Delayed diagnosis of dopa-responsive dystonia in two siblings

Background

Dopa responsive dystonia is characterized by progressive disabling dystonia, diurnal variation and a dramatic response to Levodopa.

Case characteristics

Two siblings presented with regression of motor milestones and hypertonia in lower limbs. History of diurnal variation was present in elder sibling.

Outcome

Both responded dramatically to Levodopa. The genomic DNA analysis of elder sibling revealed a novel mutation.

Message

A trial of Levodopa should be considered in a child with motor regression with diurnal variation, in the presence of extrapyramidal features.

     

Effective use of simple computer programmes and clay models to demonstrate the planning and operative steps for teaching and presentations

Background:Presenting and demonstrating a surgical procedure in the current era is difficult without good intraoperative pictures and videos. A long, complex, multi-staged surgery is better illustrated by detailed intraoperative images at various stages. Although desirable, it may be difficult due to various reasons.Results:It is a simple technique with a moderate learning curve. Once familiar with technique, one can effectively use the technique to convey the details in much more clear manner.Conclusion:It is a simple and effective way of communicating through digital images, and gives the audience a 3 dimensional idea about the concept.KEY WORDS: Clay models in plasticine surgery, photography, presentations in plastic surgery, teaching and patient education, use of clay models for presentations     

Administration of an antagonist of neurokinin receptors 1, 2, and 3 results in reproductive tract changes in beagle dogs, but not rats

SCH 206272, an antagonist of neurokinin receptors 1, 2, and 3, was administered orally by gavage for 1 month to 8- to 10-month-old dogs at doses of 0, 15, 30, or 60 mg/kg, and to 6-week-old rats at doses of 0, 30, 100, or 300 mg/kg. The most important changes occurred in the reproductive tract of the dogs at all doses. Absolute and relative group mean organ weights for the testes, prostate gland, epididymides, ovaries, and uterus were 33-86% lower than concurrent controls in groups receiving SCH 206272. Organ weight changes were not dose-related. Microscopic changes that correlated with the organ weight changes occurred in all groups receiving SCH 206272. For males, they included minimal to severe atrophy of the testes, epididymides, and prostate gland. In addition, the epididymides exhibited severe oligospermia or aspermia, minimal epithelial apoptosis and mild epithelial vacuolation. In female dogs, the ovaries and uteri appeared immature. Microscopic changes were similar in incidence and severity in dogs receiving 30 or 60 mg/kg, but were slightly less in dogs receiving 15 mg/kg. In contrast, similar findings were not observed in the reproductive tract of male or female rats, despite overlapping systemic, hypothalamic, and pituitary gland concentrations of SCH 206272.     

A class of Covariate-Adjusted Response-Adaptive Allocation Designs for Multitreatment Binary Response Trials

A class of covariate-adjusted response-adaptive randomization procedures is developed for binary treatment outcomes in a phase III clinical trial set up involving multiple treatments. The target allocation is developed by combining the ethical aspects with statistical precision under the existence of treatment covariate interaction. Relevant measures of the performance for the proposed allocation designs are studied and compared.