I. Aiello

The risk of Parkinson disease in Mediterranean people

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 per 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.

Epidemiology of Multiple Sclerosis in Northwestern Sardinia: Further Evidence for Higher Frequency in Sardinians Compared to Other Italians

The Sardinians are an ethnically homogeneous population, having a genetic structure quite different from that of all other Italian and European populations. All epidemiological studies carried out in Sardinia since 1975 indicate that this Mediterranean island shows twice the prevalence of multiple sclerosis (MS) compared to continental Italy, but the size of the Sardinian communities so far surveyed has been too small to draw definitive conclusions. To overcome this draw-back, we have studied the frequency of MS in a well-defined area of north-western Sardinia, with a population of about 270,000 in the 1991 census. Based on 276 MS cases, the prevalence on December 31st, 1991, was 102.6 per 100,000. The incidence, averaging 2 per 100,000 in the period of 1962 to 1971, rose to 5 in the period from 1977 to 1991. The present study confirms the higher frequency of MS among Sardinians compared to other Italian populations. Genetic, linguistic and historical data suggest a role of environmental and genetic factors in determining the notable difference in MS risk between Sardinia and the rest of Italy.

Pure post-stroke cerebellar cognitive affective syndrome: a case report.

Abstract.Cerebellar pathology commonly shows important motor signs and less evident cognitive dysfunction. The ’cerebellar cognitive affective syndrome’ is characterised by impairment on executive function, spatial cognition, language and behaviour. We report the case of a man with acute onset of transitory motor features and severe mental disorders. Cranial CT and brain MRI revealed extended cerebellar lesions. Neuropsychological assessment disclosed deficits of attention, executive function and memory. Auditory event–related potentials showed abnormal P300. These data suggest a pure “cerebellar cognitive affective syndrome” and strengthen the hypothesis of cerebellar cognitive function modulation.

Incidence of multiple sclerosis in the town of Sassari, Sardinia, 1965 to 1985: Evidence for increasing occurrence of the disease

Prevalence studies carried out in Sardinia from 1975 suggest an increasing occurrence of MS. However, it is questionable whether this increase represents a real change in MS incidence or simply reflects longer survival. Data from 79 patients indicated the average annual incidence for the period 1965 through 1985 was 3. 4 per 100, 000. On December 31, 1985, the prevalence rate was 69 per 100, 000. Evaluation of MS temporal trends showed a significant increase in MS incidence during the study period: values ranged around two per 100, 000 in the triennial periods 1965–1967, 1968–1970, and 1971–1973, and around five in each triennium from 197-7 onward.

Cognitive impairment and neurophysiological correlates in MS

Cognitive impairment in multiple sclerosis (MS) has received considerable interest over the last decades. Heterogeneous patterns of cognitive dysfunction have been reported in literature in relation to the subtype of the disease and the severity of specific cognitive domains affected. Event related potentials (ERPs), especially P300, have been employed to evaluate the cognitive decline in MS and neurophysiological findings agree with data obtained by neuropsychological testing. The objectivity, the reliability and the easy administration are the main features of ERP technique but more specific attention and memory tasks are needed to enhance the clinical value of the methodology. Moreover, ERP recording has the advantage of being feasible even in severe disabled patients. Finally, longitudinal ERP studies are required to investigate the natural course of cognitive dysfunction in MS, to estimate the prognostic value of subclinical defects in different clinical form of the disease and to evaluate clinical benefits of therapeutic and rehabilitative interventions.

Successful treatment of acquired pendular elliptical nystagmus in multiple sclerosis with isoniazid and base‐out prisms

We treated 3 multiple sclerosis patients who had pendular nystagmus with isoniazid (800 to 1,000 mg/d). Isoniazid abolished the nystagmus and relieved oscillopsia in 2 patients but was ineffective in the 3rd in whom the nystagmus was damped with convergence and vision improved with converging (base-out) prisms.

Incidence of multiple sclerosis in Macomer, Sardinia, 1912–1981: Onset of the disease after 1950

Intensive search of all cases of MS occurring in the Sardinian commune of Macomer since 1912 indicated that MS was absent up to the early 1950s. All 13 ascertained cases had clinical onset in the years 1952–1981. During this period, the average annual incidence was 4.8 per 100,000; the highest incidence was found in the period 1957–1961, slowly decreasing up to 1981. MS was probably introduced after 1945, when the centuries-old isolation of Macomer ended and the native population came into contact with individuals from high-and medium-risk areas.

Stroke Outcomes and Neuroimaging of Intracranial Atherosclerosis (SONIA): Design of a Prospective, Multicenter Trial of Diagnostic Tests

Background and Relevance: Intracranial atherosclerosis is responsible for 70,000 ischemic strokes each year in the USA. Noninvasive testingsuch as transcranial Doppler ultrasound (TCD) and magnetic resonance angiography (MRA) to identify intracranial atherosclerosis is in widespread use, but has not been rigorously validated against the gold standard, catheter angiography. The recently NIH-funded Warfarin-Aspirin Symptomatic Intracranial Disease (WASID) trial will compare warfarin with aspirin for stroke prevention in patients with intracranial atherosclerosis. WASID requires performance of angiography along with TCD and MRA, providing an opportunity to critically evaluate these noninvasive tests. Main Objective: The purpose of the Stroke Outcomes and Neuroimaging of Intracranial Atherosclerosis (SONIA) study is to develop the noninvasive diagnosis of intracranial atherosclerosis. The primary aim of SONIA is to define velocity values on TCD and anatomic abnormalities on MRA that identify severe (50–99%) intracranial stenosis of large, proximal arteries seen on catheter angiography. SONIA will define the criteria, or ‘cutpoints’, for an abnormal TCD or MRA and show that they perform with a reliable positive predictive value (PPV). Study Design: SONIA will be conducted in collaboration with WASID. Study-wide cutpoints defining positive TCD and MRA have been developed and reviewed by the site investigators of WASID. Hard copy angiography, TCD and MRA generated in WASID will be centrally read in SONIA. TCD and MRA cutpoints seek to achieve a target PPV of 80% for the identification of severe intracranial stenosis on angiography. Conclusions: Central readings will be used to validate the cutpoints and to develop measures of negative predictive value, and inter- and intra-observer variability. Sensitivity and specificity will be determined after adjustment for verification bias and employed in receiver-operator characteristic analyses. SONIA will use these techniques to develop TCD and MRA cutpoints that minimize the clinical consequences of test errors occurring in the noninvasive evaluation of patients with suspected intracranial atherosclerosis.

Accuracy of Death Certificates for Amyotrophic Lateral Sclerosis Varies Significantly from North to South of Italy: Implications for Mortality Studies

Objective: To evaluate the accuracy of death certificates (DCs) for amyotrophic lateral sclerosis (ALS) in different parts of Italy. Studies based on DC diagnosis for ALS have shown a reduced mortality comparing northern with southern Italy. These data are in contrast with results from other surveys on the incidence of ALS performed in Italy and other countries. Methods: Archives of neurological clinics from northern (Milano, Monza, Pavia, and Bologna) and southern Italy including islands (Napoli, Sassari, Palermo, and Messina) were searched for patients discharged with a diagnosis of ALS in the period 1970–1995. Subjects affected by definite/probable ALS according to the Scottish Motor Neuron Disease Research Group diagnostic criteria were included. DCs were obtained from the vital statistic bureau. True positive rates (TPRs) and 95% confidence intervals (CIs) for proportions were calculated for northern and southern Italy separately. Multiple logistic regression analysis was performed according to gender, age at onset, age and year of death, and interval between onset and death. Results: We found 651 patients affected by definite/probable ALS; 573 of them had died by December 31, 1996. DCs were available for 566 subjects (411 from northern Italy and 155 from southern Italy). TPR was 66.7% (95% CI 61.9–71.2) for northern Italy and 51.6% (95% CI 43.5–59.7) for southern Italy (χ2 = 10.9, p = 0.001). Logistic regression analysis showed an association between a lower accuracy of DCs and the interval between onset of symptoms and death. TPR calculations considering different death periods (1970–1982 and 1983–1996) showed comparable rates of accuracy over time. Conclusions: Mortality statistics based on official death records do not accurately reflect interregional mortality for ALS in Italy.

Effects of Long-Term L-Dopa Therapy on Carbohydrate Metabolism in Patients with Parkinson’s Disease

Carbohydrate metabolism and insulin secretion were investigated in 26 patients with Parkinsons disease before and during L-dopa treatment. Oral glucose tolerance tests were performed on 13 patients treated with L-dopa alone and on 7 patients treated with L-dopa combined with Carbidopa. Intravenous glucose tolerance tests were performed on additional 6 patients treated with L-dopa alone. Results indicate that chronic L-dopa administration does not modify glucose metabolism. It was observed only a significant decrease of insulin secretion after oral glucose in the early phase (15th day) of treatment with L-dopa alone. This temporary effect may be related to the peripheral conversion of L-dopa to dopamine since insulin secretion during combined therapy was normal. The mechanism by which L-dopa transiently inhibits insulin release is not clear. Perhaps the oral administration of L-dopa alone causes an alteration in some gastrointestinal functions which are involved in the handling of oral glucose.