Brittany Rohl

Defining the Treatment Gap: What Essential Tremor Patients Want That They Are Not Getting.

Background Patient-centeredness (i.e., providing care that is responsive to individual patient preferences) is increasingly recognized as a crucial element of quality of care. Methods A six-item patient-centeredness questionnaire was devised to assess the self-perceived needs of essential tremor (ET) patients. A link to the questionnaire was included in the monthly e-newsletter of the International Essential Tremor Foundation. The questionnaires were completed online and data were available in electronic format. Results There were 1,418 respondents. One in three respondents (i.e., 31.4%) indicated that the doctor was not even “moderately well-educated” about ET. Only 11.8% of respondents were satisfied with their care. Respondents raised a multiplicity of issues that were not being addressed in their current care. The top items were psychological services and support (33.9%), physical or occupational therapy (28.6%), handling embarrassment and social effects of tremor (15.8%), feelings of not being in control (13.7%), a detailed report and a more quantitative way of assessing tremor and tracking progression (12.7%), better counseling about current treatment and medications (11.9%), empathy, compassion and a feeling of being heard (11.6%), a treatment approach other than just medications and surgery (11.2%), and a discussion of all symptoms aside from tremor (e.g., cognition, balance). Discussion Patients with ET identified a broad range of issues that they felt were not addressed in their treatment; indeed, only one in 10 patients reported that they were satisfied with their care. It is hoped that patient-centered approaches such as this will lead to improved models for the care of patients with this common chronic disease.

Daytime sleepiness and nighttime sleep quality across the full spectrum of cognitive presentations in essential tremor

There is increasing evidence that essential tremor (ET) is a complex and heterogeneous disorder with nonmotor features including cognitive deficits and sleep problems. We are unaware of a study that has examined sleep deficits in ET across the full spectrum of cognitive presentations. Cross-sectional (baseline) data on self-reported nighttime sleep dysfunction and excessive daytime sleepiness were collected using the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS) in 96 ET cases enrolled in a prospective study. Cases underwent a comprehensive neuropsychological assessment, and were classified as ET with normal cognition (ET-NC), ET with mild cognitive impairment (ET-MCI), and ET with dementia (ET-D). PSQI scores did not significantly differ across the three ET cognitive groups (p=0.22). ESS scores were highest (more daytime sleepiness) in the ET-MCI group, followed by the ET-D and ET-NC groups, respectively (p=0.016). We examined sleep dysfunction across the cognitive spectrum in ET. We demonstrate for the first time that excessive daytime sleepiness is greater in ET-MCI than ET-NC. Unpredicted low ESS scores in the dementia group raises two possibilities: a self-report bias related to cognitive impairment and/or the possibility that currently undefined pathological heterogeneity in ET may map onto multiple presentations of non-motor deficits.

Poorer Cognitive Performance in Patients with Essential Tremor-Parkinson’s Disease vs. Patients with Parkinson’s Disease

Background Patients with essential tremor (ET) seem to be at increased risk of developing Parkinson’s disease (PD). Surprisingly, little has been written about this clinical entity, ET-PD. Cognitive dysfunction is a well-known feature of PD, and can also be an issue in patients with ET. Whether the presence of the combined diagnosis, ET-PD, is associated with additive cognitive effects as compared with PD has not been studied. Methods Thirty ET-PD patients and 53 age-matched PD patients were enrolled in a clinical-epidemiological study. Two cognitive screens, the Telephone Interview for Cognitive Status (TICS, score = 0–41) and Folstein Mini-Mental State Examination (MMSE; range 0–30), were administered. Results The MMSE score was lower in ET-PD than PD [26.5 ± 3.1 (median 28.0) vs. 28.4 ± 2.2 (median 29.0), p = 0.001]. The TICS score was lower in ET-PD than PD [31.7 ± 3.9 (32.0) vs. 35.0 ± 2.0 (35.0), p < 0.001]. Subscores of these tests that related to orientation (p < 0.001), language (p < 0.001), and working memory (p = 0.001) were lower in ET-PD than PD, whereas the delayed memory subscore was only marginally lower in ET-PD than PD (p = 0.06), and the two groups did not differ with respect to the motor/construction subscore (p = 0.22). Both global cognitive scores were inversely correlated with disease duration (for MMSE score, Spearman’s r = −0.46, p < 0.001; for TICS score, Spearman’s r = −0.53, p < 0.001). Conclusion The combined diagnosis, ET-PD, seemed to be associated with additive cognitive effects as compared with PD alone.

Cognitive Dysfunction Is Associated with Greater Imbalance and Falls in Essential Tremor

Background Essential tremor (ET) is not exclusively a tremor disorder; it is also associated with cognitive and gait dysfunction. However, a gap in knowledge is that the relationship between cognitive and gait dysfunction has not been studied in detail in ET. We examined the relationship between cognition and balance and falls in ET and hypothesized that cognitive dysfunction in ET patients would be associated with greater problems with balance and more falls. Methods ET cases were recruited into the Clinical–pathological Study of Cognition in ET. A comprehensive cognitive assessment was performed. This included the Montreal Cognitive Assessment (MoCA) to measure global cognition, multiple motor-free tests comprehensively assessing performance in each cognitive domain, and an assignment of Clinical Dementia Rating (CDR) scores. We collected data on the number of reported falls in the past year, and balance confidence was assessed using the 6-item Activities of Balance Confidence Scale. These cross-sectional analyses utilized baseline data. Results There were 199 ET cases (mean age 78.6 years). In linear regression models that considered the effects of numerous confounding variables, lower global cognition (poorer cognition) was associated with greater number of falls and reduced balance confidence (p < 0.05). In similar adjusted linear regression models, higher CDR score (poorer functional cognition) was associated with greater number of falls and reduced balance confidence (p < 0.05). We also assessed whether number of falls and balance confidence was associated with performance in specific cognitive domains. Number of falls was most closely linked with performance on tests of executive function, and balance confidence, with executive function, attention, and memory. Conclusion These data indicate that a correlate of poorer cognition in ET is greater number of falls and lower balance confidence. Cognition should enter the dialog with ET patients as an issue of clinical significance.

The Experience of Essential Tremor Caregivers: Burden and Its Correlates

Background Essential tremor (ET) is associated with physical and cognitive impairments, as well as embarrassment, avoidance of social settings, and related difficulties that negatively impact the lives of patients. In similar disease contexts, burden on friends and relatives acting as caregivers has been noted and has well-documented implications. There has been no study examining caregiver burden related to ET. Methods Data were gathered from 55 ET participants enrolled in a clinical study and their caregivers. The Zarit Burden Interview was used to assess caregiver burden. To assess clinical features that may be associated with burden, we collected several variables including the Montreal Cognitive Assessment, self-reported tremor disability, a videotaped neurological examination, questionnaires assessing ET participants’ suffering, caregivers’ perceptions of that suffering, and both caregiver and ET participant depressive symptoms. Spearman’s correlations were performed between caregiver burden and clinical features, and we created a multivariate linear regression model predicting caregiver burden. Results Many ET caregivers provide little to no care and experience little to no burden. However, some caregivers (11%) provide over 25 h of care/week, and 13% experience high levels of burden. Caregivers most commonly provided assistance with writing and cooking. Increased burden was associated with the ET participants’ decreased cognition, more caregiving tasks, more hours/week of caregiving activities, a longer duration of care, more ET participant falls/year, more medications taken by the ET participant, and more depressive symptoms in both the ET participant and the caregiver (all p < 0.05). ET participants’ suffering and their caregivers’ perceptions of suffering were both associated with increased burden. Neither tremor severity score nor self-reported tremor disability score was associated with increased caregiver burden. Using a multivariate linear regression model, we found that caregivers’ increased perception of their partners’ suffering was the best predictor of caregiver burden. Conclusion While not all relatives and friends of ET patients provide extensive care or experience high burden, there is a group reporting high levels of caregiver burden that requires the attention and counseling of clinicians. This burden is associated with primarily non-tremor symptoms of ET and with caregivers’ perception that their partners are suffering.

Self-report depressive symptoms are dissociated from tremor severity in essential tremor

BACKGROUND Depressive symptoms are associated with essential tremor (ET). However, the relationship between cognitive, functional, and motor measures with depressive symptoms in ET is not yet understood. METHODS The following measures were cross-sectionally assessed in a group of 223 subjects with ET: the Montreal Cognitive Assessment (MoCA) Scale, the Lawton Independent Activities of Daily Living (IADL) Scale, a neurologist assessment of tremor severity, and the Geriatric Depression Scale (GDS). RESULTS 20% (44) of the subjects met GDS criteria for depression (GDS ≥ 10). 43% (94) of the subjects showed at least some cognitive impairment (≤24 on the MoCA), and 15.3% (34) reported significant functional impairment (IADL score < 7). There was no significant association between GDS score and tremor scale score. The total GDS was negatively associated with the total MoCA score (Spearmans r = -0.15, p = 0.03). The total GDS was also negatively associated with the IADL score (Spearmans r = -0.19, p = 0.02), (logistic model odds ratio, OR = 4.91, p < 0.01). Over 60% of subjects who were depressed, per GDS cut-off score (≥10), were not receiving medical treatment for depression. CONCLUSIONS There was a high point prevalence of depressive symptoms in subjects with ET. Self-report depressive symptoms are dissociated from tremor severity. Hence, these data do not support the hypothesis that depression in ET represents a psychological reaction to the tremor. There appears to be a clustering of cognitive, functional, and depressive symptoms in ET. Screening of depression in ET can improve our understanding and treatment of this disorder.

Psychological Suffering in Essential Tremor: A Study of Patients and Those Who Are Close to Them

Background Although the motor and non-motor features of essential tremor (ET) have been characterized in detail, it is not known whether ET patients suffer psychologically and whether those who are close to them consider them to be suffering in this way. Methods Fifty ET patients and 50 “close others” (COs), identified by patients “as someone who knows you well and sees you often” and who can “provide a different perspective on your well-being”, reported their own depressive symptoms, daily stress, and perceptions of patient psychological suffering and patient overall suffering with validated scales. ET patients’ tremor severity, duration, disability, cognition, and number of medications were also assessed. Results ET patients reported levels of psychological suffering within the range documented in arthritis and dementia patients from previous studies, and COs perceived significantly more psychological suffering in patients than patients reported themselves. Regression models, controlling for tremor severity, duration, and disability revealed that patients’ greater psychological suffering was associated with greater patient depression. The greater perceptions of COs of patient psychological and overall suffering were associated with greater CO depression and daily stress. Sensitivity analysis showed that patients’ cognitive status or number of medications did not affect the results. Discussion Multidisciplinary teams caring for ET patients should look beyond simple clinical ET indicators. They should be aware of patient experiences and perceptions of COs of psychological and overall suffering. This will help guide the development of evidence-based, supportive interventions that improve communication about the needs of ET patients and those who are close to them.

Awareness of cognitive impairment in individuals with essential tremor

OBJECTIVE The extent to which individuals with ET who have clinically significant cognitive impairment are aware of their cognitive changes is unclear. Reduced awareness has important implications for everyday function and decision-making. METHODS 150 individuals with ET (109 Normal Cognition (ET-NC group), and 30 with MCI and 11 dementia (ET-CI group)) completed self-ratings and objective assessments of memory, language, and executive functioning. Discrepancy scores were calculated to assess awareness of cognitive functioning. One sample t-tests evaluated whether mean discrepancy scores in each group were comparable to zero (i.e., accurate). Analyses of covariance (ANCOVA) compared discrepancy scores across two groups controlling for age and education. RESULTS In the ET-NC group, discrepancy scores for language (M=-0.08, SD=1.10) and executive functioning (M=-0.01, SD=0.99) were comparable to zero. Memory discrepancy scores (M=0.32, SD=1.22) were greater than zero. In the ET-CI group, memory, (M=0.78, SD=1.01), language, (M=0.46, SD=0.95), and executive (M=0.39, SD=1.14) discrepancy scores were all greater than zero. Discrepancy scores were larger in ET-CI group than in ET-NC group for memory: F(1,148)=4.02, p=0.047, language: F(1,148)=6.16, p=0.014, and executive: F(1,148)=4.51, p=0.035. CONCLUSIONS Individuals with ET and normal cognition accurately assessed their language and executive abilities, demonstrating mild overconfidence in memory function. Individuals with ET and cognitive impairment overestimated their performance in all domains of functioning. Since ET is linked to increased risk for cognitive impairment, and such impairment may not be accurately perceived, cognitive functioning should be proactively and regularly screened in ET.

Perceived embarrassment and caregiver burden in essential tremor caregivers

Essential tremor (ET) is a progressive neurological disease associated with functional disability, diminished quality of life and, in some individuals, poorer balance, cognitive impairment, depression and sleep dysregulation. Individuals with ET may rely on family members and friends to act as informal caregivers to assist with daily activities and provide emotional support. There is a high prevalence of embarrassment among individuals with ET, which may be a result of the outwardly visible nature of tremor. Studies in populations with outwardly visible disability have shown that perception by caregivers of a care-recipients social distress can contribute to caregiver burden. We hypothesize that in ET, perception by caregivers of ET participant embarrassment is a predictor for caregiver burden. Data were collected from 57 ET participants and their caregivers. We measured ET participant embarrassment using the Essential Tremor Embarrassment Assessment (ETEA), and measured perception by caregivers of ET participant embarrassment using a modified version of the ETEA. The Zarit Burden Interview was used to measure caregiver burden. Perceived embarrassment was associated with ET participant embarrassment. In linear regression models, perceived embarrassment was a stronger predictor for caregiver burden than measures of ET participant cognitive and physical impairment. The results indicate that perception of ET participant embarrassment can be burdensome for caregivers. Clinicians may wish to address patient embarrassment and perceived embarrassment to better support caregivers and ET patients.

Self-reported physical activity in essential tremor: Relationship with tremor, balance, and cognitive function

BACKGROUND Physical inactivity may be the result of medical comorbidities. Inactivity itself may also lead to important health consequences, especially in older patients. Essential tremor (ET) patients may have a variety of physical and cognitive problems that could detrimentally impact on level of physical activity. Yet, to our knowledge, there have been no studies of physical activity in these patients. METHODS Self-reported physical activity was assessed using the Physical Activity Scale for the Elderly (PASE) in 100 ET cases (mean age 80.5years) enrolled in a clinical study. Additional clinical measures were the total tremor score, Montreal Cognitive Assessment (MOCA) score and number of steps taken off of the straight line during tandem gait (a measure of balance). RESULTS Lower PASE score was associated with older age, more tandem gait difficulty, higher total tremor score and lower MOCA score (all p<0.05). In a linear regression model that included total tremor score, MOCA score, number of steps off of the straight line during tandem gait, and age, higher total tremor score (p=0.046) and more steps off of the straight line during tandem gait (p=0.014) were independently associated with reductions in physical activity. CONCLUSIONS Several of the motor features of ET (tremor and imbalance) are independently associated with reductions in level of physical activity.