OBJECTIVES:The purpose of this study was to investigate the effect of group Cognitive Stimulation Therapy (CST) for people living with Parkinson's disease and dementia (PDD) and their care partners. METHOD:This study used a within-subjects pre-test post-test design. Participants were volunteers living with PDD and their care partners (n = 20 each). Participants living with PDD participated in 7 wk of bi-weekly 1-h long CST group classes. They completed pre and post cognition, health-related quality of life, depressive symptoms, and relationship quality assessments, care partners completed pre and post relationship quality and caregiver burden assessments, and both groups completed a satisfaction questionnaire at post. RESULTS:Among participants living with PDD, cognition improved from pre to post (p = 0.002). Care partners reported needing to provide assistance in more tasks from pre to post intervention (p = 0.04). Both groups reported 'good' to 'excellent' satisfaction on average at post. CONCLUSION:Participation in a group CST course resulted in improved cognition in this small sample of people living with PDD. In addition, participants living with PDD and their care partners were satisfied with the program, although supporting attendance may have increased burden on care partners. Although preliminary, these findings suggest that group CST may be beneficial for people living with PDD.

IMPORTANCE:Cognitive impairment is a common manifestation of Parkinson's disease (PD) that can limit participation. A clearer understanding of how people with PD (PwPD) use strategies to support their functional cognition can guide interventions designed to promote occupational performance. OBJECTIVE:To investigate strategy use by PwPD during a functional cognitive task and how it relates to task performance. DESIGN:Cross-sectional within and between groups. SETTING:Participants' homes. PARTICIPANTS:PwPD without dementia (N = 51) were allocated into two groups according to Montreal Cognitive Assessment score: ≥26 for those with PD and normal cognition (PD-NC; n = 27) and ≤25 for those with PD and mild cognitive impairment (PD-MCI; n = 24). OUTCOMES AND MEASURES:Performance outcomes (accuracy, total time, and efficiency) and strategy use on the Level 2 Adult/Older Adult version of the Weekly Calendar Planning Activity (WCPA). RESULTS:In the whole PD group, increased strategy use correlated with higher accuracy and longer total time, and four specific strategies correlated with higher accuracy (p < .04). The PD-NC group had higher accuracy and used more strategies than the PD-MCI group (p < .02), including three strategies associated with higher accuracy (cross off entered appointments, enter fixed appointments first, cross off free day; p < .03). In the PD-NC group, increased strategy use correlated with higher accuracy and longer total time (p < .02). Strategy use and WCPA performance outcomes did not correlate in the PD-MCI group (p > .10). CONCLUSIONS AND RELEVANCE:This study adds to the understanding of how PwPD select and use strategies to support their functional cognition and suggests that PwPD could potentially benefit from interventions to optimize strategy use in occupational performance. Plain-Language Summary: People with Parkinson's disease experience changes in thinking and memory that affect their ability to participate in daily activities, and they use fewer strategies to support their cognition than their peers without Parkinson's disease. We investigated the strategies people with Parkinson's disease use to support their thinking and memory during a simulated functional activity, the Weekly Calendar Planning Activity. We sought to understand whether the strategies they used were related to better performance on the activity, and we explored differences in strategy use between people with Parkinson's disease with normal cognition and those with Parkinson's disease with possible mild cognitive impairment. We found that people with Parkinson's disease who used more strategies performed more accurately. Participants with Parkinson's disease with normal cognition used strategies that supported their accuracy but took longer to complete the task. Participants with Parkinson's disease with possible mild cognitive impairment used fewer strategies and ones that did not support their performance. Our findings indicate that people with Parkinson's disease may benefit from interventions focused on selecting and using strategies to improve their daily functioning.

IMPORTANCE:Cognitive impairment is a common and disabling feature of Parkinson's disease (PD), and interventions to mitigate its negative functional consequences are in high demand. Metacognitive strategy interventions, such as the Multicontext (MC) Approach, may support daily function among people with PD (PwPD). OBJECTIVE:To determine feasibility, participant acceptance, and preliminary estimates of the MC Approach's treatment effect for PwPD. DESIGN:Quasi-randomized controlled pilot trial. Participants underwent pretreatment assessment, allocation to treatment group (MC, n = 29; control, n = 28), 10 treatment sessions, 1-wk posttreatment assessment, and 3-mo questionnaire follow-up. SETTING:Participants' homes. PARTICIPANTS:PwPD without dementia but with subjective cognitive concerns. INTERVENTION:Ten weekly sessions of the MC Approach, which aimed to develop awareness and strategies to control cognitive performance across activities via therapist mediation, functional activity performance, and homework. The control intervention used the same structure and treatment activities but did not address awareness or strategy use or use mediated learning. OUTCOMES AND MEASURES:Indicators of trial feasibility (recruitment, retention, study duration), participant acceptance (satisfaction, homework completion), and treatment effect (self-rated functional cognitive goal performance). RESULTS:We enrolled 3 participants/mo and had 87% retention. Both groups' satisfaction and homework completion were high. Compared with control participants, MC participants reported greater improvement in functional cognitive goal performance from preintervention to postintervention that was maintained at follow-up. CONCLUSIONS AND RELEVANCE:The MC Approach is a feasible, acceptable, and potentially efficacious intervention to address the functional cognitive goals of PwPD without dementia. A larger, fully randomized trial is required to provide definitive efficacy data. Plain-Language Summary: Cognitive impairment is one of the most common and disabling features of Parkinson's disease. As such, cognitive interventions that support daily function for this population are in high demand. The purpose of this study was to establish the feasibility of one such potential intervention, the Multicontext (MC) Approach, among people with Parkinson's disease (PwPD) without dementia. We also wanted to generate preliminary estimates of its effect on everyday cognitive function to inform future definitive trials. In a pilot quasi-randomized controlled trial, we found that the MC Approach is feasible, safe, and acceptable for people with PwPD. We also found that it may improve self-rated performance of daily cognitive goals. We can now proceed with a full-scale randomized controlled trial to determine its efficacy. Ultimately, this work will meet the pressing need for evidence-based cognitive interventions that improve or maintain occupational performance and participation among PwPD.

BACKGROUND:Few studies have investigated the role of school feeding in low- and middle-income countries as a means of improving childhood cognition. Peanut/milk ready-to-use food (PM-RUF) or cowpea offers an affordable, scalable option that might improve cognition. OBJECTIVES:To determine whether micronutrient-fortified PM-RUF or peanut/cowpea ready-to-use food (PC-RUF) would improve fluid cognition as assessed by 4 tests from the National Institutes of Health Toolbox Cognitive Battery when compared with a micronutrient-fortified millet porridge (FP) after a year of school feeding. METHODS:An individually randomly assigned, investigator-blinded, controlled clinical trial was conducted at 6 schools in Mion District in rural northern Ghana. Eight hundred seventy-one school children aged 5-12 y were randomly assigned and allocated to receive PM-RUF (n = 282), PC-RUF (n = 292), or FP (n = 297), each providing ∼400 kcal/d. The primary outcomes were 4 fluid cognition test scores: Dimensional Change Card Sort test, Flanker Inhibitory Control and Attention test, Pattern Comparison Processing Speed test, and a modified List Sorting Working Memory test. Secondary outcomes included a composite median ranking of the 4 primary outcomes and anthropometry changes. RESULTS:Among the 871 participants (median age, 8.8 y; 47% female), 795 (91%) completed endline cognitive testing. Median attendance rates exceeded 87% in all groups. PM-RUF group demonstrated better fluid cognition on the Dimensional Change Card Sort test [odds ratio (OR): 1.5; 95% CI: 1.1, 2.0; P = 0.016] and Pattern Comparison Processing Speed test (OR: 1.4; 95% CI: 1.0, 1.9; P = 0.026) than FP, whereas there were no significant differences on Flanker Inhibitory Control and Attention or List Sorting Working Memory tests. PC-RUF group demonstrated no improvement over FP on any cognitive tests. PM-RUF group had superior fluid cognition composite median rankings (OR: 1.5; 95% CI: 1.1, 2.0; P = 0.007). CONCLUSIONS:Among rural Ghanaian children aged 5-12 y, PM-RUF compared with FP resulted in superior fluid cognition. This trial was registered at clinicaltrials.gov as NCT04349007.

Wolfram syndrome is a rare disease characterized by diabetes, neurodegeneration, loss of vision, and audition. We recently found, in a young sample of participants (mean age 15 years), that Wolfram syndrome was associated with impairment in smell identification with normal smell sensitivity and whole-mouth taste function. However, these senses were assessed separately, and it is unknown whether smell-taste interactions are altered in Wolfram syndrome, which was the focus of this study. Participants with Wolfram syndrome (n = 36; 18.2 ± 6.8 years) and sex-age-equivalent healthy controls (n = 34) were assessed with a battery of sensory tests. Using sip-and-spit methods, participants tasted solutions containing gustatory and olfactory stimuli (sucrose with strawberry extract, citric acid with lemon extract, sodium chloride in vegetable broth, and coffee) with and without nose clips, and rated perceived taste and retronasal smell intensities using the generalized Labeled Magnitude Scale. Participants also completed n-butanol detection thresholds and the University of Pennsylvania Smell Identification Test (UPSIT). Retronasal smell increased taste intensity of sucrose, sodium chloride, and coffee solutions similarly in both groups (P values <0.03). Compared with the control group, participants in the Wolfram group had lower UPSIT scores and reduced smell sensitivity, retronasal intensity, and saltiness (P values <0.03), but rated other taste intensities similarly when wearing the nose clip. Despite impairments in orthonasal smell identification, odor-induced taste enhancement was preserved in participants with Wolfram syndrome who still had some peripheral olfactory function. This finding suggests that odor-induced taste enhancement may be preserved in the presence of reduced olfactory intensity.

The Weekly Calendar Planning Activity (WCPA) may improve understanding of functional cognition in people with Parkinson disease (PwPD) without dementia. We aimed to determine if WCPA performance (a) discriminates between PwPD with and without cognitive impairment and healthy controls and (b) correlates with other indicators of cognition and daily function. This was a cross-sectional study. Parkinson disease (PD) participants without dementia were divided into normal cognition (PD-NC, n = 25) and possible mild cognitive impairment (PD-MCI, n = 21) groups. Their WCPA performance was compared with that of a normative sample (n = 196) and correlated with neuropsychological test performance and self-reported cognition and participation. Both the PD-MCI and PD-NC groups had impaired WCPA performance. WCPA performance correlated with executive function, processing speed, and self-reported cognition and participation. The WCPA can detect functional cognitive deficits in PwPD without dementia and can inform occupational therapy interventions to support functional cognition, occupational performance, and participation in this population.

Background Wolfram Syndrome is a rare genetic disorder usually resulting from pathogenic variation in the WFS1 gene, which leads to an exaggerated endoplasmic reticulum (ER) stress response. The disorder is typically characterized by diabetes insipidus, diabetes mellitus, optic nerve atrophy, hearing loss, and neurodegenerative features. Existing literature suggests it may also have psychiatric manifestations. Objective To examine lifetime psychiatric diagnoses and medication history in Wolfram Syndrome. Method Child, adolescent, and young adult Wolfram Syndrome participants (n=39) were assessed by a child & adolescent psychiatrist to determine best estimate DSM-5 lifetime psychiatric diagnoses as well as psychoactive medication history. In addition, the Child & Adolescent Symptom Inventory-5 (CASI-5) Parent Checklist was used to determine likely psychiatric diagnoses based on symptom counts in Wolfram Syndrome patients (n=33), type 1 diabetes (n=15), and healthy comparison (n=18) groups. Results Study participants with Wolfram Syndrome had high lifetime rates of anxiety disorders (77%). Also, 31% had an obsessive-compulsive spectrum disorder, 33% had a mood disorder, 31% had a neurodevelopmental or disruptive behavior disorder, and 31% had a sleep-wake disorder. More than half of Wolfram Syndrome participants had taken at least one psychoactive medication, and one third had taken at least one selective serotonin reuptake inhibitor (SSRI). Some individuals reported poor response to sertraline but better response after switching to another SSRI (fluoxetine or citalopram). In general, people with Wolfram Syndrome often reported benefit from psychotherapy and/or commonly used psychoactive medications appropriate for their psychiatric diagnoses. Conclusions Wolfram Syndrome may be associated with elevated risk for anxiety and obsessive-compulsive spectrum disorders, which seem generally responsive to usual treatments for these disorders.

Deep-brain stimulation (DBS) of the ventro-intermediate nucleus of the thalamus (VIM) can provide substantial clinical motor benefit to Essential Tremor (ET) patients. However, the DBS impact on the functional connectivity (FC) of networks is difficult to study using standard neuroimaging modalities either due to limited temporal resolution (PET) or safety concerns from contraindications (fMRI). In this study, we tested the feasibility and sensitivity of High-Density Diffuse Optical Tomography (HD-DOT), which avoids these concerns, for mapping cortical blood flow responses to sensory stimuli and measuring resting state cortical FC in ET patients with VIM DBS OFF vs ON.

OBJECTIVE. To investigate the performance of cognitively demanding instrumental activities of daily living (IADLs) among people with Parkinson’s disease (PD) without dementia. METHOD. Seventy-seven participants with PD and 57 participants without PD underwent standardized, performance-based IADL evaluation using the Performance Assessment of Self-care Skills. Activity performance was rated for independence, adequacy, and safety. RESULTS. The PD group had lower independence and adequacy scores than the non-PD group for almost every activity. Medication management, shopping, and sharp utensil use were the activities most sensitive to group differences. In the PD group, older age, lower Mini-Mental State Examination scores, and decreased motor function were associated with poorer IADL performance. CONCLUSIONS. People with relatively early and mild PD demonstrated measurable deficits in the performance of cognitively demanding IADLs. This work highlights the importance of using objective assessments of IADL function to detect early functional changes in people with PD.

BACKGROUND : Wolfram syndrome is a rare genetic disease characterized by insulin-dependent diabetes, optic nerve atrophy, sensorineural hearing loss and neurodegeneration. Although olfactory dysfunction, a classical clinical marker of neurodegenerative processes, has been reported in Wolfram syndrome, its use as a clinical marker in Wolfram is limited due to data scarcity. In addition, it is unknown whether Wolfram syndrome affects the sense of taste. METHODS: Smell and taste perception were assessed in participants with Wolfram syndrome (n=40) who were 15.1 ± 6.0 years of age (range: 5.1- 28.7 years) and two sex- and age-matched control groups: one group with type 1 diabetes mellitus (T1D; n=25) and a healthy control group (HC; n=29). Smell sensitivity was assessed by measuring n-butanol detection thresholds and smell identification by using the University of Pennsylvania Smell Identification Test (UPSIT). Taste function was assessed using NIH Toolbox, which includes the assessment of sucrose (sweet) taste preference, and perceived intensity of sucrose, sodium chloride (salty), and quinine hydrochloride (bitter) both in the tip of the tongue (regional test) and the whole mouth. RESULTS: Smell sensitivity was not significantly different among groups; however, smell identification was impaired in Wolfram syndrome, as reflected by significantly lower UPSIT scores in Wolfram syndrome compared to HC and T1D (P<0.001). Compared to participants in the control groups, participants with Wolfram syndrome had a blunted perception of sweetness and saltiness when taste stimuli were applied regionally (P<0.05), but differences in perceived intensity were no longer significant among groups when taste stimuli were tasted with the whole mouth. Groups preferred similar sucrose concentrations. CONCLUSION : Wolfram syndrome was associated with olfactory dysfunction. However, the olfactory dysfunction was qualitative (related to smell identification) and not secondary to olfactory insensitivity or diabetes, suggesting is arising from dysfunction in central olfactory brain regions. In contrast to olfaction, and despite decreased perception of taste intensity in the anterior tongue, the sense of taste was overall well-conserved in individuals with Wolfram syndrome. Future longitudinal studies of taste and smell perception in Wolfram syndrome will be important to determine the use of the chemical senses as clinical markers of disease progression.

Current gold standard neuroimaging tools lack either necessary temporal resolution (PET) or optimal safety due to contraindications (fMRI) for measuring the neural mechanisms underlying the effects of deep brain stimulation of the subthalamic nucleus (STN DBS) in Parkinson disease (PD). In this study, we validate the feasibility of High-Density Diffuse Optical Tomography (HD-DOT) for mapping the cortical activity of the PD patients with their STN DBS ON and OFF during auditory and visual tasks and during resting state.

Introduction Wolfram syndrome is a rare genetic disease associated with a variety of progressive metabolic and neurologic impairments. Previous research has focused on Wolfram syndrome-related impairments and biomarkers for disease progression; however, information about how Wolfram syndrome impacts participation in daily activities is lacking. Method Wolfram syndrome ( n = 45; 20 children, 25 adults) participants completed an online questionnaire about activity participation. Thirty-six non-Wolfram syndrome comparison participants (11 children; 25 adults) completed a portion of the questionnaire. Symptom data from a subset of Wolfram syndrome participants ( n = 20) were also examined in relation to participation data. Results Wolfram syndrome children and adults had lower participation than non-Wolfram syndrome children and adults in almost all activity domains, and social and exercise-related activities were the most problematic. In the subset of Wolfram syndrome adults with symptom data, poorer vision, balance, gait, hearing, and overall symptom severity were related to lower participation. Conclusion Wolfram syndrome appears to negatively impact participation in a variety of activities, and this effect may increase as people age and/or Wolfram syndrome progresses. The most functionally pertinent Wolfram syndrome symptoms are those associated with neurodegeneration, especially vision loss and walking and balance problems. This study revealed symptoms and activity domains that are most relevant for people with Wolfram syndrome and, thus, can inform current practice and treatment development research.

Objective: Cross-sectional studies find altered cognition in youth with type 1 diabetes mellitus (T1DM). However, few longitudinal studies have examined the trajectories of their cognitive performance over time. The aims of this study were to explore longitudinal change in cognitive function in youth with T1DM as compared with nondiabetic sibling controls, and how glycemic control and age of onset influence cognitive performance over time.Methods: We assessed crystallized intelligence, visual-spatial ability, delayed memory, and processing speed at 3 time points using the same cognitive tasks in youth with T1DM and sibling controls. Hierarchical linear modeling examined relationships between diabetes, hyperglycemia (HbA1c values), age of onset, and cognition over 5.5 y.Results: Youth with diabetes performed worse than controls on visual-spatial ability and memory tasks over time, and did not improve as much in processing speed. Greater hyperglycemia was associated with lower crystallized intelligence and slower processing speed but better memory across all time points. There was a stronger negative relationship between hyperglycemia and visual-spatial ability for youth with earlier compared with later onset diabetes. Importantly, within-person decreases in hyperglycemia between time points were associated with improved visual-spatial ability and faster processing speed.Conclusions: On average, differences in cognitive function between youth with T1DM and nondiabetic relatives are maintained or increase during childhood and adolescence. Hyperglycemia and age of onset can have negative effects on the developmental trajectories of cognitive processes in youth with T1DM. However, treatments that lower hyperglycemia may lead to improved cognitive function in youth with T1DM.

Aims: To explore the potential influence of the Stanford Chronic Disease Self-Management Program (CDSMP) on social support in Parkinson's disease (PD). Methods: This was a quasi-experimental mixed methods design. Volunteers with PD (n=27) and care partners (n = 6) completed the CDSMP, questionnaires of social support and self-management outcomes, and an interview about social support in relation to CDSMP participation. PD participants (n = 19) who did not participate in the CDSMP completed the questionnaires for quantitative comparison purposes. Results: Regarding the quantitative data, there were no significant effects of CDSMP participation on social support questionnaire scores; however, there were some positive correlations between changes in social support and changes in self-management outcomes from pre-to post-CDSMP participation. Three qualitative themes emerged from the interviews: lack of perceived change in amount and quality of social support, positive impact on existing social networks, and benefit from participating in a supportive PD community. Conclusions: Although participants did not acknowledge major changes in social support, there were some social support-related benefits of CDSMP participation for PD participants and care partners. These findings provide a starting point for more in-depth studies of social support and self-management in this population.

OBJECTIVE:To investigate daily function among individuals with Wolfram Syndrome (WFS) and examine whether any limitations are related to disease-related symptoms.METHODS:WFS (n = 31), Type 1 diabetic (T1DM; n = 25), and healthy control (HC; n = 29) participants completed the Pediatric Quality of Life Questionnaire (PEDSQL) Self and Parent Report. PEDSQL domain scores were compared among these groups and between WFS patients with and without specific disease-related symptoms. Relationships between PEDSQL scores and symptom severity as assessed by the Wolfram Unified Rating Scale (WURS) Physical Scale were also examined.RESULTS:Across most domains, the WFS group had lower PEDSQL Self and Parent Report scores than the T1DM and HC groups. WFS participants with urinary, sleep, and temperature regulation problems had lower PEDSQL scores than those without. The WURS Physical Scale correlated with Self and Parent Report PEDSQL domains. WFS group Self and Parent Reports correlated with each other.CONCLUSIONS:The WFS group reported lower daily function compared to T1DM and HC groups. Within WFS, worse symptom severity and the specific symptoms of sleep, temperature regulation, and urinary problems were associated with poorer daily function. These findings provide rationale for an increased emphasis on identifying, treating and understanding these less well-known symptoms of WFS.

Objective: While cerebral edema and diabetic ketoacidosis (DKA) in type 1 diabetes (T1DM) have well-described acute effects on cognition, little is known about the impact of clinical presentation on longer term cognitive outcomes. We hypothesized that clinical factors (degree of hyperglycemia exposure and DKA) at the time of diagnosis would relate to cognition within 3.5 months later in children with T1DM.Methods: Cognitive testing was performed on children 7-17 years old with T1DM (n=66) within 3.5 months of diagnosis and siblings without T1DM (n=33). Overall intelligence, processing speed, and memory (including a sensitive long-delay spatial memory test; spatial delayed response or SDR) were assessed. Medical records were reviewed for hemoglobin A1c (HbA1c), DKA status, and other clinical factors at diagnosis.Results: Within the group with T1DM, 17 children presented in DKA and 49 did not. After adjusting for age, gender, and socioeconomic status, the subgroup with T1DM and DKA at diagnosis performed worse on the long-delay SDR task compared to sibling controls (p=0.006). In addition, within the group with T1DM, higher HbA1c at diagnosis was associated with worse performance on the long-delay SDR task (p=0.027). Performance on the other cognitive tasks was not different across groups or subgroups.Conclusions: DKA and degree of hyperglycemia exposure at diagnosis have implications for long-delay spatial memory function within 3.5 months of diagnosis. These findings suggest that early detection of T1DM, which decreases risk for prolonged exposure to hyperglycemia and DKA, may avoid negative effects on memory function.