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The clinical relevance and extent of cognitive impairment in adult myotonic dystrophy type 1 (DM1) and 2 (DM2) is still unclear. The aim of this study was to determine whether previously reported cognitive abnormalities progress over time and if this occurs in DM2 as it does in DM1. Fifty-six patients with DM1 and 29 patients with DM2 were subjected to muscle strength assessment, and to a complete battery of neuropsychological tests. Repeated assessment was performed in 20 DM1 and 13 DM2 over time (DM1 mean follow-up: 7.3±2.7 years; DM2 mean follow- up: 9.5±2.4 years). Muscle strength and test scores for frontal lobe functions worsened significantly over time ( p<0.01), in both DM1 and DM2. DM2 is a progressive muscle disorder, although less severe than DM1. In both DM1 and DM2 frontal cognitive impairment (attentional) worsens over time but does not extend to additional areas of cognition. [ABSTRACT FROM AUTHOR]

Allogeneic cord blood transplantation (CBT), especially from unrelated donors, is being increasingly used for treating paediatric patients with both malignant and non-malignant disorders. Recent clinical and experimental evidence suggests that human cord blood mononuclear cells (CBMC) may acquire in utero a state of tolerance towards non-inherited maternal antigens (NIMA). In order to better define this phenomenon, we measured, by means of a limiting dilution assay (LDA), the frequency of NIMA-specific CTL precursors (CTLp) in cord blood samples obtained from 13 healthy neonates. The immunophenotype of the effector cells recovered from LDA was also analysed. Data concerning both CTLp frequency and phenotype of effector cells were compared with those obtained stimulating CBMC with cells of paternal origin (NIPA) and adult PBMC with allogeneic targets. Results showed that cytotoxic cells directed towards cells of maternal origin could be detected in all cord blood samples tested. Phenotype analysis demonstrated that NIPA elicit the expansion of CD3+/CD8bright T cells, a phenotype associated with alloreactive CTL. By contrast, NIMA preferentially stimulated the expansion of CD3-/CD8dim+ cells, a phenotype associated with NK cells, which are known to be able, in certain clinical conditions, to kill allogeneic haematopoietic cells without causing GVHD. Thus, our results indicate that, when evaluated in a limiting dilution condition, NIMA-reactive cord blood cells are detectable and a preferential expansion of NK cells is observed. [ABSTRACT FROM AUTHOR]