Atypical onset of symptoms in Huntington disease: severe cognitive decline preceding chorea or other motor manifestations.

OBJECTIVE: To describe the onset of Huntington disease (HD) in a patient with atypical progression of symptoms. BACKGROUND: The authors report the case of a 39-year-old man with severe cognitive impairment and diffuse cortical atrophy before the onset of motor manifestations or symptoms of an extrapyramidal movement disorder. METHOD: Clinical examinations, neuropsychologic assessments, magnetic resonance imaging, electroencephalogram, and genetic testing were conducted. RESULTS AND CONCLUSIONS: Although HD was eventually confirmed through genetic testing, chorea was not part of the clinical picture until well after the patient had developed a frank dementia, with a decline in global intellectual functioning, memory deficits, slowed information processing speed, and executive dysfunction. This case indicates HD may present with atypical clinical features in the early course of the disease, and warrants diagnostic consideration in patients with early dementia of unknown etiology.     

Diet and premenopausal bilateral breast cancer: A case-control study

We investigated associations between diet and premenopausal bilateral breast cancer in a familial matched case-control study. We studied 140 cases from population-based registries in Los Angeles County (California) and Connecticut, and from the major hospitals in the southern parts of the Province of Quebec. Unaffected sisters of the cases served as matched controls (222 total). Dietary intake were assessed with a food frequency questionnaire. Total fat, monounsaturated fat, polyunsaturated fat, oleic acid, and linoleic acid intake was inversely associated with premenopausal bilateral breast cancer risk. Consumption of carbohydrates (and sweetened beverages) was associated with an increased risk. We observed no associations for dietary fiber, antioxidants, or major food groupings, but we did observe inverse associations for intake of low fat dairy products and tofu. These findings suggest that monounsaturated and polyunsaturated fats, as well as soy foods, might reduce the risk of premenopausal bilateral breast cancer.     

Characterisation of black-pigmented anaerobes isolated from diseased and healthy periodontal sites

Prevotella intermedia has recently been re-defined and a new species, Prevotella nigrescens has been proposed. However, there is little data available on the incidence of these new species in periodontal health or disease. Black-pigmented anaerobes isolated from diseased and healthy subgingival sites were identified by serotyping, SDS-PAGE and physiological tests. In adult periodontitis subjects, 64% of active sites, 35.7% of inactive sites and 38.5% of healthy sites yielded black-pigmented anaerobes. Of these, Porphyromonas gingivalis was found in 11% of active and 5% of healthy sites in diseased patients, Prevotella intermedia in 15.5% of active and 20.5% of healthy sites, Prevotella nigrescens in 37.7% of active and 11.5% of healthy sites and Prevotella denticola in 3% of active and 1% of healthy sites. In healthy subjects, 50% of sites yielded black-pigmented anaerobes. P. gingivalis was not found in healthy subjects but P. intermedia was found in 18% and P. nigrescens in 31% of sites. SDS-PAGE proved to be a useful method for routinely differentiating P. intermedia and P. nigrescens and two sub-types of the latter species were detected on the basis of band pattern. Only one P. nigrescens sub-type was found in any given individual and one type, typified by ATCC 25261, was more commonly found in deep pockets. However, overall both P. nigrescens and P. intermedia as species were just as frequently found at healthy sites as diseased sites. Thus, these species, in contrast to P. gingivalis , appear to be common commensals but they may act as opportunistic pathogens.     

A prototype retractor system designed to minimize ischemic brain retractor injury: initial observations

The authors have developed and patented a neurosurgical retractor system incorporating an infrared emitter and detector that allows detection of cerebral pulsations. Gentle contact with the surface of cat brains shows cerebral pulsations that correlate with arterial pulse as well as mechanical ventilation. The amplitude of cerebral pulsations decreases with higher retraction pressure and disappears at approximately 20 mmHg. The pressure on the surface of the brain decreased 50% in 5 minutes even though the position of the retractor was maintained constant. The authors postulate that monitoring cerebral pulsation may prove useful in clinical neurosurgery with respect to avoiding excessive retraction, which causes brain damage.     

Non-ulcerative fungal keratitis diagnosed by posterior lamellar biopsy*

We report a case of Fusarium solani keratitis which highlights the difficulties often associated with management of fungal corneal infections. This case demonstrates several unusual and interesting features: the occurrence of deep fungal pathology after superficial injury, the difficulties encountered in attempting to isolate and identify the causative organism, and the protracted course taken by an organism often noted to be rapidly destructive. These features necessitated an individual approach to therapy, employing unconventional medical and surgical techniques to achieve a satisfactory outcome     

An optimal three-stage design for phase II clinical trials

A phase II clinical trial in cancer therapeutics is usually a single-arm study to determine whether an experimental treatment (E) holds sufficient promise to warrant further testing. When the criterion of treatment efficacy is a binary endpoint (response/no response) with probability of response p, we propose a three-stage optimal design for testing H₀: p ≤ p₀ versus H₁: p ≥ p₁, where p₁ and p₀ are response rates such that E does or does not merit further testing at given levels of statistical significance (α) and power (1 ? β). The proposed design is essentially a combination of earlier proposals by Gehan and Simon. The design stops with rejection of H₁ at stage 1 when there is an initial moderately long run of consecutive treatment failures; otherwise there is continuation to stage 2 and (possibly) stage 3 which have decision rules analogous to those in stages 1 and 2 of Simon's design. Thus, rejection of H₁ is possible at any stage, but acceptance only at the final stage. The design is optimal in the sense that expected sample size is minimized when p = p₀, subject to the practical constraint that the minimum stage 1 sample size is at least 5. The proposed design has greatest utility when the true response rate of E is small, it is desirable to stop early if there is a moderately long run of early treatment failures, and it is practical to implement a three-stage design. Compared to Simon's optimal two-stage design, the optimal three-stage design has the following features: stage 1 is the same size or smaller and has the possibility of stopping earlier when 0 successes are observed; the expected sample size under the null hypothesis is smaller; stages 1 and 2 generally have more patients than stage 1 of the two-stage design, but a higher probability of early termination under H₀; and the total sample size and criteria for rejection of H₁ at stage 3 are similar to the corresponding values at the end of stage 2 in the two-stage optimal design.     

Familias Unidas: The Efficacy of an Intervention to Promote Parental Investment in Hispanic Immigrant Families

This paper reports a test of the efficacy of Familias Unidas, a Hispanic-specific, ecologically focused, parent-centered preventive intervention, in promoting protection against and reducing risk for adolescent behavior problems. Specifically, the intervention was designed to foster parental investment, reduce adolescent behavior problems, and promote adolescent school bonding/academic achievement, all protective factors against drug abuse and delinquency. One-hundred sixty seven Hispanic families of 6th and 7th grade students from three South Florida public schools were stratified by grade within school and randomly assigned to intervention and no-intervention control conditions. Results indicated that Familias Unidas was efficacious in increasing parental investment and decreasing adolescent behavior problems, but that it did not significantly impact adolescent school bonding/academic achievement. Summer-vacation rates of adolescent behavior problems were six times higher in the control condition than in the intervention condition. Furthermore, change in parental investment during the intervention was predictive of subsequent levels of adolescent behavior problems. The findings suggest that Familias Unidas is efficacious in promoting protection and reducing risk for adolescent problem behaviors in poor immigrant Hispanic families.