Pandemic Acceptance and Commitment to Empowerment Response (PACER) Training: Protocol for the Development and Rapid-Response Deployment.

BACKGROUND: During a global pandemic, it is critical to rapidly deploy a psychological intervention to support the mental health and resilience of highly affected individuals and communities. OBJECTIVE: This is the rationale behind the development and implementation of the Pandemic Acceptance and Commitment to Empowerment Response (PACER) Training, an online, blended, skills building intervention to increase the resilience and well-being of participants while promoting their individual and collective empowerment and capacity building. METHODS: Based on acceptance and commitment therapy (ACT) and social justice-based group empowerment psychoeducation (GEP), we developed the Acceptance and Commitment to Empowerment (ACE) model to enhance psychological resilience and collective empowerment. The PACER program consists of 6 online, interactive, self-guided modules complemented by 6 weekly, 90-minute, videoconference, facilitator-led, group sessions. RESULTS: As of August 2021, a total of 325 participants had enrolled in the PACER program. Participants include frontline health care providers and Chinese-Canadian community members. CONCLUSIONS: The PACER program is an innovative intervention program with the potential for increasing resilience and empowerment while reducing mental distress during the pandemic. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/33495.

Advanced Therapies for the Management of Dopamine Dysregulation Syndrome in Parkinson's Disease.

BACKGROUND: Dopamine Dysregulation Syndrome (DDS) is an adverse non-motor complication of dopamine replacement therapy in Parkinson's disease. The current literature on this syndrome is limited, and it remains underdiagnosed and challenging to manage. OBJECTIVE: To assess the role of advanced therapies in the management of DDS. METHODS: We performed a retrospective chart review and identified patients who fit the inclusion criteria for DDS. They were classified according to risk factors that have been identified in the literature, motor and complication scores, intervention (medical or surgical) and outcome. Multivariate analyses were performed to analyze these characteristics. RESULTS: Twenty-seven patients were identified (23 males, mean age of onset: 49 ± 8.8 years). Average levodopa equivalent daily dose was 1916.7 ± 804 mg and a history of impulse control disorders, psychiatric illness, and substance abuse was present in 89%, 70% and 3.7% of the patients, respectively. Overall 81.5% of patients had symptom resolution at follow up, on average 4.8 ± 3.5 years after management, with medication only (7/9), levodopa-carbidopa intestinal gel (1/3), deep brain stimulation of subthalamic nucleus (10/13), or globus pallidus pars interna (2/2). Reduction of medications occurred with deep brain stimulation of subthalamic nucleus (P = 0.01) but was associated with a relapse in two patients. CONCLUSION: Although the small sample size of some subgroups limits our ability to draw meaningful conclusions, our results did not suggest superiority of a single treatment option. Advanced therapies including deep brain stimulation can be considered in patients with DDS refractory to conservative measures, but outcome is variable and relapse is possible.

Functional Dyskinesias following Subthalamic Nucleus Deep Brain Stimulation in Parkinson's Disease: A Report of Three Cases.

BACKGROUND: Functional (psychogenic) dyskinesias in patients with Parkinson's disease (PD) are exceedingly rare. CASES: Herein we report three patients with PD who presented with functional dyskinesias in the first 3 months after subthalamic nucleus deep brain stimulation (DBS). All patients presented with chorea mimicking levodopa or stimulation-induced dyskinesias in the first 24 hours following stimulation adjustment. Two patients had generalized chorea and one, hemichorea. In all patients the abnormal movements could be induced or resolved with placebo/nocebo changes to the stimulation parameters. Following the diagnosis of a functional movement disorder (FMD), all patients improved with appropriate management. CONCLUSIONS: Functional chorea following DBS might mimic organic dyskinesias in PD but can be accurately diagnosed using suggestibility and placebo responses to sham stimulation adjustments. Recognizing the presence of FMD following DBS is important for proper management of these patients.

Anxiety in Parkinson's disease: Abnormal resting activity and connectivity.

Anxiety is a very common yet poorly understood symptom of Parkinson's disease. We investigated whether Parkinson's disease patients experiencing anxiety share neural mechanisms described in the general population with involvement of critical regions for the control of behaviour and movement. Thirty-nine patients with PD were recruited for this study, 20 with higher anxiety scores and 19 with lower anxiety scores. They all underwent a resting-state fMRI scan, while they were on medication. The amplitude of low-frequency fluctuation (ALFF) and seed-based connectivity were investigated to reveal the changes of the spontaneous activity and the interaction among different related regions. The results provided evidence that anxiety in Parkinson's disease is associated with the over-activation of the amygdala and impaired inter-relationship of regions involved in behavior (i.e. medial prefrontal cortex, insula) and motor control (i.e. basal ganglia).

Psychiatric associations of adult-onset focal dystonia phenotypes.

BACKGROUND: Depression and anxiety frequently accompany the motor manifestations of isolated adult-onset focal dystonias. Whether the body region affected when this type of dystonia first presents is associated with the severity of these neuropsychiatric symptoms is unknown. OBJECTIVES: The aim of this study was to determine whether depression, anxiety and social anxiety vary by dystonia onset site and evaluate whether pain and dystonia severity account for any differences. METHODS: Patients with isolated focal dystonia evaluated within 5 years from symptom onset, enrolled in the Natural History Project of the Dystonia Coalition, were included in the analysis. Individual onset sites were grouped into five body regions: cervical, laryngeal, limb, lower cranial and upper cranial. Neuropsychiatric symptoms were rated using the Beck Depression Inventory, Hospital Anxiety and Depression Scale and Liebowitz Social Anxiety Scale. Pain was estimated using the 36-Item Short Form Survey. RESULTS: Four hundred and seventy-eight subjects met our inclusion criteria. High levels of depression, anxiety and social anxiety occurred in all groups; however, the severity of anxiety and social anxiety symptoms varied by onset site group. The most pronounced differences were higher anxiety in cervical and laryngeal, lower anxiety in upper cranial and higher social anxiety in laryngeal. Increases in pain were associated with worse neuropsychiatric symptom scores within all groups. Higher anxiety and social anxiety in laryngeal and lower anxiety in upper cranial persisted after correcting for pain and dystonia severity. CONCLUSION: Anxiety and social anxiety severity vary by onset site of focal dystonia, and this variation is not explained by differences in pain and dystonia severity.

The Relationship Between Serotonin-2A Receptor and Cognitive Functions in Nondemented Parkinson's Disease Patients with Visual Hallucinations.

BACKGROUND: There is growing evidence that the serotonergic system, in particular serotonin 2A receptors, is involved in neuropsychiatric symptoms in Parkinson's disease (PD), including cognitive processing and visual hallucinations. However, the relationship between serotonin 2A receptor availability, visual hallucinations, and cognitive profile is unknown. The objective of this study was to investigate the level of serotonin 2A receptor availability in brain regions affected by visual hallucinations and to test the association with cognitive/behavioral changes in patients who have PD with visual hallucinations. METHODS: Nondemented patients who had PD with (n = 11) and without (n = 8) visual hallucinations and age-matched controls (n = 10) were recruited. All participants completed neuropsychological testing, which consisted of visuoperceptual, executive, memory, language, and frontal-behavioral function. Positron emission tomography scans using [18F]setoperone, a serotonin 2A antagonist radioligand, were acquired in patients with PD, and a parametric binding potential map of [18F]setoperone was calculated with the simplified reference tissue model using the cerebellum as a reference. RESULTS: Patients who had PD with visual hallucinations exhibited significantly lower scores on measures of executive and visuoperceptual functions compared with age-matched controls. These changes were paralleled by decreased [18F]setoperone binding in the right insula, bilateral dorsolateral prefrontal cortex, right orbitofrontal cortex, right middle temporal gyrus, and right fusiform gyrus. The psychometric correlation analysis revealed significant relationships among tests associated with visuoperceptual function, memory and learning, and serotonin 2A binding in different prefrontal and ventral visual stream regions. There was also reduced serotonin 2A receptor binding in patients who had PD with depression. CONCLUSIONS: These findings support a complex interaction between serotonin 2A receptor function and cognitive processing in patients who have PD with visual hallucinations.

Clinimetric testing of the comprehensive cervical dystonia rating scale.

INTRODUCTION: The aim of this study was to test the clinimetric properties of the Comprehensive Cervical Dystonia Rating Scale. This is a modular scale with modifications of the Toronto Western Spasmodic Torticollis Rating Scale (composed of three subscales assessing motor severity, disability, and pain) now referred to as the revised Toronto Western Spasmodic Torticollis Scale-2; a newly developed psychiatric screening instrument; and the Cervical Dystonia Impact Profile-58 as a quality of life measure. METHODS: Ten dystonia experts rated subjects with cervical dystonia using the comprehensive scale. Clinimetric techniques assessed each module of the scale for reliability, item correlation, and factor structure. RESULTS: There were 208 cervical dystonia patients (73% women; age, 59 ± 10 years; duration, 15 ± 12 years). Internal consistency of the motor severity subscale was acceptable (Cronbach's alpha = 0.57). Item to total correlations showed that elimination of items with low correlations (<0.20) increased alpha to 0.71. Internal consistency estimates for the subscales for disability and pain were 0.88 and 0.95, respectively. The psychiatric screening scale had a Cronbach's alpha of 0.84 and satisfactory item to total correlations. When the subscales of the Toronto Western Spasmodic Torticollis Scale-2 were combined with the psychiatric screening scale, Cronbach's alpha was 0.88, and construct validity assessment demonstrated four rational factors: motor; disability; pain; and psychiatric disorders. The Cervical Dystonia Impact Profile-58 had an alpha of 0.98 and its construction was validated through a confirmatory factor analysis. CONCLUSIONS: The modules of the Comprehensive Cervical Dystonia Rating Scale are internally consistent with a logical factor structure.

Subjective perceived outcome of subthalamic deep brain stimulation in Parkinson's disease one year after surgery.

OBJECTIVES: Dissatisfaction with subthalamic deep brain stimulation (STN-DBS) despite motor improvements has been observed in Parkinson's disease (PD). Hence, we compared patient's subjective perceived outcome 12 months after surgery (12mFU) with clinical measures to identify risk factors of dissatisfaction. METHODS: Patients were examined at baseline and 12mFU. Quality of life (QoL), neuropsychiatric, cognitive and neurological functioning was measured. Patients were classified concerning their subjective outcome (negative = dissatisfaction; mixed; positive = satisfaction) at 12mFU using semi-structured interviews. First, the three groups were compared concerning interview statements. Second, repeated measures ANOVAs with group as between-subjects factor were applied to find significant effects of time, group, or interaction. Third, binary logistic regression determined predictors of dissatisfaction. RESULTS: Of the 28 enrolled patients, 25% perceived their outcome as negative, 32.1% as mixed, and 42.9% as positive. Concerning interview statements, dissatisfied patients mentioned significantly less often improved QoL and reduced medication, and reported worsening of mental state, and social interaction. For the whole sample, significant improvement over time was found for motor functioning, daily dopamine dosages, and QoL. Apathy significantly worsened over time, but dissatisfied patients were overall more apathetic and depressed than the other groups. Significant interaction of group and time was identified for QoL, which only improved in the mixed and satisfied group. Finally, preoperative apathy and axial symptoms predicted dissatisfaction with STN-DBS. CONCLUSIONS: Although motor symptoms and QoL improved in the whole sample, 25% of patients showed disappointment with STN-DBS. Especially apathy predicts dissatisfaction and should be considered preoperatively.

Progression of neuropsychiatric and cognitive features due to exons 2 to 5 deletion in the epsilon-sarcoglycan gene: a case report.

Physical symptoms of myoclonus dystonia due to epsilon-sarcoglycan mutations are well documented; however, the progression of neuropsychiatric and cognitive symptoms remains unclear. We present a case of a 34-year-old woman with early childhood onset of myoclonic jerks, dystonic posture and developmental delay due to exons 2 to 5 deletion in the epsilon-sarcoglycan gene. Over time, she developed neuropsychiatric symptoms. She underwent bilateral deep brain stimulation of the ventral intermediate nucleus of the thalamus for her motor symptoms, which greatly improved but she exhibited slow deterioration of her neuropsychiatric and cognitive symptoms, particularly apathy, aggression and severe executive dysfunction.

Long-term neuropsychiatric outcomes after pallidal stimulation in primary and secondary dystonia.

OBJECTIVE: To evaluate changes in the diagnosis of Axis I psychiatric disorders in patients with primary and secondary dystonia after deep brain stimulation (DBS) of the globus pallidus internus (GPi). METHODS: Structured Clinical Interviews for the DSM-IV, Axis I psychiatric disorders, were prospectively performed before and after surgery. Diagnoses were made based on DSM-IV criteria. Psychiatric disorders were grouped into 5 categories: mood, anxiety, addiction, obsessive-compulsive disorders, and psychosis. Patients could be stratified to more than one category. Rates for unchanged diagnoses, diagnoses in remission, and new-onset diagnoses after surgery for each category were calculated. RESULTS: Fifty-seven patients with primary and secondary dystonia were included. Mean ± SD age at surgery and dystonia duration at time of surgery was 50.6 ± 13.8 and 19.0 ± 13.2 years, respectively. Preoperatively, 37 Axis I diagnoses were made in 25 patients, 43.8% of those presenting with at least 1 Axis I diagnosis (mostly mood and anxiety disorders). Mean ± SD duration of psychiatric follow-up was 24.4 ± 19.6 months. Overall, after surgery no significant changes (p = 0.16) were found in Axis I diagnoses (23 patients, 40.3%): 27 (73%) unchanged, 10 (27%) in complete remission, and 4 (12.9%) new-onset diagnoses. CONCLUSIONS: Our results support the overall psychiatric stability of patients with primary and secondary dystonia treated with GPi DBS. However, considering the high psychiatric morbidity in the dystonia population, psychiatric assessments before and after surgery are strongly recommended. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that GPi DBS does not change Axis I psychiatric diagnoses in patients with primary and secondary dystonia.

Developments in impulse control behaviours of Parkinson's disease.

PURPOSE OF REVIEW: Impulse control behaviours (ICBs) are a frequent comorbidity for patients with Parkinson's disease. They consist of impulse control disorders, dopamine dysregulation syndrome, and punding. The field continues to evolve in the understanding of impulsivity and assessment of risk factors in the development of these behaviours and their appropriate management in patients with Parkinson's disease. RECENT FINDINGS: Impulsivity is a multifaceted concept that is surprisingly common in untreated patients with Parkinson's disease. The incidence of ICBs increases with demographic, clinical, and biochemical risk factors. Treatments rely on reduction of dopamine agonists with exception of cognitive behavioural therapy and possibly repetitive transcranial magnetic stimulation. SUMMARY: Reduction of dopamine agonist dose is the mainstay of treatment of ICBs. Other forms of dopaminergic treatment such as deep brain stimulation or jejunal infusion are alternative treatments but may be complicated by dopamine agonist withdrawal syndrome. Other therapies show promise but data are insufficient to suggest their regular use.

Development of the Comprehensive Cervical Dystonia Rating Scale: Methodology.

We present the methodology utilized for development and clinimetric testing of the Comprehensive Cervical Dystonia (CD) Rating scale, or CCDRS. The CCDRS includes a revision of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS-2), a newly developed psychiatric screening tool (TWSTRS-PSYCH), and the previously validated Cervical Dystonia Impact Profile (CDIP-58). For the revision of the TWSTRS, the original TWSTRS was examined by a committee of dystonia experts at a dystonia rating scales workshop organized by the Dystonia Medical Research Foundation. During this workshop, deficiencies in the standard TWSTRS were identified and recommendations for revision of the severity and pain subscales were incorporated into the TWSTRS-2. Given that no scale currently evaluates the psychiatric features of cervical dystonia (CD), we used a modified Delphi methodology and a reiterative process of item selection to develop the TWSTRS-PSYCH. We also included the CDIP-58 to capture the impact of CD on quality of life. The three scales (TWSTRS2, TWSTRS-PSYCH, and CDIP-58) were combined to construct the CCDRS. Clinimetric testing of reliability and validity of the CCDRS are described. The CCDRS was designed to be used in a modular fashion that can measure the full spectrum of CD. This scale will provide rigorous assessment for studies of natural history as well as novel symptom-based or disease-modifying therapies.

The impact of subthalamic deep brain stimulation on caregivers of Parkinson's disease patients: an exploratory study.

To study the caregivers' perception of their own well-being 1 year after subthalamic deep brain stimulation (STN-DBS) surgery in Parkinson's disease (PD) patients, using a qualitative and quantitative approach. 25 patients and caregivers, living together in partnerships, were examined before and at 3-month and 1-year follow-up (FU) after STN-DBS surgery. Semi-structured FU interviews concerning caregivers' own well-being under STN-DBS were conducted and analyzed: caregivers were accordingly assigned to positive or negative outcome groups. Quality of life (QoL), depression, apathy and anxiety of caregivers and patients were measured. These quantitative data were compared to the 1-year FU interview outcomes. Multiple comparisons analyzed caregiver group assignments based on these measurements. Logistic regression was used to find predictors. Additionally, patients' mood ratings were used in multiple comparisons with caregivers' subjective outcome, to analyze the interaction of patient and caregiver ratings. At 3-month FU, caregivers were more indecisive concerning their own well-being than at 1-year FU. At 1-year FU, caregivers from the negative group had greater depression, anxiety and lower QoL ratings. They were significantly older compared to the positive group. Patients' depression showed significantly stronger improvement in the positive outcome group. Patients' apathy and depression ratings were significant covariates of caregivers' QoL. Our results show that at 1-year FU over 50 % of the caregivers rated their subjective well-being as negative. Especially older and more depressed caregivers are at risk. These caregivers and their partners should be monitored more closely to identify possible problems and help them adapt following surgery.

Parkinson's disease patients with subthalamic stimulation and carers judge quality of life differently.

BACKGROUND: Quality of life (QoL) improves under subthalamic deep brain stimulation (STN-DBS) in Parkinson's disease (PD), whereas social functioning may be disrupted. This disruption could negatively influence the family dynamic, leading to different perceptions of the STN-DBS outcome by patients and caregivers. METHODS: We recruited 34 PD patients for this prospective, controlled trial, 28 of whom were examined preoperatively, three months and one year after STN-DBS surgery. The primary outcome was QoL. We compared the patients' ratings and caregivers' proxy QoL ratings. The secondary outcome was social functioning. Additionally, neurological, neuropsychiatric and cognitive domains were analyzed. Changes were analyzed with repeated-measures ANOVA. Regression analysis was used to determine the association between QoL and social functioning. RESULTS: Patients' QoL improved significantly under STN-DBS (p = .003). At baseline, patients' and caregivers' QoL ratings were similar. However, one year postoperatively, QoL ratings differed significantly (p = .010), whereby QoL was rated worse by caregivers. Social functioning was positively influenced during the first months postoperatively, but did not improve longitudinally. One year postoperatively, social functioning was significantly associated with QoL ratings (patients: p = .004, caregivers: p = .002). Motor scores significantly improved, whereas verbal fluency and apathy worsened. CONCLUSIONS: Unequal perception of QoL between patients and caregivers exists under STN-DBS. The fact that social functioning does not improve longitudinally is perhaps due to patient's higher levels of apathy and reduced motivation following surgery. Our findings stress the importance of considering caregiver's input in DBS patients' outcomes and the need for pre-operative preparation.

Reluctance to start medication for Parkinson's disease: a mutual misunderstanding by patients and physicians.

Reluctance to start medication has never been investigated before in PD. We studied reluctance to start medication for PD motor symptoms, namely its prevalence, underlying reasons, drug-specificity, and associated delay in the start of PD medication. A cross-sectional observational international study was conducted. Patients with a clinical diagnosis of PD advised to start antiparkinsonian medication in the previous 5 years were invited to complete a questionnaire in three centers located in North America and Europe. An electronic online survey was sent to physicians through the mailing list of the Movement Disorder Society. 469 participants (201 PD patients, 268 physicians). 40.2% (n = 82) of the patients reported reluctance to start medication, but 88.6% (n = 234/264) of the physicians estimated that ≤20% of their patients with PD had been reluctant to start medication. The most common reasons reported by patients were the fear of side effects (n = 35, 55.6%), followed by non-acceptance of diagnosis (n = 23, 36.5%); fear of a temporally limited benefit was more commonly selected by physicians (n = 92/267, 34.5%). Patients indicated reluctance to start DAs more frequently compared with L-DOPA (OR: 2.22, 95% CI: 1.30, 9.03; p = 0.013) while physicians perceived L-DOPA to be associated with more reluctance (OR: 4.7, 95% CI: 3.41; 6.59; p < 0.0001). Patients with PD and physicians have a different perspective on the issue of reluctance to start medication. There is a need to bring physicians and patients with PD closer to a shared vision of the problem reluctance to start medication.