Biomedical subjects
Publications and source records attributed to A Pirisi.
A case of acquired ocular-motor apraxia featured by failure in voluntary initiation of lid closure, horizontal and vertical eye movements is reported. Electrographic and neuropsychological assessment pointed out several signs referable to bilateral frontal and right parietal lobe involvement, confirmed by neuroradiological finding.
On the basis of previous epidemiologic studies, Parkinson disease was thought to be evenly distributed throughout the world. These studies, however, were conducted only on North European populations. The position with regard to the Mediterranean peoples was still unknown, and we therefore studied the frequency of Parkinson disease on the island of Sardinia, where some ethnic groups of the Mediterranean stock are represented. Based on 967 accepted cases, the prevalence 100,000 population on January 1, 1972, was 65.6; the average annual incidence for the period 1961 through 1971 was 4.9. These figures are one-half of the figures established for North Europeans. Our findings suggest racial differences in predisposition to Parkinson disease. Some Negroid features are present in Sardinians. If, as seems likely, Africans prove to be relatively unsusceptible to the disease, the risk for Sardinians and other Mediterranean ethnic groups might be intermediate between North Europeans and Africans.
A case of Behr's disease (complicated optic atrophy) associated with epilepsy and with imbalance of the free amino acid pool in plasma and cerebrospinal fluid, quite similar to that identified in patients with hereditary ataxias, is reported. The latter finding stands for a closer correlation between the two clinical entities.
This investigation was conducted to clarify the epidemiology of ALS disease in Sardinia. During the years 1965-1974, the average annual incidence was found to be 0.64/100,000 inhabitants. On prevalence day, October 24th, 1974, the prevalence rate was 1.56/100,000 inhabitants. A significant male predominance was found, the average annual incidence rates for men and women being 0.88 and 0.40, respectively. The peak in both sexes was reached between 60 and 69 years. ALS distribution in the study area was uniform but its occurrence was significantly higher among agricultural workers (5.28/100,000). ALS started on average at 56.58 years and its duration was 2.5 years, being significantly longer in patients under 40-years-old. The distribution of the various clinical forms was: 66 per cent conventional forms, 20 percent bulbar and 14 per cent pseudo-polyneuritic. In the bulbar type, a female predominance was found. About 96 percent of cases were sporadic and 4 per cent familial. Familial cases presented no difference from sporadic cases. Trauma was present in 10.5 percent of the cases and gastrointestinal disfunction in 13 per cent. This probably reflects some relationship between trauma and ALS, and between malnutrition and ALS. No combination of ALS, dementia and parkinsonism was observed. Dementia was associated with ALS in four cases and Parkinson's disease in one case, separately. The combination of other disease states with ALS in the present study may be simple coincidence.
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The incidence of M.S. in Sardinia has been found to be the highest in Southern Italy. On "prevalence day", October 21st, 1971, the incidence was 12.48/100,000 inhabitants, whereas the rates in Campania and in Puglia, (regions of Southern Italy at the same latitude as Sardinia) the rates were 3.3 and 4.0 respectively. In the present study, a significant female predominance was found, the rates for women and men being 15.88 and 9.03 respectively. The incidence of MS in the study area was found to be significantly higher both in areas with a cool humid climate and in communities with the lowest socioeconomic conditions. A significantly higher incidence was computed for communities with less than 5,000 inhabitants. MS incidence had no correlation with the occupational status and dietary habits, which are uniformly distributed throughout the island. The disease started at au average of 27.3 years and its duration on "prevalence day" was 13.4 years. The age of onset was found to be low in comparison with other population studies in Europe and in North-Central Italy. The age-specific curves of onset and on "prevalence day" showed two peaks, one in the second-third decade and one in the forth-fifth decade. Most of the patients with an earlier onset showed a complete disappearance of the symptoms after the first bout.