years on 188 patients (67 behavioural variant frontotemporal dementia (bvFTD), 40 semantic dementia (SemD), 81 Alzheimer's disease (AD)) and 65 older healthy controls. Using random coefficient models, interpersonal behaviour scores at very mild, mild or moderate-to-severe disease stages were compared within and between patient groups. RESULTS: Group-level changes from premorbid personality occurred as a function of disease type and severity, and were apparent even at a very mild disease stage (Clinical Dementia Rating=0.5) for all three diseases. Decreases in interpersonal traits were associated with emotional affiliation (ie, extraversion, warmth and ingenuousness) and more rigid interpersonal behaviour differentiated bvFTD and SemD patients from AD patients. CONCLUSIONS: Specific changes in affiliative interpersonal traits differentiate degenerative brain diseases even at a very mild disease stage, and patterns of personality change differ across bvFTD, SemD and AD with advancing disease. This study describes the typical progression of change of interpersonal traits in each disease, improving the ability of clinicians and caregivers to predict and plan for symptom progression.

Advanced practice nursing: meeting the caregiving challenges for families of persons with frontotemporal dementia.

Frontotemporal dementia (FTD), once thought to be a rare cause for dementia, is now acknowledged to be the most common presenile (before age 65 years) cause of dementia. Frontotemporal dementia is associated with profound changes in behavior, personality, emotions, and cognition. The purpose of this article is to describe 2 cases of patients with FTD to illustrate salient aspects of the caregiving experience. Issues faced by caregivers are organized into 6 categories: diagnosis, behavioral symptoms, function, communication, long-term management and care, and maintenance of the caregiver's emotional and physical health. Examples of interventions directed by advanced practice nurses are described. We suggest that management of FTD requires expertise as scientific advances and discoveries about FTD continually change the landscape of care.