The effect of the Schwartz-Jampel syndrome on masticatory and facial musculatures--an electromyographic analysis.

This study had the goal to perform an electromyography evaluation of the orbicularis oris, orbicularis oculi, masseter, and temporal muscles of two siblings with Schwartz-Jampel syndrome (SJS), in different clinical activities, comparing them to healthy controls (C). The Schwartz-Jampel syndrome is a rare genetic disorder (71 cases reported in worldwide literature), in which myotonia may be observed in the facial muscles, determining a standard facie that shows an appearance of someone who is sad and weeping For the electromyography (EMG), a Myotronics--K61 electromyographer, was used, with superficial and disposable silver chloride electrodes. By means of the analysis of the obtained results, we observed significant statistical differences for the masseter muscle and for orbicularis oculi muscles (p < 0.01) among the studied groups, in which the individuals with SJS presented greater muscular activity than the normal ones, used as healthy controls. The statistical difference between the two groups was not significant for the temporal muscle, as well as for the orbicularis oris muscle, although the EMG averages were much greater in patients who were bringers of the syndrome. This high muscular activity may be related to the facial osseous alterations, evidenced in both patients with SJS, such as the mandibular migrognathia, with an atrophy of the mandibular cortex and a consequent approximation between the inferior dental root apices and the mandible base, as well as the presence of hypoplastic condyles in terms of size and height.

Evaluación microbiológica y fisicoquímica de néctares pasteurizados elaborados con pulpa de tomate de árbol (Cyphomandra betaceae Sendth) / Microbiological and physicochemical evaluation of pasteurized nectar elaborated with tree tomato (Cyphomandra betaceae Sendth)pulp

Tree tomato (Cyphomandra betaceae Sendth) is a species from high tropical regions. In Venezuela, it is cultivate at the Andean and Aragua state but its consumption is restricted as fruit-fresh, though it a nutritious and industrial potential due its provitamin A content. In this research four nectars were elaborated in proportion I L pulp/4 L of water (1:4) and addition of ascorbic acid (I: 0 per cent; II: 0.5 per cent; III: 1.0 y IV: 1.5 per cent). The nectars were pasteurized (60 degrees C for 30 min), tuned into amber bottle, and stored under refrigeration conditions (7.0 +/- 1.0 degrees C). Weekly during 21 days the mesophilic bacteria, molds, yeasts, total coliforms (MPN/mL), pH, degree Brix, acidity, total carotenoids, vitamin C and total sugars were evaluated. The mesophilic bacteria content was < 200 UFC/mL for every formulation on the first day the evaluation, molds and yeasts were < 10 UFC/mL and the total coliforms (NPN/mL) < 3 until the third evaluation. No significant differences (P > 0.05) were founded on: pH. degree Brix and total sugars. Significant differences (P < 0.05) were founded in vitamin C and total carotenoids content. Sensorial analysis did not show significant differences between formulations for the smell and flavor attributes, when the color was discriminate during the evaluations. The formulation I (without acid ascorbic) had more preference due its color. In conclusion, the nectars showed useful life of 14-21 days under refrigeration condition storage due to the adequate physicochemical and microbiological quality of the product.