Behavioral alterations associated with levetiracetam in pediatric epilepsy.

Levetiracetam (LEV) has an improved pharmacological profile and is one of the most commonly used antiepileptic drugs (AEDs). However, associations between this pharmacological profile and behavioral side effects have been extensively reported in pediatric populations. We assessed behavioral changes after initiation of LEV, prescribed by the treating neurologist, in Chilean patients with epilepsy aged 4-15 years. A behavioral questionnaire was applied at baseline and at two, four, and twelve weeks of treatment. Thirty patients were enrolled: 16 males, 14 females, average age 8 years (range: 4-14). By week four, 23.3% of patients showed significant behavioral alterations that persisted throughout the observation period. No significant alterations emerged after four weeks in the remaining patients. Family history of psychiatric disease and prior behavioral difficulties were predisposing factors for adverse behavioral effects. Although previous studies associated adverse behavioral effects with LEV in pediatric patients with epilepsy, we believe that this is the first study to use a prospective methodology and standardized tools to quantify the symptomatology. Adverse behavioral effects may significantly affect quality of life for patients and families, diminishing the tolerability of treatment. To ensure successful therapy and improve medical decision-making, it is essential to consider predisposing factors for drug-related adverse effects and to regularly assess for behavioral alterations during treatment.

Dual Targeting of Stromal Cell Support and Leukemic Cell Growth by a Peptidic PKC Inhibitor Shows Effectiveness against B-ALL.

Mesenchymal stem cells (MSC) favour a scenario where leukemic cells survive. The protein kinase C (PKC) is essential to confer MSC support to leukemic cells and may be responsible for the intrinsic leukemic cell growth. Here we have evaluated the capacity of a chimeric peptide (HKPS), directed against classical PKC isoforms, to inhibit leukemic cell growth. HKPS was able to strongly inhibit viability of different leukemic cell lines, while control HK and PS peptides had no effect. Further testing showed that 30% of primary samples from paediatric B-cell acute lymphoblastic leukaemia (B-ALL) were also strongly affected by HKPS. We showed that HKPS disrupted the supportive effect of MSC that promote leukemic cell survival. Interestingly, ICAM-1 and VLA-5 expression increased in MSC during the co-cultures with B-ALL cells, and we found that HKPS inhibited the interaction between MSC and B-ALL cells due to a reduction in the expression of these adhesion molecules. Of note, the susceptibility of B-ALL cells to dexamethasone increased when MSC were treated with HKPS. These results show the relevance of these molecular interactions in the leukemic niche. The use of HKPS may be a new strategy to disrupt intercellular communications, increasing susceptibility to therapy, and at the same time, directly affecting the growth of PKC-dependent leukemic cells.

Phenotypic variability of GLUT1 deficiency: When is necessary to suspect? / Variabilidad fenotípica del déficit de GLUT1: ¿Cuándo es necesario sospechar?

INTRODUCTION: Glucose Transporter Type 1 Deficiency Syndrome (GLUT1-DS) is caused by the SLC2A1 gene muta tion, which encodes the glucose transporter proteins to the brain Neurological manifestations occur in three main domains: seizures, abnormal movements, and cognitive disorders. The diagnosis is presumed upon the finding of low CSF glucose and confirmed by the gene molecular analysis. Ac curate diagnosis is important because it has a specific treatment, which is ketogenic diet. OBJECTIVE: To analyze two SD-GLUT1 pediatric patients with unusual phenotype. CLINICAL CASE: We present the case of two siblings who presented absence seizures and a paroxysmal movement disorder. Both patients were studied, finding low CSF glucose. The diagnosis of GLUT1-DS was confirmed with molecular analysis. Specific treatment with ketogenic diet achieved good response in both cases. Con clusions: We present their peculiar clinical characteristics that allowed us to suspect this wide phe notypic spectrum. Correct and timely diagnosis and treatment can significantly improve the quality of life of those affected.

Confirmatory factor analysis and measurement invariance of the eating disorders examination-questionnaire across four male samples in Argentina.

OBJECTIVE: The eating disorder examination-questionnaire (EDE-Q) is among the most widely used instruments in eating disorder research and clinical practice. However, the underlying structure remains a source of confusion, and contradictory results have emerged in studies among male populations. In the current study, we examined previously proposed models of EDE-Q structure in four community samples of Argentinian men. METHOD: A series of confirmatory factor analyses (CFAs) were performed for five previous factor structure models of the EDE-Q among 232 Argentinian male university students, 277 weightlifters, 275 cross-fit users, and 202 athletes. A multigroup CFA was conducted in the model we retained, to assess measurement invariance across groups. RESULTS: A respecified model of the brief eight-item one-factor proposal provided acceptable fit to the data over the original four-factor structure and three other proposed models. Results from the multigroup CFA showed that the retained model was invariant across samples. CONCLUSION: Our results provide support for retaining a one-factor EDE-Q structure over a multifactor solution for research purposes among male community samples in Argentina. These data underscore the importance of undertaking psychometric assessment of eating disorder symptom measures before their utilization in specific populations.

Development and validation of a multicultural Spanish-language version of the Muscularity-Oriented Eating Test (MOET) in Argentina.

The utility of traditional eating disorder measures in the assessment of muscularity-oriented disordered eating has been questioned. To address this limitation, the Muscularity-Oriented Eating Test (MOET) was recently developed and validated in a sample of U.S. college men. We aimed to develop a multicultural Spanish-language version of the MOET for use in Latin American samples and validate its use in a sample of Argentinian college men. Combined translation procedures were used to develop a version suitable for different Spanish-speaking populations. A total of 235 students (Mage = 23.47, SD = 5.61) participated in this study by completing a survey including the MOET. A sub-sample (n = 121) completed the MOET again after 1 week. A confirmatory factor analysis of a re-specified model of the original single-factor MOET, allowing for residual correlation between items associated to dietary rules (items 4-12), resulted in an adequate fit (χ2/df = 2.10, CFI = 0.94, TLI = 0.93, RMSEA 0.05 [90% CI = 0.04, 0.06] SRMR = 0.08). Further, the multicultural Spanish-language version of the MOET yielded evidence of internal consistency (omega = 0.83, 95% CI [0.79, 0.88], Cronbach's α = 0.83), a 1-week Intraclass Correlation Coefficient was considered for test-retest reliability (ICC = 0.82), item analysis, convergent validity with measures of eating disorder psychopathology, body dissatisfaction and weight-related behaviors, as well as for divergent validity with an unrelated construct. The availability of a multicultural Spanish-language version of the MOET may have utility in both clinical and research efforts related to muscularity-oriented disordered eating among Latino men.

How words ripple through bilingual hands: Motor-language coupling during L1 and L2 writing.

The speed of our hand movements can be affected by concurrent processing of manual action verbs (MaVs). Whereas this phenomenon is well established for native languages (L1s), it remains underexplored in late foreign languages (L2s), especially during highly automatized tasks. Here we timed keystroke activity while Spanish-English bilinguals copied MaVs, non-manual action verbs, and non-action verbs in their L1 and L2. Motor planning and execution dynamics were indexed by first-letter lag (the time-lapse between word presentation and first keystroke) and whole-word lag (the time-lapse between first and last keystroke), respectively. Despite yielding no effects on motor planning, MaVs facilitated typing execution in L1 but delayed it in L2, irrespective of the subjects' typing skills, age of L2 learning, and L2 competence. Therefore, motor-language coupling effects seem to be present in both languages though they can arise differently in each. These results extend language grounding models, illuminating the role of embodied mechanisms throughout life.

Variabilidad fenotípica del déficit de GLUT1: ¿Cuándo es necesario sospechar? / Phenotypic variability of GLUT1 deficiency: when is necessary to suspect?

Resumen: Introducción: La deficiencia del transportador de glucosa tipo 1 constituye un síndrome (SD-GLUT1), provocado por la mutación del gen SLC2A1, que codifica la proteína transportadora de glucosa al encéfalo. Las manifestaciones neurológicas se dan en tres dominios principales: crisis epilépticas, movimientos anormales y alteraciones cognitivas. El diagnóstico se presume ante el hallazgo de hipoglucorraquia y se confirma mediante el análisis molecular del gen. La importancia de precisarlo radica en que tiene tratamiento específico, la dieta cetogénica. Objetivo: Analizar dos casos clínicos de SD-GLUT1 de presentación atípica, destacando la variabilidad del fenotipo. Caso Clínico: Presentamos el caso de dos hermanos cuyas manifestaciones fueron crisis epilépticas de tipo ausencias típicas, y un trastorno paroxístico del movimiento. Los pacientes fueron estudiados encontrándose hipoglucorraquia en ambos y se confirmó diagnóstico de SD-GLUT1 con estudio molecular. El tratamiento específico con dieta cetogénica logró buena respuesta. Conclusiones: Exponemos sus características clínicas peculiares que nos permitieron sospechar este cuadro, de espectro fenotípico amplio, cuyo diagnós tico y tratamiento, correcto y oportuno, puede mejorar significativamente la calidad de vida de los afectados.

Abstract: Introduction: Glucose Transporter Type 1 Deficiency Syndrome (GLUT1-DS) is caused by the SLC2A1 gene muta tion, which encodes the glucose transporter proteins to the brain Neurological manifestations occur in three main domains: seizures, abnormal movements, and cognitive disorders. The diagnosis is presumed upon the finding of low CSF glucose and confirmed by the gene molecular analysis. Ac curate diagnosis is important because it has a specific treatment, which is ketogenic diet. Objective: To analyze two SD-GLUT1 pediatric patients with unusual phenotype. Clinical Case: We present the case of two siblings who presented absence seizures and a paroxysmal movement disorder. Both patients were studied, finding low CSF glucose. The diagnosis of GLUT1-DS was confirmed with molecular analysis. Specific treatment with ketogenic diet achieved good response in both cases. Con clusions: We present their peculiar clinical characteristics that allowed us to suspect this wide phe notypic spectrum. Correct and timely diagnosis and treatment can significantly improve the quality of life of those affected.