Space-time distribution of the ALS incident cases by onset type in the Health District of Ferrara, Italy.

OBJECTIVES: An ecological study in the resident population of the Health District (HD) of Ferrara, Italy, has been carried out to establish the distribution in space and time of the amyotrophic lateral sclerosis (ALS) incident cases according to the disease onset type and gender in the period 1964-2009. MATERIAL AND METHODS: The hypothesis of a uniform distribution was assumed. RESULTS: The incident cases of spinal onset ALS and bulbar onset ALS were evenly distributed in space and time in both men and women. The spinal onset ALS incident cases distribution according to gender was significantly different from the expected in the extra-urban population (20 observed cases in men 95% Poisson confidence interval 12.22-30.89, expected cases in men 12.19; six observed cases in women 95% Poisson confidence interval 2.20-13.06, expected cases in women 13.81), whereas no difference was found in the urban population. The spinal onset ALS incidence was higher in men than in women in the extra-urban population (difference between the rates = 1.53, 95% CI associated with the difference 0.52-2.54), whereas no difference between sexes was found in the urban population. CONCLUSIONS: The uneven distribution according to gender of the spinal onset ALS incident cases only in the extra-urban population suggests the involvement of a gender related environmental risk factor associated with the extra-urban environment. Despite some limits of the spatial analysis in the study of rare diseases, the results appear consistent with the literature data.

Incidence of childhood and adolescence epilepsy: a community-based prospective study in the province of Ferrara and in Copparo, Italy, 1996-2005.

BACKGROUND: The annual incidence of childhood and adolescence epilepsy ranges from 41 to 97 diagnoses per 100,000 people in western Countries, with a reported decline over time. We aimed at studying the incidence of epilepsy in children and adolescents (1 month to 14 years) and its temporal trend in the province of Ferrara, northern Italy. METHODS: We implemented a community-based prospective multi-source registry. All children with newly diagnosed epilepsy in the period 1996-2005 were recorded. RESULTS: The incidence rate of newly diagnosed epilepsy in the considered age range was 57 per 100,000 person-years, (95% CI: 49.3-65.9), with a peak in the first year of life (109.4; 95% CI: 69.4-164.1), without differences between the two gender. The estimates were significantly lower than those observed previously (97.3; 95% CI: 81.9-115.7). CONCLUSIONS: Incidence rates for epilepsy in the Italian population aged 1 month to 14 years are in line with those of other European and Northern American Countries. The incidence of childhood epilepsy has declined over time in our area. A reduced impact of serious perinatal adverse events could partly explain the decline.

Prevalence of self-reported symptoms related to temporomandibular disorders in an Italian population.

The aim of this study was to estimate the prevalence of temporomandibular disorders (TMD) symptoms in an Italian population sample, focusing on gender and age differences. We selected 2005 individuals by telephone survey and asked them about TMD symptoms like difficulty in jaw movement, jaw pain and joint sounds. Also, tooth-clenching and/or tooth-grinding habits were investigated. Of the study population, 8·1% reported limitations in jaw movements, 5·1% reported jaw pain and 33·3% reported joint sounds. Furthermore, 37·3% reported tooth-clenching/tooth-grinding. Confidence intervals of proportions were calculated. Significant gender differences were found for jaw limitation and pain (chi-square test; P < 0·05). Symptoms reduced with increasing age. The prevalence of TMD symptoms in the Italian population was consistent with data reported from similar studies. Gender and age differences were found for jaw pain and limitation in jaw movements.

Transient RLS during pregnancy is a risk factor for the chronic idiopathic form.

OBJECTIVE: Pregnancy is a risk factor for transient restless legs syndrome, which usually recovers during the postdelivery period. The goal of the present survey is to investigate whether restless legs syndrome during pregnancy represents a risk factor for later development of restless legs syndrome. METHODS: A long-term follow-up study, planned as an extension of a previous survey on restless legs syndrome during pregnancy, was carried out. After a mean interval of 6.5 years, 207 parous women were contacted again to compare the incidence of restless legs syndrome among subjects who never experienced the symptoms with those who reported restless legs syndrome during the previously investigated pregnancy. RESULTS: Seventy-four women who experienced restless legs syndrome during previous pregnancy, and 133 who did not, were included in the study. The incidence of restless legs syndrome was 56% person/year in women who experienced the transient pregnancy restless legs syndrome form, and 12.6% person/year in subjects who did not, with a significant 4-fold increased risk of developing chronic restless legs syndrome in women who presented restless legs in the previous pregnancy. Considering further new pregnancies during the follow-up period, the restless legs symptoms reappeared in 58% of the cases, while they emerged for the first time in only 3% of women who had never experienced restless legs syndrome. CONCLUSIONS: The transient pregnancy restless legs syndrome form is a significant risk factor for the development of a future chronic idiopathic restless legs syndrome form, and for a new transient symptomatology in a future pregnancy.

Myasthenia gravis: a changing pattern of incidence.

The annual incidence of myasthenia gravis (MG) ranges from 3 to 30 per 1,000,000 people. Since the mid-1980s, an increasing incidence has been reported, mainly due to late-onset MG. Whether the increase was due to population aging, improved diagnosis and case collection, or a true excess of incidence cases is still under debate. We used a complete enumeration approach by reviewing all possible sources of case collection in the province of Ferrara, Italy, to estimate the MG incidence and its temporal trend over the study period (1985-2007). The mean annual age-adjusted incidence of MG was 18 per 1,000,000, without any significant temporal trend. The incidence rates in the period 1985-1990 were 14 both for early and late-onset MG. Thereafter, a significant increase in incidence of late-onset MG (p < 0.05), and a decrease in early onset MG were detected (p < 0.01). These findings were related to nonthymoma MG. The median age at onset of the disease steadily increased over time. A changing pattern of MG incidence with an increase in frequency of late-onset and a decrease of early onset MG was found in the last years, giving a significant shift to older age at onset of the disease. Unknown environmental factors may have driven this change in MG epidemiology.

Cerebral blepharoptosis: two new cases, literature review and proposal for diagnostic criteria.

Cerebral ptosis (CP) consists of acute and bilateral drooping eyelids due to a right or left middle cerebral artery territory stroke, without involvement of brain stem, third cranial nerve or oculosympathetic fibres. The pathogenesis of CP is still unknown, but most authors have hypothesised that eyelid control is hemispherically lateralised. Two new cases of CP and a complete literature review are reported in this paper. Because no unanimous criteria for CP diagnosis are currently available, a proposal for new criteria is also included.

Restless legs syndrome and pregnancy.

OBJECTIVE: To perform a large and detailed epidemiologic study on restless legs syndrome (RLS) during pregnancy and the puerperium. METHODS: A structured clinical interview, assessing symptoms since the beginning of pregnancy, was performed to a population of 642 pregnant women at the time of delivery and at follow-up evaluation (1, 3, and 6 months after delivery). Main hematologic tests were also evaluated. A woman was considered affected if she met the International RLS Study Group criteria for RLS diagnosis. RESULTS: Twenty-six percent of women were affected by RLS during their pregnancy. The disease was strongly related to the third trimester of pregnancy and tended to disappear reaching the time of delivery. Affected women presented lower values of hemoglobin and mean corpuscular volume compared with healthy subjects (both groups received the same supplemental iron and folate therapy). CONCLUSIONS: Pregnancy is associated with transient restless legs syndrome.