A case of unilateral neglect in Huntington's disease

Unilateral neglect, an attentional deficit in detecting and acting on information coming from one side of space, is a relatively common consequence of right hemisphere stroke. Although neglect has been observed following damage to a variety of brain structures, to date no reports exist of neglect phenomena arising from Huntington's Disease (HD). However, reports in the animal and human literature suggest that neglect is possible following damage to a primary site for Huntington's pathology, the basal ganglia. Here we present a patient (BG) with genetically proven HD who, in the context of the mild intellectual, executive and attentional impairments associated with the disorder, showed a remarkably severe and stable neglect for left space. MRI and electrophysiological results make it unlikely that the spatial bias could be accounted for by basic sensory loss. In addition, behavioural investigation indicated that, although BG's neglect operated in a very striking manner along body-centred co-ordinates (missing almost all information presented to her left), more general limitations in visual attention were apparent to the left-side of information presented entirely to the right of the body midline. Further evidence is presented showing that the neglect was manifest on tactile and auditory tasks as well as those presented in the visual domain. The presence of neglect in HD in this case is novel and somewhat puzzling, particularly as flourodeoyglucose positron emission tomography revealed bilateral caudate hypoperfusion. Reducing the statistical threshold on this analysis revealed a potential frontal hypometabolism that was more marked in the right than left hemisphere. However, as this was only apparent at a threshold below that normally considered acceptable (due to the resulting number of false positives), this possible account of the neglect must be viewed very cautiously.     

Management of talon cusp affecting the primary central incisor: a case report

Talon cusps are relatively rare dental anomalies that manifest as accessory cusplike structures and project from the cingulum area of the anterior teeth. The condition can occur in both the primary and permanent dentitions. However, the occurrences of anomalous cusps are rather infrequent in the primary dentition. Little has been written about the treatment of talon cusps in the primary dentition compared with their counterparts in the permanent dentition. The purpose of this article was to document the management of a patient with a maxillary primary incisor affected by a talon cusp and the long-term follow up.     

Characterizing self-rated health during a period of changing health status

Self-rated health (SRH) is among the most frequently assessed health perceptions. The purpose of this study was to assess the tenability of the recently proposed distinctions of SRH, as a spontaneous assessment of overall health, or as an enduring self-concept. Individuals (n = 449) undergoing total joint replacement for hip or knee osteoarthritis in Toronto, Canada were followed over 6 months of recovery. Health questionnaires, completed pre-surgery, and at 3 and 6 months post-surgery, included measures of pain, physical function, sports/recreation, fatigue, anxiety, depression, social participation, passive/active recreation, and community access. Structural equation modeling was used for the analyses. SRH was found to be responsive to current and changing mental well-being throughout the six months of recovery. Current SRH strongly predicted future SRH. In this clinical sample undergoing significant changes in health status, SRH displayed both enduring and spontaneous features; evidence is provided that both operate simultaneously. SRH may prove to be a simple yet critical health measure for identifying individuals who would benefit most from targeted interventions for improving overall health.     

Potassium iodide in refractory,recurrent pediatric Sweet syndrome: Guidance in dosing and monitoring

We describe a 5‐month‐old boy with clinical and histopathologic presentation of Sweet syndrome. He responded to systemic corticosteroids, with multiple flares on tapering; potassium iodide was added, which provided complete resolution of Sweet syndrome. Potassium iodide has been used in only a few cases, and no standard dosage has been established in children. We discuss calculation of a pediatric dosage for potassium iodide in Sweet syndrome.     

Functional interactions in brain networks underlying epileptic seizures in bilateral diffuse periventricular heterotopia.

OBJECTIVE: Our aim was to investigate relationships between heterotopic and remote cortical structures at seizure initiation, in a patient with bilateral periventricular nodular heterotopias (BPNH) explored by intracerebral electrodes. METHODS: Stereoelectroencephalography (SEEG) was performed in a man with BPNH and refractory epilepsy to investigate the hypothesis of right temporal lobe epilepsy and the possible involvement of heterotopic structures during seizures. SEEG signals were analyzed with quantification of functional coupling between different brain structures during seizures, using nonlinear regression. We have used Z-score transformation of correlation values to reflect the change from the preictal period. Relationships between BPNH and cortical structures were investigated using analysis of stimulation-induced potentials. RESULTS: Three spontaneous seizures were recorded and analyzed. Signal analysis of interdependencies in two seizures demonstrated a large initial network involving both heterotopia and cortical structures. Stimulations of heterotopia induced responses in remote cortical structures. CONCLUSIONS: Distinct epileptogenic networks were identified, in which leader structures were either the heterotopic or the mesial temporal structures, with functional connections between heterotopic and cortical areas. SIGNIFICANCE: These results confirm that a vast epileptogenic network, including heterotopic and cortical neurons, may be responsible for seizure generation in BPNH. This may explain certain surgical failures in this group.     

Effectiveness of a Tai chi Qigong program in promoting health-related quality of life and perceived social support in chronic obstructive pulmonary disease clients

Purpose  

This paper evaluates the effectiveness of a 3-month Tai chi Qigong (TCQ) program in promoting the psychosocial functional health of clients with chronic obstructive pulmonary disease (COPD) in Hong Kong.     

Cost-effectiveness of drotrecogin alfa (activated) for the treatment of severe sepsis in Germany

Drotrecogin alfa (activated) (Xigris; Eli Lilly and Company, Indianapolis, IN) significantly reduced mortality in severe sepsis in the PROWESS trial. We evaluate the cost-effectiveness of drotrecogin alfa (activated) as an adjunct to standard therapy from the German healthcare payer’s perspective with respect to patients with 1) severe sepsis and 2) severe sepsis and multiple organ failure the approved European indication. Hospital resource use based on PROWESS was valued using German unit costs. German life-tables and long-term survival assumptions determined life-years gained. European and German healthcare resource use data are examined in the sensitivity analysis. We assumed a unit price of €237.50 for drotrecogin alfa (activated). Per patient treated, drotrecogin alfa (activated) increased costs by €7,500, and hospital costs by €900 for all patients (€7,400 and €1,500 respectively for the approved indication) and survival by 0.59 life years (0.87 life years respectively for the approved indication). Thus drotrecogin alfa (activated) cost €14,100 (€17,700 discounting life years at 3%) per life year gained for all patients (€10,200 and €12,900, respectively, for the approved indication). Testing the unit cost of drotrecogin alfa (activated), pattern of resource use, and survival benefit, demonstrated that cost-effectiveness lies well within the range of other life saving interventions in Germany representing good economic value.