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Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease caused by prions that is randomly distributed in all countries, with an overall yearly mortality rate of about 1-2 cases per million people. On a few occasions, however, sporadic CJD occurred with higher than expected rates, but further investigations failed to recognize any convincing causal link. In Italy, cluster analyses of sporadic CJD cases have not been performed previously., Objective: To investigate the geographical distribution of sporadic CJD using municipality geographical data of Apulia with the aim of detecting spatial clusters of disease., Patients and Methods: Patients included in this study were diagnosed as probable or definite sporadic CJD and were residents of the Apulia Region (Italy). Bayesian hierarchical models with spatially structured and unstructured random components were used to describe the spatial pattern of the disease and to assess the extent of heterogeneity among municipalities. The Kulldorff-Nagarwalla scan test and the flexible spatial scan statistic were used for detecting spatial clusters., Results: Smoothed Bayesian relative risks above the null value were observed in a few adjacent municipalities in the north and middle areas of Apulia. However, both the circular scanning method and the flexible spatial scan statistic identified only a single cluster in the central part of the region., Conclusion: Geographical analyses and tests for spatial randomness identified a restricted area with an unusually high number of sporadic CJD cases in the Apulia region of Italy. Environmental and genetic risk factors other than mutations in the prion protein gene however, need to be investigated., (© 2019 S. Karger AG, Basel.)

A water-oil microemulsion and an aqueous solution, both carrying pertechnetate, were injected subcutaneously in rabbits; release was observed by imaging the administration sites with a gamma-camera. Disappearance from the injection site of pertechnetate in aqueous solution was about ten times faster than that of pertechnetate in a microemulsion.

Both CBF and CBV were evaluated by gamma-camera SPECT in 14 patients with classic migraine, all studied while symptom-free. Nuclear data were correlated with CT and MRI. A decreased regional CBF was observed in 13 of the 14 patients. The decreased perfusion was localized in the frontal lobe in 6 patients, the temporal lobe in one, the parietal lobe in 11 and the occipital lobe in 5 patients. The parieto-occipital cortex was involved more often than the frontal cortex; the association of hypoperfusion with parieto-occipital cortex was quite high. The right parieto-occipital regions were affected more often than the left ones. Regional CBV was increased in 8 patients. There was good topographical concordance between decreased CBF and increased CBV, but the increase of CBV was in general more evident at the periphery of the hypoperfusion. It is of interest that the only patient with a normal CBF study had a pathological CBV study. Apparently, CBF derangements are very common in symptom-free patients with classic migraine, a CBF decrease being often accompanied by a CBV increase. In these patients both CT and MRI have a lower diagnostic yield than SPECT.

In newly diagnosed diabetics treated with Monotard (porcine monocomponent (MC) Lente insulin) for five years, no antibodies against porcine or bovine proinsulin were observed, but 2 of 13 subjects developed a-component antibodies. In newly diagnosed diabetics, treatment for the same period with conventional Lente insulin induced both proinsulin and a-component antibodies (in 5 and 8 of 10 cases, respectively). In 31 patients transferred from conventional Lente to Monotard, proinsulin and a-component antibody levels were significantly lower than in 22 patients maintained on conventional Lente after the 5-year follow-up period. No significant differences were noted between bovine and procine proinsulin antibodies. Insulin antibody production was similar to that of proinsulin antibody.