Stigma, coping strategies, distress and wellbeing in individuals with cervical dystonia: a cross-sectional study.

Cervical dystonia (CD) is a movement disorder which causes sustained muscle contractions in the neck leading to abnormal postures and repetitive movements. As it is a highly visible condition, people with CD can experience stigma, which may lead to unhelpful coping strategies and increased psychological distress. This study investigated whether adaptive and maladaptive coping strategies mediate the relationship between stigma and psychological outcomes in people with CD. A total of 114 adults with CD completed measures of stigma, coping, health-related quality of life (HRQOL), psychological distress (depression, anxiety, stress), and psychological wellbeing at one time point. Participants' levels of distress were high, compared to the general population. Correlational analyses showed increased stigma and maladaptive coping (e.g. substance use, behavioural disengagement) were both significantly related to increased distress, lower wellbeing and lower HRQOL, whereas higher adaptive coping (e.g. acceptance, humour) was only related to higher wellbeing. In a parallel mediation model, maladaptive coping strategies mediated the relationship between stigma and distress, HRQOL and wellbeing, but adaptive coping strategies did not. These findings suggest that maladaptive coping may play an important role in explaining the relationship between stigma and some aspects of distress and wellbeing in CD. Interventions which focus on reducing different aspects of maladaptive coping may be helpful to improve wellbeing as well as reducing stigma.

Impact of Depression on Cognitive Function and Disease Severity in Idiopathic Cervical Dystonia Patients: One-Center Data in Cross-Sectional Study.

Background: Cervical dystonia is a highly disabling hyperkinetic movement disorder with a lot of nonmotor symptoms. One symptom with a high prevalence is depression, which may negatively affect dystonia patients. The aim of the study was to investigate the impact of depression on disease severity and cognitive functions in cervical dystonia patients. Methods: Patients with cervical dystonia were interviewed and divided into two groups, based on the Patient Health Questionnaire-9: those with no depression or mild depressive features and those with moderate, moderately severe, and severe depression. The severity of dystonia and cognitive functions were assessed and compared in both groups. Results: A total of 52 patients were investigated. Self-assessment of the disease was more negative in clinically significant depressive signs group (p = 0.004), with a tendency for patients with clinically significant depressive features to have a slightly higher score on objective dystonia scales (TSUI and TWSTRS), but without statistically significant differences (p = 0.387 and p = 0.244, respectively). Although not statistically significant, a slightly higher MoCA scale score was registered in cervical dystonia patients with clinically insignificant depressive signs. There was a tendency for worse results in the abstraction category in patients with clinically significant depression (p = 0.056). Conclusions: Patients with clinically significant depression have a more negative self-assessment of the disease and perform worse in abstraction tasks.

Neuropsychological correlates of prospective memory: A comparison between tremor-dominant Parkinson's disease and cervical dystonia.

Cervical Dystonia (CD) and Parkinson's disease, particularly tremor-dominant motor phenotype (TD-PD), showed a selective deficit of time-based prospective memory (TBPM). The two movement disorders are mainly characterized by dysfunctions of basal-ganglia and prefrontal cortex but it is reported that cerebellum also plays a key role in their pathogenesis. These cerebral structures are specifically involved in TBPM rather than in event-based PM (EBPM), but until now no study directly compared these two components of PM between CD and TD-PD patients. Therefore, the present study aimed at investigating if differences in PM functioning between CD and TD-PD patients might exist and if the type of movement disorder moderated the relationship between deficit of PM and deficit of executive functions and retrospective memory. Thirty TD-PD, 27CD patients and 29 healthy subjects (HCs), matched for demographic features, underwent neuropsychological tests for PM, executive functions, retrospective memory and self-rated questionnaires. The three groups did not differ on neuropsychological variables except for TBPM where TD-PD and CD patients performed worse than HCs; moreover, TD-PD performed worse than CD patients. Moderation analysis indicated that the type of movement disorder moderated the relationship between executive dysfunction and TBPM, but not EBPM. In conclusion, selective deficit of TBPM characterizes both CD and TD-PD but it is associated with executive dysfunction only in TD-PD. It might be possible to speculate that the involvement of the cerebellum, responsible for internal timing processes, could explain the impairment of TBPM in both movement disorders. This issue deserves to be explored in future neuroimaging studies.

Botulinum toxin injection changes resting state cerebellar connectivity in cervical dystonia.

In cervical dystonia, functional MRI (fMRI) evidence indicates changes in several resting state networks, which revert in part following the botulinum neurotoxin A (BoNT) therapy. Recently, the involvement of the cerebellum in dystonia has gained attention. The aim of our study was to compare connectivity between cerebellar subdivisions and the rest of the brain before and after BoNT treatment. Seventeen patients with cervical dystonia indicated for treatment with BoNT were enrolled (14 female, aged 50.2 ± 8.5 years, range 38-63 years). Clinical and fMRI examinations were carried out before and 4 weeks after BoNT injection. Clinical severity was evaluated using TWSTRS. Functional MRI data were acquired on a 1.5 T scanner during 8 min rest. Seed-based functional connectivity analysis was performed using data extracted from atlas-defined cerebellar areas in both datasets. Clinical scores demonstrated satisfactory BoNT effect. After treatment, connectivity decreased between the vermis lobule VIIIa and the left dorsal mesial frontal cortex. Positive correlations between the connectivity differences and the clinical improvement were detected for the right lobule VI, right crus II, vermis VIIIb and the right lobule IX. Our data provide evidence for modulation of cerebello-cortical connectivity resulting from successful treatment by botulinum neurotoxin.

Experiences of Parents of Infants Diagnosed With Mild or Severe Grades of Congenital Muscular Torticollis.

PURPOSE: The primary purpose of this study was to describe the experiences of parents of infants diagnosed with congenital muscular torticollis (CMT). A secondary purpose was to compare the experiences of parents of infants with mild grades versus severe grades of involvement based on the CMT severity classification system. METHODS: Through semistructured interviews, a qualitative phenomenological approach of inquiry was used to investigate the lived experiences of 12 parents. RESULTS: Eight themes common to both groups of parents were identified. Findings indicated having an infant with CMT has a significant effect on the parents and other caregivers. Two themes were unique to parents of the infants with severe CMT. CONCLUSIONS: Parents are faced with a diagnosis that requires regular therapy visits and a challenging home program. A multimodal approach by clinicians for teaching and supporting parents during the episode of care may best address their unique challenges and stresses.

Prevalence of non-motor symptoms and their association with quality of life in cervical dystonia.

OBJECTIVES: Non-motor symptoms (NMS) are commonly present along with motor impairment in patients with cervical dystonia (CD) and have a significant impact on health-related quality of life (HRQoL). However, the prevalence of NMS and their association with dystonia are still unclear. The aim of our study was to assess the prevalence of depression, anxiety, fatigue, apathy, pain, sleep problems, and excessive daytime sleepiness (EDS) in CD using different evaluation approaches and to explore their association with HRQoL relative to that of motor symptoms. MATERIALS AND METHODS: We enrolled 102 Slovak patients with CD. The severity of both motor and non-motor symptoms was assessed using validated scales. HRQoL was determined by the 36-item Short Form Health Survey (SF-36). Association of NMS with poor HRQoL was assessed using multiple regressions. RESULTS: The most frequent NMS in our sample were sleep impairment (67.3%), anxiety (65.5%), general and physical fatigue (57.5% and 52.9%, respectively), depression (47.1%), mental fatigue (31.4%), apathy (30.4%), reduced activity (29.4%), EDS (20.2%), and reduced motivation (18.6%). Univariate analysis showed that NMS, but not motor symptoms, were significantly linked to poor HRQoL, with EDS being most commonly associated with poor HRQoL, followed by disrupted sleep, depression, and fatigue. CONCLUSIONS: The prevalence of NMS among patients with CD is high, and some NMS are strongly associated with poor HRQoL, while motor impairment was not associated with the severity of NMS or poor HRQoL. Actively diagnosing and treating NMS should therefore be a routine part of the clinical management of patients with CD.

Neuropsychiatric profile of patients with craniocervical dystonia: A case-control study.

OBJECTIVE: Depression, anxiety, and obsessive-compulsive disorder have been widely reported in patients with dystonia. On the other hand, cognitive impairment, frontal lobe function, impulsiveness and pseudobulbar affect are less studied. The objective of the study is to assess these neuropsychiatric symptoms along with the quality of life of subjects with craniocervical dystonia. PATIENTS AND METHODS: A cross-sectional study was carried out in patients with craniocervical dystonia. Sex- and age-matched healthy controls were included. Neuropsychiatric assessment included the Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), Barrat Impulsiveness Scale (BIS-11), Center for Neurologic Study-Lability Scale (CNS-LS), Anxiety Rating Scale (HAM-A), Hamilton Depression Rating Scale (HAM-D), and the 12-item Short Form Health Survey (SF-12). RESULTS: A total of 44 patients with craniocervical dystonia and 44 controls were included. The mean age was 57 ± 13.7 years. Depression (56.1 % vs 9.1 %, p < 0.001), anxiety (56.8 % vs 6.8 %, p < 0.001), and pseudobulbar affect (31.8 % vs 9.1 %, p = 0.02) were more common in the dystonia group in comparison to controls. No difference between groups was found in impulsiveness (p = 0.65), MoCA score (p = 0.14) or executive dysfunction (p = 0.42). Quality of life was worst in the dystonia group with 90.9 % (p = 0.03) and 61.4 % (p < 0.001) of the subjects scoring under average in the Physical Composite Score (PCS) and Mental Composite Score (MCS) of the SF-12. CONCLUSION: MoCA scores ≤18, pseudobulbar affect, depression and anxiety are more prevalent in subjects with craniocervical dystonia in comparison to sex- and age-matched healthy controls. Regarding quality of life, MCS is more affected that the PCS in subjects with dystonia.

The effects of a relaxation program featuring aquatic therapy and autogenic training among people with cervical dystonia (a pilot study).

Classic physical interventions for cervical dystonia (CD) have focused on treating motor components or, on motor components and relaxation programs. However, no CD treatment study has focused on a relaxation program alone. We developed a pilot study to assess whether a therapy completely based on a relaxation program could improve the physical and mental symptomatologies of patients with CD. Fifteen persons were included in the experimental group, which received individual sessions of aquatic (Watsu) therapy (WT) and autogenic training (AT). In addition, 12 persons were included in passive control group. We administered different questionnaires related to quality of life (SF-36), pain (Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Visual Analog Scale (VAS)) and mood (Beck Depression Inventory (BDI-II) and State-Trait Anxiety Inventory (STAI)). A significant interaction was observed between treatment and time with regard to the SF-36, VAS, and TWSTRS within the experimental group (p < 0.01). The BDI-II showed depression decrease as a simple effect (p < 0.05), and the STAI did not change. No effects were found with regard to the control group. In this exploratory study, we found that a therapy based on whole body relaxation improved the symptoms of patients with CD. This knowledge enables a disease-management strategy that uses a holistic perspective and moves beyond the dystonic focus.

Sensorimotor Perceptive Rehabilitation Integrated (SPRInt) program: exercises with augmented movement feedback associated to botulinum neurotoxin in idiopathic cervical dystonia-an observational study.

BACKGROUND: Idiopathic cervical dystonia (ICD) is a focal dystonia affecting neck muscles. Botulinum neurotoxin (BoNT) is the first-line treatment of ICD and different physical therapies (including exercise) are often proposed as adjunct treatments. However, the actual effectiveness of exercise in ICD is unclear. The aim of the current work is to assess the potential effectiveness of the Sensorimotor Perceptive Rehabilitation Integrated (SPRInt) exercise program as adjunct therapy for ICD. METHODS: Fifteen ICD patients received BoNT injections in the neck muscles and, 12 weeks later, received BoNT a second time and SPRInt started. SPRInt consists in 18 exercise sessions in which augmented feedback of movement (including visual and acoustic feedback) is extensively used. Dystonia burden was measured by the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Patients were evaluated immediately before, 6 and 12 weeks after each BoNT injection. RESULTS: Six weeks after the first BoNT injection (i.e., at BoNT peak effect), TWSTRS total score was better than baseline and remained improved at 12 weeks. TWSTRS disability domain slightly improved 6 weeks after the first BoNT injection, but after 6 more weeks returned to its baseline level. Disability improved more at SPRInt end (i.e., 6 weeks after the second BoNT injection), being even lower than after toxin alone. With a single-subject analysis, 4/10 patients who did not improve disability after BoNT improved after SPRInt plus BoNT. CONCLUSIONS: SPRInt plus BoNT can be more effective than BoNT alone in improving cervical dystonia patients' difficulties in the activities of daily living. TRIAL REGISTRATION: www.ClinicalTrials.gov, identifier NCT03247868 (https://register.clinicaltrials.gov).

Validation of a self-completed Dystonia Non-Motor Symptoms Questionnaire.

OBJECTIVE: To develop and validate a novel 14-item self-completed questionnaire (in English and German) enquiring about the presence of non-motor symptoms (NMS) during the past month in patients with craniocervical dystonia in an international multicenter study. METHODS: The Dystonia Non-Motor Symptoms Questionnaire (DNMSQuest) covers seven domains including sleep, autonomic symptoms, fatigue, emotional well-being, stigma, activities of daily living, sensory symptoms. The feasibility and clinimetric attributes were analyzed. RESULTS: Data from 194 patients with CD (65.6% female, mean age 58.96 ± 12.17 years, duration of disease 11.95 ± 9.40 years) and 102 age- and sex-matched healthy controls (66.7% female, mean age 55.67 ± 17.62 years) were collected from centres in Germany and the UK. The median total NMS score in CD patients was 5 (interquartile range 3-7), significantly higher than in healthy controls with 1 (interquartile range 0.75-2.25) (P < 0.001, Mann-Whitney U-test). Evidence for intercorrelation and convergent validity is shown by moderate to high correlations of total DNMSQuest score with motor symptom severity (TWSTRS: rs  = 0.61), clinical global impression (rs  = 0.40), and health-related quality of life measures: CDQ-24 (rs  = 0.74), EQ-5D index (rs  = -0.59), and scale (rs  = -0.49) (all P < 0.001). Data quality and acceptability was very satisfactory. INTERPRETATION: The DNMSQuest, a patient self-completed questionnaire for NMS assessment in CD patients, appears robust, reproducible, and valid in clinical practice showing a tangible impact of NMS on quality of life in CD. As there is no specific, comprehensive, validated tool to assess the burden of NMS in dystonia, the DNMSQuest can bridge this gap and could easily be integrated into clinical practice.

Comorbidity and retirement in cervical dystonia.

BACKGROUND: Cervical dystonia (CD) is the most common form of dystonia. The onset of CD is usually before 60 years of age and it may cause severe functional and psychosocial impairment in everyday life. Recently non-motor symptoms have been reported to occur in CD substantially affecting the quality of life. METHODS/PATIENTS: We studied comorbidities of patients with primary focal CD in Finland based on ICD-10 codes obtained from the care registry and patient records of 937 confirmed adult isolated focal CD patients between the years 2007-2016. The retirement months and diagnosis of retirement were calculated from pension registry information. The results were compared with 3746 age and gender-matched controls. RESULTS: Most prominent comorbidities with primary focal CD were depression (14%), anxiety (7%), and back pain (11%). The retirement age was significantly younger in CD patients compared to control group controls (59.0 years, 95% CI 58.5-59.5 vs. 61.7 years, 95% CI 61.6-61.9) years, p < 0.001). For dystonia patients the most common diagnoses for retirement due to sickness were dystonia (51%), depression (14%), and anxiety (8%). Patients with anxiety and depression retired earlier than other dystonia patients. DISCUSSION: Cervical dystonia considerably reduces working ability and leads to earlier retirement. Anxiety and depression are most notable comorbidities and their co-occurrence further reduces working ability. Our results suggest that more health care resources should be administered in treatment of CD to longer maintain working ability of CD patients. Further, psychiatric comorbidities should be taken into consideration in CD treatment.

Maladaptive striatal plasticity and abnormal reward-learning in cervical dystonia.

In monogenetic generalized forms of dystonia, in vitro neurophysiological recordings have demonstrated direct evidence for abnormal plasticity at the level of the cortico-striatal synapse. It is unclear whether similar abnormalities contribute to the pathophysiology of cervical dystonia, the most common type of focal dystonia. We investigated whether abnormal cortico-striatal synaptic plasticity contributes to abnormal reward-learning behavior in patients with focal dystonia. Forty patients and 40 controls performed a reward gain and loss avoidance reversal learning task. Participant's behavior was fitted to a computational model of the basal ganglia incorporating detailed cortico-striatal synaptic learning rules. Model comparisons were performed to assess the ability of four hypothesized receptor specific abnormalities of cortico-striatal long-term potentiation (LTP) and long-term depression (LTD): increased or decreased D1:LTP/LTD and increased or decreased D2: LTP/LTD to explain abnormal behavior in patients. Patients were selectively impaired in the post-reversal phase of the reward task. Individual learning rates in the reward reversal task correlated with the severity of the patient's motor symptoms. A model of the striatum with decreased D2:LTP/ LTD best explained the patient's behavior, suggesting excessive D2 cortico-striatal synaptic depotentiation could underpin biased reward-learning in patients with cervical dystonia. Reversal learning impairment in cervical dystonia may be a behavioral correlate of D2-specific abnormalities in cortico-striatal synaptic plasticity. Reinforcement learning tasks with computational modeling could allow the identification of molecular targets for novel treatments based on their ability to restore normal reward-learning behavior in these patients.

Improvement of quality of life and mental health in patients with spasmodic torticollis after microvascular decompression.

OBJECTIVE: Although not life threatening, spasmodic torticollis (ST) impairs patients' daily activity, socialization and work. The aim of this study was to evaluate the quality of life (QOL) and mental health in patients with ST after microvascular decompression (MVD). PATIENTS AND METHODS: From June 2014 to June 2017, patients with ST who underwent MVD in our department were included in this study. Toronto Western Sparse Torticollis Rating Scale (TWSTRS) were used to evaluate the ST symptoms. Quality of life was assessed by the craniocervical dystonia questionnaire (CDQ-24). Beck Depression Inventory (BDI-II) and Beck Anxiety Inventory (BAI) were used to evaluate the mental health. Intraoperative findings and follow-up results were analyzed. RESULTS: A total of 104 consecutive patients were enrolled in this study. At the 12 months follow-up, the total effective rate was 81.73%. After MVD surgery, 88(84.62%) ST patients experienced QOL improvement. The severity of ST symptoms was positively correlated with the CDQ-24 score(r = 0.31, P = 0.02). Forty-eight patients (46.16%) with ST have moderate to severe depression and nine (8.65%) have depression preoperatively. Pain and disability domains of TWSTRS were found have high relation with BDI-II score(r = 0.27, P = 0.02; r = 0.33, P = 0.03). There was a positive correlation of educational levels with the BDI-II scores(r = 0.45, P = 0.02). CONCLUSION: ST affects patients' QOL both physically and mentally. MVD for ST not only provides high spasm-relief rate but also leads to significantly higher QOL after surgery. Not only ST symptoms, but also psychiatric status of patients should be routinely followed. Psychological care and psychopharmaceuticals should also be considered for these patients.

The Prevalence and Correlation of Non-motor Symptoms in Adult Patients with Idiopathic Focal or Segmental Dystonia.

Background: Idiopathic focal dystonia is a motor syndrome associated with dysfunction of basal ganglia circuits. Observations have suggested that many other non-motor symptoms may also be part of the clinical picture. The aim was to assess the prevalence and correlation of non-motor symptoms in patients with common idiopathic focal or segmental dystonia. Methods: In a single-center cross-sectional case-control study, we evaluated the presence of pain, neuropsychiatric symptoms, and sleep alterations in 28 patients with blepharospasm, 28 patients with cervical dystonia, 24 patients with writer's cramp, and 80 control subjects matched for sex, age, and schooling. We obtained clinical and demographic data, and evaluated patients using the Fahn-Marsden Dystonia Rating Scale and other specific scales for dystonia. All subjects completed the following questionnaires: Beck Depression Inventory, Beck Anxiety Inventory, Social Phobia Inventory, Apathy Scale, Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index, Brief Pain Scale, and the World Health Organization Quality of Life brief scale. Results: The patients presented more symptoms of depression, anxiety, and apathy than the control subjects. They also reported worse quality of sleep and more pain complaints. Patients with blepharospasm were the most symptomatic subgroup. The patients had worse quality of life, and the presence of pain and symptoms of apathy and depression were the main influences for these findings, but not the severity of motor symptoms. Discussion: Patients with dystonia, especially those with blepharospasm, showed higher prevalence of symptoms of depression, anxiety, apathy, worse quality of sleep, and pain. These symptoms had a negative impact on their quality of life.

Evaluation of anxiety and depression scales and quality of LIFE in cervical dystonia patients on botulinum toxin therapy and their relatives.

OBJECTIVE: In this study, quality of life and psychiatric comorbid disorders were investigated in patients with cervical dystonia and their spouses and we also investigated the effect of botulinum toxin (BTX) treatment on these parameters. MATERIAL AND METHOD: Thirty patients with cervical dystonia (CD) on BTX treatment and their spouses (n = 30) were included. Beck Depression Scale (BDS), State-Trait Anxiety Inventory I and II (STAI-I, STAI-II), Hospital Anxiety Scale (HAS), Hospital Depression Scale (HDS) for psychiatric comorbid disease assessment, Toronto Western Spasmodic Torticollis Scale (TWSTRS) for disease activity assessment, and Craniocervical Dystonia Questionnaire (CDQ-24), Cervical Dystonia Impact Profile (CDIP-58), and Short Form 36 (SF-36) questionnaires for quality of life assessment were used. BDS, STAI-I and STAI-II, HAS, HDS, and SF-36 scales were also obtained from the spouses. The same tests were applied both before and 8 weeks after the BTX treatment. CONCLUSION: In our study, an increase in psychiatric comorbid disorders such as depression and anxiety was observed and the quality of life was adversely affected in all areas in patients. In the spouses of the patients, the rates of psychiatric comorbid disorders such as depression and anxiety were found to be increased when compared to healthy subjects while vitality, mental health, and general health perception were found to be negatively affected. Patients showed improvements in anxiety level, disease activity, and overall quality of life scales after BTX treatment.

Cortical modulation of brainstem circuits is abnormal in cervical dystonia.

OBJECTIVE: In healthy subjects, magnitude of hand-evoked blink reflex (BR) is increased if the stimulated hand is positioned in the peripersonal space. Here, we aimed to investigate the modulation of hand-evoked BR in peripersonal space to understand alterations of cortical modulations of subcortical structures in cervical dystonia. PATIENTS AND METHOD: We recruited 23 patients with idiopathic cervical dystonia and 21 healthy subjects, all of whom had hand-evoked BR. The reflex was recorded while stimulated hand was close to face ('peripersonal space) and far away from face (extrapersonal space). Comparisons were done between two conditions in each group. RESULTS: In extrapersonal space, magnitude of the reflex was bigger in patients compared to healthy subjects. In peripersonal space, magnitude of the reflex was increased in healthy subjects whereas it was reduced in patients. Comparisons of patients with and without sensory trick disclosed reflex magnitude was reduced in peripersonal space in patients with sensory trick whereas it did not change between two conditions in patients without sensory trick. DISCUSSION: Enhancement of hand-evoked BR is a normal phenomenon that provides evidence for top-down modulation of its neural circuitry. In cervical dystonia, the normal modulation is lost which suggests abnormal modulatory effect of higher-order centers upon brainstem circuits. The pattern of alteration also differs if there is no sensory trick.

Mind the gap: temporal discrimination and dystonia.

BACKGROUND AND PURPOSE: One of the most widely studied perceptual measures of sensory dysfunction in dystonia is the temporal discrimination threshold (TDT) (the shortest interval at which subjects can perceive that there are two stimuli rather than one). However the elevated thresholds described may be due to a number of potential mechanisms as current paradigms test not only temporal discrimination but also extraneous sensory and decision-making parameters. In this study two paradigms designed to better quantify temporal processing are presented and a decision-making model is used to assess the influence of decision strategy. METHODS: 22 patients with cervical dystonia and 22 age-matched controls completed two tasks (i) temporal resolution (a randomized, automated version of existing TDT paradigms) and (ii) interval discrimination (rating the length of two consecutive intervals). RESULTS: In the temporal resolution task patients had delayed (P = 0.021) and more variable (P = 0.013) response times but equivalent discrimination thresholds. Modelling these effects suggested this was due to an increased perceptual decision boundary in dystonia with patients requiring greater evidence before committing to decisions (P = 0.020). Patient performance on the interval discrimination task was normal. CONCLUSIONS: Our work suggests that previously observed abnormalities in TDT may not be due to a selective sensory deficit of temporal processing as decision-making itself is abnormal in cervical dystonia.

Social cognition and idiopathic isolated cervical dystonia.

For a long time, cervical dystonia (CD) has been characterised only by disturbances in motor functioning. Despite accumulating evidence for symptomatology in various non-motor domains, to date no study has investigated social cognition in CD. The aim of this study was to compare performance of CD patients and healthy controls in neurocognitive and socio-cognitive domain. Twenty-five non-depressed patients with CD and 26 healthy controls underwent neuropsychological testing. This involved assessment of cognitive status (general intellect, verbal memory, and executive function), and socio-cognitive functions using a Theory of mind task and self-report on empathy and emotion regulation. In comparison to controls, CD patients displayed significantly decreased cognitive abilities, particularly in executive function and verbal memory tasks. Difficulties in inferring mental states on both cognitive and affective levels were also observed. The largest discrepancies were detected in understanding intentionality in others. Poorer performance in cognitive and socio-cognitive tasks was unrelated to severity of the disease. This is the first evidence of compromised socio-cognitive functions in CD patients, highlighting this domain as another facet of non-motor symptoms of this disease. Future studies should advance our understanding of the extent, nature, and time course of these deficits in other aspects of social cognition in this patient population.

The relationship between the dopaminergic system and depressive symptoms in cervical dystonia.

PURPOSE: Cervical dystonia (CD) is associated with tremor/jerks (50%) and psychiatric complaints (17-70%). The dopaminergic system has been implicated in the pathophysiology of CD in animal and imaging studies. Dopamine may be related to the motor as well as non-motor symptoms of CD. CD is associated with reduced striatal dopamine D2/3 (D2/3) receptor and increased dopamine transporter (DAT) binding. There are differences in the dopamine system between CD patients with and without jerks/tremor and psychiatric symptoms. METHODS: Patients with CD and healthy controls underwent neurological and psychiatric examinations. Striatal DAT and D2/3 receptor binding were assessed using [123I]FP-CIT and [123I]IBZM SPECT, respectively. The ratio of specific striatal to non-specific binding (binding potential; BPND) was the outcome measure. RESULTS: Twenty-seven patients with CD and 15 matched controls were included. Nineteen percent of patients fulfilled the criteria for a depression. Striatal DAT BPND was significantly lower in depressed versus non-depressed CD patients. Higher DAT BPND correlated significantly with higher scores on the Unified Myoclonus Rating Scale (UMRS). The striatal D2/3 receptor BPND in CD patients showed a trend towards lower binding compared to controls. The D2/3 BPND was significantly lower in depressed versus non-depressed CD patients. A significant correlation between DAT and D2/3R BPND was found in both in patients and controls. CONCLUSIONS: Alterations of striatal DAT and D2/3 receptor binding in CD patients are related mainly to depression. DAT BPND correlates significantly with scores on the UMRS, suggesting a role for dopamine in the pathophysiology of tremor/jerks in CD.

[The efficacy of botulinotherapy in the correction of the pain syndrome and quality of life of patients with cervical dystonia]. / Éffektivnost' botulinoterapii v korrektsii stepeni bolevogo sindroma i kachestva zhizni patsientov s tservikal'noi distoniei.

AIM: To evaluate the severity of pain, emotional status and humoral serotonin in patients with cervical dystonia (CD) before and after the botulinotherapy. MATERIAL AND METHODS: A simple, open, comparative study of clinical characteristics of hyperkinesis, pain and emotional status, quality of life and contents of serum and blood platelet serotonin in 48 patients (32 women and 16 men) with CD, in age from 37 to 53 years, before and one month after the botulinotherapy with disport in dose of 500--1000 U was carried out. A control group included 15 healthy people. RESULTS: All patients (100%) complained of involuntary movements and pain in the neck. The overall score on a scale of dystonic movements in the group of patients was 16,7±7,7 points, on TWSTRS - 46,48±6,2 points, on the Visual Analogue Scale, the average level of pain was 6,4±1,08 points. The degree of depression according to the Hamilton scale was significantly higher (p<0.05) compared to the control group. The level of trait and state anxiety measured with the Spielberger-Khanin scale was significantly higher (p<0.005) in patients with CD than in the controls. The correlation analysis revealed a direct dependence of the intensity of pain subscale TWSTRS with the degree of anxiety on the Hamilton scale and the amount of final points of dystonic movements. The level of serotonin in the serum was significantly lower in patients compared to the controls. After botulinotherapy, pain scores, anxiety and depression have significantly decreased and the level of blood platelet serotonin has increased. CONCLUSION: Botulinotherapy with dysport in CD patients reduces the degree of pain, depression, improves quality of life and stimulates the serotoninergic system.