A unique subpopulation of Tbr1-expressing deep layer neurons in the developing cerebral cortex

Cells of the subplate (SP) and deep cortical plate (CP) are among the pioneer neurons of the developing cerebral cortex, an important group of early-born cells that impact cortical organization and function. Similarities between pioneer neurons in different cortical positions and heterogeneities in pioneer cells in the same cortical location, however, have made it difficult to appreciate the characteristics and functions of particular sets of these cells. Here, we provide a tool to illuminate a unique subset of SP and deep CP neurons: expression of a Tbrain-1 (Tbr1)-driven transgene. Transgene-expressing cells were consistently positive for neuronal but not glial markers, were born early in corticogenesis, representing just a subset of SP and deep CP neurons, were morphologically complex during the formation of the cortex, and were maintained into maturity. This analysis reveals a novel group of pioneer neurons and demonstrates unrecognized diversity within this cortical population. In the future, this information will help to uncover the roles of discrete pioneer populations in cortical development.     

Homozygosity for a Thr575Met missense mutation in the catalytic domain associated with factor XI deficiency

In this study we investigated an asymptomatic 55-year-old Lebanese woman with factor XI deficiency. The F11 gene was analyzed and a cross reacting material positive (CRM+) variant, Thr575Met, was identified in homozygosity in the proband, and in heterozygosity in four of her siblings.     

Botulinum toxin injections do not improve freezing of gait in Parkinson disease

Freezing of gait (FOG) is common in patients with Parkinson disease (PD) and responds poorly to medical treatment. Botulinum toxin A (BTX-A) injections into calf muscles decreased FOG in previous open-label studies. The authors conducted a randomized double-blind placebo-controlled crossover study of BTX-A vs placebo in 12 subjects with PD and FOG. No significant improvement with BTX-A was found using subjective and objective measures.     

Cranial suture simulator for ultrasound diagnosis of craniosynostosis

Background: In evaluating the effectiveness of ultrasound as a screening tool for craniosynostosis it was discovered that sonologists and sonographers needed more experience scanning and visualizing cranial sutures on ultrasound. Objective: To create an ultrasound simulator to train radiologists and technologists to locate and recognize patent and fused cranial sutures in children. Materials and methods: The hypoechoic appearance of patent sutures was simulated by cutting lines into life-sized plastic doll heads and filling them with a commercial hypoechogenic material. Fused hyperechoic sutures were simulated by not cutting into the hard plastic region of a suture. The simulators teaching value was evaluated on three radiology residents and three fellows. Subjects performed pre-training scans on unknown simulators, received feedback and an opportunity to scan a training simulator, and then performed post-training scans on random unknown simulators. Accuracy was recorded as percentage of correctly demonstrated sutures. Results: The suture simulator reproduces the sonographic appearance of patent and fused cranial sutures. Accuracy of acquisition, interpretation, and overall diagnosis increased from 64 to 91%, 79 to 91%, 61 to 97%, respectively, between pre and post training scans. Conclusion: An ultrasound simulator can reproduce the appearance of patent and fused cranial sutures in children and can be used to train radiologists and technologists in the performance of a screening protocol.     

Project ENABLE: a palliative care demonstration project for advanced cancer patients in three settings

At the end of the 1990s, based on data from two major studies of end-of-life (EOL) care, the Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatment (SUPPORT), and the Hospitalized Elderly Longitudinal Project (HELP), a consensus panel report documented the problems and needs of patients with cancer and other life-limiting diagnoses at end-of-life. A national program of The Robert Wood Johnson Foundation (RWJF), Promoting Excellence in End-of-Life Care, attempted to address these needs by funding demonstration projects to test various approaches to improve identified deficits. In 1998, Project ENABLE (Educate, Nurture, Advise Before Life Ends), one of four RWJF-funded cancer center/hospice collaborations of the Promoting Excellence program, began to address these issues. The jointly sponsored Norris Cotton Cancer Center (NCCC)/Hospice of Vermont and New Hampshire (Hospice VNH) program provided an integrated approach to the management of life-limiting cancer. Project ENABLE was aimed at alleviating the symptoms of disease and treatment, enhancing clinician and patient/family communication, offering support for families, friends and other caregivers, addressing emotional and spiritual needs of dying people and providing conceptual and administrative structure to provide EOL care consistent with patients' values and preferences. Although patient symptom data is not yet available, other measures of success included improved access to hospice and palliative care services from the time of diagnosis and a sustained palliative care program at two of the three sites in which the program was implemented.     

Insurance coverage of smoking cessation treatment for state employees

Public health experts recommend that health insurance include coverage for smoking cessation treatment as an evidence-based strategy to reduce smoking. As employers, states can implement this policy for more than 5 million individuals nationwide. This study identified the extent to which states require smoking cessation treatment insurance coverage for their employees; of 45 states, 29 required coverage for at least 1 US Public Health Service (PHS)-recommended treatment, and only 17 of 45 provided coverage that was fully consistent with PHS recommendations.     

Heat illness in the U.S. mining industry

BACKGROUND: Heat illness is relatively common in the underground metalliferous mines of South Africa and Australia. Little is known about heat illness in other forms of mining and there have been no studies of heat illness in the US mining industry. METHODS: Mine Safety and Health Administration (MSHA) accident, injury, illness, and employment data were used to study heat illness reported by the US mining industry from January 1, 1983 to December 31, 2001. RESULTS: Five hundred thirty eight cases of heat illness were reported. None of these cases were fatal. Four hundred twenty seven cases (79.4%) occurred in the summer months of June, July, and August. Incidence rates of heat illness in underground mining ranged from 0.00275/10(6) person-hours for coal, to 0.168/10(6) person-hours for metal, rate-ratio = 61.1 (P < 0.001). Incidence rates in surface mining ranged from 0.0265/10(6) person-hours for coal, to 0.0644/10(6) person-hours for stone, rate-ratio = 2.43 (P < 0.001). Incidence rates in mills/preparation plants ranged from 0.0255/10(6) person-hours for coal, to 0.417/10(6) person-hours for stone, rate-ratio = 16.4 (P < 0.001). CONCLUSIONS: Heat illness occurs most frequently in stone mills, metal mills, and underground metal mines. Preventive measures should target the summer months of June, July, and August.     

Neuroimaging findings in glutaric aciduria type 1

Objective To review the imaging features of glutaric aciduria type 1 (GA-1) in a group of 20 patients, the largest published series to date. To document the findings not previously reported and compare our findings with the imaging characteristics of GA-1 previously reported in the literature.Materials and methods For 14 patients the original scans were examined and in the remaining 6, where the imaging was unavailable, the radiology reports were consulted. Nine patients had serial cranial US examinations, 13 had 18 CT scans performed and 14 patients had 39 MRI scans.Results Widening of the sylvian fissures and of the fluid spaces anterior to the temporal lobes was seen in 93% of cases. The mesencephalic cistern was also widened in 86%. Abnormal high-signal intensity on T2-weighted (T2-W) images was seen in the basal ganglia and periventricular white matter in 64% of children. Subdural collections were found in 3 patients, all of which resolved spontaneously. Four neonates followed with serial cranial US showed bilateral multiple caudothalamic cysts. Abnormal high signal on T2-W images was seen in the dentate nucleus, substantia nigra and the pontine medial lemniscus in 79, 43 and 64%, respectively.Conclusions Widening of the sylvian fissure, mesencephalic cistern and expansion of CSF spaces anterior to the temporal lobes are cardinal signs of GA-1. If combined with abnormalities of the basal ganglia and white matter, GA-1 should be strongly suspected.Presented at 37th Annual Congress of ESPR, Lisbon, Portugal, May 2000