Ubiquitin-positive achromatic neurons in corticobasal degeneration

A 66-year-old woman presented with an alien limb syndrome without dementia. The course of her illness was unremitting and at autopsy 6 years later her diagnosis was confirmed as corticobasal degeneration without Alzheimer-type pathology. Although the presence of ballooned achromatic cortical neurons and cell loss from the substantia nigra distinguishes such patients, the site and density of achromatic neurons has not previously been quantified. We show that immunohistochemistry for the cell stress protein ubiquitin selectively stains these achromatic neurons, whereas they do not stain for abnormally phosphorylated tau protein. Phosphorylated neurofilament antibodies recognise both ballooned and non-ballooned neurons. In this case, high densities of ubiquitin-positive ballooned neurons were found in frontal cortical regions with the highest densities in layers V and VI of the anterior cingulate cortex. In addition, high densities of ubiquitin-positive ballooned neurons were found in the insular cortex, claustrum and amygdala. These results confirm past reports of frontal pathology, but show that there is also considerable pathology in insular and parahippocampal cortical regions and some subcortical regions. Our findings suggest that the distribution and staining characteristics of ballooned neurons in corticobasal degeneration may help to differentiate these cases pathologically, while the absence of dementia appears to be an important clinical criterion.     

Some practical perspectives for educating gifted children

This article identifies common characteristics of educationally related programs that form a common basis for understanding and working with gifted programs. Special approaches and programs for educational enrichment as well as specific activities that have been successful are discussed.     

Universities as resources to state health agencies.

In a survey of the 50 State health agencies in the spring of 1992, officials were asked about their manpower and research needs in the specific areas of administration, behavioral and social science, education and information, environmental health, environmental protection, epidemiology, laboratory, law, occupational health, policy and planning, and statistics. In all, 40 agencies (80 percent) responded. Indepth telephone interviews to determine whether universities and schools and graduate programs in public health filled these needs completed the data collection process. Agency officials indicated that their resources were least adequate in environmental protection, behavioral and social science, and occupational health. They did not feel their research needs were being met. There was a general feeling that universities and schools and programs in public health have different agendas than State agencies and that practical solutions to the shortage of research resources are not forthcoming from these sources. Suggestions are made as to what can be done to improve relationships between those who train public health personnel and those who employ them.     

CD4-specific monoclonal antibody can prolong cardiac allograft survival without T-cell depletion

Current immunosuppressive regimens for clinical transplantation are immunologically non-specific, are associated with acute and chronic toxic side-effects [1] and are unable to prevent chronic graft loss in a significant proportion of patients. Additionally, new and increasingly powerful drugs are being introduced to induce non-specific immunosuppression, and therefore this is likely to be followed by an increase in related complications such as the induction of cancers. Hence, there is a need for an alternative approach. It has been shown that long-term survival of murine cardiac grafts can be induced by the monoclonal antibody YIS 191 that depletes CD4 ⁺T cells in vivo [2]. In this study, we have investigated the ability of a non-depleting antibody to produce better graft survival.     

Inhibition of mediator release in systemic mastocytosis is associated with reversal of bone changes

A 59-year-old male presented with systemic mastocytosis with extensive skeletal involvement resulting in vertebral compression fractures and bone pain. Histomorphometric analysis of bone revealed increased mast cells, elevated static parameters of bone resorption, and low bone formation. Serum calcium, phosphorus, and alkaline phosphatase were normal; however, serum 1,25-dihydroxyvitamin D3 and osteocalcin levels were low. Histamine levels in plasma and urine were elevated. Following therapy with ketotifen, the patient had resolution of bone pain along with decreased flushing and pruritus. Elevated plasma and urine histamine levels normalized, as did 1,25-dihydroxyvitamin D3 and osteocalcin levels. Indices of low bone formation improved on therapy. Eroded surfaces improved but remained elevated. This case is the first demonstration that bone symptoms and histomorphometric change in systemic mastocytosis are reversed with inhibition of mast cell degranulation. The role of mast cells and their products in bone metabolism is poorly understood, but the therapy of bone disease in systemic mastocytosis should include inhibition of the release of mast cell products along with the use of histamine antagonist.