Future research and collaboration: the "SINERGIE" project on HCV (South Italian Network for Rational Guidelines and International Epidemiology).

The SINERGIE (South Italian Network for Rational Guidelines and International Epidemiology) project is intended to set up a collaborative network comprising virologists, clinicians and public health officials dealing with patients affected by HCV disease in the Calabria Region. A prospective observational data-base of HCV infection will be developed and used for studies on HCV natural history, response to treatment, pharmaco-economics, disease complications, and HCV epidemiology (including phylogenetic analysis). With this approach, we aim at improving the identification and care of patients, focusing on upcoming research questions. The final objective is to assist in improving care delivery and inform Public Health Authorities on how to optimize resource allocation in this area.

The effect of the 1993 European revision of the AIDS case definition in Italy: implications for modelling the HIV epidemic.

OBJECTIVES: To evaluate the effect of the 1993 European AIDS definition on reducing pre-AIDS mortality and to what degree an earlier diagnosis can be made. DESIGN: Prospective observational study. METHODS: All patients diagnosed between January 1993 and December 1994 and reported to the National AIDS Registry from four Italian regions, who met only the new criteria for the 1993 case definition (AIDS-1993) were studied. Follow-up of patients who did not eventually meet the 1987 definition (AIDS-1987), or had not died from other causes (pre-AIDS-1987 death), was censored at the last available clinical visit before 1 April 1996. We analysed the data using Kaplan-Meier non-parametric survival analysis and Cox proportional hazards model. RESULTS: A total of 74 (4.1%) individuals met only the new criteria. Of these, 49 (62.2%) were men, 42 (56.8%) had pulmonary tuberculosis, 22 (29.7%) had recurrent bacterial pneumonia, and 10 (13.5%) had cervical cancer. During follow-up, 35 (45.3%) individuals developed an AIDS-1987 disease, and 10 (13.5%) died without fulfilling the AIDS-1987 definition. Pre-AIDS-1987 death accounted for 22.2% (10 out of 45) of the subsequent outcomes observed prior to 1 April 1996. Using Kaplan-Meier technique, we estimated that after 9.8 months 50% of these individuals were diagnosed with AIDS-1987 disease, or died without such a diagnosis. Individuals with lower CD4+ count at the time of the AIDS-1993 diagnosis progressed more rapidly to AIDS-1987 than those with a higher count. In contrast, pre-AIDS-1987 mortality was strongly associated with injecting drug use, whereas no association was found with CD4+ count. CONCLUSIONS: Approximately 50% of individuals with one of the three new AIDS-defining diseases will develop an AIDS-1987 disease or will die within 1 year. Time from AIDS-1993 to AIDS-1987 disease is strongly associated with CD4+ count at diagnosis. AIDS_1993 diagnosis reduced the pre-AIDS-1987 mortality in injecting drug users. Furthermore, approximately 20% of individuals diagnosed with AIDS-1993 disease are expected to die without developing an AIDS-1987 disease. These data should be useful for correcting the AIDS incidence curve in Europe for the effect of the changes in the AIDS definition.

Early-onset progressive encephalopathy with migrant, continuous myoclonus.

Three unusual cases of focal continuous myoclonus with onset during the first months of life, lasting from dozens of minutes to hours, are reported. During disease evolution, prolonged bilateral myoclonic seizures and generalized tonic-clonic seizures occur. Subsequently, a progressive encephalopathy with hypotonia and ataxia appears. A net worsening of the neurological condition is observed after the age of 4-5 years. Cortical atrophy is shown by CCT and MRI. Neurometabolic screening is not contributory. Repeated polygraphic recordings show continuous and segmental myoclonic jerks, localized in different muscles, at frequencies ranging between 0.5-1 c/s and 6-8 c/s. Moreover action myoclonus is recorded. During the first period of disease the EEG does not show any paroxysmal activity. As to the classification, this syndrome corresponds to an early onset progressive encephalopathy of unknown origin, similar in some aspects to Alper's disease. Another problem is the interpretation of the myoclonic phenomena. Some important aspects suggest a cortical origin of the diverse myoclonic phenomena observed in these cases.

Occlusion of unilateral carotid artery in Down syndrome.

The association between moyamoya phenomena and Down syndrome (DS) is reported in the literature. This paper reports a case of DS, which at age 9 presented right hemiparesis, secondary to the occlusion of the left internal carotid artery; cerebral angiography (CAG) showed a collateral circulation that mimicks the moyamoya phenomenon. Clinical recovery was almost complete; a second CAG after 15 months showed a persistent occlusion of the left internal carotid artery and an opacification of the left middle cerebral artery from abnormal vessels; but the collateral circulation is not enhanced. This case proves that in DS cerebrovascular occlusions may present moyamoya-like phenomena. These differ however from the true moyamoya disease in a number of aspects: the arterial occlusion is unilateral, the evolution is favorable and revascularization does not occur through the peculiar abnormal vessels of the moyamoya syndrome.