Fixation instability and oculomotor abnormalities in Friedreich's ataxia

Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnormalities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients showed various combinations of cerebellar, vestibular and brain-stem oculomotor signs. Our patients did not comprise a homogeneous group with regard to their oculomotor findings. There was no correlation between the severity of any of the so-called cerebellar oculomotor disturbances and the number of SWJ. We tried to correlate the extent of oculomotor disturbances with floccular atrophy and atrophy of the dorsal vermis on MRI in seven of the patients. None of the oculomotor features (including SWJ) correlated with flocculus or dorsal vermis size. Furthermore, floccular and vermal measurements on MRI were normal. Accordingly, we think it unlikely that the oculomotor disturbances, including SWJ, are attributable to cerebellar pathology per se.     

The influence of contamination of culture medium with hepatitis B virus on the outcome of in vitro fertilization pregnancies.

Heat-inactivated human serum is added to the culture medium used for in vitro fertilization and other forms of assisted conception. Because one batch of pooled serum contained hepatitis B virus, an epidemic occurred among women participating in the treatment program. Seventy-nine women had serologic proof of hepatitis B infection. This incident gave the opportunity to study the effect of hepatitis B virus on pregnancy outcome and the newborn. The situation is unique because the preimplantation embryo was exposed to hepatitis B virus or the pregnancy was complicated by a (sub)clinical infection. Twenty-four women were or became pregnant while having an acute hepatitis B infection. Five pregnancies ended in abortion. The remaining 19 pregnancies ended in the birth of 24 children. No evidence for any harmful effect of exposure to hepatitis B virus in the embryonic or fetal period on the newborn could be found.     

Paraovarian cysts associated with prenatal diethylstilbestrol exposure. Comparison of the human with a mouse model

The association of prenatal exposure to diethylstilbestrol (DES) and abnormalities in structures of müllerian (paramesonephric) origin has been well documented. In a murine model, exposure to DES in utero results in persistent mesonephric remnants in adult female mice. Six women exposed prenatally to DES had paraovarian cysts excised during routine gynecologic surgery; and in 4, histologic abnormalities were observed, including thickened fibromuscular walls with tall columnar epithelium in a papillary or pseudoglandular configuration. Four of 25 nonexposed and 8 of 9 DES-exposed infertile women undergoing surgery for infertility had paraovarian cysts, and the difference was statistically significant (P less than 0.02). These findings raise the possibility that structures derived from the mesonephric ducts or tubules may also be affected in women exposed in utero to DES.     

The nonverbal language of the health educator

Communication in the classroom is often a problem for both students and teachers. Previous research has shown that non-verbal teaching behavior can have a profound effect on students when mixed with verbalization and may serve as a major source of confusion. Misinterpretation is common and in the area of health education may foster the development of negative health attitudes. Health education is a shared experience of students and their teacher, one which should be comfortable and supportive. It is the educators responsibility to develop teaching behaviors that are encouraging, inspiring, and comfortable. This can be accomplished by using a variety of techniques requiring a minimum of time, equipment and energy. The purpose of this article is to demonstrate the need for cognizance of personal teaching behaviors and to suggest methods to evaluate these behaviors.     

Assessing vestibular function: which tests, when?

Vertigo and dizziness are common complaints encountered in clinical practice. The patient’s history and a thorough otoneurological evaluation are essential for identifying the specific pathology behind the patient’s complaints. If the patient reports an illusion of movement (vertigo), this most likely indicates an imbalance within the vestibular system. A sensation of rotatory movement together with a spontaneous nystagmus suggests a lesion involving the semicircular canals, while an illusion of linear movement indicates a disturbance of the otoliths. Nystagmus of central origin or caused by a peripheral vestibular lesion can usually be distinguished by other features in the history or on clinical examination. While peripheral vestibular lesions usually lead to a mixed horizontal-torsional or vertical-torsional nystagmus, a pure vertical or pure torsional nystagmus is always caused by a central lesion. With simple bedside tests such as head-shaking nystagmus and rapid head impulses deficits in labyrinthine function can clearly be detected. For a more thorough investigation of vestibular function at the level of individual semicircular canals and the otoliths, modern techniques are now available such as three-dimensional eye movement vector analysis for the evaluation of individual semicircular canal function, measurement of the subjective visual vertical for utricular, and click-evoked myogenic potentials for saccular testing. Received: 3 October 1999/Accepted: 7 December 1999     

Interoperability—Making Information Systems Work Together

Disaster literature suggests that children's and adolescents’ post-disaster reactions vary according to their developmental levels. Preschool children show less psychological problems as compared to older children and adolescents, but they have a higher incidence of trauma-specific fears and behavioral problems (e.g., dependency, clinging). School-age children's disaster responses include sleep and eating disturbances, behavioral problems, and poor school performance. Adolescents tend to exhibit symptoms such as posttraumatic stress disorder, depression, anxiety, belligerence, and pessimistic views about the future (Korol, Green, &; Gleser, ).     

Usefulness of transcutaneous triggering of conventional implanted pulse generators by an activity-sensing pacemaker for predicting effectiveness of rate response pacing

A noninvasive procedure has been developed to assess effectiveness of activity-sensing rate response ventricular (VVIR) pacing systems in patients with implanted conventional fixed-rate ventricular (VVI) pacemakers capable of triggered mode operation (VVT). A VVIR pacemaker (activity threshold medium or low, rate response 6 to 10, upper rate 125 or 150 beats/min) was strapped to the chest wall (prepectoral area) of patients with previously implanted fixed-rate VVI pacemakers. In 17 of the 18 patients included in the study, the implanted pacemaker was capable of triggered mode operation (VVT). Triggering of the conventional pacemaker was achieved by reprogramming it to the VVT mode (high sensitivity, short refractory) and connecting the output of the "strapped-on" VVIR pacemaker (5 volts, 1.5-ms pulse width) to 2 standard cutaneous electrodes positioned so as to be in proximity to the implanted intracardiac electrode system. Patients underwent symptom-limited treadmill exercise tests during both VVI and VVIR pacing. Triggering conventional VVI pulse generators by a "strapped-on" VVIR pacing system proved feasible in 16 of 17 cases and improved overall heart rate response (fixed rate 86 +/- 22 vs VVIR 118 +/- 7 beats/min) and exercise duration (fixed rate 6.6 +/- 4.9 vs VVIR 10.1 +/- 4.8 minutes) (mean values for all patients studied during exercise testing). Triggering of a previously implanted permanent pacemaker by a strapped-on activity-triggered device may be useful to assess rate response pacing before implantation of a VVIR device in patients in whom elective pulse generator replacement is planned.     

The effect of enteral supplementation of specific neutral and acidic oligosaccharides on the faecal microbiota and intestinal microenvironment in preterm infants

We aimed to determine the effects of enteral supplementation of a prebiotic mixture of neutral and acidic oligosaccharides (scGOS/lcFOS/pAOS) on the faecal microbiota and microenvironment in preterm infants. Furthermore, we determined the influence of perinatal factors on the development of the faecal microbiota. In a randomised controlled trial, preterm infants with gestational age <32 weeks and/or birth weight <1,500 g received enteral supplementation of scGOS/lcFOS/pAOS or placebo (maltodextrin) between days 3 and 30 of life. Faecal microbiota, as measured with fluorescent in situ hybridisation (FISH), and microenvironment [short-chain fatty acids (SCFAs), pH, sIgA] were measured at four time points: before the start of the study and at days 7, 14 and 30 of life. In total, 113 preterm infants were included. Enteral supplementation of the prebiotic mixture increased the total bacteria count at day 14 (Exp 3.92; 95 % confidence interval [CI] 1.18–13.04, p?=?0.03), but not at day 30 (Exp 1.73; 95 % CI 0.60–5.03, p?=?0.31). There was a trend toward increased bifidobacteria counts. There was a delayed intestinal colonisation of all bacteria. Enteral supplementation of the prebiotic mixture decreased the faecal pH (Exp 0.71; 95 % CI 0.54–0.93, p?=?0.01) and there was a trend toward increased acetic acid compared to the placebo group (Exp 1.09; 95 % CI 0.99–1.20, p?=?0.10). There was no effect on sIgA (Exp 1.94; 95 % CI 0.28–13.27, p?=?0.50). Antibiotics decreased the total bacteria count (Exp 0.13; 95 % CI 0.08–0.22, p?<?0.001). Enteral supplementation of a prebiotic mixture of neutral and acidic oligosaccharides increases the postnatal intestinal colonisation. However, the extensive use of broad-spectrum antibiotics in preterm infants decreased the growth of all intestinal microbiota, thereby, delaying the normal microbiota development.