Clinical outcomes of pediatric hemispherectomy following unsuccessful subhemispheric resection for refractory epilepsy: a case review study.

OBJECTIVE: Epilepsy surgery remains one of the most underutilized procedures in epilepsy despite its proven superiority to other available therapies. This underutilization is greater in patients in whom initial surgery fails. This case series examined the clinical characteristics, reasons for initial surgery failure, and outcomes in a cohort of patients who underwent hemispherectomy following unsuccessful smaller resections for intractable epilepsy (subhemispheric group [SHG]) and compared them to those of a cohort of patients who underwent hemispherectomy as the first surgery (hemispheric group [HG]). The objective of this paper was to determine the clinical characteristics of patients in whom a small, subhemispheric resection failed, who went on to become seizure free after undergoing a hemispherectomy. METHODS: Patients who underwent hemispherectomy at Seattle Children's Hospital between 1996 and 2020 were identified. Inclusion criteria for SHG were as follows: 1) patients ≤ 18 years of age at the time of hemispheric surgery; 2) initial subhemispheric epilepsy surgery that did not produce seizure freedom; 3) hemispherectomy or hemispherotomy after the subhemispheric surgery; and 4) follow-up for at least 12 months after hemispheric surgery. Data collected included the following: patient demographics; seizure etiology; comorbidities; prior neurosurgeries; neurophysiological studies; imaging studies; and surgical details-plus surgical, seizure, and functional outcomes. Seizure etiology was classified as follows: 1) developmental, 2) acquired, or 3) progressive. The authors compared SHG to HG in terms of demographics, seizure etiology, and seizure and neuropsychological outcomes. RESULTS: There were 14 patients in the SHG and 51 patients in the HG. All patients in the SHG had Engel class IV scores after their initial resective surgery. Overall, 86% (n = 12) of the patients in the SHG had good posthemispherectomy seizure outcomes (Engel class I or II). All patients in the SHG who had progressive etiology (n = 3) had favorable seizure outcomes, with eventual hemispherectomy (1 each with Engel classes I, II, and III). Engel classifications posthemispherectomy between the groups were similar. There were no statistical differences in postsurgical Vineland Adaptive Behavior Scales Adaptive Behavior Composite scores or postsurgical full-scale IQ scores between groups when accounting for presurgical scores. CONCLUSIONS: Hemispherectomy as a repeat surgery after unsuccessful subhemispheric epilepsy surgery has a favorable seizure outcome, with stable or improved intelligence and adaptive functioning. Findings in these patients are similar to those in patients who had hemispherectomy as their first surgery. This can be explained by the relatively small number of patients in the SHG and the higher likelihood of hemispheric surgeries to resect or disconnect the entire epileptogenic lesion compared to smaller resections.

Intuitive Eating Intervention and Diet Quality in Adults: A Systematic Literature Review.

INTRODUCTION: Intuitive eating interventions aim to improve individual health and promote sustainable changes to one's relationship with food. However, there is no evidence-based consensus on the impact of intuitive eating interventions on diet quality. This systematic review aimed to investigate intuitive eating interventions and their impact on diet quality. METHOD: PubMed, Embase, CINAHL, PsycInfo, and Cochrane databases were systematically searched to October 2021 for studies reporting interventions that encompassed the principles of intuitive eating and measured diet quality. Other health outcomes were used for secondary analysis. Findings were synthesized narratively. RESULTS: Seventeen papers reporting 14 intervention studies (n = 3,960) were included in the review. All studies found a positive or neutral effect on diet quality following an intuitive eating intervention. A favorable change in eating behavior following these interventions was also observed. DISCUSSION: Intuitive eating promotes an attunement to the body, which aids in improving diet quality because of increased awareness of physiological cues. The reduction of emotional and binge eating may also increase diet quality. IMPLICATIONS FOR RESEARCH AND PRACTICE: Findings from the current review suggest that intuitive eating interventions are most effective face-to-face, in a group setting, and sustained for at least 3 months.

Concordance of functional MRI memory task and resting-state functional MRI connectivity used in surgical planning for pediatric temporal lobe epilepsy.

OBJECTIVE: Assessing memory is often critical in surgical evaluation, although difficult to assess in young children and in patients with variable task abilities. While obtaining interpretable data from task-based functional MRI (fMRI) measures is common in compliant and awake patients, it is not known whether functional connectivity MRI (fcMRI) data show equivalent results. If this were the case, it would have substantial clinical and research generalizability. To evaluate this possibility, the authors evaluated the concordance between fMRI and fcMRI data collected in a presurgical epilepsy cohort. METHODS: Task-based fMRI data for autobiographical memory tasks and resting-state fcMRI data were collected in patients with epilepsy evaluated at Seattle Children's Hospital between 2010 and 2017. To assess memory-related activation and laterality, signal change in task-based measures was computed as a percentage of the average blood oxygen level-dependent signal over the defined regions of interest. An fcMRI data analysis was performed using 1000 Functional Connectomes Project scripts based on Analysis of Functional NeuroImages and FSL (Functional Magnetic Resonance Imaging of the Brain Software Library) software packages. Lateralization indices (LIs) were estimated for activation and connectivity measures. The concordance between these two measures was evaluated using correlation and regression analysis. RESULTS: In this epilepsy cohort studied, the authors observed concordance between fMRI activation and fcMRI connectivity, with an LI regression coefficient of 0.470 (R2 = 0.221, p = 0.00076). CONCLUSIONS: Previously published studies have demonstrated fMRI and fcMRI overlap between measures of vision, attention, and language. In the authors' clinical sample, task-based measures of memory and analogous resting-state mapping were similarly linked in pattern and strength. These results support the use of fcMRI methods as a proxy for task-based memory performance in presurgical patients, perhaps including those who are more limited in their behavioral compliance. Future investigations to extend these results will be helpful to explore how the magnitudes of effect are associated with neuropsychological performance and postsurgical behavioral changes.

Precision medicine in pediatric temporal epilepsy surgery: optimization of outcomes through functional MRI memory tasks and tailored surgeries.

OBJECTIVE: The goal of epilepsy surgery is both seizure cessation and maximal preservation of function. In temporal lobe (TL) cases, the lack of functional MRI (fMRI) tasks that effectively activate mesial temporal structures hampers preoperative memory risk assessment, especially in children. This study evaluated pediatric TL surgery outcome optimization associated with tailored resection informed by an fMRI memory task. METHODS: The authors identified focal onset TL epilepsy patients with 1) TL resections; 2) viable fMRI memory scans; and 3) pre- and postoperative neuropsychological (NP) evaluations. They retrospectively evaluated preoperative fMRI memory scans, available Wada tests, pre- and postoperative NP scores, postoperative MRI scans, and postoperative Engel class outcomes. To assess fMRI memory task outcome prediction, the authors 1) overlaid preoperative fMRI activation onto postoperative structural images; 2) classified patients as having "overlap" or "no overlap" of activation and resection cavities; and 3) compared these findings with memory improvement, stability, or decline, based on Reliable Change Index calculations. RESULTS: Twenty patients met the inclusion criteria. At a median of 2.1 postoperative years, 16 patients had Engel class IA outcomes and 1 each had Engel class IB, ID, IIA, and IID outcomes. Functional MRI activation was linked to NP memory outcome in 19 of 20 cases (95%). Otherwise, heterogeneity characterized the cohort. CONCLUSIONS: Functional MRI memory task activation effectively predicted individual NP outcomes in the context of tailored TL resections. Patients had excellent seizure and overall good NP outcomes. This small study adds to extant literature indicating that pediatric TL epilepsy does not represent a single clinical syndrome. Findings support individualized surgical intervention using fMRI memory activation to help guide this precision medicine approach.

A clinically applicable functional MRI memory paradigm for use with pediatric patients.

OBJECTIVE: Clinically employable functional MRI (fMRI) memory paradigms are not yet established for pediatric patient epilepsy surgery workups. Seeking to establish such a paradigm, we evaluated the effectiveness of memory fMRI tasks we developed by quantifying individual activation in a clinical pediatric setting, analyzing patterns of activation relative to the side of temporal lobe (TL) pathology, and comparing fMRI and Wada test results. METHODS: We retrospectively identified 72 patients aged 6.7-20.9 years with pathology (seizure focus and/or tumor) limited to the TL who had attempted memory and language fMRI tasks over a 9-year period as part of presurgical workups. Memory fMRI tasks required visualization of autobiographical memories in a block design alternating with covert counting. Language fMRI protocols involved verb and sentence generation. Scans were both qualitatively interpreted and quantitatively assessed for blood oxygenation level dependent (BOLD) signal change using region of interest (ROI) masks. We calculated the percentage of successfully scanned individual cases, compared 2 memory task activation masks in cases with left versus right TL pathology, and compared fMRI with Wada tests when available. Patients who had viable fMRI and Wada tests had generally concordant results. RESULTS: Of the 72 cases, 60 (83%), aged 7.6-20.9 years, successfully performed the memory fMRI tasks and 12 (17%) failed. Eleven of 12 unsuccessful scans were due to motion and/or inability to perform the tasks, and the success of a twelfth was indeterminate due to orthodontic metal artifact. Seven of the successful 60 cases had distorted anatomy that precluded employing predetermined masks for quantitative analysis. Successful fMRI memory studies showed bilateral mesial temporal activation and quantitatively demonstrated: (1) left activation (L-ACT) less than right activation (R-ACT) in cases with left temporal lobe (L-TL) pathology, (2) nonsignificant R-ACT less than L-ACT in cases with right temporal lobe (R-TL) pathology, and (3) lower L-ACT plus R-ACT activation for cases with L-TL versus R-TL pathology. Patients who had viable fMRI and Wada tests had generally concordant results. SIGNIFICANCE: This study demonstrates evidence of an fMRI memory task paradigm that elicits reliable activation at the individual level and can generally be accomplished in clinically involved pediatric patients. This autobiographical memory paradigm showed activation in mesial TL structures, and cases with left compared to right TL pathology showed differences in activation consistent with extant literature in TL epilepsy. Further studies will be required to assess outcome prediction.

Balanced carbohydrate ratios are associated with improved diet quality in Australia: A nationally representative cross-sectional study.

BACKGROUND: Carbohydrate quality influences major health outcomes; however, the best criteria to assess carbohydrate quality remain unknown. OBJECTIVE: The objectives were to: i) evaluate whether a diet that meets a carbohydrate ratio (simple, modified or dual ratio) is associated with higher nutrient intakes and diet quality, and ii) model the impact of substituting carbohydrate foods that meet the proposed ratios in place of foods that do not, on nutrient intakes. DESIGN: A secondary analysis of cross-sectional data from the 2011-12 Australian National Nutrition and Physical Activity Survey. PARTICIPANTS/SETTING: National data from participants aged 2 years and older (n = 12,153). MAIN OUTCOME MEASURES: Ratios were defined as (i) simple ratio, 10:1 (10g carbohydrate:≥1g dietary fiber); (ii) modified ratio, 10:1:2 (10g carbohydrate:≥1g dietary fiber:≤2g free sugars); and (iii) dual ratio, 10:1 & 1:2 (10g carbohydrate:≥1g dietary fiber & ≤2g free sugars per 1g dietary fiber). Ratios were compared to nutrient intakes obtained via automated multiple-pass 24-hour dietary recall and diet quality calculated using the Australian Healthy Eating Index. STATISTICAL ANALYSES PERFORMED: Substitution dietary modelling was performed. Data were analyzed using paired and independent sample t-tests. RESULTS: Ratio adherence was highest for simple (50.2% adults; 28.6% children), followed by dual (40.6% adults; 21.7% children), then modified (32.7% adults; 18.6% children) ratios. Participants who met any ratio reported higher nutrient intake and diet quality compared to those who failed to meet the respective ratio (P < .001 for all), with the greatest nutrient intakes found for those who met modified or dual ratios. Dietary modelling improved nutrient intakes for all ratios, with the greatest improvement found for the dual ratio. CONCLUSIONS: All carbohydrate ratios were associated with higher diet quality, with a free sugars constraint in the dual ratio providing the greatest improvements.

Contribution of dietary snacking behaviours to discretionary energy intake and anthropometric measures in Australian adults: A comparison using an objective vs subjective definition for snacking.

AIM: There is limited information on the snacking behaviour of Australian adults, and the role of snacking in the diet may depend on how it is defined. This study aimed to compare the dietary snacking behaviours and associated nutritional intake and body composition in Australian adults, using an objective vs a subjective definition for snacking. METHODS: Cross-sectional data were analysed from the 2011 to 2012 National Nutrition and Physical Activity Survey (n = 8361, 19+ years). Objective snacking was defined based on time of day between main meals. Subjective snacking was self-reported by participants. RESULTS: Using the objective definition, 88.2% of adults were snack consumers; where snacking contributed 20.0% (SD 20.0%) of total daily energy intake and 27.0% (SD 31.4%) of total daily discretionary energy. 41.3% (SD 37.1%) of snacking energy intake came from discretionary foods. Using the subjective definition, 98.5% of adults were snack consumers, where discretionary foods contributed 52.6% (SD 35.2%) of all snacking energy. The proportion of objective and subjective snacking energy from discretionary foods did not differ across body mass index groups. CONCLUSIONS: Objective snacking energy contributed more to core food groups than discretionary, was not associated with anthropometric measures, and contributed less to total discretionary energy than the evening meal. When snacking was defined subjectively, more than half of snacking energy was discretionary, suggesting that adults are more likely to perceive discretionary foods as snacks. Differences between snacking definitions means that associations between self-reported snacking and diet or health outcomes, should be interpreted with caution.

Awake Mapping of the Auditory Cortex during Tumor Resection in an Aspiring Musical Performer: A Case Report.

INTRODUCTION: Preoperative functional MRI (fMRI) and intraoperative awake cortical mapping are established strategies to identify and preserve critical language structures during neurosurgery. There is growing appreciation for the need to similarly identify and preserve eloquent tissue critical for music production. CASE REPORT: A 19-year-old female musician, with a 3- to 4-year history of events concerning for musicogenic seizures, was found to have a right posterior temporal tumor, concerning for a low-grade glial neoplasm. Preoperative fMRI assessing passive and active musical tasks localized areas of activation directly adjacent to the tumor margin. Cortical stimulation during various musical tasks did not identify eloquent tissue near the surgical site. A gross total tumor resection was achieved without disruption of singing ability. At 9-month follow-up, the patient continued to have preserved musical ability with full resolution of seizures and without evidence of residual lesion or recurrence. CONCLUSION: A novel strategy for performing an awake craniotomy, incorporating preoperative fMRI data for music processing with intraoperative cortical stimulation, interpreted with the assistance of a musician expert and facilitated gross total resection of the patient's tumor without comprising her musical abilities.

Family in Rehabilitation, Empowering Carers for Improved Malnutrition Outcomes: Protocol for the FREER Pilot Study.

BACKGROUND: Interventions to improve the nutritional status of older adults and the integration of formal and family care systems are critical research areas to improve the independence and health of aging communities and are particularly relevant in the rehabilitation setting. OBJECTIVE: The primary outcome aimed to determine if the FREER (Family in Rehabilitation: EmpowERing Carers for improved malnutrition outcomes) intervention in malnourished older adults during and postrehabilitation improve nutritional status, physical function, quality of life, service satisfaction, and hospital and aged care admission rates up to 3 months postdischarge, compared with usual care. Secondary outcomes evaluated include family carer burden, carer services satisfaction, and patient and carer experiences. This pilot study will also assess feasibility and intervention fidelity to inform a larger randomized controlled trial. METHODS: This protocol is for a mixed-methods two-arm historically-controlled prospective pilot study intervention. The historical control group has 30 participants, and the pilot intervention group aims to recruit 30 patient-carer pairs. The FREER intervention delivers nutrition counseling during rehabilitation, 3 months of postdischarge telehealth follow-up, and provides supportive resources using a novel model of patient-centered and carer-centered nutrition care. The primary outcome is nutritional status measured by the Scored Patient-Generated Subjective Global Assessment Score. Qualitative outcomes such as experiences and perceptions of value will be measured using semistructured interviews followed by thematic analysis. The process evaluation addresses intervention fidelity and feasibility. RESULTS: Recruitment commenced on July 4, 2018, and is ongoing with eight patient-carer pairs recruited at the time of manuscript submission. CONCLUSIONS: This research will inform a larger randomized controlled trial, with potential for translation to health service policies and new models of dietetic care to support the optimization of nutritional status across a continuum of nutrition care from rehabilitation to home. TRIAL REGISTRATION: Australian New Zealand Clinical Trials Registry Number (ACTRN) 12618000338268; https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=374608&isReview=true (Archived by WebCite at http://www.webcitation.org/74gtZplU2). INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/12647.

Patients' Experiences and Perspectives of Telehealth Coaching with a Dietitian to Improve Diet Quality in Chronic Kidney Disease: A Qualitative Interview Study.

BACKGROUND: Dietary behavior change interventions for the self-management of chronic kidney disease (CKD) have the potential to slow disease progression and reduce metabolic complications. Telehealth-delivered dietary interventions may assist in the self-management of CKD, although their acceptability by patients is unknown. OBJECTIVE: This study aims to describe the acceptability and experiences of a telehealth coaching intervention that utilized telephone calls and tailored text messages to improve diet quality in patients with stage 3 to 4 CKD. DESIGN: Semistructured interview study of adults with CKD. PARTICIPANTS/SETTING: Adults with stage 3 to 4 CKD (n=21) aged 28 to 78 (mean 62) years, who completed a 12-week telehealth-delivered dietary intervention in Queensland, Australia, were interviewed from March to July 2017. DATA ANALYSIS: Interviews were transcribed verbatim and analyzed thematically. RESULTS: Five themes were identified: valuing relationships (receiving tangible and perceptible support, building trust and rapport remotely, motivated by accountability, readily responding to a personalized approach, reassured by health professional expertise); appreciating convenience (integrating easily into lifestyle, talking comfortably in a familiar environment, minimizing travel and wait time burden); empowered with actionable knowledge (comprehending diet-disease mechanisms, practical problem solving for sustainable dietary behavior); increasing diet consciousness (learning from recurrent feedback, prompted by reiteration of messages); making sense of complexity (contextualizing and prioritizing comorbidities, gaining confidence to make dietary decisions, setting and achieving realistic goals). CONCLUSIONS: Among adults with stage 3 to 4 CKD, individualized telehealth coaching for improving diet quality was convenient for patients, and they felt supported and empowered to navigate recommendations and prioritize dietary behavior changes. Telehealth-delivered dietary interventions appear to be well accepted by patients as a way of providing regular, tailored contact with a health professional to support dietary management in CKD.

Feasibility and acceptability of telehealth coaching to promote healthy eating in chronic kidney disease: a mixed-methods process evaluation.

OBJECTIVE: To evaluate the feasibility and acceptability of a personalised telehealth intervention to support dietary self-management in adults with stage 3-4 chronic kidney disease (CKD). DESIGN: Mixed-methods process evaluation embedded in a randomised controlled trial. PARTICIPANTS: People with stage 3-4 CKD (estimated glomerular filtration rate [eGFR]15-60 mL/min/1.73 m2). SETTING: Participants were recruited from three hospitals in Australia and completed the intervention in ambulatory community settings. INTERVENTION: The intervention group received one telephone call per fortnight and 2-8 tailored text messages for 3 months, and then 4-12 tailored text messages for 3 months without telephone calls. The control group received usual care for 3 months then non-tailored education-only text messages for 3 months. MAIN OUTCOME MEASURES: Feasibility (recruitment, non-participation and retention rates, intervention fidelity and participant adherence) and acceptability (questionnaire and semistructured interviews). STATISTICAL ANALYSES PERFORMED: Descriptive statistics and qualitative content analysis. RESULTS: Overall, 80/230 (35%) eligible patients who were approached consented to participate (mean±SD age 61.5±12.6 years). Retention was 93% and 98% in the intervention and control groups, respectively, and 96% of all planned intervention calls were completed. All participants in the intervention arm identified the tailored text messages as useful in supporting dietary self-management. In the control group, 27 (69%) reported the non-tailored text messages were useful in supporting change. Intervention group participants reported that the telehealth programme delivery methods were practical and able to be integrated into their lifestyle. Participants viewed the intervention as an acceptable, personalised alternative to face-face clinic consultations, and were satisfied with the frequency of contact. CONCLUSIONS: This telehealth-delivered dietary coaching programme is an acceptable intervention which appears feasible for supporting dietary self-management in stage 3-4 CKD. A larger-scale randomised controlled trial is needed to evaluate the efficacy of the coaching programme on clinical and patient-reported outcomes. TRIAL REGISTRATION NUMBER: ACTRN12616001212448; Results.

Cognitive characteristics of mitochondrial diseases in children.

INTRODUCTION: This retrospective descriptive study was undertaken to further define the intelligence profiles of children with mitochondrial disorders, in the context of seizures and age of symptom onset. METHODS: We retrospectively identified forty-nine pediatric patients with definitive mitochondrial disease diagnoses and complete intelligence or adaptive functioning testing data. Patients were 0-216 months at onset of symptoms and 61-250 months of age at testing. Twenty-four of 49 patients had seizures. Twenty-one of the 24 patients with seizures had medically intractable seizures. All patients had Wechsler Intellectual Quotient (IQ) testing, except nine patients with seizures who were unable to engage in IQ testing and were assessed with a structured parent interview measure, the Vineland Adaptive Behavior Scales. We used descriptive and exploratory data analysis methods to characterize test results. RESULTS: Distribution of ages for patients with the Vineland assessment was younger than those given the Wechsler. The median overall score (combining Wechsler and Vineland summary scores) for all patients was 85 (interquartile range [IQR]: 50, 102), with the group without seizures obtaining a higher median Full Scale IQ (FSIQ) of 100 (IQR: 86, 109), compared to the group with seizures with a median FSIQ of 67 (IQR: 49.5, 89), a difference that is both statistically and clinically different (Δ = 33; 95% CI: 9, 52). The adaptive function measure was composed of patients only with intractable epilepsy and yielded the lowest overall median summary score of 43 (IQR: 37, 50). This general trend in differences between the FSIQ scores of the groups with and without seizures was also seen across all subscale measures analyzed-IQ index scores and two subtest scores, Digit Span and Coding-though differences were not always statistically different. Vargha-Delaney's A effect sizes ranged between 0.68 and 0.90, trends that mirrored those of distributional and median differences. Groups without versus with seizures differed most distinctly in Performance IQ (PIQ), with the group without seizures' median PIQ being 100 (IQR 94, 112) versus the group with seizures' median PIQ being 63 (IQR 54, 84), a difference of 37 points (95% CI). DISCUSSION: Results suggest that patients with mitochondrial diseases with seizures and early onset disease represent a worse cognitive phenotype, as compared with those with no seizures, who can have average intelligence. Results are discussed in the context of current literature.

Improved Access to and Impact of Registered Dietitian Nutritionist Services Associated with an Integrated Care Model in a High-Risk, Minority Population.

BACKGROUND: Integrated health care models create opportunities for registered dietitian nutritionists (RDNs) to provide nutrition-related care and engage in multidisciplinary teams to improve clinical outcomes. While benefits of integrated care (IC) have been reported, little is known about the impact of the RDN within an IC model. OBJECTIVE: Our primary objective was to identify whether IC vs traditional care (TC) increases the number of RDN patient visits. Our secondary objective was to evaluate clinical outcomes of patients seeing an RDN vs not, regardless of care model. DESIGN: This was a retrospective cohort study. PARTICIPANTS/SETTING: Patients were aged 3 to 94 years and from a patient-centered medical home in Boston, MA. MAIN OUTCOME MEASURES: We measured 3-month total and average number of patients seen by the RDN in TC vs IC. Changes in adult hemoglobin A1c, weight, and pediatric body mass index (measured as kg/m2) among high-risk patients seen by an RDN compared to patients not seen by an RDN. STATISTICAL ANALYSIS: Data were obtained from electronic medical records and analyzed utilizing Mann-Whitney U test, analysis of covariance, and paired sample t tests. RESULTS: The RDN saw 145 patients (137 adult, 8 pediatric) in the TC model compared to 185 patients (135 adult, 50 pediatric) in the IC model. Mean number of patients seen per session was 3.20 in the TC model vs 4.63 in the IC model (P=0.004). Adult hemoglobin A1c within-group differences decreased by 0.42%±1.49% (P=0.007) for patients seen by an RDN and decreased 0.15%±1.47% (P=0.012) for patients not seen by an RDN. Adult weight within-group differences decreased 1.0±7.2 kg (P=0.15) for patients seen by a RDN and increased 0.1±5.6 kg (P=0.70) for patients not seen by a RDN. Pediatric BMI showed no change between or within groups. CONCLUSIONS: The IC model increased 3-month total number of patients seen by an RDN. High-risk patients who saw an RDN had a significant decrease in hemoglobin A1c.

Corticospinal tract atrophy and motor fMRI predict motor preservation after functional cerebral hemispherectomy.

OBJECTIVE The potential loss of motor function after cerebral hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function. METHODS Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)-DTI maps in 25 patients undergoing functional hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI. RESULTS Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661-0.998) and 100% specificity (95% CI 0.715-1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572-0.982) and 100% specificity (95% CI 0.715-1.00). Interpreting the fMR images, the reviewers achieved a Fleiss' kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01). CONCLUSIONS Functional hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.

Reporting of Telehealth-Delivered Dietary Intervention Trials in Chronic Disease: Systematic Review.

BACKGROUND: Telehealth-delivered dietary interventions are effective for chronic disease management and are an emerging area of clinical practice. However, to apply interventions from the research setting in clinical practice, health professionals need details of each intervention component. OBJECTIVE: The aim of this study was to evaluate the completeness of intervention reporting in published dietary chronic disease management trials that used telehealth delivery methods. METHODS: Eligible randomized controlled trial publications were identified through a systematic review. The completeness of reporting of experimental and comparison interventions was assessed by two independent assessors using the Template for Intervention Description and Replication (TIDieR) checklist that consists of 12 items including intervention rationale, materials used, procedures, providers, delivery mode, location, when and how much intervention delivered, intervention tailoring, intervention modifications, and fidelity. Where reporting was incomplete, further information was sought from additional published material and through email correspondence with trial authors. RESULTS: Within the 37 eligible trials, there were 49 experimental interventions and 37 comparison interventions. One trial reported every TIDieR item for their experimental intervention. No publications reported every item for the comparison intervention. For the experimental interventions, the most commonly reported items were location (96%), mode of delivery (98%), and rationale for the essential intervention elements (96%). Least reported items for experimental interventions were modifications (2%) and intervention material descriptions (39%) and where to access them (20%). Of the 37 authors, 14 responded with further information, and 8 could not be contacted. CONCLUSIONS: Many details of the experimental and comparison interventions in telehealth-delivered dietary chronic disease management trials are incompletely reported. This prevents accurate interpretation of trial results and implementation of effective interventions in clinical practice.

Impact of epilepsy surgery on development of preschool children: identification of a cohort likely to benefit from early intervention.

OBJECT: Outcomes of focal resection in young children with early-onset epilepsy are varied in the literature due to study differences. In this paper, the authors sought to define the effect of focal resection in a small homogeneous sample of children who were otherwise cognitively intact, but who required early surgical treatment. Preservation of and age-appropriate development of intelligence following focal resection was hypothesized. METHODS: Cognitive outcome after focal resection was retrospectively reviewed for 15 cognitively intact children who were operated on at the ages of 2-6 years for lesion-related, early-onset epilepsy. Intelligence was tested prior to and after surgery. Effect sizes and confidence intervals for means and standard deviations were used to infer changes and differences in intelligence between 1) groups (pre vs post), 2) left versus right hemisphere resections, and 3) short versus long duration of seizures prior to resection. RESULTS: No group changes from baseline occurred in Full Scale, verbal, or nonverbal IQ. No change from baseline intelligence occurred in children who underwent left or right hemisphere surgery, including no group effect on verbal scores following surgery in the dominant hemisphere. Patients with seizure durations of less than 6 months prior to resection showed improvement from their presurgical baseline in contrast to those with seizure duration of greater than 6 months prior to surgery, particularly in Wechsler Full Scale IQ and nonverbal intelligence. CONCLUSIONS: This study suggests that surgical treatment of focal seizures in cognitively intact preschool children is likely to result in seizure remediation, antiepileptic drug discontinuation, and no significant decrement in intelligence. The latter finding is particularly significant in light of the longstanding concern associated with performing resections in the language-dominant hemisphere. Importantly, shorter seizure duration prior to resection can result in improved cognitive outcome, suggesting that surgery for this population should occur sooner to help improve intelligence outcomes.

High-fat diets and seizure control in myoclonic-astatic epilepsy: a single center's experience.

PURPOSE: To determine the efficacy of the Modified Atkins Diet (MAD) and Ketogenic Diet (KD) in seizure control within a population of myoclonic-astatic epilepsy (MAE) patients. METHODS: This was a retrospective, single center study evaluating the seizure control by high fat diets. Seizure diaries kept by the parents performed seizure counts. All patients met the clinical criteria for MAE. RESULTS: Nine patients met the clinical criteria. We found that both the MAD and KD were efficacious in complete seizure control and allowed other medications to be stopped in seven patients. Two patients had greater than 90% seizure control without medications, one on the KD and the other on the MAD. Seizure freedom has ranged from 13 to 36 months, and during this time four patients have been fully weaned off of diet management. One patient was found to have a mutation in SLC2A1. CONCLUSION: Our results suggest that strictly defined MAE patients respond to the MAD with prolonged seizure control. Some patients may require the KD for seizure freedom, suggesting a common pathway of increased requirement for fats. Once controlled, those fully responsive to the Diet(s) could be weaned off traditional seizure medications and in many, subsequently off the MAD or KD.

Mitochondrial electron transport chain deficiency, cardiomyopathy, and long-term cardiac transplant outcome.

Organ transplantation in multisystemic mitochondrial cytopathies is usually not performed because of perceived untoward complications. We report three patients with demonstrated oxidative phosphorylation defects and dilated cardiomyopathy who underwent cardiac transplant. All three patients tolerated immunosuppression medications and have had an excellent long-term outcome. Our results suggest that with proper patient selection in this population, cardiac transplantation is feasible and can have good outcomes.

Functional magnetic resonance imaging for presurgical evaluation of very young pediatric patients with epilepsy.

OBJECT: The authors describe their experience with functional MR (fMR) imaging in children as young as 5 years of age, or even younger in developmental age equivalent. Functional MR imaging can be useful for identifying eloquent cortex prior to surgical intervention. Most fMR imaging clinical work has been done in adults, and although children as young as 8 years of age have been included in larger clinical series, cases in younger children are rarely reported. METHODS: The authors reviewed presurgical fMR images in eight patients who were 8 years of age or younger, six of whom were 5 or 6 years of age. Each patient had undergone neuropsychological testing. Three patients functioned at a below-average level, with adaptive functioning age scores of 3 to 4 years. Self-paced finger tapping (with passive movement in one patient) and silent language tasks were used as activation tasks. The language task was modified for younger children, for whom the same (not novel) stimuli were used for extensive practice ahead of time and in the MR imaging unit. Patient preparation involved techniques such as having experienced staff present to work with patients and providing external management during imaging. Six of eight patients had extensive training and practice prior to the procedure. In the two youngest patients, this training included use of a mock MR unit. RESULTS: All cases yielded successful imaging. Finger tapping in all seven of the patients who could perform it demonstrated focal motor activation in the frontal-parietal region, with expected activation elsewhere, including in the cerebellum. Three of four patients had the expected verb generation task activations, with left-hemisphere dominance, including a 6-year-old child who functioned at the 3-year, 9-month level. The only child (an 8-year-old) who was not prepared prior to the imaging session for the verb generation task failed this task due to movement artifact. CONCLUSIONS: Despite the challenges of successfully using fMR imaging in very young and clinically involved patients, these studies can be performed successfully in children with a chronological age of 5 or 6 years and a developmental age as young as 3 or 4 years.