Multimodal Approach for the Treatment of Complex Hypothalamic Hamartomas.

Hypothalamic hamartomas (HHs) are rare congenital lesions formed by heterotopic neuronal and glial cells attached to the mammillary bodies, tuber cinereum, and hypothalamus.They often present with an intractable epilepsy typically characterized by gelastic seizures but commonly associated with other types of refractory seizures. The clinical course is progressive in most of the cases, starting with gelastic seizures in infancy and deteriorating into complex seizure disorders that result in catastrophic epilepsy associated with cognitive decline and behavioral disturbances.Hamartomas are known to be intrinsically epileptogenic and the site of origin for the gelastic seizures. As antiepileptic drugs are typically ineffective in controlling HH-related epilepsy, different surgical options have been proposed as a treatment to achieve seizure control. Resection or complete disconnection of the hamartoma from the mammillothalamic tract has proved to achieve a long-lasting control of the epileptic syndrome.Usually, symptoms and their severity are typically related to the size, localization, and type of attachment. Precocious puberty appears mostly in the pedunculated type, while epileptic syndrome and behavioral decline are frequently related to the sessile type. For this reason, different classifications of HHs have been developed based on their size, extension, and type of attachment to the hypothalamus.The bigger and more complex hypothalamic hamartomas typically present with severe refractory epilepsy, behavioral disturbances, and progressive cognitive decline posing a formidable challenge for the control of these symptoms.We present here our experience with the multimodal treatment for complex hypothalamic hamartomas. After an in-depth review of the literature, we systematize our approach for the different types of hypothalamic hamartomas.

Temporo-parieto-occipital disconnection (TPO) by robot-assisted magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) for refractory epilepsy in a pediatric patient: Proof-of-principle case report and surgical nuances.

Magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) has been proven safe and effective for the treatment of focal epilepsy of different etiologies. It has also been used to disconnect brain tissue in more extensive or diffuse epilepsy, such as corpus callosotomy and hemispherotomy. In this study, we report a case of temporo-parieto-occipital disconnection (TPO) surgery performed using MRIgLITT assisted by a robotic arm for refractory epilepsy of the posterior quadrant. A highly realistic cadaver simulation was performed before the actual surgery. The patient was a 14-year-old boy whose seizures began at the age of eight. The epilepsy was a result of a left perinatal ischemic event that caused a porencephalic cyst, and despite receiving multiple antiepileptic drugs, the patient continued to experience daily seizures which led to the recommendation of surgery. A Wada test lateralized language in the right hemisphere. Motor and sensory function was confirmed in the left hemisphere through MRI functional studies and NexStim®. The left MRIgLITT TPO disconnection was achieved using five laser fibers. The patient followed an excellent postoperative course and was seizure-free, with no additional neurological deficits 24 months after the surgery.

Sound symbolic associations in Spanish emotional words: affective dimensions and discrete emotions.

Sound symbolism refers to non-arbitrary associations between word forms and meaning, such as those observed for some properties of sounds and size or shape. Recent evidence suggests that these connections extend to emotional concepts. Here we investigated two types of non-arbitrary relationships. Study 1 examined whether iconicity scores (i.e. resemblance-based mapping between aspects of a word's form and its meaning) for words can be predicted from ratings in the affective dimensions of valence and arousal and/or the discrete emotions of happiness, anger, fear, disgust and sadness. Words denoting negative concepts were more likely to have more iconic word forms. Study 2 explored whether statistical regularities in single phonemes (i.e. systematicity) predicted ratings in affective dimensions and/or discrete emotions. Voiceless (/p/, /t/) and voiced plosives (/b/, /d/, /g/) were related to high arousing words, whereas high arousing negative words tended to include fricatives (/s/, /z/). Hissing consonants were also more likely to occur in words denoting all negative discrete emotions. Additionally, words conveying certain discrete emotions included specific phonemes. Overall, our data suggest that emotional features might explain variations in iconicity and provide new insight about phonemic patterns showing sound symbolic associations with the affective properties of words.

Exercise recommendations for patients with myositis: a narrative review of safety and efficacy.

Idiopathic inflammatory myopathies (IIM) are marked by progressive muscle weakness and lasting disability. Therapies targeting patient well-being include the use of prescription drugs as well as exercise. Maintaining or increasing muscular strength and endurance as well as cardiorespiratory fitness (CRF) improves quality of life (QoL) as well as functional status in IIM patients. This narrative review highlights exercise interventions in patients of different IIM subtypes with the intent to provide a summary table with exercise recommendations that will safely and effectively improve QoL in myositis patients.

The impact of emotional valence on generalization gradients.

Generalization enables individuals to respond to novel stimuli based on previous experiences. The degree to which organisms respond is determined by their physical resemblance to the original conditioned stimulus (CS+), with a stronger response elicited by more similar stimuli, resulting in similarity-based generalization gradients. Recent research showed that cognitive or conceptual dimensions also result in gradients similar to those observed with manipulations of physical dimensions. Such findings suggest that attributes beyond physical similarity play a role in shaping generalization gradients. However, despite its adaptive relevance for survival, there is no study exploring the effectiveness of affective dimensions in shaping generalization gradients. In two experiments (135 Spanish and 150 English participants, respectively), we used an online predictive learning task, in which different stimuli (words and Gabor patches) were paired with the presence - or absence - of a fictitious shock. After training, we assessed whether valence (i.e., hedonic experience) conveyed by words shape generalization gradients. In Experiment 1, the outcome expectancy decreased monotonically with variations in valence of Spanish words, mirroring the gradient obtained with the physical dimension (line orientation). In Experiment 2, conducted with English words, a similar gradient was observed when non-trained (i.e., generalization) words varied along the valence dimension, but not when words were of neutral valence. The consistency of these findings across two different languages strengthens the reliability and validity of the affective dimension as a determinant of generalization gradients. Furthermore, our data highlight the importance of considering the role of affective features in generalization responses, advancing the interplay between emotion, language, and learning.

Pediatric Type 1 Diabetes: Is Age at Onset a Determining Factor in Advanced Hybrid Closed-Loop Insulin Therapy?

BACKGROUND: The integration of continuous glucose monitoring systems with insulin infusion pumps has shown improved glycemic control, with improvements in hyperglycemia, hypoglycemia, Hb1Ac, and greater autonomy in daily life. These have been most studied in adults and there are currently not many articles published in the pediatric population that establish their correlation with age of debut. METHODS: Prospective, single-study. A total of 28 patients (mean age 12 ± 2.43 years, 57% male, duration of diabetes 7.84 ± 2.46 years) were included and divided into two groups according to age at T1D onset (≤4 years and >4 years). Follow-up for 3 months, with glucometric variables extracted at different cut-off points after the start of the closed-loop (baseline, 1 month, 3 months). RESULTS: Significant improvement was evidenced at 1 month and 3 months after closed-loop system implantation, with better glycemic control in the older age group at baseline at TIR (74.06% ± 6.37% vs. 80.33% ± 7.49% at 1 month, p < 0.003; 71.87% ± 6.58% vs. 78.75% ± 5.94% at 3 months, p < 0.009), TAR1 (18.25% ± 4.54% vs. 14.33% ± 5.74% at 1 month, p < 0.006; 19.87% ± 5.15% vs. 14.67% ± 4. 36% at 3 months, p < 0.009) and TAR2 (4.75% ± 2.67% vs. 2.75% ± 1.96% at 1 month, p = 0.0307; 5.40% ± 2.85% vs. 3% ± 2.45% at 3 months, p < 0.027). CONCLUSIONS: the use of automated systems such as the MiniMedTM780G system brings glucometric results closer to those recommended by consensus, especially in age at T1D onset >4 years. However, the management in pediatrics continues to be a challenge even after the implementation of these systems, especially in terms of hyperglycemia and glycemic variability.

Tendon Transfers to Improve Grip and Pinch in Patients with Sporadic Inclusion Body Myositis.

Background: Sporadic inclusion body myositis (sIBM) is a rare and slowly progressive skeletal muscle disease that can cause hand dysfunction, which is a major source of disability. Tendon transfers have been reliably used to improve function in other neuromuscular settings. Given that sIBM patients often present with flexion impairments and mostly functioning extensors, we investigated the potential opportunity for tendon transfer surgery to improve hand dysfunction in sIBM patients. Methods: We conducted a scoping review for studies of sIBM and tendon transfers, extracted descriptions of hand function and surgical technique, and recorded results in terms of hand function. We also conducted an institutional review board-approved survey with 470 participants to determine baseline patient-reported function and to determine participant perceptions and expectations for tendon transfer surgery to improve hand function in sIBM. Results: We identified three published case reports on tendon transfers in sIBM patients with subjectively improved grip and pinch strength, but standardized measures of hand function or quality-of-life were not reported. Within the surveyed cohort, half of participants reported that they would consider surgery, yet only 8% had been referred to a hand surgeon. Fifty four percent of participants reported that they would consider surgery if there would be 1-2 years of benefit after surgery. All participants who would consider surgery also had significant upper extremity disability. Discussion: Tendon transfer surgery has the potential to improve quality-of-life for sIBM patients, and there is significant patient interest in this approach. To objectively assess its efficacy, we propose conducting a surgical trial.

The bright side of words: Norms for 9000 Spanish words in seven discrete positive emotions.

In recent years, assumptions about the existence of a single construct of happiness that accounts for all positive emotions have been questioned. Instead, several discrete positive emotions with their own neurobiological and psychological mechanisms have been proposed. Of note, the effects of positive emotions on language processing are not yet properly understood. Here we provide a database for a large set of 9000 Spanish words scored by 3437 participants in the positive emotions of awe, contentment, amusement, excitement, serenity, relief, and pleasure. We also report significant correlations between discrete positive emotions and several affective (e.g., valence, arousal, happiness, negative discrete emotions) and lexico-semantic (e.g., frequency of use, familiarity, concreteness, age of acquisition) characteristics of words. Finally, we analyze differences between words conveying a single emotion ("pure" emotion words) and those denoting more than one emotion ("mixed" emotion words). This study will provide researchers a rich source of information to do research that contributes to expanding the current knowledge on the role of positive emotions in language. The norms are available at https://doi.org/10.6084/m9.figshare.21533571.v2.

EpiGe: A machine-learning strategy for rapid classification of medulloblastoma using PCR-based methyl-genotyping.

Molecular classification of medulloblastoma is critical for the treatment of this brain tumor. Array-based DNA methylation profiling has emerged as a powerful approach for brain tumor classification. However, this technology is currently not widely available. We present a machine-learning decision support system (DSS) that enables the classification of the principal molecular groups-WNT, SHH, and non-WNT/non-SHH-directly from quantitative PCR (qPCR) data. We propose a framework where the developed DSS appears as a user-friendly web-application-EpiGe-App-that enables automated interpretation of qPCR methylation data and subsequent molecular group prediction. The basis of our classification strategy is a previously validated six-cytosine signature with subgroup-specific methylation profiles. This reduced set of markers enabled us to develop a methyl-genotyping assay capable of determining the methylation status of cytosines using qPCR instruments. This study provides a comprehensive approach for rapid classification of clinically relevant medulloblastoma groups, using readily accessible equipment and an easy-to-use web-application.t.

Masked priming under the Bayesian microscope: Exploring the integration of local elements into global shape through Bayesian model comparison.

To investigate whether local elements are grouped into global shapes in the absence of awareness, we introduced two different masked priming designs (e.g., the classic dissociation paradigm and a trial-wise probe and prime discrimination task) and collected both objective (i.e., performance based) and subjective (using the perceptual awareness scale [PAS]) awareness measures. Prime visibility was manipulated using three different prime-mask stimulus onset asynchronies (SOAs) and an unmasked condition. Our results showed that assessing prime visibility trial-wise heavily interfered with masked priming preventing any prime facilitation effect. The implementation of Bayesian regression models, which predict priming effects for participants whose awareness levels are at chance level, provided strong evidence in favor of the hypothesis that local elements group into global shape in the absence of awareness for SOAs longer than 50 ms, suggesting that prime-mask SOA is a crucial factor in the processing of the global shape without awareness.

A multidisciplinary approach to posterior quadrant disconnective epilepsy surgery in pediatric patients.

PURPOSE: Extensive lesions of the posterior quadrant are a relevant cause of pediatric drug-resistant epilepsy. Early surgery is the best treatment in these cases, but conventional multilobar resections carry a significant risk in pediatric patients. Despite temporo-parieto-occipital (TPO) disconnection being the preferable technique due to the preservation of motor function, studies reporting long-term longitudinal outcomes are still limited. This study aims to analyze seizures and developmental outcomes after TPO disconnection. METHODS: A prospective analysis was carried out on 12 children who underwent TPO disconnection. RESULTS: TPO disconnection was performed in 12 pediatric patients aged between 14 months and 18 years (median 6.29 years). The average age of seizure onset was 0.97 ± 1.22 years. Causes of TPO included perinatal ischemia in 4 patients and malformation of cortical development (MCD) in 8 patients. The presenting seizure types were focal motor impaired awareness seizures in 7 children and generalized in 5. The affected hemisphere was the right in 9 patients and the left in 3. In half of the patients, the temporal approach was performed through T1, and in the other 50%, it was performed through T2. After neuropsychological examination, 2 children improved, 7 remained stable, 2 patients presented stagnation and 1 declined. Regarding postoperative complications, nonresorptive hydrocephalus and an asymptomatic caudate nucleus infarct were observed. After a median follow-up of 2 years, 9 patients were in Engel's Class I seizure outcome. CONCLUSION: TPO disconnection is a safe and effective motor-sparing epilepsy surgery for children with refractory seizures located in the posterior quadrant that prevents further cognitive deterioration.

What makes a word a good representative of the category of "emotion"? The role of feelings and interoception.

The words we use to describe emotions vary in terms of prototypicality; that is, some of these words may be more representative of the semantic category of emotion than others (e.g., anger refers more clearly to an emotion than boredom). Based on a multicomponential conception of emotions, the aim of the present study was to examine the contribution of several variables to emotion prototypicality. Some of those variables are related to the distinct components of emotions: evaluation, action, body expression, internal body sensations (interoception), and feelings. Other variables are related to the concreteness/abstractness distinction: sensory experience, social interaction, thought, and morality. We collected ratings for these variables for a large set of words (1,286) which varied in emotion prototypicality. A regression analysis revealed that the variables that most contributed to emotion prototypicality were feelings and interoception. Furthermore, a factor analysis identified two underlying factors: socioemotional polarity and emotional experience. The scores of each word in both factors were used to create a two-dimensional space and a density plot which provides relevant information about the organization of emotion concepts in memory. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Image-guided radiation therapy (IGRT) in prostate cancer in México, survey of SOMERA (Sociedad Mexicana de Radioterapeutas/Mexican Society of Radiation Oncologists) with recommendations on its implementation and process.

Background: Prostate cancer is one of the main tumors worldwide, its treatment is multidisciplinary, includes radiotherapy in all stages: curative, radical, adjuvant, salvage and palliative. Technological advances in planning systems, image acquisition and treatment equipment have allowed the delivery of higher doses limiting toxicity in healthy tissues, distributing radiation optimally and ensuring reproducibility of conditions. Image-guided radiotherapy (IGRT) is not standard in guidelines, only recommended with heterogeneity in its own process. Materials and methods: A survey was conducted to members of the Mexican Society of Radiation Oncologists (SOMERA), to know the current status and make recommendations about its implementation and use, taking into account existing resources. Results: Responses of 541 patients were evaluated, 85% belonged to the intermediate-high risk group, 65% received adjuvant or salvage radiotherapy (RT), 80% received intensity-modulated radiation therapy (IMRT) using doses up to 80 Gy/2 Gy. Cone beam computed tomography (CBCT) was performed on 506 (93.5%), (100% IMRT) and 90% at a periodicity of 3-5/week. 3D treatment with 42% portal images 1/week. Online correction strategies (36% changes before treatment), following a diet and bladder and rectal control. Evidence and recommendations are reviewed. Conclusions: IGRT should be performed in patients with prostate cancer. In Mexico, despite limitations in the distribution of human and technological resources, it is routinely applied. More information is still needed on clinical evidence of its benefits and the process should be implemented according to infrastructure, following institutional guidelines, recommending to report the initial experience that helps to standardize national conduct.

Selection within working memory impairs perceptual detection.

There is broad consensus supporting the reciprocal influence of working memory (WM) and attention. Top-down mechanisms operate to cope with either environmental or internal demands. In that sense, it is possible to select an item within the contents of WM to endow it with prioritized access. Although evidence supports that maintaining an item in this privileged state does not rely on sustained visual attention, it is unknown whether selection within WM depends on perceptual attention. To answer this question, we recorded electrophysiological neural activity while participants performed a retro-cue task in which we inserted a detection task in the delay period after retro-cue presentation. Critically, the onset of to-be-detected near threshold stimuli was unpredictable, and thus, sustained perceptual spatial attention was needed to accomplish the detection task from the offset of the retro-cue. At a behavioral level, we found decreased visual detection when a WM representation was retro-cued. At a neural level, alpha oscillatory activity confirmed a spatial shift of attention to the retro-cued representation. We interpret the convergence of neural oscillations and behavioral data to point towards the theory that selection within WM could be accomplished through a perceptual attentional mechanism.

Emoji-SP, the Spanish emoji database: Visual complexity, familiarity, frequency of use, clarity, and emotional valence and arousal norms for 1031 emojis.

This article presents subjective norms for 1031 emojis in six dimensions: visual complexity, familiarity, frequency of use, clarity, emotional valence, and emotional arousal. This is the largest normative study conducted so far that relies on subjective ratings. Unlike the few existing normative studies, which mainly comprise face emojis, here we present a wide range of emoji categories. We also examine the correlations between the dimensions assessed. Our results show that, in terms of their affective properties, emojis are analogous to other stimuli, such as words, showing the expected U-shaped relationship between valence and arousal. The relationship between affective properties and other dimensions (e.g., between valence and familiarity) is also similar to the relationship observed in words, in the sense that positively valenced emojis are more familiar than negative ones. These findings suggest that emojis are suitable stimuli for studying affective processing. Emoji-SP will be highly valuable for researchers of various fields interested in emojis, including computer science, communication, linguistics, and psychology. The full set of norms is available at: https://osf.io/dtfjv/ .

Pain E-motion Faces Database (PEMF): Pain-related micro-clips for emotion research.

A large number of publications have focused on the study of pain expressions. Despite the growing knowledge, the availability of pain-related face databases is still very scarce compared with other emotional facial expressions. The Pain E-Motion Faces Database (PEMF) is a new open-access database currently consisting of 272 micro-clips of 68 different identities. Each model displays one neutral expression and three pain-related facial expressions: posed, spontaneous-algometer and spontaneous-CO2 laser. Normative ratings of pain intensity, valence and arousal were provided by students of three different European universities. Six independent coders carried out a coding process on the facial stimuli based on the Facial Action Coding System (FACS), in which ratings of intensity of pain, valence and arousal were computed for each type of facial expression. Gender and age effects of models across each type of micro-clip were also analysed. Additionally, participants' ability to discriminate the veracity of pain-related facial expressions (i.e., spontaneous vs posed) was explored. Finally, a series of ANOVAs were carried out to test the presence of other basic emotions and common facial action unit (AU) patterns. The main results revealed that posed facial expressions received higher ratings of pain intensity, more negative valence and higher arousal compared with spontaneous pain-related and neutral faces. No differential effects of model gender were found. Participants were unable to accurately discriminate whether a given pain-related face represented spontaneous or posed pain. PEMF thus constitutes a large open-source and reliable set of dynamic pain expressions useful for designing experimental studies focused on pain processes.

Completion of disconnective surgery for refractory epilepsy in pediatric patients using robot-assisted MRI-guided laser interstitial thermal therapy.

OBJECTIVE: Since 2007, the authors have performed 34 hemispherotomies and 17 posterior quadrant disconnections (temporoparietooccipital [TPO] disconnections) for refractory epilepsy at Sant Joan de Déu Barcelona Children's Hospital. Incomplete disconnection is the main cause of surgical failure in disconnective surgery, and reoperation is the treatment of choice. In this study, 6 patients previously treated with hemispherotomy required reoperation through open surgery. After the authors' initial experience with real-time MRI-guided laser interstitial thermal therapy (MRIgLITT) for hypothalamic hamartomas, they decided to use this technique instead of open surgery to complete disconnective surgeries. The objective was to report the feasibility, safety, and efficacy of MRIgLITT to complete hemispherotomies and TPO disconnections for refractory epilepsy in pediatric patients. METHODS: Eight procedures were performed on 6 patients with drug-resistant epilepsy. Patient ages ranged between 4 and 18 years (mean 10 ± 4.4 years). The patients had previously undergone hemispherotomy (4 patients) and TPO disconnection (2 patients) at the hospital. The Visualase system assisted by a Neuromate robotic arm was used. The ablation trajectory was planned along the residual connection. The demographic and epilepsy characteristics of the patients, precision of the robot, details of the laser ablation, complications, and results were prospectively collected. RESULTS: Four patients underwent hemispherotomy and 2 underwent TPO disconnection. Two patients, including 1 who underwent hemispherotomy and 1 who underwent TPO disconnection, received a second laser ablation because of persistent seizures and connections after the first treatment. The average precision of the system (target point localization error) was 1.7 ± 1.4 mm. The average power used was 6.58 ± 1.53 J. No complications were noted. Currently, 5 of the 6 patients are seizure free (Engel class I) after a mean follow-up of 20.2 ± 5.6 months. CONCLUSIONS: According to this preliminary experience, laser ablation is a safe method for complete disconnective surgeries and allowed epilepsy control in 5 of the 6 patients treated. A larger sample size and longer follow-up periods are necessary to better assess the efficacy of MRIgLITT to complete hemispherotomy and TPO disconnection, but the initial results are encouraging.

CROWD-5e: A Croatian psycholinguistic database of affective norms for five discrete emotions.

The present study introduces affective norms for a set of 3022 Croatian words on five discrete emotions: happiness, anger, sadness, fear, and disgust. The words were rated by 1239 Croatian native speakers. Each participant rated 251 or 252 words for one discrete emotion on a five-point Likert scale. The analyses revealed a significant relationship between discrete emotions, emotional dimensions (valence and arousal), and other psycholinguistic properties of words. In addition, small sex differences in discrete emotion ratings were found. Finally, the analysis of the distribution of words among discrete emotions allowed a distinction between "pure" words (i.e., those mostly related to a single emotion) and "mixed" words (i.e., those related to more than one emotion). The new database extends the existing Croatian affective norms collected from a dimensional conception of emotions, providing the necessary resource for future experimental investigation in Croatian within the theoretical framework of discrete emotions.