Non-natively folded variants of superoxide dismutase 1 (SOD1) are thought to contribute to the pathogenesis of familial amyotrophic lateral sclerosis (ALS), however the relative toxicities of these variants are controversial. Here, we aimed to decipher the relationships between the different SOD1 variants (aggregated, soluble misfolded, soluble total) and the clinical presentation of ALS in the SOD1 G93A mouse. Using a multi-approach strategy, we found that the CNS regions least affected by disease had the most aggregated SOD1. We also found that the levels of aggregated SOD1 in the spinal cord were inversely correlated with the disease progression. Conversely, in the most affected regions, we observed that there was a high soluble misfolded/soluble total SOD1 ratio. Taken together, these findings suggest that soluble misfolded SOD1 may be the disease driver in ALS, whereas aggregated SOD1 may serve to sequester the toxic species acting in a neuroprotective fashion.
In 1993, the American Speech-Language-Hearing Association (ASHA) recommended caseloads of 40 for speech-language pathologists (SLPs) practicing in the public schools, a recommendation that was not feasible for most school districts. In addition, new laws and policies substantially increased the paperwork, responsibilities, and time expenditures required of the school-based SLP. ASHA subsequently instigated extensive reviews of the problem and, in 2002, suggested that schools instead use a “workload analysis approach,” considering all of the required activities from prereferral to dismissal, in order to determine the number of children an SLP could appropriately serve. In our study, we attempted to catalog the status of Texas' efforts in using workload considerations. More than 400 school-based respondents indicated the percentages of time they spent fulfilling each professional responsibility at their school. Responses suggest that variation in caseload numbers corresponds to clinician role (SLP vs. supervising SLP vs. SLP Assistant). Most clinicians indicated that they were fairly satisfied with their job; however, many were employed 5 or fewer years in the schools and the average workweek was 48–49 hours. Although it appears that Texas is using workload considerations to some extent, efforts should continue to optimize the quality of treatment for Texas schoolchildren and retention of SLPs in the schools.
When imitation skills are not present in young children, speech and language skills typically fail to emerge. There is little information on practices that foster the emergence of imitation skills in general and verbal imitation skills in particular. The present study attempted to add to our limited evidence base regarding accelerating the development of speech/language in young children who have failed to achieve expected language milestones. Imitation therapy (Zedler, 1972) was utilized for five non-verbal 18-19-month-old children to determine if imitative behavior in the form of sound production could be initiated and increased. Treatment was administered until children reached criteria for consistent imitative sound productions. Following 8-9 weeks of therapy, all five children exhibited significant increases in both the number of vocalizations and the variety of phonemes produced, and they demonstrated regular spontaneous verbal imitation and emergence of phonetically consistent forms. Imitation therapy appears to be a promising practice that merits further investigation.
Purpose The purpose of this review article is to review the progress made in the treatment and education of persons with disabilities in Zambia, to identify barriers that have hindered change and initiatives that have facilitated positive changes in Zambia, and to highlight the initial steps toward the establishment of the profession of speech-language pathology in Zambia. Conclusions There have been remarkable strides in the education of children with disabilities and the establishment of support for persons with disabilities since the Republic of Zambia became an independent nation in 1964. Zambia's limited resources, understanding of disabilities, and cultural and social barriers have hampered efforts to provide special services to children. Despite these challenges, many policies have been adopted and laws passed to protect the rights of those with disabilities, including access to quality equitable and inclusive education programs. Though implementation of the stated ideals has been difficult, and poverty continues in many areas, preparation of special education teachers and inclusion practices has improved. One vital service for children with disabilities, speech/language therapy, remains outside the new provisions. Speech therapy is not currently provided in the schools and is rarely available elsewhere. Though there are currently no Zambian college or university preparation programs for speech therapists, plans to bring speech pathology to Zambia are underway.
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