Neglect as a deficit determined by an imbalance between multiple spatial representations.

A previous study on neglect suggested that at least two hand parameters are crucial in producing an amelioration of neglect: the hand (left or right) and the spatial position of the hand (left or right). The improvement observed in perceiving left targets when the left hand acts in the left space can be due either to proprioceptive or to visual cuing. The stimulated left hand located in the left space may act as a powerful visual cue for the enhancement of the left visuo-spatial representation, in the same way as any other visual stimulus presented in the periphery of the visual field. Alternatively, it may be that the perceived hand location (due to the activation of the proprioceptive system) acts as an attentional field able to enhance the representation of the left space. In order to disentangle these two hypotheses, in the present study a naming task was executed by a group of neglect patients and by a control group. The subjects had to name all the objects depicted on a sheet of paper which were reflected on a mirror that inverted right and left space. While doing the naming performance, the subjects passively moved either the right or the left hand, in the left or right space. Stimuli and hand were reflected in the mirror that inverted right and left space and direct view of the stimuli and of the stimulated hand was prevented by a board. The results show that patients were more accurate at naming stimuli reflected in the left side of the mirror when the left hand was located and moved on the left side. In this condition, however, the left hand was seen in the right side of the mirror. It is therefore clear that the better performance was not due to visuo-spatial cuing but to a proprioceptive cuing effect. The results are discussed in terms of the relevance of personal and peripersonal spatial activation in the modulation of extrapersonal visual neglect. The coactivation of different spatial representations seems to be very influential on stimulus coding, thus confirming that spatial awareness is strictly related to the joint activity of multiple brain maps.

Derangement of non-specific immunity in Down syndrome subjects: low leukocyte chemiluminescence activity after phagocytic activation.

Metabolic activation of peripheral blood leukocytes (chemiluminescence) from 27 children with Down syndrome (DS) and 23 age and sex-matched control children after phagocytic stimulation by opsonized zymosan particles was investigated through a chemiluminescence assay. Using autologous plasma or serum as opsonizing media, phagocytic activity of circulating leukocytes was significantly decreased in DS subjects. A further decrease of phagocytic activity was found in neutrophils from DS children, when normal heterologous plasma or sera were used. On the other hand, sera or plasma from DS subjects significantly increased phagocytic activation of leukocytes from normal donors. In DS subjects opsonizing agents such as serum immunoglobulins and complement fractions were in the normal ranges of concentration. Thus, the impaired chemiluminescence of neutrophils was mainly due to a metabolic impairment at the cellular level. A decreased production of radicals derived from the oxygen metabolism in neutrophils may be an important step of immune derangement leading to the increased incidence of infectious diseases frequently associated with DS.

Defective self-recognition in subjects of far-advanced age.

The immunocompetence of 22 subjects aged 85-104 years (mean 90 +/- 1 years) was studied and compared to 21 young subjects aged 19-37 years (mean 30 +/- 1 years). The absolute lymphocyte number and the percentage of T and B lymphocytes in the peripheral blood was similar in the two groups. A marked decrease in phytohemagglutinin response of T-enriched lymphocytes from old subjects was observed. Autologous mixed lymphocyte reaction (MLR) was also profoundly reduced in old subjects. No difference between male and female subjects was observed. The responsiveness of enriched T lymphocytes to allogeneic irradiated non-T cells was only slightly impaired in the old individuals. Non-T cells from old and young subjects functioned equally well as stimulatory cells in allogeneic MLR. The data suggest that an alteration of T lymphocytes with regulatory function and of self-recognition is present in aged humans.

Enhancing effect of lithium and potassium ions on lectin-induced lymphocyte proliferation in aging and Down's syndrome subjects.

The effect of different concentrations of LiCl or KCl (0.6-20 meq/liter) on PHA-stimulated lymphocytes from young, old, and Down's syndrome subjects was studied. LiCl showed a dramatic enhancing effect on [3H]thymidine incorporation induced by a suboptimal dose of PHA in old subjects and Down's syndrome patients. An increase of [3H]thymidine incorporation in human lymphocytes stimulated by a suboptimal dose of PHA was also observed with KCl. This effect was higher in old subjects than that observed in young and Down's subjects. LiCl and KCl can modulate and partially restore the derangement in early events of mitogen stimulation which seems to be present in lymphocytes from both old and Down's syndrome subjects.

Frequency and prognostic value of HLA antigens in osteosarcoma patients.

A homogeneous group of 53 Caucasian subjects with high-grade osteosarcoma (OS) was typed for HLA-A and B locus antigens. Although no significant differences in the distribution of these antigens were found in comparison with 425 local controls, a trend towards an increase of HLA-B18 and decrease of HLA-B12 was observed. All the patients underwent amputation plus adjuvant chemotherapy and among the 29 patients with a follow-up longer than one year, 9 out of 10 subjects with HLA-A3 antigens developed metastases within a few months. None of the OS patients had the HLA-A3, B7 haplotype which is present in linkage-disequilibrium in the control population.

DNA repair after gamma radiation and superoxide dismutase activity in lymphocytes from subjects of far advanced age.

DNA repair after gamma radiation was studied in purified T lymphocytes from young and aged subjects. Two different assays were employed. In the first, T lymphocytes were stimulated with phytohemagglutinin (PHA) for 72 h and then treated with hydroxyurea, irradiated with 30 K rads and pulsed with [3H]thymidine (TdR) for 4 h. In the second, T lymphocytes were first irradiated with graded doses of gamma rays (200-800 rads) and then stimulated with PHA, cultured for 72 h and pulsed with 3H-TdR for the last 6 h of culture. T lymphocytes from aged subjects showed a lack of DNA repair synthesis in the first assay whereas only minor differences were found in the second assay between the two groups, i.e., a certain degree of radioresistance in aged lymphocytes. Lymphocyte superoxide dismutase activity showed great individual variations in both groups and a slight increase in old subjects.

Genetic and hormonal characterization of cryptic 21-hydroxylase deficiency.

Cryptic 21-hydroxylase deficiency has been previously described in asymptomatic family members of patients with classical congenital adrenal hyperplasia (CAH). These family members were detected by high baseline 17-hydroxyprogesterone levels found in the course of family studies. The hormonal responses to ACTH of the family members with cryptic 21-hydroxylase deficiency were determined and compared to the responses of patients with CAH, patients with acquired adrenal hyperplasia, family members predicted to be heterozygous for CAH, family members predicted to be unaffected, and the general population. The ACTH-stimulated levels of 17-hydroxyprogesterone and delta 4-androstenedione in the cryptic family members were elevated above the level of the general population or family members heterozygous for classical CAH, but below that of patients with CAH. The hormonal profile of patients with cryptic 21-hydroxylase deficiency is similar to that of patients with acquired adrenal hyperplasia. The response of family members heterozygous for the cryptic gene (21-OH CRYPTIC/21-OH NORMAL) was indistinguishable from that of family members heterozygous for the classical CAH gene (21-OH CAH/21-OH NORMAL). These studies support our previous proposal that patients with cryptic 21-hydroxylase deficiency are genetic compounds, having one gene for a severe enzyme deficiency and one gene for a mild 21-hydroxylase deficiency. Thus, the 21-hydroxylase genotype in cryptic 21-hydroxylase deficiency is 21-OH CAH/21-OH CRYPTIC.

Longitudinal study of clinical, neurophysiological and immunological parameters in multiple sclerosis. Preliminary investigation.

Thirty subjects affected with multiple sclerosis, of which 22 were female and eight male, with an average of 30 +/- 7, years were studied, for a period of 10-15 months,. clinically using Kurtzkes' report form, neurophysiologically (responses tested: VEP, BAEP, SEP, ESG) and immunologically (Rosette Et, Ea, EAC). Of the 30 cases, 19 showed poussées in the last three years and 11 were in the stabilization phase. The Kurtzke reports and the neurophysiological tests permitted an accurate assessment of lesion levels and their rate of evolution. The immunological tests showed a notable ability to differentiate between subjects with recent poussées and those in the stabilization phase presenting overall values significantly below the norm. In the follow-up, after treatments with methisoprinol, the immunological tests also revealed modifications of notable interest.

HLA genotypes and HLA-linked genetic markers in Italian patients with classical 21-hydroxylase deficiency.

HLA genotype and HLA-linked marker data for 40 unrelated patients from central Italy and 2 unrelated patients from Sardinia with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21-OH-def) were analyzed. The results confirm that the HLA-linked 21-OH-def gene is associated with several different HLA determinants and complete HLA haplotypes, although the only determinant with significantly increased frequency was the complement C2 allele C2B. The HLA antigens B8 and DR3 were found in significantly decreased frequencies. The haplotype A3, Cw6, Bw47, BfF, DR7, which is exceptionally rare in the general population but which has been found in many other 21-OH-def patients from diverse geographical origins, was also found in one of the Italian patients. This and other HLA haplotype associations found among the Italian patients may represent mutations that have occurred on HLA haplotypes with genetic linkage disequilibrium or, alternatively, may represent mutations that have not yet had time to become randomly associated with different HLA complex determinants. The marked negative associations with B8 and DR3 could, however, result from an interaction between the gene products of the HLA complex and the 21-OH-def phenotype.

Cryptic 21-hydroxylase deficiency in families of patients with classical congenital adrenal hyperplasia.

Serum androgens and 17-hydroxyprogesterone concentrations and HLA genotypes were determined in 124 families of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH). In 8 pedigrees, we discovered 16 pubertal or postpubertal family members of either sex who had biochemical evidence of 21-hydroxylase deficiency but were without clinical symptoms of excess virilism, amenorrhea, or infertility. We designated these family members as individuals with cryptic 21-hydroxylase deficiency. Within each generation, the family members with cryptic 21-hydroxylase deficiency were HLA identical. It is proposed that these family members are genetic compounds, having 21-hydroxylase deficiency as a result of two recessive gene defects: 1) a severe 21-hydroxylase gene defect present in the index case with classical CAH (21-OHCAH) and 2) a mild 21-hydroxylase gene defect (21-OHCRYPTIC). Thus, the CAH genotype in the family members with cryptic 21-hydroxylase deficiency is 21-OHCAH/21-OHCRYPTIC. Lod score analysis for linkage between the cryptogenic 21-OH trait and HLA gave a combined Lod score for males and females of theta = 0.00 of 3.409. Close genetic linkage between HLA and 21-OHCRYPTIC was thus established. This study provides support for the previously reported heterogeneity of 21-hydroxylase deficiency which may result from allelic variability at the locus for steroid 21-hydroxylase.

[Variation of the TG lymphocyte subpopulation in the aged]. / Variazioni della subpopolazione linfocitaria TG nei soggetti anziani.

Peripheral blood from 20 healthy subjects of whom 10 were young (15 - 30) and 10 aging (73 - 85) was examined for the proportions of TG lymphocyte population by two different methods TG cells proportions were significantly (P 0.001) increased in aging subjects when compared with the young subjects.

[T-lymphocyte subpopulations at high altitudes. Morococha, Peru - 4560 meters above sea-level]. / Studio della sottopopolazione linfocitaria T in alta quota.-Morococha, Peru - m 4560 su l.m.

We have studied T cell subpopulation in 10 miners of Morococha-Peru and 3 caucasian people as controls. T cells were identifed by E rosette test. Miners show a less number of T cells as compared to caucasian people: respectively 70+/-2% and 77,9+/-3,7%.

[Receptors for murine red blood cells on normal lymphocytes and in chronic lymphocytic leukemia]. / Recettori per emazie di topo sui linfociti normali e nella leucemia linfatica cronica

The ability of unfractionated and purified B and T peripheral lymphocytes to form rosettes EAC and E with sheep and mouse red blood cells was assessed in thirty healthy controls and in five patients with chronic lymphocytic leukaemia. E mouse rosettes is a marker of B cells in man and its percentage in peripheral blood lymphocytes is 9.3% in normal subjects on the contrary patients with CLL had a high rosette count with mouse red blood cells (mean 57%) and a low count with sheep red blood cells (mean 13.7%).