Jennifer Merrilees

An open-label study of memantine treatment in 3 subtypes of frontotemporal lobar degeneration

There are currently no Food and Drug Administration-approved treatments for frontotemporal lobar degeneration (FTLD). The objectives of this study were to explore the tolerability of memantine treatment in FTLD and to monitor for possible effects on behavior, cognition, and function. Forty-three individuals who met clinical criteria for FTLD [21 with frontotemporal dementia (FTD), 13 with semantic dementia (SD), and 9 with progressive nonfluent aphasia (PA)] received 26 weeks of open-label treatment with memantine at a target dose of 20 mg daily. Concurrent treatment with acetylcholinesterase inhibitors was prohibited. Cognitive and functional outcome measures included the Mini Mental State Examination, Alzheimers Disease Assessment Scale-Cognitive (ADAS-cog), clinical dementia rating-sum of boxes, Neuropsychiatric Inventory (NPI), Frontal Behavior Inventory, Executive Interview (EXIT25), Texas Functional Living Scale (TFLS), Geriatric Depression Scale, and Unified Parkinsons Disease Rating Scale-motor scale. Most subjects were able to tolerate the target dose of memantine. A transient improvement was observed on the total NPI score primarily in the FTD group. Variable declines were observed on the ADAS-cog, EXIT25, Frontal Behavior Inventory, NPI, TFLS, and UPDRS scores. The FTD and SD groups declined on most of the cognitive and behavioral outcome measures, but remained stable on the UPDRS, whereas the progressive nonfluent aphasia group remained relatively stable on the ADAS-cog, NPI, and TFLS, but declined on the UPDRS. Memantine was well-tolerated in these subjects. Future placebo-controlled trials of memantine in FTLD are warranted and may have greater power to detect behavioral and cognitive effects if focused on the FTD and SD clinical syndromes.

Cognitive and behavioral challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis

Abstract Frontotemporal dementia (FTD) is a progressive neurological condition caused by degeneration of the frontal and/or anterior temporal lobes resulting in personality, behavioral, and cognitive changes. Amyotrophic lateral sclerosis (ALS) is caused by degeneration of lower motor and pyramidal neurons, leading to loss of voluntary muscle movement. The common molecular pathological and anatomical overlaps between FTD and ALS suggest that the two disorders are strongly linked. In some patients FTD precedes ALS; in others ALS occurs first, while in still others the two disorders begin simultaneously. The association between ALS and FTD creates unique challenges for family caregivers. This paper provides a guide for healthcare providers caring for patients with FTD-ALS exhibiting behavioral, cognitive, and emotional symptoms. Strategies are suggested to help minimize the impact of negative symptoms.

A model for management of behavioral symptoms in frontotemporal lobar degeneration.

Frontotemporal lobar degeneration is a neurologic condition that manifests profound behavioral, personality, and language symptoms. These changes, often unique to each person, may include social misconduct, eating disturbances, apathy, emotional blunting, childlike behavior, habits and rituals, executive dysfunction, and marked difficulty with speech and language. These symptoms occur as the result of cell loss in discrete areas of the brain, the frontal and/or anterior temporal lobes. Little is known about effective management of the behavioral symptoms that result from this disease. The purpose of this paper is to describe a model that could be used in the assessment and potential management of frontotemporal lobar degeneration behavioral symptoms. Suggestions for interventions are given for some of the more common FTD behaviors. The impact of behavioral symptoms on the family caregiver is discussed along with strategies for supportive services and recommendations for future study.

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.

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 caregivers 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.

Sleep in Persons With Frontotemporal Dementia and Their Family Caregivers

Background:Dementia is associated with disruptions in sleep and sleep quality for patients and their family caregivers. Little is known about the impact of frontotemporal dementia (FTD) on sleep. Objective:The purpose of this study was to characterize sleep in patients with FTD and their family caregivers. Methods:Twenty-two patient–caregiver dyads were enrolled: Thirteen behavioral variant FTD (bvFTD) and nine semantic dementia (SD). Sleep and sleep quality data were collected for 2 weeks using diaries and Actiwatches. Results:Patients with bvFTD and SD spent more time in bed at night compared to their caregivers. Nighttime behaviors were reported more frequently by caregivers for the bvFTD patients and strongly correlated with caregiver distress. Actigraphy data showed normal sleep efficiency and timing of the nighttime sleep period for both patients and their caregivers. Caregivers of patients with bvFTD reported poorer sleep quality compared to the SD caregivers. A greater number of bvFTD caregivers compared to SD reported negative aspects of sleep quality for themselves and used sleep medications more frequently. Conclusion:The clinical manifestations of bvFTD appear to be associated with different and more distressing impacts on the caregiver sleep quality than SD.

Life enhancing activities for family caregivers of people with frontotemporal dementia.

Aberrant psychological and behavioral symptoms are common in patients with dementia. These symptoms have negative consequences for family caregivers, causing stress and burden. Frontotemporal dementia (FTD) symptoms cause more pronounced stress and burden on caregivers than those associated with Alzheimer dementia. In this randomized, attention control pilot study, we delivered 5-weekly, one-on-one, positive affect intervention sessions to family caregivers of people with FTD. The program, Life Enhancing Activities for Family Caregivers: LEAF was conducted in-person or by videoconference with caregivers across the United States. Measures of affect, caregiver mood, stress, distress, and caregiver burden were assessed at baseline, end of sessions, and 1 month after completion. Twenty-four caregivers (12 intervention and 12 attention control) participated. At the end of the intervention, scores on positive affect, negative affect, burden, and stress all improved in the intervention compared with the control group. These scores continued to show improvement at the assessment done 1 month after intervention. Subjects were receptive to the skills and the delivery methods. The positive emotion skill-building intervention proved feasible especially in the internet videoconference delivery format. The intervention promoted positive affect and improved psychological outcomes for family caregivers of people with FTD.

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.

Evaluating and treating neurobehavioral symptoms in professional American football players: Lessons from a case series

SummaryIn the aftermath of multiple high-profile cases of chronic traumatic encephalopathy (CTE) in professional American football players, physicians in clinical practice are likely to face an increasing number of retired football players seeking evaluation for chronic neurobehavioral symptoms. Guidelines for the evaluation and treatment of these patients are sparse. Clinical criteria for a diagnosis of CTE are under development. The contribution of CTE vs other neuropathologies to neurobehavioral symptoms in these players remains unclear. Here we describe the experience of our academic memory clinic in evaluating and treating a series of 14 self-referred symptomatic players. Our aim is to raise awareness in the neurology community regarding the different clinical phenotypes, idiosyncratic but potentially treatable symptoms, and the spectrum of underlying neuropathologies in these players.

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.