M. Carreras

Motor neuron disease in the province of Ferrara, Italy, in 1964–1982

We carried out an intensive incidence, prevalence, and mortality survey of motor neuron disease (MND) in the province of Ferrara, northern Italy. Based on 72 patients, the mean incidence per year for the period 1964 through 1982 was 0.98 cases per 100,000. On December 31, 1981, the prevalence rate was 3.95 per 100,000. In the 19-year period the average mortality rate was 0.83 per 100,000 per year. The disease was more common in men, in individuals aged 50 to 70 years, and in residents in rural areas engaged in agricultural work. A retrospective case-control study, confirming a significantly higher frequency of MND in farmers and persons living in rural areas, revealed that the disease was more common in the lower social classes to which most unskilled and heavy laborers belong. In addition, a significantly increased risk for MND was found in patients with previous histories of trauma, but confounding variables may account for this association.

Systemic lupus erythematosus presenting with neurological disorders

SummarySix patients are described who developed a wide variety of neurological manifestations heralding systemic lupus erythematosus (SLE), which included epileptic seizures, stroke, peripheral polyradiculoneuro pathy similar to Guillain-Barré syndrome, transverse my elopathy and multifocal disorders with remitting course mimicking multiple sclerosis. The peculiarity of these cases was that the neurological disorders remained the only manifestations of SLE for many years and the nervous system appeared to be the main target even after the development of systemic SLE. In five patients the prognosis was favourable and corticosteroid treatment led to prolonged remission.

The frequency of multiple sclerosis in Mediterranean Europe: an incidence and prevalence study in Barbagia, Sardinia, insular Italy

Recent investigations on multiple sclerosis (MS) distribution in Italy, based on relatively small population groups, contradict the current thesis, derived from studies undertaken in populations exceeding 300,000, of a lower spreading of the disease in Mediterranean Europe. Intensive surveys on small populations strengthen the suggestion that Italy is a high‐risk area, although MS studies based on prevalence rather than incidence could also be biased by geographic variables making prevalence unsuitable for comparing MS prevalence rates found in different years and/or zones. We report a further epidemiologic study in Barbagia, Sardinia, insular Italy, undertaken both to establish the MS incidence in the last 20 years, and to estimate a new prevalence rate. Based on 31 probable incident cases, the mean incidence per year for the years 1961‐1980 was 2.9 per 100,000 (3.2 if age‐ and sex‐standardized to the Italian population). On October 24, 1981, the prevalence per 100,000 was 65.3 (77.9 if standardized to the Italian population). These results confirm that in Barbagia, MS occurs more frequently than expected in a Mediterranean area, and give further support to the idea that MS frequency in Italy is similar to that established for most central and northern European countries.

Immunogenetic heterogeneity and associated autoimmune disorders in myasthenia gravis: a population-based survey in the province of Ferrara, northern Italy.

Introduction ‐ The well‐established relationship between myasthenia gravis (MG) and HLA antigens varies among different ethnic groups. In Caucasians B8 and/or DR3 alleles have been found associated with young MG women without thymoma and with high titres of acetylcholine‐receptor antibody (AChR Ab). An increased frequency of haplotype HLA‐A3, B7 and/or DR2 has been observed in older MG patients with low AChR Ab levels. So far, there is no convincing evidence for an association between a specific haplotype HLA and ocular MG or MG with thymoma. MG subjects often show other concurrent autoimmune disorders suggesting a more general inherited predisposition to autoimmunity. We performed a community‐based study to verify the HLA‐A, B, C, DR and DQ profile on ethnically homogeneous MG patients and with the aim to estimate the frequency of concurrent autoimmune diseases and to compare HLA phenotypes to autoimmune status in different MG patients groups. Methods ‐ Forty‐seven patients, living in the province of Ferrara, were followed‐up in our neurologic department and typed for HLA Antigens. In addition a set of immunological laboratory tests was performed. Results ‐ We found a trend towards an increased B8 and DR3 frequencies in total affected population; an association between B8 allele and early onset of generalized MG sustained by thymic hyperplasia. The DR3 allele is statistically associated with the presence of additional autoimmune disorders. Conclusions ‐ Our data support the hypothesis of a genetically‐based heterogeneity of the disease and show an increased prevalence of associate autoimmune conditions in MG patients.

Multiple sclerosis in southern Europe. A prevalence study in the sociosanitary district of Copparo, northern Italy.

The prevalence of multiple sclerosis (MS) in the Italian studies presently available is reported to be between 3.3 and 19.5 per 100,000. All these studies, however, have been undertaken in populations exceeding 300,000 and probably offer underestimates of MS frequency. Current sanitary organization in Italy is inadequate for epidemiological search and many patients with MS may be overlooked when large populations are studied. So, accurate estimates of MS frequency are possible only as a result of intensive surveys in small population groups. Prevalence figures of 53 and 32 per 100,000 have been recently reported by Dean et al. who studied the frequency of MS in small population groups in Sicily. The present study reports the results of a similar survey in the district of Copparo (population 45,153) in northern Italy. The prevalence of probable MS found in Copparo was 31 per 100,000. This result confirms the findings of Dean et al. in southern Italy and supports Deans opinion that the true frequency of MS in Italy as a whole is higher than that indicated by the Italian studies.

Predisposing factors in Bell's palsy: a case-control study

SummaryThe frequency of diabetes mellitus reported in subjects affected by Bells palsy varies widely. In this investigation, a case-control study, we encountered a frequency of 24.8%. In addition, arterial hypertension and lipid disturbances were found to affect subjects with Bells palsy more frequently than controls. These findings appear to suggest a primarily ischaemic pathogenesis for most cases of idiopathic peripheral facial paralysis. Furthermore, the finding of significantly lower taste impairment in diabetics than in non-diabetics with Bells palsy may support the hypothesis of a vascular rather than a metabolic pathogenesis in these cases also. In fact, the vessels supplying the distal portion of the facial nerve, probably more affected in the diabetic patients in order to preserve taste sensation, have such a particular anatomical configuration that this might favour the onset of a diabetic small vessel disease which, in turn, would represent a factor of easier decompensation.

Multiple sclerosis: does epidemiology contribute to providing etiological clues?

The epidemiological approach has undoubtedly contributed to our knowledge of Multiple Sclerosis (MS) by providing some etiological hypotheses in spite of the fact that a definitive basis for the conclusive resolution of its enigma is still lacking. Epidemiological studies have indicated that MS has an uneven geographical distribution and a changing incidence over time at least in several areas of the world: this suggests an etiological role of both genetic and environmental factors. The racial difference in disease risk, the results of familial and twin studies as well as the association between MS and some HLA markers, support the great importance of genetic factors. On the other hand, the evidence of temporal trends and the data from migrant studies seem to underline the etiological contribution of environmental factors. In the light of these results much of the present views have emerged interpreting the disease as caused by multiple factors acting at a susceptible age in genetically predisposed subjects.

Prognosis of Childhood Epilepsy: A Community-Based Study in Copparo, Italy

We performed a community-based study among children and adolescents with idiopathic and cryptogenic epilepsy and onset of the seizures between 0 and 19 years of age on the prognosis of being seizure-free. The study population was recruited during a descriptive investigation in the Local Health Service of Copparo (USL 34), Ferrara, Northern Italy. We included 111 patients (61 males and 50 females). The average length of follow-up was 18.8 years (ranging from 7 to 24 years). The cumulative probability of being in remission was 81.2% at 15 years after onset and the estimated percentage of patients in remission without therapy was 56% for the same time period. At 15 years after the onset of epilepsy, approximately 20% of patients continued to have seizures; nearly 25% continued to take antiepileptic drugs but had been free of seizures for at least 5 years; nearly 56% had been without seizures and free of medication for at least 5 years. Seizure type, gender, age at onset of the illness, epileptic abnormalities on EEG, family history of convulsive disorders, number and frequency of seizures prior to the start of treatment were found not to be helpful as prognostic factors. This community study, carried out on patients without the well known factors that adversely affect prognosis, confirms that the prospect of seizure control and for withdrawal of therapy is (generally) good.

Intracranial gliomas in Ferrara, Italy, 1976 to 1991

Introduction ‐ We planned a descriptive study on the incidence of intracranial gliomas spanning a 16‐year period (1976–1991) in the Local Health Service 31 of Ferrara, Northern Italy. Material and methods ‐ We used a complete enumeration approach by reviewing all the possible sources of case collection available in the study area. Results ‐ The mean annual incidence rate was 5.8 new cases per 100000 population (6.96 for men and 4.78 for women; p < 0.05), resulting in 4.7 per 100000 when directly adjusted to the Italian population. The age‐specific incidences showed a small peak in childhood, an increase with age, reaching a maximum in the age group 60 to 64 and then a decline in the elderly. This pattern is similar for both sexes. The adjusted rates increased from 3.94 per 100000 population in the first five‐year period to 5.6 per 100000 in the third (a nonsignificant difference). The distribution of cases within the study area was substantially uniform. Conclusion ‐ The incidence rates of Ferrara fell into the middle‐high values so far reported and confirmed the male preponderance found in previous studies. The age‐related pattern is similar to that observed, with few exceptions, in other surveys. Like other authors we did not find a significant temporal trend, although the incidence rates tended to increase with time. The data encourage further, wider epidemiological studies of a prospective nature.

Ictal Prosopagnosia and Epileptogenic Damage of the Dominant Hemisphere. A Case History

This case report concerns a patient whose episodes of prosopagnosia appeared to be epileptic manifestations arising from the left occipital lobe. The underlying pathology, as revealed by radiologic and surgical findings, was an atrophic-hypoplastic lesion of the left hemisphere, more prominent in the posterior sections, likely dating from early life. Removal of an arachnoidal cyst of the occipital pole and excision of the damaged gray matter were followed by the disappearance of the prosopagnosic seizures for the two years between the date of the surgical intervention and the publication of this article.