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Biomedical subjects

S Benton

Publications and source records attributed to S Benton.

11 recordsLinked to original sources
PubMed2006

Assistive technology--behaviourally assisted.

In considering the recurrent problems involved in technology led initiatives within the public sector, this paper seeks to identify change management requirements needed to help avoid these latent pitfalls in the widespread introduction of Assistive Technology. It develops a change process approach based on current clinical psychology techniques used in assessing sources and level of resistance to behavioural change and applies them to managing effective benefits realisation.

Activities of Daily Living↗
PubMed2002

Benign paroxysmal torticollis of infancy: four new cases and linkage to CACNA1A mutation.

Benign paroxysmal torticollis of infancy (BPTI) is a disorder characterized by recurrent episodes of head tilt secondary to cervical dystonia. Attacks are often accompanied by vomiting, pallor, and ataxia, settling spontaneously within hours or days. Episodes begin within the first 12 months of life and resolve by 5 years. We report four patients with BPTI. Symptoms started from 3 months of age, with head tilting lasting between 10 minutes and 2 months; the shorter episodes were followed by vomiting, apathy, and unsteadiness. Head tilt became less prominent after infancy, replaced by vertigo and eventually by migraine headaches. Two patients came from a kindred with familial hemiplegic migraine linked to CACNA1A mutation. BPTI may be regarded as a migraine aura equivalent. The syndrome poses interesting questions regarding varying phenotypic expression of calcium channelopathies at different stages of development.

Adolescent↗
PubMed2000

An unknown combination of infantile spasms, retinal lesions, facial dysmorphism and limb abnormalities.

A female patient is presented with infantile spasms, punched-out retinal lesions, facial dysmorphism, short upper arms, short thumbs, left lower limb hypoplasia with foot deformity, a hemivertebra, atrial septal defect, growth retardation and severe developmental delay. There is some similarity to patients with Aicardi syndrome (AS), but the retinal lesions in our patient are different and she does not have agenesis of the corpus callosum, one of the diagnostic features of AS. She might represent an atypical form of this syndrome with additional features, usually not present in AS. As there is no diagnostic test for AS yet, this diagnosis cannot be confirmed nor rejected with certainty. However, it might be more likely that our patient has another, possibly unique, condition.

Abnormalities, Multiple↗
PubMed1998

Lucifer Yellow filling of area X-projecting neurons in the high vocal center of female canaries.

The avian high vocal center (HVC) is a complex forebrain nucleus that coordinates the sensorimotor integration necessary for song learning and production. It receives auditory and potentially somatosensory input, and sends major projections to vocal motor and anterior forebrain nuclei. The HVC has at least four morphological classes of neurons for which the connectivity remains uncertain. Previous studies have alluded to the functional identity of the cell classes, but none have provided the definitive evidence necessary for subsequent identification of behaviorally relevant changes within known neuronal populations. The cell filling technique we have adapted for use in the song system provides a method by which hodologically identified classes can be described with precision, and song related changes in their morphology can be readily identified. Neurons in female canaries (Serinus canarius) that project to Area X of the anterior forebrain pathway were retrogradely labeled, selectively filled with Lucifer Yellow in a fixed slice preparation, and converted to a Golgi-like stain through an immunocytochemical reaction. We have identified Area X-projecting neurons as belonging to the thick dendrite class of Nixdorf et al. [B.E. Nixdorf, S.S. Davis, T.J. DeVoogd, Morphology of golgi-impregnated neurons in hyperstriatum ventralis, pars caudalis in adult male and female canaries, J. Comp. Neurol. 284 (1989) 337-349] and have shown definitively that they are among the HVC neurons that can receive direct auditory input, as this cell class has short dendrites that extend into the shelf region ventral to HVC that is known to receive auditory inputs. Well-filled axons had collaterals that ramified and terminated within the nucleus, demonstrating a network through which Area X-projecting cells can contribute to intrinsic HVC communication.

Animals↗
PubMed1998

Anterior forebrain pathway is needed for stable song expression in adult male white-crowned sparrows (Zonotrichia leucophrys).

The anterior forebrain pathway of the avian song system is involved in juvenile song learning, but its function in adult song behavior is not known. This report uses lesions to study the role of a particular forebrain nucleus, IMAN, in the seasonal regeneration of song in adult white-crowned sparrows (Zonotrichia leucophrys oriantha). White-crowned sparrows, even when acoustically isolated as juveniles, crystallize a single song which they maintain throughout adulthood. The lateral portion of the magnocellular nucleus of the anterior neostriatum (1MAN) was lesioned bilaterally in adult males maintained on short days (8 h of light). Daylength was increased to 16 h following the surgeries, and all birds were recorded in the post-lesion singing season. Lesioned birds showed a large decrease in song note frequency following the lesions, significantly larger than did intact, age-matched controls. Further changes were seen in the post-lesion songs of seven of 11 successfully lesioned males. These changes included variability in song pattern, loss of frequency control and addition of new notes, some of which had been practiced during juvenile song development. These changes seemed especially large in birds that had either been acoustically isolated or had not fully copied a tape-tutor song during juvenile song development. These results are the first to indicate that the motor memories for song elements that had been practiced and discarded early in life are retained, and they suggest that 1MAN affects seasonal song expression by selectively reinforcing a particular song pattern.

Animals↗
PubMed1989

Motion-deblurring in human vision.

If photographs are taken of moving objects at slow shutter speeds the images of the objects are blurred. In human vision, however, we are not normally conscious of blur from moving objects despite the fact that the temporal response of the photoreceptors is sluggish. It has been suggested that there are motion-deblurring mechanisms specifically to aid the visual system in the analysis of the shape of retinally moving targets. Models of motion deblurring have been influenced by the finding that certain very precise spatial pattern discriminations are unaffected by motion. An example is vernier hyperacuity, in which the observer must detect the direction of offset between two lines with abutting ends. With a stationary stimulus, observers can detect a vernier cue of less than 10 arcsec and acuity is unaffected by retinal-image motion of up to 3 deg s-1 We confirm this finding, but provide evidence against any general deblurring mechanism by showing that another kind of hyperacuity, discrimination of the distance between two parallel lines (spatial interval acuity), is interfered with by motion. This argues against a general deblurring mechanism, such as a neural network 'shifter circuit', and we point out that the high level of vernier acuity for moving stimuli is susceptible to an alternative explanation.

Humans↗
PubMed1989

Joubert syndrome.

Joubert syndrome is an autosomal recessive condition in which there is a variable combination of central nervous system defects with a distinctive congenital retinal dystrophy, ocular motor abnormalities, and respiratory abnormalities in early infancy. The retinal dystrophy has been previously classified as a variant of Leber's congenital amaurosis. We report electrophysiologic and eye movement findings in a series of seven consecutive children with Joubert syndrome. Unlike patients with Leber's congenital amaurosis, all but one of these children had preserved flash and pattern-reversal visual evoked potentials. Six of the seven children had abnormalities of smooth pursuit, optokinetic nystagmus, and saccades. Six of the children had nystagmus: three had a pendular torsional nystagmus and three had a form of see-saw nystagmus. An alternating hyperdeviation was present in five of the patients, two of whom also had a tonic deviation of their eyes laterally. All seven patients had cerebellar vermis hypoplasia on a magnetic resonance imaging scan. Developmentally delayed children with an absent or highly attenuated electroretinogram should be investigated for Joubert syndrome.

Blindness↗
PubMed1980

Vision in the temporal crescent in occipital infarction.

Four patients with hemianopias due to occipital infarction are described. In each of these patients there was useful residual vision in the unpaired temporal crescent. Moving stimuli were particularly well perceived in this part of the field. Preservation of the temporal crescent results in strikingly incongruous fields. The significance of this part of the visual field is discussed in relation to perception of movement and to the fixation reflex, and also with regard to recent reports of residual visual ability in patients with striate cortex lesions, and to the possible role of the monocular temporal field in striate and tectal visual systems.

Cerebral Infarction↗
PubMed1980

Effect of lecithin on disability and plasma free-choline levels in Friedreich's ataxia.

Four patients with Friedreich's ataxia took part in an open trial, in which they consumed 50-100g/day lecithin granules (containing approximately 22% phosphatidycholine) for 16 weeks, but no improvement resulted. Several unwanted effects including diarrhoea, nausea, depression, "hot flushes" and weakness were experienced. Resting levels of free-choline in plasma were within the range found in 19 normal subjects. Sixteen other patients with Friedreich's ataxia also had normal free-choline levels. Treatment with lecithin significantly increased plasma free-choline levels, but there was a trend for these to fall towards baseline levels, despite continued ingestion of lecithin.

Adolescent↗