Our objective was to describe incidence and clinical features of ALS from a prospective population-based study in Emilia Romagna Region (ERR). From 2009 onwards, a prospective registry recorded all incident cases of ALS among residents in the ERR (population, 4.4 million inhabitants), involving 17 neurological departments. For each patient, detailed demographic and clinical information was collected by caring physicians. Results showed that from 1 January 2009 to 31 December 2011, 347 patients received a new diagnosis of ALS with a crude incidence rate of 2.63/100,000/year. There was micro-geographic heterogeneity throughout ERR, with higher incidence rates in the low density population (3.27/100,000) (p < 0.01). ALS patients have been more frequently employed in agriculture than the general ERR population (8.64% vs. 4.6%, p < 0.01). Clinical features were similar to those described in previous population based studies. In conclusion, we report incidence rates similar to those reported by European registries, reflecting good accuracy of our prospective study. We confirmed previous studies reporting higher incidence rates in rural areas and among agricultural workers. Although genetics has been gaining increasing importance in ALS aetiology, some epidemiological data are still unexplained. Identifying geographical areas or populations with high incidence rates can be a starting point for identifying environmental risk factors. Further studies having this specific aim can shed light on these topics.

Very few studies examined trend over time of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and factors influencing it; previous studies, then, included only patients attending tertiary ALS Centres. We studied ALSFRS-R decline, factors influencing this trend and survival in a population-based setting. From 2009 onwards, a prospective registry records all incident ALS cases among residents in Emilia Romagna (population: 4.4 million). For each patient, demographic and clinical details (including ALSFRS-R) are collected by caring physicians at each follow-up. Analysis was performed on 402 incident cases (1279 ALSFRS-R assessments). The average decline of the ALSFRS-R was 0.60 points/month during the first year after diagnosis and 0.34 points/month in the second year. ALSFRS-R decline was heterogeneous among subgroups. Repeated measures mixed model showed that ALSFRS-R score decline was influenced by age at onset (p < 0.01), phenotype (p = 0.01), body mass index (BMI) (p < 0.01), progression rate at diagnosis (ΔFS) (p < 0.01), El Escorial Criteria-Revised (p < 0.01), and FVC% at diagnosis (p < 0.01). Among these factors, at multivariate analysis, only age, site of onset and ΔFS independently influenced survival. In this first population-based study on ALSFRS-R trend, we confirm that ALSFRS-R decline is not homogeneous among ALS patients and during the disease. Factors influencing ALSFRS-R decline may not match with those affecting survival. These disease modifiers should be taken into consideration for trials design and in clinical practice during discussions with patients on prognosis.

Independently from other prognostic factors, patients who received riluzole for a longer period of time survived longer, but further population based studies are needed to verify if long-tem use of riluzole prolongs survival.

Neuroimaging is increasingly used in the study of peripheral nerve diseases, and sometimes may have a pivotal role in the diagnostic process. We report on three patients with atypical chronic inflammatory polyradiculoneuropathy (CIDP) in whom magnetic resonance imaging (MRI) and nerve Ultrasound (US) were crucial for a correct diagnostic work-out. A literature review on MRI and US in acquired demyelinating polyneuropathies is also provided. Awareness of the imaging features of CIDP will assist in confirmation of the diagnosis, institution of the appropriate therapy, and prevention of inadequate or delayed treatment in atypical CIDP.

Our series confirms that radiation therapy has a major role in the management of early glottic cancer, with results comparable to surgical approaches and with better voice preservation.

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Papain-treated microfilariae of Wuchereria bancrofti have been used as antigen for indirect fluorescent assay: 0% of non-endemic sera, 8% of healthy exposed Polynesians, 48% of clinical patients and 96% of microfilaraemic subjects were positive by this test. The geometric mean titres were 22, 41, 147 and 605 respectively. Untreated microfilariae were unsuitable for diagnostic purposes. Dirofilaria immitis adult sections showed low reactivity, giving poor discrimination between non-endemic and microfilaraemic sera. The geometric mean titres were 6 and 61 respectively.