Human Schwann cells exhibit long-term cell survival, are not tumorigenic and promote repair when transplanted into the contused spinal cord.

The transplantation of rodent Schwann cells (SCs) provides anatomical and functional restitution in a variety of spinal cord injury (SCI) models, supporting the recent translation of SCs to phase 1 clinical trials for human SCI. Whereas human (Hu)SCs have been examined experimentally in a complete SCI transection paradigm, to date the reported behavior of SCs when transplanted after a clinically relevant contusive SCI has been restricted to the use of rodent SCs. Here, in a xenotransplant, contusive SCI paradigm, the survival, biodistribution, proliferation and tumorgenicity as well as host responses to HuSCs, cultured according to a protocol analogous to that developed for clinical application, were investigated. HuSCs persisted within the contused nude rat spinal cord through 6 months after transplantation (longest time examined), exhibited low cell proliferation, displayed no evidence of tumorigenicity and showed a restricted biodistribution to the lesion. Neuropathological examination of the CNS revealed no adverse effects of HuSCs. Animals exhibiting higher numbers of surviving HuSCs within the lesion showed greater volumes of preserved white matter and host rat SC and astrocyte ingress as well as axon ingrowth and myelination. These results demonstrate the safety of HuSCs when employed in a clinically relevant experimental SCI paradigm. Further, signs of a potentially positive influence of HuSC transplants on host tissue pathology were observed. These findings show that HuSCs exhibit a favorable toxicity profile for up to 6 months after transplantation into the contused rat spinal cord, an important outcome for FDA consideration of their use in human clinical trials.

SUDDEN VISION LOSS FROM CENTRAL RETINAL ARTERY OCCLUSION AS A PRESENTING SYMPTOM OF MITRAL VALVE PAPILLARY FIBROELASTOMA.

PURPOSE: We describe a central retinal artery occlusion with cilioretinal sparing in a young male patient who was found to have mitral valve papillary fibroelastoma. METHODS: At the initial examination, a 33-year-old Hispanic man had visual acuity of 20/200 in his left eye, and 2 weeks later, visual acuity improved to 20/20. Diagnosis required transesophageal echocardiography to localize the lesion. RESULTS: Mitral valve papillary fibroelastoma involving the mitral valve was successfully treated with tumor resection. CONCLUSION: Routine echocardiography should be performed in all patients presenting with central retinal artery occlusion as it may diagnose treatable cardiogenic etiologies and present further potentially life-threatening embolic events.

Collaborating with Culturally Competent Prenatal Education among Hispanic Communities.

INTRODUCTION: Hispanics represent the largest minority group in the United States. In Kansas, the population of Hispanics has been increasing; unfortunately, their infant mortality rate has increased as well. Baby Talk is a prenatal education program promoting maternal and infant health through risk-reduction strategies and healthy decision-making. The aim of this pilot project was to develop and evaluate a Spanish curriculum for Baby Talk. METHODS: A collaborative partnership between bilingual community members and health professionals from different origins, nationalities, and Spanish dialects was formed to create a culturally and linguistically appropriate Spanish Baby Talk curriculum. This interventional pilot mixed methods research study employed quantitative and qualitative methods to evaluate participant knowledge, intentions, satisfaction, and perceptions of the new curriculum. RESULTS: Fifteen pregnant women participated in Spanish Baby Talk. Of those, 12 participated in either phone interviews (n = 6) or a focus group (n = 6). All respondents described their experience with the Spanish Baby Talk program as "excellent". Significant increases in knowledge were seen related to topics such as benefits of full-term pregnancy and benefits of breastfeeding. Four themes were identified from the focus group and interviews: 1) lack of accessible community resources; 2) sense of community; 3) Spanish Baby Talk strengths; and 4) areas for improvements. CONCLUSIONS: Findings suggested that the Spanish Baby Talk curriculum was linguistically appropriate and resulted in increases in knowledge and intentions related to health and safety behaviors. Areas for improvement were related to marketing the program and referring to resources that provide material supports (i.e., diapers) to continue the move towards a culturally competent program.

Infant Safe Sleep Promotion: Increasing Capacity of Child Protective Services Employees.

Sleep-related infant deaths, including Sudden Infant Death Syndrome (SIDS), are the number one cause of death in infants between 28 days and one year of life. Nearly half of families experiencing a sleep-related infant death in Kansas were involved with the Department of Children and Families Child Protective Services (CPS), making CPS staff a priority for safe sleep training. This study assessed the impact of the two-day Kansas Infant Death and SIDS (KIDS) Network Safe Sleep Instructor (SSI) train-the-trainer program on CPS staffs' knowledge of the American Academy of Pediatrics safe sleep recommendations. Training was attended by 43 participants, 27 (63%) of whom were employed by CPS. CPS staff had significantly lower baseline knowledge on the 10-item pretest (t = 3.33, p = 0.002), but both CPS and other attendees showed significant improvement by posttest (t = 8.53, p < 0.001 and t = 4.44, p < 0.001, respectively). Following SSI certification, CPS SSIs provided more safe sleep training to professionals than other SSIs (1051 vs. 165, respectively), and both groups of SSIs were able to significantly increase the knowledge of their trainees. Overall, the KIDS Network SSI training was successful. The innovative partnership with CPS allowed for provision of training to a group not historically targeted for safe sleep education.

RETINAL MANIFESTATIONS OF SPINOCEREBELLAR ATAXIA TYPE 7 IN TWO CONSECUTIVE GENERATIONS.

PURPOSE: To report a diagnosis of spinocerebellar ataxia Type 7 (SCA-7) first diagnosed in the daughter followed by the father, with proven genetic testing and display of progressive anticipation of disease penetrance. METHODS: A 5-year-old African American female admitted for failure to thrive underwent full ocular examination and fundus photography, with genetic confirmation of SCA-7. The father carried a previous diagnosis of possible solar retinopathy; however, with further genetic testing, he was also found to have SCA-7. RESULTS: The patient was admitted for failure to thrive with suspicion of ataxia neurodegenerative disorder. Visual acuity was hand motion. Fundus examination showed retinal pigment epithelium pigmentary changes in the macula and peripheral retina. Further genetic workup revealed 96 CAG repeat expansion compared with a normal of <20 repeats. Ocular examination of patient's father displayed a milder form of retinopathy with genetic testing showing 47 CAG repeat expansion. Diagnosis of SCA-7 was made displaying genetic anticipation. CONCLUSION: Spinocerebellar ataxia Type 7 is a disease of expanded CAG repeats showing genetic anticipation. Patients display progressive cerebellar ataxia, dysarthria dysphagia, slow saccadic eye movements, and cone photoreceptor loss leading to progressive vision loss. CAG repeat length tends to expand with transmission resulting in dramatic symptoms in offspring sometimes resulting in diagnosis before parents' diagnosis. Awareness of this condition may help in earlier diagnosis and unnecessary testing resulting in more effective counseling for the patient and their family.

Permissive Schwann cell graft/spinal cord interfaces for axon regeneration.

The transplantation of autologous Schwann cells (SCs) to repair the injured spinal cord is currently being evaluated in a clinical trial. In support, this study determined properties of spinal cord/SC bridge interfaces that enabled regenerated brainstem axons to cross them, possibly leading to improvement in rat hindlimb movement. Fluid bridges of SCs and Matrigel were placed in complete spinal cord transections. Compared to pregelled bridges of SCs and Matrigel, they improved regeneration of brainstem axons across the rostral interface. The regenerating brainstem axons formed synaptophysin(+) bouton-like terminals and contacted MAP2A(+) dendrites at the caudal interface. Brainstem axon regeneration was directly associated with glial fibrillary acidic protein (GFAP(+)) astrocyte processes that elongated into the SC bridge. Electron microscopy revealed that axons, SCs, and astrocytes were enclosed together within tunnels bounded by a continuous basal lamina. Neuroglycan (NG2) expression was associated with these tunnels. One week after injury, the GFAP(+) processes coexpressed nestin and brain lipid-binding protein, and the tips of GFAP(+)/NG2(+) processes extended into the bridges together with the regenerating brainstem axons. Both brainstem axon regeneration and number of GFAP(+) processes in the bridges correlated with improvement in hindlimb locomotion. Following SCI, astrocytes may enter a reactive state that prohibits axon regeneration. Elongation of astrocyte processes into SC bridges, however, and formation of NG2(+) tunnels enable brainstem axon regeneration and improvement in function. It is important for spinal cord repair to define conditions that favor elongation of astrocytes into lesions/transplants.