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This report discusses a rare case of a soft palate deformity in a young girl due to lipofibromatosis (LPF). This rare benign pediatric soft tissue tumour usually arises in the distal extremities. We believe this case represents the first report of lipofibromatosis involving only the maxillary bone.

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Limited evidence suggests that the SARS-CoV-2 infection can accelerate the progression of neurodegenerative diseases, but this has been not verified in the spinocerebellar ataxias (SCA). The objective of this study is to assess the impact of COVID-19 on the mental health and motor features of SCA2. A follow-up study was carried out in 170 Cuban SCA2 subjects and 87 community controls between 2020 and 2021. All subjects underwent a structured questionnaire to assess the risks of exposure to COVID-19, the confirmation of COVID-19 diagnosis, and the Hospital Anxiety and Depression Scale (HADS). Moreover, 36 subjects underwent the Scale for the Assessment and Rating of ataxia (SARA). The risk of exposure to SARS-CoV-2 and the frequency of COVID-19 were similar between the ataxia cohort and the community controls. Within the ataxia group, significantly increased HADS scores existed at the 2nd visit in both groups, but this increase was more evident for the infected group regarding the depression score. Moreover, a significant within-group increase of SARA score was observed in the infected group but not the non-infected group, which was mainly mediated by the significant increase of the speech item score in the infected group. Similar results were observed within the subgroup of preclinical carriers. Our study identified no selective vulnerability nor protection to COVID-19 in SCA2, but once infected, the patients experienced a deterioration of mental health and speech function, even at preclinical disease stage. These findings set rationales for tele-health approaches that minimize the detrimental effect of COVID-19 on SCA2 progression and identify SCA2 individuals as clinical model to elucidate the link between SARS-CoV-2 infection and neurodegeneration.

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Grain mold and stalk rot are among the fungal diseases that cause significant losses in sorghum worldwide and are caused by different Fusarium spp. The presence of Fusarium species in sorghum grains causes yield losses and mycotoxin contamination, which represents a risk to consumers. In this study, Fusarium graminearum species complex (FGSC) had a high incidence, followed by Fusarium fujikuroi species complex (FFSC) and F. incarnatum-equiseti species complex. Within FFSC, F. proliferatum, F. andiyazi, F. fujikuroi, F. thapsinum, F. verticillioides and F. subglutinans were identified, and this was the first report of F. fujikuroi in sorghum. The most frequent toxins found in sorghum samples were deoxynivalenol (DON) and zearalenone (ZEN). The presence of fumonisins and nivalenol (NIV) was detected at low levels. This study adds new knowledge about the occurrence of Fusarium species and mycotoxins in sorghum grains. Furthermore, this is the first report in Uruguay on fungicide sensitivity for Fusarium isolates from sorghum, which constitutes an important starting point for defining management practices to minimize fungal infection and mycotoxin contamination.

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BACKGROUND: Antisynthetase syndrome is an inflammatory myopathy that is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies. Only 30% of those who suffer from the disease can be identified. We present three Hispanic cases of antisynthetase syndrome with unusual clinical pictures were extended myositis panel results enable disease diagnosis and treatment. CASE PRESENTATION: A 57-year-old Hispanic/Latino female with an erythematous scaly plaque, unresolved fever and non-immune haemolytic anaemia in whom inpatient work-up for fever of unknown origin was positive for anti-PL12 positive myositis extended panel. A 72-year-old Hispanic/Latino male with amyopathic weakness syndrome and mechanic hands in whom impatient work-up was relevant for proximal muscle uptake and anti-PM75 and AntiPL-12 myositis extended panel. And a 67-year-old Hispanic/Latino male with progressive interstitial lung disease and unresolved fever ended in myositis extended panel positive for antiPL-7. After systemic immunosuppressor treatment, patients had favourable clinical and paraclinical responses during outpatient follow-up. CONCLUSIONS: The high variability of the antisynthetase syndrome in these cases demonstrates the importance of identification through an expanded panel and highlights the probability that this is a variable disease and that we need to include emerging molecular tests to promote the timely treatment of patients.

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Although there are no convincing evidences of detrimental effect of SARS-CoV2 infection on the cerebellum, the COVID-19 pandemic could impact the life quality of patients with cerebellar ataxias, but few studies have addressed this concern. To assess the motor and mental health changes caused by the COVID-19 pandemics in Cuban patients with cerebellar ataxias, three hundred four patients with cerebellar ataxias and 167 healthy controls were interviewed for risks of exposure to COVID-19, and the self-perception of the pandemics' impact on the disease progression and on the mental health. All subjects underwent the Hospital Anxiety and Depression Scale. The patients reported low exposition to SARS-CoV2 infection, but one case was confirmed with a mild COVID-19. Overall, depressive and anxiety symptoms were significantly and marginally increased in patients, respectively, with higher scores in cases with severe and moderate ataxia. Positive patient's impression of psychopathological changes was associated to increased age, age at onset, and anxiety. Sixty-seven patients had a positive self-perception of ataxia progression, which was mainly influenced by higher anxiety scores but not by the adherence to at-home exercise programs. However, the practice of physical exercise was related with lower depression and anxiety scores, but this therapeutical effect was not significantly influenced by the disease stage. We demonstrated the negative effect of the COVID-19 pandemic on the mental and motor deficits in Cuban patients with cerebellar ataxias and the positive effect of the at-home physical exercise programs on their mental well-being. These findings give rationales to develop tele-medicine approaches to minimize these health impacts and to study the long-term effects of such sequelae and accordingly define their treatments.

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The prevalence estimations of hereditary ataxias are biased since most epidemiological studies are confined to isolated geographical regions and few nationwide studies are available. The study aims to assess the prevalence, distribution, and neurological features of the Cuban population with hereditary ataxias. A nationwide epidemiological study of hereditary ataxias was conducted in Cuba between March 2017 and June 2018. Patients were scheduled at the Cuban ataxia research center, various hospitals, or at their homes. Demographic and clinical variables were obtained through standardized questionnaires and validated clinical tools. Overall, 1001 patients were diagnosed with hereditary ataxias for a nationwide prevalence of 8.91 cases/100.000 inhabitants. Spinocerebellar ataxia type 2 (SCA2) was the commonest subtype, with highest prevalences at Holguín province (47.86/100.000), and a broad dissemination in the whole country. Most of neurological features were common between all SCA cohorts, but the frequencies of some of them varied between distinct subtypes. Within the SCA2 cohort, significant influences of long mutation size and higher disease duration over the muscle atrophy and oculomotor disorders were observed. Besides, higher disease durations were associated with resting tremor and dysphagia, whereas shorter disease durations were associated with hyperreflexia. The spreading of SCA2 to whole country and the documented raising of its prevalence set the rationales for higher-scope medical care and research strategies, supported in collaborative research networks. The wide epidemiological, clinical, and genetic characterization of this founder SCA2 population identifies this homogeneous cohort as an attractive source for the development of future clinical-genetic and therapeutic researches.

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Se realizó un estudio de tipo no experimental, descriptivo, transversal en el Hospital Pediátri-co Universitario "José Luís Miranda", provincia de Villa Clara, Cuba; durante el período 2007 ­ 2012. Se trabajó con la totalidad de la población de estudio, la que estuvo integrado por 56 casos interpretados mediante el método diagnóstico del Síndrome Neurológico Infec-cioso: meningoencefalitis bacteriana, cuyos participantes fueron seleccionados a partir de las Tarjetas de Declaración Obligatoria (EDO). Los datos fueron recopilados mediante la revi-sión de documentos (historias clínicas) y procesados mediante estadísticas descriptivas. El 42,86% de los pacientes correspondió a niños menores de 1 año; así mismo el 21,43% resultó causado por meningococo y una cifra similar por neumococo. Se observó un predominio del género masculino; la etiología fue mayoritariamente neumocóccica y meningocóccica; los síntomas más frecuentes fueron fiebre, cefalea y vómitos escasos; así como la complicación más importante observada resultó el fallecimiento de algunos pacientes.

A non-experimental, descriptive, cross-sectional study was carried out at the "José Luís Miranda" University Pediatric Hospital, in the province of Villa Clara-Cuba, during the period 2007 ­ 2012. The total population of the study was composed of 56 cases interpreted by the diagnostic method of Infectious Neurological Syndrome: bacterial meningoencephali-tis, whose participants were selected from the Obligatory Declaration Cards (ODC). The data was collected by reviewing documents (medical records) and these were processed using descriptive statistics. 42.86% of the patients were children under one year of age; likewise, 21.43% was caused by meningococcus and a similar number by pneumococcus. A predomi-nance of the masculine gender was observed. The etiology was mostly pneumococcal and meningococcal, the most frequent symptoms were fever, headache and poor vomiting; as well as the most important complication observed was the death of some patients.

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ASBTRACT: BACKGROUND: The complexity of current organizations implies a potential overload for workers. For this reason, it is of interest to study the effects that mental workload has on the performance of complex tasks in professional settings. OBJECTIVE: The objective of this study is to empirically analyze the relation between the quality of decision-making, on the one hand, and the expected and real mental workload, on the other. METHODS: The study uses an ex post facto prospective design with a sample of 176 professionals from a higher education organization. Expected mental workload (Pre-Task WL) and real mental workload (Post-Task WL) were measured with the unweighted NASA-Task Load Index (NASA-TLX) questionnaire; difference between real WL and expected WL (Differential WL) was also calculated; quality of decision-making was measured by means of the Decision-Making Questionnaire. RESULTS: General quality of decision-making and Pre-Task WL relation is compatible with an inverted U pattern, with slight variations depending on the specific dimension of decision-making that is considered. There were no verifiable relations between Post-Task WL and decision-making. The subjects whose expected WL matched the real WL showed worse quality in decision-making than subjects with high or low Differential WL. CONCLUSIONS: The relations between mental workload and decision-making reveal a complex pattern, with evidence of nonlinear relations.

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Asbtract Background The complexity of current organizations implies a potential overload for workers. For this reason, it is of interest to study the effects that mental workload has on the performance of complex tasks in professional settings. Objective The objective of this study is to empirically analyze the relation between the quality of decision-making, on the one hand, and the expected and real mental workload, on the other. Methods The study uses an ex post facto prospective design with a sample of 176 professionals from a higher education organization. Expected mental workload (Pre-Task WL) and real mental workload (Post-Task WL) were measured with the unweighted NASA-Task Load Index (NASA-TLX) questionnaire; difference between real WL and expected WL (Differential WL) was also calculated; quality of decision-making was measured by means of the Decision-Making Questionnaire. Results General quality of decision-making and Pre-Task WL relation is compatible with an inverted U pattern, with slight variations depending on the specific dimension of decision-making that is considered. There were no verifiable relations between Post-Task WL and decision-making. The subjects whose expected WL matched the real WL showed worse quality in decision-making than subjects with high or low Differential WL. Conclusions The relations between mental workload and decision-making reveal a complex pattern, with evidence of nonlinear relations.

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Shubin and Alberch (Evol Biol 1986;20:319-387) proposed a scheme of tetrapod limb development based on cartilage morphogenesis that provides the arguments to interpret the homologies of skeletal elements and sets the basis to explain limb specialization through later developmental modification. Morphogenetic evidence emerged from the study of some reptiles, but the availability of data for lizards is limited. Here, the study of adult skeletal variation in 41 lizard taxa and ontogeny in species of Liolaemus and Tupinambis attempts to fill in this gap and provides supporting evidence for the Shubin-Alberch scheme. Six questions are explored. Is there an intermedium in the carpus? Are there two centralia in the carpus? Is there homology among proximal tarsalia of reptiles? Does digit V belong to the digital arch? Is the pisiform an element of the autopodium plan? And should the ossification processes be similar to cartilage morphogenesis? We found the following answers. Some taxa exhibit an ossified element that could represent an intermedium. There is one centrale in the carpus. Development of proximal tarsalia seems to be equivalent with that observed among reptiles. Digit V could arise from the digital arch. Pisiform does not arise as part of the limb plan. And different patterns of ossification occur following a single and conservative cartilaginous configuration. Lizard limb development shows an early pattern common to other reptiles with clear primary axis and digital arch. The pattern then becomes lizard-specific with specialization involving some reduction in prechondrogenic elements.

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