Socioeconomic burden of amyotrophic lateral sclerosis, myasthenia gravis and facioscapulohumeral muscular dystrophy

Neuromuscular disorders (NMD) are chronic devastating diseases. The aim of this multicenter cross-sectional study was to evaluate the socioeconomic impact of three NMDs in Germany. Patients (n = 107) with amyotrophic lateral sclerosis (ALS), myasthenia gravis (MG) or facioscapulohumeral muscular dystrophy (FSHD) were recruited consecutively in seven centers in Germany. The health-economic data were collected using a “bottom-up” approach consisting of comprehensive questionnaires and patient diaries. Costs were evaluated from the societal perspective in 2009 Euros (EUR). Total annual costs from the societal perspective were EUR 36,380 (95% CI 27,090–47,970) per patient in ALS, EUR 26,240 (95% CI 17,770–37,940) in FSHD and EUR 14,950 (95% CI 10,470–21,730) in MG. The main components of costs were the expenditures of health insurance and the loss of productivity of patients and their caregivers. The following independent cost-driving factors were identified: disease severity, assistance in activities of daily living (ADL), dementia and younger age in ALS, disease severity in FSHD and assistance in ADL, disease severity and assistance in ADL in MG. The socioeconomic burden of NMDs in Germany is considerable. Further studies evaluating both the health-economic and clinical effects of NMD treatment as well as disease management programs and benchmarking activities are necessary.     

Assessment of health-related quality of life in patients with myasthenia gravis in Belgrade (Serbia)

The aim of this study was to assess factors that might influence the health-related quality of life (HRQoL) in patients with myasthenia gravis (MG). A cross-sectional study was performed including 230 consecutive patients with MG. Severity of the disease was estimated according to the MGFA classification and QMG score. HRQoL was assessed by the SF-36 questionnaire. Depressive and anxiety symptoms were assessed using the Hamilton rating scales for depression and anxiety, respectively. Social support was measured by the Multidimensional Scale of Perceived Social Support (MSPSS), and acceptance of the disease by the Acceptance of Illness Scale. The significant demographic predictors of worse HRQoL in MG patients were older age (p?=?0.025) and lower education (p?=?0.012). Among clinical features, significant independent contributing factors of worse HRQoL were more severe form of the disease according to MGFA (p?=?0.001) and higher QMG score (p?=?0.001). Finally, psychosocial predictors of worse quality of life were lower MSPSS score (p?=?0.001), poor acceptance of the disease (p?=?0.001), as well as higher levels of anxiety and depression (p?=?0.001). Our study revealed that the HRQoL in patients with MG is similarly reduced in its psychological and physical aspects. These results may have a practical implication pointing out that different aspects of psychosocial support should be added to the regular therapeutic protocols.     

Fatigue,self-efficacy and psychiatric symptoms influence the quality of life in patients with myasthenia gravis in Tianjin,China

BackgroundMuscle weakness related to myasthenia gravis (MG) limits the daily functioning of patients. MG patients often experience subjective symptoms including psychiatric disorders, fatigue, and reduction in self-efficacy. All of which ultimately influence their life. The relationship between the subjective symptoms and health-related quality of life (HRQoL) has never been systematically explored among MG patients.ObjectiveThis study aimed to evaluate the HRQoL of patients with MG in China, and to assess the impact of potential predictors of HRQoL.MethodsThis was a cross-sectional observational study in patients with confirmed diagnosis of MG. Patients with MG were assessed using the 36-Item Short-Form Health Survey (SF-36) questionnaire, Self-Rating Depression Scale (SDS), Self-Rating Anxiety Scale (SAS), Fatigue Severity Scale (FSS), and Self-Efficacy for Managing Chronic Disease 6 items scale (SES6G). Disease severity was evaluated by two specialists at the same time.ResultsPatients had significantly lower mean SF-36 scores for the categories role physical and general health (GH). The mean physical and mental composite scores were 57.76 ± 21.28 and 60.03 ± 23.75. Sex and unemployment influenced the QoL. Financial burden was negatively associated with total SF-36 scores. Compared to the control group, patients with generalized symptoms (MGFA II and III) had lower SF-36 scores, but the patients with pure ocular symptoms (MGFA I) had not significant difference, except GH. The SF-36 scores were highly correlated with the severity of the disease, the states of mood, fatigue, and self-efficacy.ConclusionsThe decrease in the HRQoL of patients with MG was related not only to the gender, severity of disease, and unemployment but also to the subjective experience including depressive and anxiety disorders, fatigue, and self-efficacy. In the course of treatment, the evaluation of HRQoL should be included in the routine assessment of patients with MG. Psychosocial treatment, social support, and health education should be advocated.     

Factors influencing depression endpoints research (FINDER): study design and population characteristics.

Factors influencing outcomes of depression in clinical practice, especially health-related quality of life (HRQoL), are poorly understood. The Factors Influencing Depression Endpoints Research (FINDER) study is a European prospective, observational study designed to estimate the HRQoL of adults with a clinically diagnosed depressive episode at baseline, and 3 and 6 months after commencing antidepressant medication. We report here the study design and baseline patient characteristics. HRQoL was assessed by the 36-item Short-Form Health Survey (SF-36) and European Quality of Life-5 Dimensions (EQ-5D). Patient ratings on Hospital Anxiety and Depression Scale (HADS) and pain Visual Analogue Scale (VAS) were also obtained. Results (n=3468) showed that SF-36 mental component summary (mean 22.2) was more than two SDs below general population norms (mean 50.0) and one SD below clinical depression norms (mean 34.8); the physical component summary (mean 46.1) was similar to general population (mean 50.0) and clinical depression norms (mean 45.0). Mean EQ-5D scores were also lower than general population norms. Mean HADS-Depression and -Anxiety subscores were 12.3 and 13.0, respectively. Fifty-six percent of patients reported an overall pain VAS score of at least 30mm and 70% of these patients had no physical explanation for their pain. Further investigation into factors associated with HRQoL in depression after treatment initiation is warranted.     

Quality of life predictors in patients with chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic disease which can lead to many functional impairments, and like most other chronic disorders it might significantly affect quality of life (QoL). Information about QoL in patients with CIDP from developing countries is still lacking. We, therefore, sought to complete these data mosaic by investigating QoL in patients with CIDP from Serbia and surrounding countries. Our study comprised 106 patients diagnosed with CIDP. QoL was investigated using the Serbian version of the SF-36 questionnaire. The Medical Research Council 0–5 point scale, INCAT motor and sensory scores, Krupp’s Fatigue Severity Scale, and Beck Depression Inventory were also used. Factors that significantly correlated with SF-36 total score in univariate analysis were included in the multiple linear regression analysis. Physical domains of the SF-36 were more affected than mental, and the overall score was 56.6 ± 25.4. Significant predictors of worse SF-36 score in our patients with CIDP were severe fatigue (β = ? 0.331, p < 0.01), higher INCAT motor score (β = ? 0.301, p < 0.01), depression (β = ? 0.281, p < 0.01), being unemployed/retired (β = ? 0.188, p < 0.05), and shorter duration of CIDP (β = + 0.133, p < 0.01). QoL was reduced in CIDP patients, especially in physical domains. Patients with presence of fatigue and depression, with more severe motor disability, unemployed/retired ones, and those with shorter duration of the disease need special attention of clinicians since they could be at higher risk to have worse QoL.     

Health-related quality of life in patients with narcolepsy

BACKGROUND: To evaluate the health-related quality of life (HRQoL) in patients suffering from narcolepsy. METHODS: Subjects included 75 narcoleptic patients diagnosed at the Hephata Klinik, Germany, who met the International Classification of Sleep Disorders (ICSD) criteria for narcolepsy. A standardized telephone interview was used to inquire about the disease and its burdens to the patients. HRQoL was recorded using the 36-item short-form Medical Outcomes Study (SF-36) as well as the Euroqol (EQ-5D). Frequency and factors of influence on decreased HRQoL were evaluated by using bivariate and multivariate analyses. RESULTS: Patients with narcolepsy had considerably lower scores on all eight domains of the SF-36 compared to the general German population. In particular, scores were poor for the dimensions "physical role", "vitality", and "general health perception". Forty-eight percent of the patients reported problems in at least one of the EQ-5D items; most frequent were problems in the dimension "usual activity" (63.8%), "pain/discomfort" (61.7%) and "anxiety/depression" (41.1%). Difficulty maintaining "self-care" was documented only by 6.8%. The mean VAS score was 60.7%. Interestingly, signs and symptoms of narcolepsy, except for irresistible sleep episodes (p<0.03), had only a minor impact on HRQoL. Multivariate analyses confirmed a strong influence of employment status, living with a partner, excessive daytime sleepiness (EDS) and professional advancement. CONCLUSIONS: HRQoL is considerably reduced in patients, with narcolepsy affecting the different dimensions to various degrees. Factors other than clinical signs and symptoms are associated also with poor HRQoL. Measures should be taken to integrate those factors into healthcare guidelines in order to improve the quality of life in patients with narcolepsy.     

Quality of life in adult patients with limb–girdle muscular dystrophies

Although limb–girdle muscular dystrophies (LGMD) can cause permanent disability, to date there are no studies that examined quality of life (QoL) in these patients. Our aim was to evaluate QoL in patients with LGMD, and to identify the most significant predictors of QoL. The study comprised 46 patients with diagnosis of limb–girdle muscular weakness. QoL in patients was evaluated using two scales—SF-36 questionnaire and the Individualized Neuromuscular Quality of Life questionnaire (INQoL). Following scales were also applied: Epworth Sleepiness Scale (ESS), Hamilton Scale for Depression (HamD), and Krupp’s Fatigue Severity Scale (FSS). Mean SF-36 score was 52.4 ± 23.5, and physical composite score was worse than mental. Total INQoL score was 46.1 ± 20.4, with worst results obtained for weakness, fatigue and independence, while social relationships and emotions showed better results. Significant predictors of worse SF-36 score in LGMD patients were higher fatigue level (β = ? 0.470, p < 0.01) and use of assistive device (β = ? 0.245, p < 0.05). Significant predictors of worse INQoL score were higher fatigue level (β = 0.514, p < 0.01) and presence of cardiomyopathy (β = ? 0.385, p < 0.01). It is of special interest that some of the identified factors that correlated with worse QoL in LGMD patients were amenable to treatment.     

Caregivers of patients with amyotrophic lateral sclerosis: investigating quality of life,caregiver burden,service engagement,and patient survival

Few studies in amyotrophic lateral sclerosis (ALS) have profiled disease-specific features of the condition in conjunction with assessment of caregivers’ burden, distress, quality of life, and investigated patient survival. Eighty-four ALS patients and their primary caregivers were enrolled. Patients completed ALS-specific measures of physical and cognitive function, while caregivers completed measures of anxiety, depression, caregiver burden, and quality of life. Patient-caregiver dyads were interviewed about their health-service utilisation. Survival data were obtained through the Irish register for ALS. Participants were dichotomised into low/high groups according to the severity of self-reported caregiver burden, based on statistically derived cut-off scores. High-burdened caregivers (n = 43) did not significantly differ from low-burdened caregivers (n = 41) with respect to disease-specific characteristics, i.e., ALSFRS-R, bulbar- or spinal-onset ALS, disease duration, or survival data. However, significant differences were reported on subjective measures of anxiety (p < 0.000), depression (p < 0.001), distress (p < 0.000), and quality of life (p < 0.000). These data demonstrate the limited impact of ALS patient-related variables, i.e., ALSFRS-R and onset, on caregiver burden in ALS, and identify the importance of the psychological composition of caregivers. This study suggests that the subjective experience of individual caregivers is an important factor influencing the severity of experienced caregiver burden.     

Is health-related quality of life an independent predictor of survival in patients with chronic heart failure?

OBJECTIVE: The aim of this study was to examine whether the physical and mental components of health-related quality of life (HRQoL) are independent predictors of survival in patients with chronic heart failure (CHF). METHODS: A cohort of 231 outpatients with CHF was followed prospectively for 986 days (median; interquartile range=664-1120). Generic HRQoL was measured with the Short Form-36 Health Survey (SF-36), disease-specific HRQoL was measured with the Kansas City Cardiomyopathy Questionnaire, and depression was measured with the self-reported Patient Health Questionnaire. RESULTS: Both generic and disease-specific HRQoL were predictive of survival on univariate analyses. After adjustment for prognostic factors such as age, gender, degree of left ventricular dysfunction, and functional status, only the mental health component of SF-36 and the disease-specific HRQoL remained significant. When depression was included, both measures also lost their predictive power. CONCLUSION: Our data suggest that the prognostic value of patients' HRQoL reflects confounding with the severity of disease and comorbid depression.     

White matter hyperintensities and quality of life in acute lacunar stroke

White matter hyperintensities (WMH) are common in stroke. The influence of WMH on health-related quality of life (HRQoL) following a lacunar stroke is unknown. This study evaluated the impact of WMH on HRQoL in acute lacunar stroke. A cohort of 160 patients with acute lacunar stroke admitted to the stroke unit of a university-affiliated regional hospital in Hong Kong was recruited. Three months after the index stroke, a research assistant administered the Short Form-36 (SF-36) to assess HRQoL. The severity of WMH was evaluated with magnetic resonance imaging (MRI). In univariate analysis, the severity of deep WMH (DWMH) negatively correlated with patients’ vitality (VT; p < 0.05), social function (SF; p < 0.001), role-emotional (RE; p < 0.01), mental health (MH; p < 0.01), and mental component summary (MCS; p < 0.001) scores of HRQoL. DWMH was independently associated with all of the above five SF-36 scores (p < 0.05) in linear regression analysis. These findings suggest that DWMH has a significant impact on the HRQoL of stroke survivors. The importance of DWMH in the long-term HRQoL in lacunar stroke warrants further investigation.     

Factors that influence health-related quality of life in Australian adults with Charcot-Marie-Tooth disease

Health-related, quality of life (HRQoL) is an important outcome in clinical trials of patients with Charcot-Marie-Tooth disease (CMT). In a cross-sectional survey of 295 Australian adults with CMT, HRQoL was measured using the Short Form-36 (SF-36) and predictors of reduced HRQoL were identified with a CMT-specific health status questionnaire. People with CMT demonstrated lower HRQoL scores than the general Australian population in all SF-36 dimensions. The disparity between people with CMT and normative data was greater for physical dimensions than for mental health dimensions. SF-36 scores were generally lower in older vs younger people, but not between men and women, or between CMT types. HRQoL in CMT was predicted strongly by lower limb weakness and to a lesser extent by leg cramps, suggesting clinical trials targeting weakness and cramps may improve HRQoL in patients with CMT.     

Treatment for Depression and Health-Related Quality of Life among Adults with Arthritis

Depression treatment has been proven to relieve depressive symptoms and pain and may therefore improve the health-related quality of life (HRQoL) among adults with arthritis. The objective of the current study was to examine the HRQoL associated with depression treatment among adults with arthritis and depression. A retrospective longitudinal cohort study design using data from the Medical Expenditure Panel Survey (2009–2012) was adopted. The study sample consisted of adults (≥ 21 years) with co-existing arthritis and depression (N = 1692). Depression treatment was categorized into: antidepressants only, psychotherapy with or without antidepressants, and neither antidepressants nor psychotherapy. Multivariable Ordinary Least Square (OLS) regressions, which controlled for observed selection bias with inverse probability treatment weights (IPTW) were built to examine the association between depression treatment categories and the HRQoL scores. The OLS regression controlled for factors in the biological, psychological and social domains that may affect HRQoL. A majority of individuals reported taking antidepressants only (52%), 24.4% reported receiving psychotherapy with or without antidepressants and 23% did not receive either antidepressants or psychotherapy. In multivariable OLS regression with IPTWs, adults using only antidepressants had marginally higher physical component summary scores (beta = 0.96, p value = 0.096) compared to no depression treatment. There were no significant associations between depression categories and mental component summary scores. HRQoL was not affected by depression treatment in adults with coexisting arthritis and depression. Improvement in HRQoL may require a collaborative care approach and such intense care may not be replicated in real-world practice settings.     

Longitudinal predictors of caregiver burden in amyotrophic lateral sclerosis: a population-based cohort of patient–caregiver dyads

Objective

Caregiver burden is a recognised consequence of caring for a patient with neurodegeneration. Amyotrophic lateral sclerosis (ALS) differs from other neurodegenerations by its rapid progression and impairment of motor, cognitive, and behavioural function, which contribute to caregiver burden. However, longitudinal factors that determine the extent of caregiver burden, and in particular the impact of psychological distress among caregivers, have not been fully established.

Methods

Patients with ALS (n = 85) and their primary caregivers (n = 85) completed three serial evaluations. Caregiver burden was measured using the Zarit Burden Interview (ZBI). Anxiety and depression were evaluated using the Hospital Anxiety and Depression Scale (HADS). The Edinburgh Cognitive-Behavioural ALS Screen (ECAS) was used to determine cognitive function in patients. The ALS Functional Rating Scale (ALSFRS-R) measured disease progression.

Results

Using the ZBI, caregivers were categorised as high or low burden. In the low burden group, anxiety scores from the HADS predicted caregiver burden (r = 0.410, F = 3.73, p = 0.033), whereas the depression sub-score from the HADS was predictive of caregiver burden in the high burden group (r = 0.501, F = 5.87, p = 0.006) for cross-sectional analyses. Longitudinally, an elevated score on the HADS at Time 1 was the largest predictor of caregiver burden across serial assessments.

Conclusion

In a patient cohort with relatively preserved cognitive function (65%), anxiety and depression at Time 1, as measured by the HADS, were the best predictors of caregiver burden at Time 3. This observation provides a mechanism by which caregiver burden can be identified by health-care professionals and a stepped care programme of intervention initiated.

     

Quality of life in 188 patients with myasthenia gravis in China

Myasthenia gravis (MG) is a kind of chronic autoimmune disease which can weaken patients' motor function and, furthermore, produce negative impact on the health-related quality of life (HRQoL). The primary purpose of this research was to evaluate factors that might affect the HRQoL of MG patients. A cross-sectional clinical research was carried out including 188 successive patients with MG. Myasthenia Gravis Foundation of America (MGFA) classification and Quantitative Myasthenia Gravis (QMG) score were applied to assess the severity of the disease. The Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36) was used to estimate the HRQoL. Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Rating Scale (HARS) were utilized to measure the depression and anxiety symptom. Factors may influence the HRQoL of MG patients include age, educational level, occupation, the situation of the thymus, the type of MG and generalized myasthenia gravis (GMG), the severity of the disease and the psychological disorder. Higher QMG and HARS scores were two significant factors that can prognosticate lower Physical Composite Score (PCS) and Mental Composite Score (MCS), while older age was just a significant factor which has prognostic value for lower PCS. The results of this research may have a potential guiding significance for the clinical treatment strategy and improve the quality of life in patients with MG consequently. In addition to the treatment of physical symptoms, the psychological symptoms such as anxiety and depression should be concerned as well.     

Psychometric evaluation of health-related quality of life measures in women after different types of delivery

OBJECTIVE: We examined the psychometric properties of three internationally established measures for health-related quality of life (HRQoL) in women after vaginal delivery (VD), elective cesarean section (CS), and emergency CS and the relationship of HRQoL scores with blood loss after delivery. METHODS: This is a prospective longitudinal study. One hundred forty-one consecutive patients (71 after VD, 36 after elective CS, and 34 after emergency CS) were enrolled in two university hospitals and one general hospital from June 2003 to March 2004. Women completed the Multidimensional Fatigue Inventory (MFI) and the EQ-5D classification of own health between 12 and 24 h after VD or between 24 and 48 h after CS. Subsequent assessments, additionally including the Short Form 36 (SF-36), were made 1, 3, and 6 weeks after delivery. We analyzed feasibility (response, completion time, reported difficulties, item nonresponse), reliability (Cronbach's alpha), discriminative validity between groups by type of delivery, and responsiveness over time (Wilcoxon's signed rank tests and effect sizes). RESULTS: The MFI, SF-36, and EQ-5D proved to be highly feasible and reliable (alpha>.7 for all scales of MFI and SF-36). The measures were able to discriminate between groups by mode of delivery and to detect moderate recovery in physical and small recovery in mental status over time in the first 6 weeks after delivery. The suboptimal total questionnaire response of 60% after 6 weeks was attributable to low response among women of non-Dutch ethnic origin. The significant correlation between Hb level and mean physical HRQoL scores found at T=0 disappeared 1 week postpartum. CONCLUSION: The combination of MFI, SF-36, and EQ-5D showed good psychometric performance and is a good choice to measure HRQoL after delivery in scientific studies. Development of a shorter set is needed for use in routine clinical practice.     

Cognitive impairment, health-related quality of life and vocational status at early stages of multiple sclerosis: a 7-year longitudinal study

The association between cognitive impairment, health-related quality of life (HRQoL) and vocational status has been studied in recent years in cross-sectional studies in multiple sclerosis (MS), but longitudinal data are still lacking. This study assesses this association in a sample of 65 newly diagnosed MS patients followed longitudinally. Each patient underwent a standardised clinical assessment, cognitive tests and the HRQoL SEP-59 questionnaire six months after the MS diagnosis (baseline) and seven years later (y7).Vocational status was also established at baseline and at y7 in MS patients. The HRQoL at baseline was severely reduced in MS patients compared with healthy subjects. The independent predictors for HRQoL composite scores at y7 were the baseline depression score and the memory Z-score. Accordingly, 81.5 % of MS patients worked at baseline and only 54.4 % worked at y7. Among the MS patients who did not work at y7, 72.7 % of them were cognitively impaired, while 27.3 % were unimpaired at baseline. The vocational status at y7 was significantly associated with the baseline IPS Z-score, EDSS and age. Vocational status at y7 and its change over 7 years was significantly associated with cognitive deterioration. IPS or memory dysfunction in the early stages of MS is correlated with a decreased level in health perception, independent of fatigue, depression and physical disability. Cognitive impairment at the diagnosis of MS increases the risk of changing vocational status in MS patients seven years later.     

High-intensity interval training in facioscapulohumeral muscular dystrophy type 1: a randomized clinical trial

Increasing evidence suggests that high-intensity training (HIT) is a time-efficient exercise strategy to improve fitness. HIT has never been explored in neuromuscular diseases, likely because it may seem counterintuitive. A single session of high-intensity exercise has been studied without signs of muscle damage in facioscapulohumeral muscular dystrophy type 1 (FSHD1). We aimed to determine whether HIT is safe and effective in FSHD1 in a randomized, controlled parallel study. Untrained adults with genetically verified FSHD1 (n = 13) able to perform cycle-ergometer exercise were randomized to 8 weeks of supervised HIT (n = 6) (3 × 10-min cycle-ergometer-HIT/week) or 8 weeks of usual care (n = 7). Following this, all participants performed 8 weeks of unsupervised HIT (3 × 10-min cycle-ergometer-HIT/week). Primary outcome was fitness, maximal oxygen uptake/min/kg body weight. Furthermore, workload, 6-min walk distance, 5-time sit-to-stand time, muscle strength, and daily activity levels were measured. Pain, fatigue, and plasma-CK were monitored. Twelve patients completed the randomized part of the study. Plasma-CK levels and pain scores were unaffected by HIT. Supervised HIT improved fitness (3.3 ml O₂/min/kg, CI 1.2–5.5, P < 0.01, n = 6, NNT = 1.4). Unsupervised HIT also improved fitness (2.0 ml O₂/min/kg, CI 0.1–3.9, P = 0.04, n = 4). There was no training effect on other outcomes. Patients preferred HIT over strength and moderate-intensity aerobic training. It may seem counterintuitive to perform HIT in muscular dystrophies, but this RCT shows that regular HIT is safe, efficacious, and well liked by moderately affected patients with FSHD1, which suggests that HIT is a feasible method for rehabilitating patients with FSHD1.     

Association between depression and survival in Chinese amyotrophic lateral sclerosis patients

To determine the prevalence of depression, to identify correlated factors for depression, and to explore the impact on the progression or survival of amyotrophic lateral sclerosis (ALS) by depression in a Chinese population. A total of 166 ALS patients were recruited. Diagnosis of depression disorders and the severity of depression were established by using the fourth diagnostic and statistical manual of mental disorders, Hamilton Depression Rating Scale-24 items (HDRS-24) and Beck Depression Inventory (BDI). Major depression was found in 15 patients (9.6 %). The multiple regression analysis showed that a lower ALS Functional Rating Scale-Revised (ALSFRS-R) score was correlated with increasing HDRS scores and BDI scores (P = 0.018 and P = 0.012). No significant difference in the median survival time between ALS patients with and without depression was revealed by Kaplan–Meier analysis (log-rank P = 0.282). Cox hazard model showed that the presence of depression in ALS was unrelated to the survival, while the severity of depression in ALS was correlated with the survival. The presence and severity of depression in ALS did not correlate with the progression of ALS. Major depression in ALS is uncommon. Depression evaluation should be given to ALS patients, especially those with lower ALSFRS-R score. The severity of depression may be associated with the survival; however, depression does not worse the progression of ALS.     

Predictors of health-related quality of life in patients with epilepsy and psychogenic nonepileptic seizures

Epilepsy and psychogenic nonepileptic seizures (PNES) are associated with reduced health-related quality of life (HRQoL). The present study investigated the profile, relationship, and predictive power of illness perceptions, psychological distress (depression and anxiety), seizure activity, and demographic factors on HRQoL in these patient groups. Patients with epilepsy (n = 62) and PNES (n = 45) were recruited from a United Kingdom hospital and from membership-led organizations for individuals living with seizures. Patients completed a series of self-report questionnaires assessing: anxiety (GAD-7), depression (NDDI-E), illness perceptions (B-IPQ), HRQoL (NEWQOL-6D), and seizure frequency and severity (LSSS-3). Correlational and hierarchical multiple regression analyses were conducted. Patients with epilepsy reported higher HRQoL and scored lower on measures of depression and anxiety. Patients with PNES perceived their condition as more threatening overall. In both conditions, HRQoL was negatively correlated with more severe illness perceptions and psychological distress. In epilepsy and PNES, psychological distress (epilepsy: 27%; PNES: 24.8%) and illness perceptions (epilepsy: 23.1%; PNES: 23.3%) accounted for the largest amount of variance in HRQoL. Clinical factors were found not to be significant predictors, while demographic factors predicted HRQoL in epilepsy (12.6%), but not in PNES. Our findings support the notion that psychological factors are a stronger predictor of HRQoL in epilepsy and PNES than condition-related and demographic variables. Prior research suggests that anxiety and depression are key predictors of HRQoL; this study demonstrates that the relationship between illness perceptions and HRQoL is similarly close. These findings highlight the importance of addressing patients' beliefs about their condition.     

Determinants and status of quality of life after long-term botulinum toxin therapy for cervical dystonia

The aim of this study was to assess health-related quality of life (HRQoL), using the Short Form Health Survey-36 (SF-36), in 70 cervical dystonia (CD) patients after long-term botulinum toxin (BTX) treatment (median 5.5 years), and to identify factors determining reduced HRQoL. We used combined patient-and physician-based measures to assess both CD severity [Toronto Western Spasmodic Torticollis Rating Scale, (TWSTRS)] and effect of long-term BTX treatment, and the Hospital Anxiety and Depression Scale (HAD) and General Health Questionnaire-30 to assess psychological distress. Mean SF-36 domain scores of the CD patients were reduced by <1 SD compared with age- and gender-matched population samples. High TWSTRS total scores and high HAD-depression (HAD-D) scores were the main factors associated with reduced scores in the physical and mental SF-36 domains, respectively. Patients evaluated to have a 'good effect' of long-term BTX treatment ( n  = 47), had significantly lower median TWSTRS total score, and a 3× lower frequency of high HAD-D scores, than those evaluated to an 'unsatisfactory effect' ( n  = 23). In conclusion, most CD patients enjoy a good HRQoL after long-term BTX therapy. Reduced HRQoL was associated with more severe disease and/or depressive symptoms.