Prevalence figures for inherited neuromuscular disorders are important both for health care planning purposes and for evaluating the need for DNA diagnostic services for eugenic approaches. We screened for the prevalence of myotonic dystrophy (MyD) through extensive inquiry of neurologic and primary health services of Guipúzcoa (Basque Country, northern Spain) between 1989 and 1991. Typical adult-onset and neonatal cases and relatives at risk; suffering from a partial syndrome, were included. In the latter, molecular typing was performed with DNA probes close to the MyD gene to demonstrate the MyD gene carrier status. The high prevalence detected (26.5 cases per 100,000 population) could be explained by methodological factors, but intrinsic factors, such as a possible founder genetic effect or the quick growth of the Guipúzcoa population since the last century may contribute to one of the highest MyD prevalence in the world. In the future, the methodological basis for epidemiologic surveys of MyD must combine molecular technology with more-extensive family inquiries.

DNA samples from 231 unselected patients with cataracts were studied to determine the frequency of the DM mutation in cataract patients. A previous epidemiological study established a high prevalence of DM in the population of Guipuzcoa (Basque Country, Spain), 26-5 cases/100 000. We have found two carriers (0'9%) of the DM mutation in patients who are not related to any previously known DM family. The screening of the DM mutation in cataract patients should be restricted to young patients or people with multicoloured and iridescent opacities, in which the risk of carrying the DM premutation could be higher. Our results suggest that subjects with 38 to 80 repeats could constitute the genetic reservoir of the DM mutation.

Objective: Use of some restrictive procedures, including physical restraint, has been controversial. For children within the foster care system, who have already suffered various degrees of abuse and neglect, restrictive procedures could add to their emotional and behavioral problems. The current study was conducted to determine whether a behavioral staff training program would help reduce the use of restrictive procedures at two group facilities housing children in the foster care system. Method: Pre- and posttraining measures (incident reports) were obtained within a nonconcurrent multiple-baseline design to document the use of restrictive procedures. Results: The data revealed decreases in reports of several restrictive procedures (e.g., take downs, physical holds, and physical and pharmacological restraints) at both facilities. Conclusions: The results suggest that training direct care staff personnel, including social workers, in positive behavioral strategies might reduce staff use of restrictive procedures and result in fewer injuries to both children and staff.

No abstract

Objectives: An extensive literature base exists for behavioral parent training; however, few studies have focused on training direct care staff at group home and residential facilities for children. This study was conducted to determine whether a behavioral staff training program consisting of classroom training and in-home feedback would improve staff interactions with children at two group foster care homes. Method: Staff at both homes were trained, and direct observation measures were obtained on positive and negative interactions and lack of interactions. A multiple-baseline design across homes was used to assess staff interaction behavior. Results: Increases in both positive interactions and tool use were observed in the treatment phase, as were decreases in negative interactions for both group homes. Conclusions: The results suggest that training direct care staff, including social workers, to use behavioral strategies change patterns of interaction, resulting in a greater likelihood of improved relationships between staff and children.