Reproductive life events and Alzheimer's disease in Italian women: a retrospective study.

The aim of our study was to understand the complex relationship between the major reproductive life events in women with Alzheimer's disease. In a retrospective, case-control study, 275 women with Alzheimer's disease (AD) and 276 control patients from a hospital population were enrolled from July 2007 to December 2010. The AD patients presented with later menopause (P < 0.01), lower occurrence of surgical menopause (P < 0.04), reduced hormone-replacement therapy intake (P < 0.0001), and a longer reproductive life span (P < 0.01), compared to controls. No significant differences were found with respect to age at puberty, number of pregnancies, previous abortions, or contraceptive therapy. A higher education level appeared to have a protective role against the risk of developing AD. In women, menopause and hormone-replacement therapy can differentially modulate the clinical manifestations of AD, but these factors do not play a predictive role in its development.

Epidemiology and geographical variation of myasthenia gravis in the province of Pavia, Italy.

BACKGROUND AND AIMS: Previous studies have reported a prevalence estimate of myasthenia gravis (MG) from 7.7 to 11.1 per 100,000 inhabitants in Europe. Moreover, the study of the geographical distribution of MG should be useful to generate specific hypotheses. The aims are to estimate MG prevalence and to investigate its geographical variation in a delimited area in Northern Italy. METHODS: The primary source of data was the MG database of the Neurological Institute of Pavia and all other sources of case collection in and outside the province. We adopted a Bayesian approach to analyze MG geographical variation within the finest geographical grid. RESULTS: We identified 119 live MG prevalent cases resident in the province of Pavia on December 31, 2008. The overall crude prevalence was 24 per 100,000 inhabitants. The Bayesian analysis identified a small cluster of higher MG prevalence in the northern area of the province. CONCLUSIONS: The estimated MG prevalence sets the province of Pavia among the high-risk areas. The identification of high/low MG risk areas deserves further investigation of genetic and environmental factors possibly related to a major risk of the disease in that area.

A multicenter, randomized, placebo-controlled trial of levetiracetam in children and adolescents with newly diagnosed absence epilepsy.

PURPOSE: To evaluate the potential efficacy of levetiracetam as an antiabsence agent in children and adolescents with newly diagnosed childhood or juvenile absence epilepsy. METHODS: Patients were randomized in a 2:1 ratio to receive de novo monotherapy with levetiracetam (up to 30 mg/kg/day) or placebo for 2 weeks under double-blind conditions. Responder status (primary end point) was defined as freedom from clinical seizures on days 13 and 14 and from electroencephalographic (EEG) seizures during a standard EEG recording with hyperventilation and intermittent photic stimulation on day 14. The double-blind phase was followed by an open-label follow-up. KEY FINDINGS: Nine of 38 patients (23.7%) were responders in the levetiracetam group, compared with one of 21 (4.8%) in the placebo group (p = 0.08). Seven of 38 patients (18.4%) were free from clinical and EEG seizures during the last 4 days of the trial (including 24-h EEG monitoring on day 14) compared with none of the patients treated with placebo (p = 0.04). Seventeen patients remained seizure-free on levetiracetam after 1 year follow-up. Of the 41 patients who discontinued levetiracetam due to lack of efficacy (n = 39) or adverse events (n = 2), 34 became seizure-free on other treatments. SIGNIFICANCE: Although superiority to placebo just failed to reach statistical significance for the primary end point, the overall findings are consistent with levetiracetam having modest efficacy against absence seizures. Further controlled trials exploring larger doses and an active comparator are required to determine the role of levetiracetam in the treatment of absence epilepsy.

How parkinsonism influences life: the patients' point of view.

To explore the experience of living with parkinsonism, a survey form was sent to the members of a patients' association; 1,256 forms were analysed. The mean age was 65.75 ± 9.29 years; 64.4% males. A family history was reported by 19.2%. Basic abilities were preserved in 75% of the responders; the ability to do indoor and outdoor activities was preserved in 42 and 28%, respectively. 70% of the responders liked to meet other people and about 50% liked discussing their condition. 80.3% of the responders lived with partner, while 7.8% did not live with family. Of the patients' partners, 38.9% took drugs, and 9.4% themselves needed assistance. Care programmes for parkinsonians should take into account the disease duration, the degree of disability, the presence of caregiver/s, and the level of caregiver burden; but it should also be appreciated that social habits, need of help, and severity of symptoms influence disability.

Risk factors for tumor occurrence in patients with myasthenia gravis.

There is still uncertainty regarding risk factors for cancer occurrence in patients with myasthenia gravis (MG). The objective of this study is to determine the prevalence of extrathymic neoplasms in patients with MG and the factors associated with tumor occurrence. The archives of four tertiary MG centers were consulted and patients were interviewed on the main clinical features of the disease, the presence and type(s) of extrathymic neoplasms and other autoimmune disorders, and their symptomatic and immunosuppressant treatments (with detailed schedules). A retrospective cohort survey was undertaken comparing the demographic and clinical variables of patients with extrathymic neoplasms to those of the remaining MG population. 2,479 patients were traced and interviewed personally or through informants. The sample included 1,490 women and 989 men (mean age 54.7 years). Other autoimmune disorders were present in 216 cases (8.7%). Thymectomy was performed in 1,549 cases (62.5%), thymic hyperplasia and thymoma being the most common findings. Acetylcholinesterase-inhibitors were the most common treatment (93.5%), followed by steroids (64.3%), azathioprine (35.0%), plasma exchange (13.2%), immunoglobulins (7.5%), cyclosporine (5.3%), and cyclophosphamide (5.0%). 221 patients (8.9%) had one or more extrathymic tumors, 168 of which occurred after disease onset. Patients with and without extrathymic neoplasms were followed for 14.8 and 13.9 years, respectively. Variables shown by multivariate analysis to be associated with increased neoplastic risk included older age, thymoma and immunoglobulin use. Extrathymic tumors are a common finding in patients with MG and tend to be associated with age, thymoma, and immunoglobulin use.

Disability and mortality in a cohort of multiple sclerosis patients: a reappraisal.

The aim of the study was to evaluate how the natural history of multiple sclerosis (MS) had changed over a 15-year period. We compared disability and mortality in a cohort of 83 MS patients hospitalised in the Neurological Institute of Pavia, northern Italy, from January 1, 1990, to December 31, 1991, with a similar cohort of 52 patients analysed in the past. After the follow-up, an unfavourable course (death or relevant disability) was observed in 41% of the patients in the new cohort, compared to 63.5% of the patients in the old one. The percentage of deceased patients was reduced from 25 to 6%. The analysis of the pooled data of the two cohorts indicates a recent tendency of firstly hospitalised patients having a shorter disease duration and a lower disability level, which could explain the relevant decrease both in mortality and disability. Finally, our findings confirmed that age at onset, early disability and a short interval between onset and secondary progression increase the risk of an unfavourable course.

Nontraumatic spinal cord injury: an Italian survey.

OBJECTIVE: To describe the demographic and clinical characteristics and the clinical course of patients with nontraumatic spinal cord injury (SCI). DESIGN: A multicenter prospective study. SETTING: Thirty-two rehabilitation centers in several Italian regions. PARTICIPANTS: Patients with nontraumatic SCI (N=330) on first admission (February 1, 1997-January 31, 1999) to rehabilitation centers. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Indicators of rehabilitation process quality were efficient bladder and bowel management. The indica-tor of neurologic recovery was improvement in American Spinal Injury Association Impairment Scale (AIS) level at discharge. The indicator of rehabilitation outcome was return home. Length of stay (LOS) was also measured as an indicator of the care process. RESULTS: Of the 330 patients, 30% exhibited an improvement in AIS classification at discharge, and 73% returned home. In multivariate analysis, a longer LOS was associated with vascular etiology, complete lesions, residence outside the district of the rehabilitation center, and presence of clinical complications. Neurologic improvement was related to incompleteness of the lesion and longer LOS. Factors predicting a return home were married status, incompleteness of lesion, clinical improvement, efficient bowel and bladder management, absence of pressure ulcers, and longer LOS. CONCLUSIONS: Patients showed long waiting times between diagnosis and initiation of rehabilitation, a good chance of improvement on the AIS, and low rates of home returns.

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 (chi(2) = 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.

Reproductive life milestones in women with Parkinson's disease.

Reproductive life milestones were studied in 150 unselected women with idiopathic Parkinson's disease (PD) and in 300 postmenopausal healthy women (PM). Duration of reproductive life was found to be similar in the two groups. The women with PD reported significantly more premenstrual symptoms, fewer deliveries and abortions, and less use of contraception. Time and mode of menopause onset were similar in PD and PM, but the PD women reported significantly more hot flushes, less insomnia, depression, urinary incontinence and dyspareunia, and less recourse to hormone replacement therapy than the PM women. Women diagnosed with PD before the menopause reported more premenstrual symptoms and contraceptive use compared with those with postmenopausal PD onset, as well as a premenstrual worsening of PD symptoms in more than 50% of cases. Our data indicate poor adaptation of neuronal pathways to the hormonal fluctuations of reproductive life in women with PD, supporting the existence of a qualitative relationship between PD and reproductive events.