Congenital supratentorial and infratentorial peripheral neurogenic tumor: a clinical, ultrastructural, and immunohistochemical study

Congenital neoplasms of the central nervous system are extremely rare, although they have been well documented since the earliest reports from the mid-19th century. Medulloblastoma, other primitive neuroectodermal tumors, and various types of gliomas have comprised the majority of cases. This report describes a highly unusual infratentorial and supratentorial tumor presenting as a scalp mass in a neonate who experienced in utero distress. The supratentorial mass extended through a defect in the skull to the parietooccipital lobe, and the infratentorial aspect involved the 9th and 10th cranial nerves in the region of the cerebellopontine angle. A complex spindle cell neoplasm incorporating peripheral nerve sheath and vascular characteristics was further characterized by electron microscopy and immunohistochemistry. Based upon these studies, the tumor was interpreted as a congenital schwannoma with divergent vascular differentiation. The child has been tumor-free for approximately 2 years after the initial operative procedure.     

Macrophage activation and Fcgamma receptor-mediated signaling do not require expression of the SLP-76 and SLP-65 adaptors

The Src-homology 2 domain-containing, leukocyte-specific phosphoprotein of 76 kDa (SLP-76) is a hematopoietic adaptor that plays a central role during immunoreceptor-mediated activation of T lymphocytes and mast cells and collagen receptor-induced activation of platelets. Despite similar levels of expression in macrophages, SLP-76 is not required for Fc receptor for immunoglobulin G (IgG; FcgammaR)-mediated activation. We hypothesized that the related adaptor SLP-65, which is also expressed in macrophages, may compensate for the loss of SLP-76 during FcgammaR-mediated signaling and functional events. To address this hypothesis, we examined bone marrow-derived macrophages (BMM) from wild-type (WT) mice or mice lacking both of these adaptors. Contrary to our expectations, SLP-76(-/-) SLP-65(-/-) BMM demonstrated normal FcgammaR-mediated activation, including internalization of Ig-coated sheep red blood cells and production of reactive oxygen intermediates. FcgammaR-induced biochemical events were normal in SLP-76(-/-) SLP-65(-/-) BMM, including phosphorylation of phospholipase C and the extracellular signaling-regulated kinases 1 and 2. To determine whether macrophages functioned normally in vivo, we infected WT and SLP-76(-/-) SLP-65(-/-) mice with sublethal doses of Listeria monocytogenes (LM), a bacterium against which the initial host defense is provided by activated macrophages. WT and SLP-76(-/-) SLP-65(-/-) mice survived acute, low-dose infection and showed no difference in the number of liver or spleen LM colony-forming units, a measure of the total body burden of this organism. Taken together, these data suggest that neither SLP-76 nor SLP-65 is required during FcgammaR-dependent signaling and functional events in macrophages.     

Indirect hypothalamo-cerebellar pathway? Demonstration of hypothalamic efferents to the lateral reticular nucleus

Summary Hypothalamic efferents to the lateral reticular nucleus (NRL) have been demonstrated in the cat by means of anterograde and retrograde axonal transport of the wheat germ agglutinin — horseradish peroxidase (WGA-HRP) complex. Pressure injections of the WGA-HRP complex into the hypothalamus resulted in anterograde labelling of branching terminal axons both in the NRL and in an adjacent area, presumably the ventrolateral catecholaminergic cell group (A1). After microiontophoretical ejections of the WGA-HRP complex into the NRL from a ventral approach, retrogradely labelled neurons were found in the lateral, dorsal, posterior and anterior hypothalamic areas and in the tubero-mammillary, dorsomedial and periventricular nuclei. The projection is bilateral with a clear ipsilateral predominance and has its main origin in the lateral hypothalamic area. The locations of hypothalamic cells projecting to the NRL are somewhat different from those giving rise to hypothalamo-cerebellar and hypothalamo-spinal connections. The present demonstration of a hypothalamic input to one of the major precerebellar relay nuclei introduces a new possible indirect route through which the cerebellum may be influenced by the hypothalamus. The different indirect and direct hypothalamo-cerebellar pathways and their potential functional importance are discussed.     

Immunological parameters in girls with Turner syndrome

Disturbances in the immune system has been described in Turner syndrome, with an association to low levels of IgG and IgM and decreased levels of T- and B-lymphocytes. Also different autoimmune diseases have been connected to Turner syndrome (45, X), thyroiditis being the most common.     

A point mutation in the 5' splice site of the dystrophin gene first intron responsible for X-linked dilated cardiomyopathy

X-linked dilated cardiomyopathy (XLDC) is a familial heart disease presenting in young males as a rapidly progressive congestive heart failure, without clinical signs of skeletal myopathy. This condition has recently been linked to the dystrophin gene in some families and deletions encompassing the genomic region coding for the first muscle exon have been detected. In order to identify the defect responsible for this disease at the molecular level and to understand the reasons for the selective heart involvement, a family with a severe form of XLDC was studied. In the affected members, no deletions of the dystrophin gene were observed. Analysis of the muscle promoter, first exon and intron regions revealed the presence of a single point mutation at the first exon-intron boundary, inactivating the universally conserved 5' splice site consensus sequence of the first intron. This mutation introduced a new restriction site for MseI, which cosegregates with the disease in the analyzed family. Expression of the major dystrophin mRNA isoforms (from the muscle-, brain- and Purkinje cell-promoters) was completely abolished in the myocardium, while the brain- and Purkinje cell- (but not the muscle-) isoforms were detectable in the skeletal muscle. Immunocytochemical studies with anti- dystrophin antibodies showed that the protein was reduced in quantity but normally distributed in the skeletal muscle, while it was undetectable in the cardiac muscle. These findings indicate that expression of the muscle dystrophin isoform is critical for myocardial function and suggest that selective heart involvement in dystrophin- linked dilated cardiomyopathy is related to the absence, in the heart, of a compensatory expression of dystrophin from alternative promoters.      

Hypertension control: meeting the 1990 objectives for the nation.

Hypertension (high blood pressure) is 1 of 15 health priorities of the Public Health Service set forth in the report, "Promoting Health/Preventing Disease: Objectives for the Nation." The nine objectives for hypertension include improved health status, reduced risk factors, increased public-professional awareness, improved services and protection, and improved surveillance-evaluation systems. A number of Federal agencies, coordinated by the National Heart, Lung, and Blood Institute, are working to reach the hypertension objectives in cooperation with State, local, and voluntary agencies and organizations. A great deal of progress has been made toward the objectives, as reflected by a variety of indicators. By 1980, for example, 34.1 percent of the population with hypertension had their blood pressure controlled at less than 160/95 mm Hg, but in 1972 only 16.5 percent were so controlled. Since 1972, the age-adjusted death rate has dropped 42 percent for stroke and 27 percent for coronary heart disease. Data indicate that the national goal for sodium ingestion (3-6 grams daily) may already have been met. Fifty-one percent of the population understand that hypertension may lead to stroke, meeting another objective. Public knowledge about hypertension as a "major likely cause of heart trouble" almost doubled in the 6-year period for which data are available. In 1982, 30 percent of processed food in grocery stores had sodium content labeling and almost 50 percent had calorie labeling, according to studies conducted by the Bureau of Foods of the Food and Drug Administration. Efforts are underway to develop a methodology for assessing incidence of hypertension and categories of hypertension control, a need spelled out in another objective.