Hyperactive vestibulo-ocular reflex in cerebellar degeneration: pathogenesis and treatment

We studied a patient with a cerebellar degeneration and hyperactive vestibulo-ocular reflex (VOR). He complained of oscillopsia and blurred vision with head movement. A twofold increase in VOR gain (peak eye velocity/peak head velocity) at high frequencies was associated with a VOR time constant of 6 seconds (low normal). Visual cancellation ("suppression") of the VOR and smooth pursuit were also abnormal. We hypothesized that his high VOR gain was due to dysfunction of olivocerebellar projections. Physostigmine reduced his VOR gain, consistent with the hypothesis that these projections are cholinergic.     

Muscle energy metabolism in human phosphofructokinase deficiency as recorded by 31P nuclear magnetic resonance spectroscopy

31P nuclear magnetic resonance studies of a patient with phosphofructokinase deficiency in muscle provided the following new findings: First, ATP metabolism is disturbed at rest and during exercise. At rest, ATP levels are lower than normal and continue to decline during exercise. Second, exercise kinetics are normal, suggesting a normal mitochondrial fuel supply although glycolysis is blocked. Third, no "phosphate trapping" is observed during prolonged low-level exercise. Fourth, postexercise recovery is abnormally prolonged by the slow dephosphorylation of sugar phosphates, which has an in vivo half-life of about nine minutes. These findings demonstrate how muscle tissue adapts to a block in a major bioenergetic pathway.     

Optimizing Disclosure of HIV Status to a Diverse Population of HIV-Positive Youth at an Urban Pediatric HIV Clinic

PurposeThe purpose of the study was to increase the proportion of youth living with HIV (YLWH) aged ≥11 years who undergo developmentally appropriate disclosure about their HIV status.MethodsA quality improvement project was initiated at an urban pediatric HIV clinic between July 2018 and March 2020. The primary outcome measure was the proportion of YLWH aged ≥11 years who were disclosed to about their HIV status. The proportion of undisclosed YLWH who had documented nondisclosure status was also assessed as a process measure. Plan-Do-Study-Act (PDSA) cycles for change included monthly clinic staff check-ins to discuss new disclosures, quarterly team meetings to discuss strategies to improve disclosure, and modifying a clinic note template to prompt providers to document disclosure status. Annotated run charts were used to analyze the data.ResultsBefore the first PDSA cycle, 26/46 (57%) of the target population of YLWH aged ≥11 years had their HIV status disclosed to them, and none of the undisclosed youth had disclosure status documented in their medical record. After 20 months and six PDSA cycles, the proportion of YLWH aged ≥11 years disclosed to about their HIV status increased to 80% and the proportion of undisclosed YLWH with documentation of their disclosure status increased to 100%.ConclusionsSeveral interventions integrated throughout the pediatric HIV care process were associated with an increase in the proportion of YLWH with developmentally appropriate HIV disclosure and documentation of disclosure status, an important psychosocial aspect of care in these individuals.     

An Exploration of Dyadic Relationship Approach-Avoidance Goals and Relationship and Sexual Satisfaction in Couples Coping with Endometriosis

Endometriosis affects women of reproductive age and is associated with higher levels of sexual and relational distress. Despite the relational context of endometriosis, the research pertaining to dyadic relationship goals is lacking. An exploration of the relationship goals of couples coping with endometriosis can facilitate the understanding of potential protective mechanisms that mitigate the relational components of the condition. Guided by the approach-avoidance theoretical framework, the current cross-sectional study aimed to examine the role relationship goals play in sexual and relationship satisfaction in couples coping with endometriosis. Approach goals relate to the pursuit of a positive outcomes, whereas avoidance goals relate to the avoidance of negative outcomes. Women with endometriosis and their partners (N?=?61) completed an online survey measuring relationship goals and relationship and sexual satisfaction. The study results indicated that, for women, their own and their partner’s higher relationship approach goals were linked to higher sexual satisfaction. For partners of women with endometriosis, their own higher relationship approach goals were associated with their own higher relationship satisfaction. Higher relationship avoidance goals in both women with endometriosis and partners were associated with higher relationship satisfaction. The study’s findings highlight relationship goals as relevant to the relational and sexual experience of couples coping with endometriosis. When treating women with endometriosis, the inclusion of partners and consideration of factors beyond the physical illness are important for a holistic management approach.

     

Mission impossible? The definition and functions of the medical director

Seeks to throw light on both strategic and managerial aspects of the post of medical director. The medical director is a key executive member of the Trust Board, yet the role remains ill-defined. The existing published guidance gives some indication of what medical directors should be doing but gives limited information about "best practice" or how to cope with the demands being made on them. A survey of medical directors in 1994 revealed the diversity of the tasks that they can be called on to perform, often with inadequate managerial and secretarial support. The survey also indicated that problems have occurred as a result of the heavy workload and unexpected multiple facets of the role. Concludes that, to make the job somewhat easier, there should be a clear core job specification which is tailored to suit the individual circumstances. This should be combined with generous support and training, together with a suitable reward package.     

Potassium homeostasis in vacuolate plant cells

Plant cells contain two major pools of K+, one in the vacuole and one in the cytosol. The behavior of K+ concentrations in these pools is fundamental to understanding the way this nutrient affects plant growth. Triple-barreled microelectrodes have been used to obtain the first fully quantitative measurements of the changes in K+ activity (aK) in the vacuole and cytosol of barley (Hordeum vulgare L.) root cells grown in different K+ concentrations. The electrodes incorporate a pH-selective barrel allowing each measurement to be assigned to either the cytosol or vacuole. The measurements revealed that vacuolar aK declined linearly with decreases in tissue K+ concentration, whereas cytosolic aK initially remained constant in both epidermal and cortical cells but then declined at different rates in each cell type. An unexpected finding was that cytoplasmic pH declined in parallel with cytosolic aK, but acidification of the cytosol with butyrate did not reveal any short-term link between these two parameters. These measurements show the very different responses of the vacuolar and cytosolic K+ pools to changes in K+ availability and also show that cytosolic K+ homeostasis differs quantitatively in different cell types. The data have been used in thermodynamic calculations to predict the need for, and likely mechanisms of, active K+ transport into the vacuole and cytosol. The direction of active K+ transport at the vacuolar membrane changes with tissue K+ status.     

Lateral deviation of the eyes on forced lid closure in patients with cerebral lesions

We examined 35 patients with unilateral cerebral lesions to determine the incidence of lateral deviation of the eyes under forcefully closed lids and the reliability of this sign in predicting the side of the lesion. Only patients with radiologically confirmed unilateral lesions were studied. Over 70% of patients had contralateral ocular deviation (Cogan's "spasticity of conjugate gaze"), 20% had ipsilateral deviation, and less than 9% of the patients had no deviation. Lateral ocular deviation was as sensitive, but not as specific, as a unilateral Babinski plantar response in determining the side with the lesion. Contralateral deviation was more common with parietotemporal localization, suggesting that the phenomenon reflects an underlying disturbance of attentional mechanisms.     

The cardiovascular effects of bromocriptine in Parkinsonism

1 The cardiovascular effects of bromocriptine, a dopamine receptor agonist, were investigated in twenty-eight Parkinsonian patients.

2 Bromocriptine caused a significant impairment of postural compensation with a fall in systolic pressure and an absence of the rise in diastolic pressure after standing for 1 min when patients taking active drug were compared to the same patients on placebo. The hypotensive effect persisted for at least 6 weeks of treatment. There was also a significant reduction in supine heart rate.

3 One patient had marked falls in supine and erect blood pressure after a single oral dose of bromocriptine (2.5 mg) and a further patient developed paroxysmal atrial tachyarrhythmias. Both blood pressure and heart rate changes reversed spontaneously after stopping bromocriptine.

4 It is proposed that dopaminergic mechanisms either in the central nervous system or the periphery contribute to cardiovascular regulation in man.

     

Configurational coding, familiarity and the right hemisphere advantage for face recognition in sheep

This study examined characteristics of visual recognition of familiar and unfamiliar faces in sheep using a 2-way discrimination task. Of particular interest were effects of lateralisation and the differential use of internal (configurational) vs external features of the stimuli. Animals were trained in a Y-maze to identify target faces from pairs, both of which were familiar (same flock as the subjects) or both of which were unfamiliar (different flock). Having been trained to identify the rewarded face a series of stimuli were presented to the sheep, designed to test for the use of each visual hemifield in the discriminations and the use of internal and external facial cues. The first experiment showed that there was a left visual hemifield (LVF) advantage in the identification of 'hemifaces', and 'mirrored hemifaces' and 'chimeric' faces and that this effect was strongest with familiar faces. This represents the first evidence for visual field bias outside the primate literature. Results from the second experiment showed that, whilst both familiar and unfamiliar faces could be identified by the external features alone, only the familiar faces could be recognised by the internal features alone. Overall the results suggest separate recognition methods for socially familiar and unfamiliar faces, with the former being coded more by internal, configurational cues and showing a lateral bias to the left visual field.