NEURONS AND ASTROCYTES SHED EXTRACELLULAR VESICLES THAT SEEM TO VEHICLE ANGIOGENIC FACTORS

Tumors preceding ALS onset and ALS: results from a case-control study.

Determinants of depressive symptoms among spouse caregivers of patients with Parkinson's disease

COGNITIVE IMPAIRMENT IN PATIENTS WITH PARKINSON’S DISEASE

Clinical and magnetic resonance imaging predictors of disease progression in multiple sclerosis: a nine-year follow-up study.

Objective: The objective of this paper is to identify clinical or magnetic resonance imaging (MRI) predictors of long-term clinical progression in a large cohort of multiple sclerosis (MS) patients. Methods: A total of 241 relapsing–remitting (RR) MS patients were included in a nine-year follow-up (FU) study. The reference MRIs were acquired at baseline (BL) as part of a multicenter, cross-sectional, clinical-MRI study. Volumetric MRI metrics were measured by a fully automated, operator-independent, multi-parametric segmentation method. Clinical progression was evaluated as defined by: conversion from RR to secondary progressive (SP) disease course; progression of Expanded Disability Status…

Brain cells shed angiogenic and/or pro-apoptotic factors by extracellular vesicles

Correlation between fatigue and brain atrophy and lesion load in multiple sclerosis patients independent of disability.

Abstract Background Fatigue is a major problem in multiple sclerosis (MS), and its association with MRI features is debated. Objective To study the correlation between fatigue and lesion load, white matter (WM), and grey matter (GM), in MS patients independent of disability. Methods We studied 222 relapsing remitting MS patients with low disability (scores ≤ 2 at the Kurtzke Expanded Disability Status Scale). Lesion load, WM and GM were measured by fully automated, operator-independent, multi-parametric segmentation method. T1 and T2 lesion volume were also measured by a semi-automated method. Fatigue was assessed by the Fatigue Severity Scale (FSS), and patients divided in high-fatigue (FS…

A three-cell type in vitro-model of BBB

LEVITIRACETAM IN THE TREATMENT OF CREUTZFIELDT-JACOB DISEASE

Studio delle frequenze alleliche di +1188 A/C di IL12B (p40) in soggetti affetti da Sclerosi Multipla.

Lack of association between estrogen receptor 1 gene polymorphisms and multiple sclerosis in southern Italy in humans

Estrogen receptor 1 gene polymorphisms (ESR1) have been found to be associated with multiple sclerosis (MS) in both Japanese and Finnish populations. We investigated the association between ESR1 polymorphisms (PvuII and XbaI) and MS in a study of 132 MS patients and 129 controls from the same geographic background (southern Italy). Allelic and genotypic frequencies were not different between MS patients and population controls for either the PvuII or XbaI polymorphism. This result suggests that the association between a given disease and a genomic characteristic must be confirmed by separate investigations in different populations. © 2002 Elsevier Science Ireland Ltd. All rights reserved.

Sleep and sleepiness in Parkinson’s disease: result of a “spontaneous” observational study on rotigotine

Clinical features and disease out come in patients with pediatric and young adult onset Multiple Sclerosis

Depression and associated factors among spouse-caregivers of patient with Parkinson’s disease

High Prevalence and Fast Rising Incidence of Multiple Sclerosis in Caltanissetta, Sicily, Southern Italy

<i>Background:</i> Epidemiological studies conducted in Sicily and Sardinia, the two major Mediterranean islands, showed elevated incidence and prevalence of multiple sclerosis (MS)and a recent increase in disease frequency. <i>Objective:</i> To confirm the central highlands of Sicily as areas of increasing MS prevalence and elevated incidence, we performed a follow-up study based on the town of Caltanissetta (Sicily), southern Italy. <i>Methods:</i> We made a formal diagnostic reappraisal of all living patients found in the previous study performed in 1981. All possible information sources were used to search for patients affected by MS diagnosed accordi…

PREGNANCIES MODIFY RELAPSES AND PROGRESSION IN MULTIPLE SCLEROSIS

Height as a potential indicator of early life events predicting Parkinson's disease: a case-control study.

Aim of this study was to investigate the relationship between height in young adult age and Parkinson's disease (PD) risk. We included 266 persons affected by idiopathic PD. Patients were matched by age and sex to 266 controls by a random selection from the municipality of residence. We collected information about height preceding PD from official documents where these characteristics referred to young adult age (nearly 30 years). We compared height in cases and controls by calculating differences in mean distribution and by χ2 analyses. Crude and adjusted odds ratios (OR) and 95% confidence intervals (CI) were calculated by logistic regression models. Mean height was significantly lower in…

BOTH NEURONS AND ASTROCYTES RELEASE EXTRACELLULAR VESICLES

TRANSCRANIAL DIRECT CURRENT STIMULATION FOR TREATMENT OF FREEZING OF GAIT IN PARKINSON’S DISEASE. A CROSS-OVER STUDY

Gender-related effect of clinical and genetic variables on the cognitive impairment in multiple sclerosis

BACKGROUND: Cognitive impairment may occur at any time during the course of multiple sclerosis (MS), and it is often a major cause of disability in patients with the disease. The APOE-epsilon4 allele is the major known genetic risk factor for late onset familial and sporadic Alzheimer's Disease (AD), and it seems to be implicated in cognitive decline in normal elderly persons. OBJECTIVE: To investigate the clinical and genetic variables that can be associated with the cognitive decline in patients with MS. METHODS: Five-hundred and three patients with clinically definite MS underwent a battery of neuropsychological tests and, according to the number of failed tests, were divided into cognit…

EFFETTO DI TERRENI CONDIZIONATI DA NEURONI ED ASTROCITI SULLE PROPRIETÀ BARRIERA DI CELLULE DEI CAPILLARI CEREBRALI.

Neurons and astrocytes shed extracellular membrane vesicles containing angiogenic factors

Binding of flunitrazepam to differentiating neurons cultured in a chemically defined, hormone-supplemented medium

[3H]Flunitrazepam (FNZ) binding to cortical neurons from fetal rat brain was investigated in vitro. The use of a synthetic medium specific for neurons made it possible to plot a developmental curve of3H-FNZ binding in an almost pure neuronal culture. Detectable specific binding was present in vitro at time 0 (that is, the 16th gestational day). A progressive increase of binding, due to an increment in the number of recognition sites, was observed on the subsequent days. The affinity of the specific binding sites to3H-FNZ was enhanced by the addition of exogenous GABA, whereas the density was not affected. © 1990 Plenum Publishing Corporation.

Myocardial 123metaiodobenzylguanidine uptake in genetic Parkinson's disease.

Myocardial (123)Metaiodobenzylguanidine (MIBG) enables the assessment of postganglionic sympathetic cardiac innervation. MIBG uptake is decreased in nearly all patients with Parkinson's disease (PD). Our objective was to evaluate MIBG uptake in patients with genetic PD. We investigated MIBG uptake in 14 patients with PD associated with mutations in different genes (Parkin, DJ-1, PINK], and leucine-rich repeat kinase 2 -LRRK2), in 15 patients with idiopathic PD, and 10 control subjects. The myocardial MIGB uptake was preserved in 3 of the 4 Parkin-associated Parkinsonisms, in I of the 2 patients with DJ-1 mutations, in 1 of the 2 brothers with PINK] mutations, in 3 of the 6 unrelated patient…

Remarkably increasing incidence of Multiple Sclerosis. A follow-up study in calatanissetta, Sicily

The HLA locus and multiple sclerosis in Sicily

In vitro models of blood-brain barrier and application in the study of the multiple sclerosis

Primary Progressive and progressive relapsing multiple sclerosis show different clinical and instrumental findings and disability progression? A comparison study on a Sicilian database

First italian case with a de novo T666M mutation in the CACNA1A gene associated with hemiplegic migraine/progressive cerebellar and episodic ataxia

Epidemiology of multiple sclerosis in Sicily

From epidemiological data obtained over more than 20 years by surveys conducted in different parts of Sicily, it is evident that Sicily is a high-risk area for multiple sclerosis (MS). This is in sharp contrast with the gradient hypothesis. High frequencies have been found in different parts of the island having different geoclimatic features, but at least two cities (Monreale and Enna) had had a prolonged Norman domination. This is in agreement with the hypothesis that MS originated in Northern Europe and spread around the world throughout the raids of the northern peoples. The increase in frequency estimated by follow-up and incidence studies is well established and is only in part linked…

INFLUENCE OF REPETITIVE TRANSCRANIAL MAGNETIC STIMULATION (RTMS) ON ACTION TREMOR IN MULTIPLE SCLEROSIS PATIENTS

Brain atrophy and lesion load in a large population of patients with multiple sclerosis

Objective: To measure white matter (WM) and gray matter (GM) atrophy and lesion load in a large population of patients with multiple sclerosis (MS) using a fully automated, operator-independent, multiparametric segmentation method. Methods: The study population consisted of 597 patients with MS and 104 control subjects. The MRI parameters were abnormal WM fraction (AWM-f), global WM-f (gWM-f), and GM fraction (GM-f). Results: Significant differences between patients with MS and control subjects included higher AWM-f and reduced gWM-f and GM-f. MRI data showed significant differences between patients with relapsing-remitting and secondary progressive forms of MS. Significant correlations bet…

Natalizumab therapy of relapsing-remitting multiple sclerosis: an evaluation of the safety profile and a comparison of efficacy with interferon beta therapy

Levetiracetam in the treatment of vascular chorea: a case report

Impact of sociodemographic characteristics on cognitive performance in an elderly Sicilian population.

<i>Objective:</i> To assess cognitive levels in an elderly Sicilian population and to evaluate the role of education and other sociodemographic characteristics in cognitive performance. <i>Background:</i> The pattern of cognitive performance in the elderly has not been investigated extensively in well-defined Italian populations. This study was conducted as part of a door-to-door survey of common neurologic disorders (the Sicilian Neuro-Epidemiologic Study project). <i>Methods:</i> Thirteen physicians administered an Italian version of the Short Portable Mental Status Questionnaire (SPMSQ) to all subjects aged 50 years or older who, on November 1, 1987, r…

Stroke in the young in sicily: Prevalence and clinical features

Few epidemiological reports focus on the prevalence of stroke in the young population. As part of a neuroepidemiological survey on the total population of three Sicilian municipalities, we assessed the prevalence of stroke in the young, describing the clinical features of each patient. We screened 14,832 subjects younger than 40 years with a door-to-door method and a two-phase study design. Phase 1 consisted of a questionnaire and a brief neurological examination; phase 2 was an extensive evaluation performed by a neurologist. On the prevalence day (November 1, 1987), we ascertained six cases of stroke (prevalence rate, 40.5/100,000). Three were hemorrhagic, one ischemic, and two of uncerta…

Analisi dei Polimorfismi di Citochine Th2 nella Sclerosi Multipla.

Acute myeloid leukemia in Italian patients with multiple sclerosis treated with mitoxantrone

none 25 no Abstract OBJECTIVES: To evaluate the incidence and dose-dependency of mitoxantrone (MTX)-associated acute myelocytic leukemia (AML) in the network of Italian multiple sclerosis (MS) clinics. METHODS: We performed a multicenter retrospective cohort study of patients treated with MTX in MS centers under the Italian national health care system between 1998 and 2008. Demographic, disease, treatment, and follow-up information were collected using hospital records. RESULTS: Data were available for 3,220 patients (63% women) from 40 Italian centers. Follow-up (mean ± SD) was 49 ± 29 months (range 12-140 months). We observed 30 cases of AML (incidence 0.93% [95% confidence interval 0.60%…

Polycistic ovary syndrome and antiepileptic drugs: a cross-sectional study

Clinical features, disease course and prognosis in patients with paediatric and young adult onset multiple sclerosis

Background. Multiple sclerosis (MS) patients develop their first symptoms before adolescence approximately in 5%. Studies comparing clinical and prognostic characteristics in early onset MS, did not show homogeneous results. Objective. Aim of this study was to investigate characteristics of early onset MS patients and compare them with patients who experienced MS onset later. Methods. MS patients diagnosed as affected by MS according to Poser's criteria, were included if they experienced symptoms onset before age 25. They were classified in three groups. Group A: patients who had their onset until they were 16 years old Group B: individuals with onset between 17 and 20 yearsGroup C: patient…

Use of antiepileptic drugs in women with polycystic ovary syndrome

Screening for PINK1 mutations in patients with early- and late-onset Parkinson's Disease

SHEDDING OF ANGIOGENIC FACTORS BY BRAIN CELLS

A case-control study of amyotrophic lateral sclerosis

A retrospective case-control study was conducted using 46 patients affected by amyotrophic lateral sclerosis and 92 closely matched healthy controls. Cases were ascertained through typical clinical and instrumental findings. Putative risk factors (bone fractures or major trauma, exposure to domestic animals, surgical operations, disease among first degree relatives and others) were investigated anamnestically using a standard questionnaire. Using Mantel-Haenzsel estimates of the odds ratio, no association was found between amyotrophic lateral sclerosis and the investigated variables.

Teaching Research Methods to Young Neurologists: The Erice International Courses.

No

Levetiracetam, a new option Treatment for Creutzfeldt-Jacob Disease.

Clinical features and lifestyle of patients with amyotrophic lateral sclerosis in Campania: brief overview of an Italian database

Background. Physical activity and occupational exposures appeared to play a relevant role in pathogenesis of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease of unknown origin. Materials and methods. We aimed to make an overview of the clinical characteristics and life - style (occupation and sport) of a population of 395 patients with ALS from campania, in southern Italy. Results. ALS onset resulted anticipated of about 11 years in industry workers, whilst the more frequent site of onset among farmers was upper limbs. compared to non-athletes, athletes, particu- larly soccer players, showed a 7 years anticipation of ALS onset, with higher mortality after 5 years. Discussion…

Population-based case-control study of essential tremor.

Current epidemiologic data on the association between environmental factors and essential tremor (ET) are scanty. In a population-based case-control study we investigated the relationship between some putative risk factors and ET. In the present study, we included all subjects identified during a door-to-door prevalence survey in a Sicilian community, affected by ET, and alive on 1 November 1987 (n=31). Of the 31 prevalent cases of ET, we were able to contact 28 subjects. Twenty-eight controls were randomly selected from the general population and matched to each case for age and sex. Exposure variables were investigated by interviewing the patients and their controls or close relatives, us…

A SPG4 GENE MUTATION CAUSES AUTOSOMAL DOMINANT SPASTIC PARAPLEGIA IN AN ITALIAN FAMILY

Lifestyle factors and multiple sclerosis: a case control study

A population-based survey of cognitive performance in a Sicilian elderly community.

<i>Background:</i> The global burden of neurodegenerative diseases is increasing. Previous studies reported differences related to age, gender and socioeconomic status. We calculated the cognitive performances of a Sicilian population aged 65 years or older, by means of a door-to-door population-based survey in Bagheria city, Sicily. <i>Methods:</i> A door-to-door survey was carried out in the city of Bagheria, Sicily (prevalence day September 30, 2006). A cohort of 2,200 persons was randomly stratified, obtaining a 25% sample of the whole population. Cognitive function was assessed by Mini Mental State Examination (MMSE). Percentile distributions by age, gender, edu…

Stroke incidence and survival in three Sicilian municipalities

We investigated the incidence of first-ever stroke in three Sicilian municipalities over the years 1984–1987. Case ascertainment involved two approaches: a doorto-door two-phase prevalence survey and a study of death certificates. Only first-ever strokes occurring within the study time interval were included, and diagnoses were based on specified criteria. We found 138 subjects who had experienced a first stroke over 73 488 person-years; the average incidence rate (new cases per 1000 population per year) was 1.9 overall, 1.7 in men, and 2.1 in women. Incidence increased steeply with age and was similar in men and women. Incidence was similar in the three study municipalities. The overall ca…

MRI abnormalities following repeated and incoming seizures

Neuroimaging, an important diagnostic tool frequently used in the evaluation of patients with epilepsy, has mainly the aim to identify structural abnormalities needing a treatment and to contribute to the definition of the aetiology. Brain magnetic resonance imaging (MRI) in epilepsy is more sensitive than computerized tomography (CT) scan for detecting abnormalities. Status epilepticus (SE) and repeated incoming seizures may determine extensive and transient or long lasting pronounced MRI changes. We describe a case of a 41-year-old woman with a history of brain neoplasm, whose contrast-enhanced MRI images following repeated and incoming seizures were characterized either by reversible and…

Brain atrophy evolution and lesion load accrual in multiple sclerosis: a 2-year follow-up study

Background To investigate in a large cohort of patients with multiple sclerosis (MS), lesion load and atrophy evolution, and the relationship between clinical and magnetic resonance imaging (MRI) correlates of disease progression. Methods Two hundred and sixty-seven patients with MS were studied at baseline and two years later using the same MRI protocol. Abnormal white matter fraction, normal appearing white matter fraction, global white matter fraction, gray matter fraction and whole brain fraction, T2-hyperintense, and T1-hypointense lesions were measured at both time points. Results The majority of patients were clinically stable, whereas MRI-derived brain tissue fractions were signifi…

Incidence and lifetime prevalence of Bell's palsy in two Sicilian municipalities

Introduction - In a door-to-door two-phase survey of common neurologic diseases conducted in two Sicilian municipalities, we investigated the incidence and the lifetime prevalence of Bell's palsy (BP). Material & methods - During phase 1, we administered a screening instrument for facial palsy to 11,901 adult persons. During phase 2, study neurologists using specified diagnostic criteria evaluated those subjects who screened positive. Results - We found 73 subjects who had experienced BP during their life. The lifetime prevalence as of November 1, 1987, was 642.8 cases per 100,000 population age 15 years and above. The prevalence increased with age and was similar in men and women. Eighteen…

Dropped head as an unusual presenting sign of myasthenia gravis.

Prominent or isolated weakness of cervical extensor muscles is a relatively rare clinical sign. Commonly, this is known as "dropped-head syndrome". This abnormal flexion of the head may occur in a variety of neuromuscular diseases and in a few non-neurological disorders as well. The case we describe concerns a 61-year-old woman with dropped-head syndrome as the unique complaint of myasthenia gravis.

Age at onset of parkinson's disease in women is associated with age at menopause.

Prevalence of Parkinson's disease and other types of parkinsonism in the Aeolian Archipelago, Sicily.

Abstract Objective To estimate prevalence of Parkinson's disease (PD) and other types of parkinsonism in the Aeolian Archipelago, Sicily. Methods We studied the frequency of PD and other types of parkinsonism in the Aeolian Archipelago (population 13,431). All potential cases were identified from available medical information sources. To ensure the completeness of the case-findings, a screening questionnaire was also mailed to residents aged 40 years and over. Subjects were considered prevalent if they fulfilled the SNES diagnostic criteria for PD, on prevalence day (January 1, 2001). Results We identified 17 patients with parkinsonism from medical sources, and 4 from mail-survey. Prevalenc…

Sleep disturbances and dysautonomic dysfunction are associated in patients with Parkinson’s disease

Cholesterol levels and risk of hemorrhagic transformation after acute ischemic stroke.

<i>Background:</i> The association between cholesterol levels and hemorrhagic transformation (HT) is still controversial. Studies investigating this issue are influenced by treatments as some are characterized by a higher risk of HT. The aim of our study was to evaluate, in a hospital-based series of patients not treated with thrombolysis, the relationship between cholesterol levels and HT. <i>Methods:</i> We retrospectively collected information about total cholesterol (TC) and low-density lipoprotein cholesterol (LDLC) levels at admission in a consecutive series of 240 patients with anterior ischemic stroke (IS). The TC and LDLC levels were arranged in 3 groups acc…

Insidious onset of Pisa syndrome after rasagiline therapy in a patient with Parkinson’s disease

N.A.

Rat CNS cell culture. Enhancement of neuronal survival and delay of glial proliferation by serum from patients with multiple sclerosis. A morphological study.

The addition of serum from multiple sclerosis (MS) patients to the culture medium of dissociated cells from cerebral hemispheres of rat embryos caused a delay in glial proliferation and an enhancement of neuronal survival. Sera from normal individuals and patients with other neurological diseases failed to show this effect. These morphological observations are interpreted as the outcome of inhibition of in vitro gliogenesis.

Inhibition of glial proliferation in vitro by serum from patients with multiple sclerosis

Primary cell cultures from fetal rat CNS have been employed to evaluate the effects caused by the addition of serum from patients affected by multiple sclerosis (MS). MS-serum supplemented media caused a decrease in [3H]-thymidine incorporation into the cultures, thus indicating an inhibitory effect on proliferating glial cells. Sera from patients in remission stage of the disease showed an inhibitory effect not significatively lower than those from patients in acute stage. These results suggest that glial cells may be a target of circulating factors present in MS.

Hypoxanthine and purine compounds in plasma from patients with multiple sclerosis and psoriasis

Lesion load may predict long-term cognitive dysfunction in multiple sclerosis patients

Background: Magnetic Resonance Imaging (MRI) techniques provided evidences into the understanding of cognitive impairment (CIm) in Multiple Sclerosis (MS). Objectives: To investigate the role of white matter (WM) and gray matter (GM) in predicting long-term CIm in a cohort of MS patients. Methods: 303 out of 597 patients participating in a previous multicenter clinical-MRI study were enrolled (49.4% were lost at follow-up). The following MRI parameters, expressed as fraction (f) of intracranial volume, were evaluated: cerebrospinal fluid (CSF-f), WM-f, GM-f and abnormal WM (AWM-f), a measure of lesion load. Nine years later, cognitive status was assessed in 241 patients using the Symbol Dig…

A case-control study on cigarette, alcohol, and coffee consumption preceding Parkinson's disease

<i>Objective:</i> To investigate the association between cigarette smoking, alcohol drinking, coffee consumption and Parkinson’s disease (PD). <i>Methods:</i> We selected subjects affected by idiopathic PD, with a Mini-Mental State Examination of ≧24, and controls matched 1 to 1 with cases by age (± 2 years) and sex. Controls were randomly selected from the resident list of the same municipality of residence of the cases. We assessed cigarette smoking, alcohol drinking, and coffee consumption preceding the onset of PD or the corresponding time for controls using a structured questionnaire, which also evaluated the duration and dose of exposure. Using conditional logi…

Transcranial direct current stimulation for treatment of freezing of gait: A cross-over study

Background and objective Progression of Parkinson's disease (PD) is frequently characterized by the occurrence of freezing of gait (FOG) representing a disabling motor complication. We aim to investigate safety and efficacy of transcranial direct current stimulation of the primary motor cortex of PD patients with FOG. Methods In this cross-over, double-blind, sham-controlled study, 10 PD patients with FOG persisting in “on” state underwent anodal and sham direct current stimulation for 5 consecutive days. Clinical assessment over a 1-month period was performed. Results A significant improvement of gait, as assessed by the Stand Walk Sit test, with reduction in number and duration of FOG epi…

Diabetic and non-diabetic subjects with ischemic stroke: Differences, subtype distribution and outcome

Abstract Background and aim Diabetes mellitus increases the risk of stroke, and pathophysiological changes of diabetic cerebral vessels may differ in comparison with non-diabetic ones; nonetheless, the clinical and prognostic profile of stroke in diabetic patients is not yet fully understood. On this basis, the aim of our study was to evaluate cerebrovascular risk factor prevalence in diabetic stroke patients in comparison with non-diabetics, to analyze whether diabetics have a different prevalence of stroke subtypes as classified by the TOAST classification, and determine whether diabetics and non-diabetics have a different prognosis. Methods and results We enrolled 102 diabetics and 204 n…

A post-marketing comparison study on disability modification in progressive multiple sclerosis after interferon beta-1b or mitoxantrone therapy

Multiple sclerosis in southern Europe: Monreale City, Italy. A twenty-year follow-up incidence and prevalence study.

BACKGROUND: Several follow-up studies showed increasing prevalence and incidence rates for multiple sclerosis (MS). OBJECTIVE:To ascertain, throughout a follow-up study, the incidence and prevalence of MS in the city of Monreale, Sicily, southern Italy. METHODS:We calculated crude and age- and sex-specific prevalence rates on December 31, 2000, and determined incidence rates for the period January 1, 1992 to December 31, 2000. RESULTS:The prevalence of MS was 71.2 per 100,000 population (48,5/100,000 in men; 93,0/100,000 in women). The incidence rate of MS for the period 1992-2000 was 4.0/100,000 per year. CONCLUSION:This study showed a nonsignificant increase in MS incidence rates in Monre…

Neuronal cell cultures: A tool for investigations in developmental neurobiology

The aim of this review is to describe environmental requirements for survival of neuronal cells in culture, and secondly to survey the complex interplay between hormones, neurotrophic factors, transport- and extracellular matrix- proteins, which characterize the developmental program of differentiating neurons. An overall reconsideration of the literature in this vast field is above the limits of the present paper; since progress and refinement in the techniques of neuronal cell cultures have paralleled the advancement in Developmental Neurobiology, we will run instead through the main steps which form the conceptual framework of neuronal cell cultures. © 1992 Plenum Publishing Corporation.

Frequency and determinants for hemorragic transformation of cerebral infarction

Inferior altitudinal hemianopia associated with a tumor in the posterior fossa: report of a case

Altitudinal hemianopias may be related to chiasmatic involvement. A case in which horizontal inferior hemianopia is associated with a tumor in the posterior fossa producing a triventricular hydrocephalus is described. The visual field defect is interpreted as a consequence of the pressure by a dilated third ventricle upon the chiasmatic area.

Corticobasal syndrome-like variant of Creutzfeldt–Jakob disease: clinical description of two cases

A whole genome screen for linkage disequilibrium in multiple sclerosis performed in a continental Italian population

We have systematically screened the genome for evidence of linkage disequilibrium (LD) with multiple sclerosis (MS) by typing 6000 microsatellite markers in case-control and family based (AFBAC) cohorts from the Italian population. DNA pooling was used to reduce the genotyping effort involved. Four DNA pools were considered: cases (224 Italian MS patients), controls (231 healthy Italians), index (185 index cases from trio families) and parents (the 370 parents of the patient included in the Index pool), respectively. After refining analysis of the most promising 14 markers to emerge from this screening process, only marker D2S367 retained evidence for association. © 2003 Elsevier B.V. All r…

PINK1 MUTATIONS AND THE RISK OF PARKINSON'S DISEASE IN FAMILY MEMBERS OF SOUTHERN ITALY

Answer to: The possible risk of cancer in multiple sclerosis patients: a controversial issue

Triiodothyronine-Induced Shortening of Chromatin Repeat Length in Neurons Cultured in a Chemically Denned Medium

Abstract: At the time of terminal differentiation, mammalian cortical neurons undergo a dramatic change in the structural organization of their chromatin: the nucleosomal repeat length shortens from ∼200 base pairs in fetuses to a value of 165 base pairs after birth. These events occur several days after the end of neuronal proliferation. Previously, we reported that rat cortical neurons cultured in a very selective synthetic medium were not yet programmed to these events at the end of mitotic cycles. Herein, we report that addition of triiodothyronine to neuronal cultures induces a shortening of the chromatin repeat length comparable to the natural one. Copyright © 1987, Wiley Blackwell. A…

Cognitive dysfunction in patients with relapsing-remitting multiple sclerosis

Cognitive dysfunction is considered one of the clinical markers of multiple sclerosis (MS). However, in the literature there are inconsistent reports on the prevalence of cognitive dysfunction, and separate data for the relapsing-remitting (RR) type of the disease are not always presented. In this study, we submitted 461 RRMS patients to a battery of neuropsychological tests to investigate their impairment in various cognitive domains. As a consequence of the exclusion criteria, the sample is not fully representative of the entire population of RRMS patients. In this selected sample, when only the eight scores of a core battery (Mental Deterioration Battery) were considered (with respectiv…

Cellular mechanism of action of thyroid hormones.

Abstract It has emerged in the last decade that the molecular mechanism of action of thyroid hormones resembles that of steroids; thyroid hormones indeed exert their effects mainly by directly regulating gene expression, on association with specific chromatin-bound receptors. Of the two thyroid hormones, thyroxine (T4) appears to be a sort of prohormone, whereas triiodothyronine (T3) seems to be the active form; in this respect, T4-deiodination, which occurs at the level of the target tissues, may be crucial in the local homeostasis of T3. Moreover, many cellular compartments, other than the nucleus, can bind thyroid hormone, and at least some of these further sites might play some role in …

Neutralizing antibodies in multiple sclerosis patients treated with 375 microg interferon-beta-1b.

Prevalence of Parkinson's disease and other types of parkinsonism: A door-to-door survey in three Sicilian municipalities

We investigated the prevalence of Parkinson's disease and other types of parkinsonism in a Sicilian population using a door-to-door two-phase approach. This design called for the administration of a brief screening instrument to all subjects who, on November 1, 1987, were residents of Terrasini (Palermo Province), Santa Teresa di Riva (Messina Province), and Riposto (Catania Province), Sicily (N = 24,496). Study neurologists using specified diagnostic criteria extensively investigated those subjects who screened positive. We found 63 subjects affected by Parkinson's disease, 21 with secondary parkinsonism, and seven with unspecified parkinsonism. The crude prevalence per 100,000 population …

Oligodendroglioma cells shed microvesicles which contain TRAIL as well as molecular chaperones and induce cell death in astrocytes.

Microvesicles (MVs) shed from G26/24 oligodendroglioma cells were previously reported to cause a reproducible, dose-dependent, inhibitory effect on neurite outgrowth, and eventually neuronal apoptosis, when added to primary cultures of rat cortical neurons. These effects were reduced but not abolished by functional monoclonal antibodies against Fas-L. In order to investigate whether MVs contain other factors able to induce cell death, we tested them for TRAIL and found clear evidence of its presence in the vesicles. This finding suggests the possibility that Fas-L and TRAIL cooperate in inducing brain cell death. Aimed at understanding the route through which the vesicles deliver their mess…

Tumor diagnosis after PD onset: a case-control study

VALIDATING SCREENING INSTRUMENTS FOR NEUROEPIDEMIOLOGIC SURVEYS - EXPERIENCE IN SICILY

In a hospital setting in Sicily, we assessed a screening instrument developed for a prevalence survey of parkinsonism, peripheral neuropathies, stroke, and epilepsy. The subjects consisted of (1) hospital patients with any of the above-mentioned diseases, to investigate sensitivity; and (2) hospital visitors free of all these diseases, to investigate specificity. The standard for comparison was a clinical evaluation based on specified criteria. Trained interviewers administered the screening instrument, asking subjects to answer symptom questions and to perform simple physical tasks. For the questions and tasks together, the sensitivity estimates were 100% for parkinsonism (n = 21), 96% for…

Effect of the serum from multiple sclerosis patients on an in vitro model of blood-brain barrier.

Multiple sclerosis (MS) is characterized by focal inflammatory demyelination, largely due to autoimmune responses against different components of the myelin sheet. It is also generally accepted that the pathogenesis of MS consists of inflammatory and neurodegenerative phases, where demyelination should produce partially reversible clinical deficits that can remit, due to limited remyelination, while axonal degeneration produces permanent non-remitting clinical damage. It is also assumed that nervous system inflammation is initiated by autoreactive, myelin-specific T cells that permeate the blood-brain barrier and trigger a series of events leading to tissue destruction. In addition to antib…

Multiple sclerosis survival: a population-based study in Sicily.

Background and purpose: There are few population-based surveys on multiple sclerosis (MS) survival. To investigate MS survival in MS patients recruited during surveys conducted in Sicily. Methods: Multiple sclerosis patients identified during previous surveys were randomly matched to two referent subjects by residence, year of birth, and gender. Living status was obtained by municipality records (end of follow-up June, 30th 2007) and, for the deceased, date and causes of death were searched. Kaplan-Meier plots were used to calculate differences in mortality between MS patients and referent subjects. MS risks for mortality with 95% confidence intervals (CI) were also calculated. Results: We …

FREQUENCY AND DETETRIMINANTS OF HEMORRHAGIC TRASFORMATION OF CEREBRAL INFARCTION

A CASE-CONTROL STUDY ON THE ASSOCIATION BETWEEN DIABETES AND PD

Tumor diagnosis preceding Alzheimer’s disease: a case-control study

Frequency and determinants for hemorrhagic transformation of posterior cerebral stroke : Posterior ischemic stroke and hemorrhagic transformation.

BACKGROUND: hemorrhagic transformation is a threatening ischemic stroke complication. Frequency of hemorrhagic transformation differs greatly among studies, and its risk factors have been usually studied in patients with anterior ischemic stroke who received thrombolytic therapy. We evaluated, in a hospital-based series of patients with posterior ischemic stroke not treated with thrombolysis, frequency and risk factors of hemorrhagic transformation. Patients with posterior circulation stroke were seen in our Department during the period January 2004 to December 2009. Demographic and clinical information were collected. We estimated risk for spontaneous hemorrhagic transformation by means of…

MRI activity and neutralising antibody as predictors of response to interferon beta treatment in multiple sclerosis

Objective: To prospectively validate MRI activity and neutralising anti-interferon antibody (NAb) during the first 6 months of interferon β treatment as response indicators in multiple sclerosis (MS). Methods: Patients with relapsing–remitting MS were followed during the first 2 years of treatment. Neurological assessments were performed every 3 months or when a relapse was suspected. MRI scans performed at baseline and at 3, 4, 5 and 6 months after the start of treatment were assessed centrally for disease activity: new T2 or gadolinium enhancing T1 lesions. NAb were assessed using the MxA protein assay; positivity was defined as two consecutive titres ⩾20 NU/ml. We evaluated the predictiv…

Prevalence of lumbosacral radiculopathy in two Sicilian municipalities

Objectives - As part of a door-to-door survey of neurologic diseases, we investigated the prevalence of lumbosacral radiculopathy (LR) in two Sicilian municipalities (N = 14,540, as of November 1, 1987). Material & methods - During phase 1, we administered a brief screening instrument to subjects in the study population. In phase 2, study neurologists using specified diagnostic criteria investigated those subjects who screened positive. Our case finding was restricted to subjects who manifested symptoms of LR in the six months preceding the prevalence day. Results - We found 143 subjects affected by LR (112 definite, 31 possible) yielding a crude prevalence of 9.8 cases per 1,000 population…

DOES MOTOR CORTEX HIGH FREQUENCY RTMS MODULATE INTRACORTICALINHIBITION IN PATIENTS WITH PARKINSON'S DISEASE ?

Oligodendroglioma cells synthesize the differentiation-specific linker histone H1˚ and release it into the extracellular environment through shed vesicles

Chromatin remodelling can be involved in some of the epigenetic modifications found in tumor cells. One of the mechanisms at the basis of chromatin dynamics is likely to be synthesis and incorporation of replacement histone variants, such as the H1° linker histone. Regulation of the expression of this protein can thus be critical in tumorigenesis. In developing brain, H1° expression is mainly regulated at the post-transcriptional level and RNA-binding proteins (RBPs) are involved. In the past, attention mainly focused on the whole brain or isolated neurons and little information is available on H1° expression in other brain cells. Even less is known relating to tumor glial cells. In this st…

ANODAL TRANSCRANIAL DIRECT CURRENT STIMULATION (TDCS) APPLIED TO THE MOTOR CORTEX AMELIORATES FREEZING OF GAIT IN PATIENTS AFFECTED BY PARKINSON’S DISEASE

Background: Progression of Parkinson’s disease (PD) is frequently characterized by the occurrence of freezing of gait (FOG), which generally does not improve with dopaminergic therapy and with current available surgical therapies. Recent evidences show that motor symptoms may be ameliorated by means of non-invasive brain stimulation techiniques in PD (transcranial current direct stimulation, TDCS; repetitive transcranial magnetic stimulation, RTMS). Objective: To investigate the efficacy of anodal TDCS in the treatment of FOG in PD. Excitatory anodal tDCS was applied unilaterally to the motor and premotor cortices controlateral to the most affected leg. Methods: randomized, double-blind, sh…

Atherothrombotic Stroke: Plasmatic Modification Of Homocysteine and Other Thiols

Qual e' la percentuale dei pazienti con Malattia di Parkinson eleggibili per la stimolazione cerebrale profonda del subtalamo? Risultati di un questionario

Grey matter and white matter atrophy in a large population of patients with multiple sclerosis: a 2 years follow-up study based on a fully automated MR segmentation method.

Risk of Parkinson disease in women: Effect of reproductive characteristics

Objective: To investigate the association between some fertile life characteristics and Parkinson disease (PD) in women. Methods: Women affected by PD and control subjects were matched one to one by age (±2 years). One hundred thirty-one women with idiopathic PD and 131 matched control subjects were interviewed. Controls were randomly selected from the resident list of the same municipality of residence of cases. All subjects had a Mini-Mental State Examination score of ≥24. Cumulative length of pregnancies, age at menarche, age and type of menopause, and estrogen use before and after menopause were investigated in cases and controls through a structured questionnaire. Models of matched pai…

Progressive necrotic encephalopathy following tacrolimus therapy for liver transplantation.

Previously described neurologic damage induced by immunosuppressive treatments includes transient or reversible central nervous system involvement. We describe a 57-year-old man who underwent liver transplantation and was started on immunosuppressive therapy with tacrolimus (FK506). Six months later, he started complaining of a progressive motor and sensory impairment of the left side, together with cognitive impairment. Brain MRI showed an enlarging lesion of the white matter with peripheral contrast enhancement. PET study indicated severe hypometabolism in the right hemisphere and spectroscopic MRI showed a peak of choline and relative reduction of other metabolites. Findings of CSF exami…

Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases

Following an outbreak of bovine spongiform encephalopathy (BSE) in dairy cows in the United Kingdom (UK), 153 definite and probable human cases of new variant Creutzfeldt-Jakob disease (nvCJD) have been reported, almost exclusively in the UK. Although exposure to the BSE agent is the most plausible interpretation for the occurrence of nvCJD, the causal link between the BSE prion and nvCJD is still debated. This review discusses the pros and cons of nvCJD as a separate nosographic entity, the scientific basis for a correlation between BSE and nvCJD, the validity of the current diagnostic criteria for CJD and nvCJD, the contribution of epidemiology to the detection of a causal relation betwee…

In patient’s with Parkinson disease, autonomic symptoms are frequent and associated with other non-motor symptoms

Background: Autonomic symptoms and sleep disorders are common non-motor symptoms of Parkinson disease (PD), which are correlated with poor quality of life for patients. Purpose: To assess the frequency of autonomic symptoms in a consecutive series of PD patients and to correlate them with other motor and non-motor symptoms. Methods: All consecutive non-demented PD patients who underwent an extensive evaluation including Hoehn and Yahr staging, Unified Parkinson’s Disease Rating Scale, Beck’s Depression Inventory, Neuropsychiatric Inventory, PDQ-39 Scale, the Parkinson’s diseases Sleep Scale, the Epworth Sleepiness Scale and SCOPA-AUT scale were enrolled. Comorbidity has been also considered…

Height as a predictor of Parkinson’s disease: a case-control study.

IMMUNOSUPPRESSANT TREATMENT AND CANCER RISK IN A COHORT OF PATIENTS AFFECTED BY MULTIPLE SCLEROSIS

Attrition and Use of Proxy Respondents and Auxiliary Information in the Sicilian Neuroepidemiologic Study

Two-phase prevalence surveys with screening (phase 1) and examination (phase 2) are useful for some chronic diseases. Attrition, which may bias estimates, occurs in either phase because some eligible subjects die before contact, some refuse to cooperate, some are incapacitated, and some are unreachable. This investigation relates to a survey of neurologic diseases conducted in three municipalities of Sicily (prevalence date, November 1, 1987) and considers the attrition experienced and the use of proxy respondents in phase 1 and auxiliary information in phase 2 to offset, in part, this attrition. Regarding case finding, the salvage effort was more productive for decreased and incapacitated …

Natalizumab: a country-based surveillance program

Natalizumab is a humanized monoclonal antibody with a selective adhesion-molecule inhibitor effect, and a demonstrated efficacy in decreasing the frequency of relapses and progression of disability in relapsing-remitting multiple sclerosis (RR MS). After the approval of FDA and EMEA in MS cases unresponsive to immunomodulating therapy or in severe MS patients also not previously treated with interferons, and considering the concern on the possible side effects, an accurate program of surveillance was organized in our country by a combined effort of AIFA, Cineca, Department of Pharmacology of University of Bologna, and a group of neurologists appointed by the National Society of Neurology (S…

Blood lipids, homocysteine, stress factors, and vitamins in clinically stable multiple sclerosis patients

Abstract Multiple Sclerosis (MS) patients present a decrease of antioxidants and neuroprotective and immunoregulatory vitamins and an increase of total homocysteine (tHcy), cholesterol (CHL), HDL-cholesterol, and of cellular stress markers, variably associated with the different phases of the disease. We compared the blood levels of uric acid, folic acid, vitamins B12, A, and E, tHcy, CHL, HDL-cholesterol, and triglycerides in forty MS patients during a phase of clinical inactivity with those of eighty healthy controls, matched for age and sex. We found higher levels of tHcy (p = 0.032) and of HDL-cholesterol (p = 0.001) and lower levels of vitamin E (p = 0.001) and the ratio vitamin E/CHL …

Reversible Pisa Syndrome in a patient with Parkinson's disease on rasagiline therapy

Long-term survival of Parkinson’s disease: a population-based case-control study.

In a set of a population-based study, long-term survival of 59 prevalent PD patients was compared with that of individuals free of neurological diseases matched 1:2 by sex and age of enrolment. PD individuals, compared with reference subjects, showed a two-fold increased risk of death (OR 2.1; 95 % CI 1.4, 3.1). Among causes of death, pneumonia and cachexia were significantly more frequent among PD patients than among individuals free of neurological diseases. We confirmed in a long-term follow-up study an increased mortality among PD individuals compared with that of the general population.

Idiopathic Parkinson’s Disease and Associated Conditions: Results From a Case-Control Study

LATE MORTALITY OF HEMORRHAGIC TRANSFORMATION OF ISCHEMIC STROKE

Introduction. Hemorrhagic transformation (HT), a complication of ischemic stroke (IS) is supposed to influence patient’s prognosis. Aim of our study was to evaluate, in a hospital-based series of patients not treated with thrombolysis, the relationship between HT and late mortality. Methods. Mortality of individuals with spontaneous HT was compared to that of individuals without. Medical records of patients diagnosed with anterior IS during the period 2004-2006 were reviewed. Living status was obtained from the public record office of the municipality of Palermo or indirectly by telephone interview. Outcome measure was 90 days survival after IS onset. Kaplan-Meier estimates were used to con…

CD45 and multiple sclerosis: the exon 4 C77G polymorphism (additional studies and meta-analysis) and new markers

We re-evaluated the association with multiple sclerosis (MS) of the C77G splicing regulatory variation in the CD45 gene and screened for new mutations the three alternatively spliced exons (#4, 5 and 6). No association with C77G was detected in two groups of patients (total=448) and controls (total=559) from Northern and Southern Italy. When excluding the first published study indicating a positive association, a meta-analysis of the five further studies conducted to date (including the present one) led to a non-significant combined odds ratio (OR) of 1.11. None of the four newly identified nucleotide substitutions, namely C77T (Pro59Pro) in exon 4, G69C (Asp121His) in exon 5, T127A (Ile187…

Modulation of action tremor by repetitive transcranial magnetic stimulation in multiple sclerosis patients

Background: Patients affected by Multiple Sclerosis (MS) can show a tremor of their upper limbs, mostly during the performing of finalised action (action tremor) or at the maintaining of a position against gravity (postural tremor), as key clinical feature of their disease. Objectives: In order to reduce the tremor, patients underwent to repetitive transcranial magnetic stimulation (rTMS) of the primary motor cortex of left hemisphere. Methods: Eight patients affected by a middle form of MS were enrolled into the study. A standard neurological examination was carried out and the individual degree of disability was established employing the Expanded Disability Status Score (EDSS). At EDSS pa…

Gender Effect on Parkinson’s disease

Chronic acquired hepatocerebral degeneration or Parkinson Disease? A case report

Drug-induced parkinsonism: Prevalence, clinical features and follow-up study in three Sicilian communities [1]

Accuracy of death certificates for motor neuron disease and multiple sclerosis in the province of Palermo in southern Italy

Mortality studies based on death certificates (DCs) are relatively inexpensive and easy to conduct. Therefore, they are frequently used to evaluate variations of geographical and temporal patterns, particularly in uncommon diseases. Recent surveys of motor neuron disease (MND) and multiple sclerosis (MS) based on official mortality statistics in Italy showed a decreasing trend of mortality from northern to southern Italy. To evaluate if DCs are homogeneously recorded in Italy and whether or not they can be considered a good instrument for mortality studies, we assessed the accuracy of DCs for MND and MS in the province of Palermo, Italy, and compared our results with those reported in other…

Determinants of depressive symptoms among spouse caregivers of patients with Parkinson’s disease

Refsum disease. Clinical and morphological report on a case

An atypical case of Refsum disease is reported together with the peripheral nerve morphological data. The body fluids must be assayed for phytanic acid whenever an atypical chronic peripheral neuropathy is observed.

Linkage disequilibrium screening for multiple sclerosis implicates JAG1 and POU2AF1 as susceptibility genes in Europeans.

By combining all the data available from the Genetic Analysis of Multiple sclerosis in EuropeanS (GAMES) project, we have been able to identify 17 microsatellite markers showing consistent evidence for apparent association. As might be expected five of these markers map within the Major Histocompatibility Complex (MHC) and are in LD with HLA-DRB1. Individual genotyping of the 12 non-MHC markers confirmed association for three of them — D11S1986, D19S552 and D20S894. Association mapping across the candidate genes implicated by these markers in 937 UK trio families revealed modestly associated haplotypes in JAG1 (p=0.019) on chromosome 20p12.2 and POU2AF1 (p=0.003) on chromosome 11q23.1.

In vitro model of blood-brain barrier and application on the study of the multiple sclerosis.

Tumor diagnosis preceding Parkinson's disease: A case-control study

Lower cancer risk in Parkinson’s disease (PD) patients compared to the general population has been reported. However, most of the studies were based on death certificates. We designed a case– control study to estimate the association of tumor preceding PD onset and PD. PD patients were matched by age and gender to PD-free individuals, randomly selected from the municipalities of residence of cases. Occurrence of tumors preceding PD onset was assessed through a structured questionnaire. Neoplasms were categorized as benign, malignant, or of uncertain classification, and endocrine-related or not. Odds ratios (OR) were calculated using conditional logistic regression and adjusted for tumor cat…

Second asymptomatic carotid surgery trial (ACST-2): a randomised comparison of carotid artery stenting versus carotid endarterectomy.

Summary Background Among asymptomatic patients with severe carotid artery stenosis but no recent stroke or transient cerebral ischaemia, either carotid artery stenting (CAS) or carotid endarterectomy (CEA) can restore patency and reduce long-term stroke risks. However, from recent national registry data, each option causes about 1% procedural risk of disabling stroke or death. Comparison of their long-term protective effects requires large-scale randomised evidence. Methods ACST-2 is an international multicentre randomised trial of CAS versus CEA among asymptomatic patients with severe stenosis thought to require intervention, interpreted with all other relevant trials. Patients were eligib…

Metanalisi degli studi su fattori di rischio ambientali nella sclerosi multipla

Cigarette Smoking, coffee consumption and multiple sclerosis: a case-control study

Che cos'è la Sclerosis Multipla

EXTRACELLULAR FACTORS INVOLVED IN THE INDUCTION OF BLLOD-BRAIN BARRIER

How many parkinsonian patients are suitable candidates for deep brain stimulation of subthalamic nucleus? Results of a questionnaire.

We used a CAPSIT-based questionnaire to estimate the percentage of parkinsonian patients suitable for subthalamic nucleus (STN) deep brain stimulation (DBS) in a movement disorders clinic. We found that out of 641 consecutive PD patients only 1.6% fulfilled strict STN-DBS criteria. When we applied more flexible criteria, the percentage of eligibility increased to 4.5%. Most patients (60%) were ineligible because they did not satisfy multiple questionnaire items. Items related to disease severity were responsible for the largest number of exclusions. This knowledge will help make decisions on resource allocation in centres wishing to start DBS surgery.

SHEDDING OF EXTRACELLULAR VESICLES FROM BRAIN CELLS

Dropped head as an unusual presenting sign of myastenia gravis

Serum acid homocysteine, oxidative stress markers and vitamins in multiple sclerosis

Cholesterol level and hemorrhagic trasformation after ischemic stroke

A screening instrument for a Sicilian neuroepidemiological survey in the elderly.

Abstract We evaluated the sensitivity and specificity of a screening instrument developed for use in a two-phase neuroepidemiological survey in Sicily. The Sicilian Epidemiological Dementia Study (SEDES) project will evaluate the prevalence and incidence of dementia, parkinsonisms and essential tremor in four Sicilian municipalities. It is a two-phase door-to-door survey. To identify subjects with possible neurological disorders, in this study, we developed a screening instrument including a symptoms questionnaire and simple physical tasks for parkinsonisms and essential tremor. The Mini-Mental State Examination (MMSE) was chosen for screening dementia. The symptoms questionnaire and simple…

Clinical Features and disease outcome in pt with pediatric and young adult onset multiple sclerosis

Multiple sclerosis severity score: Using disability and disease duration to rate disease severity

Background: There is no consensus method for determining progression of disability in patients with multiple sclerosis (MS) when each patient has had only a single assessment in the course of the disease. Methods: Using data from two large longitudinal databases, the authors tested whether cross-sectional disability assessments are representative of disease severity as a whole. An algorithm, the Multiple Sclerosis Severity Score (MSSS), which relates scores on the Expanded Disability Status Scale (EDSS) to the distribution of disability in patients with comparable disease durations, was devised and then applied to a collection of 9,892 patients from 11 countries to create the Global MSSS. I…

Environmental risk factors for Parkinson's Disease

Height as an indicator of eraly life events predicting Parkinson's disease: A case-control study

Familiarity for epilepsy among multiple sclerosis patients with seizures: a pilot case-control study.

Extracellular membrane vesicle shedding and the blood-brain barrier

Association between multiple sclerosis, cancer risk, and immunosuppressant treatment: a cohort study

Abstract Background The association between multiple sclerosis (MS) and cancer has long been investigated with conflicting results. Several reports suggest an increased cancer risk among MS patients treated with immunosuppressant (IS) drugs. Methods We performed a cohort study including MS patients recruited at the Neurological Department of the University of Palermo. Mean follow-up period was ten years for the whole cohort. We calculated cancer incidence among patients treated with IS. Incidence rates were compared in the cohort by calculating the relative risk according to length and dose of exposure to IS. Cancer incidence among MS patients was compared to cancer incidence in the general…

Prevalence of diabetic neuropathy with somatic symptoms

In a door-to-door survey conducted in two municipalities of Sicily, we ascertained the prevalence of diabetic neuropathy. Our case-finding was restricted to subjects with somatic symptoms. During phase 1, we administered a screening instrument for peripheral neuropathy to 14,540 persons residing in Santa Teresa di Riva (Messina Province) and Terrasini (Palermo Province). During phase 2, neurologists evaluated those subjects who had screened positive. Diagnoses were based on clinical criteria only, and were reviewed by an adjudication panel. We found 39 persons (27 women, 12 men) affected by diabetic neuropathy. The crude prevalence, as of November 1, 1987, was 268.2 cases per 100,000 popula…

Sleep quality in caregivers of Parkinson’s disease and Alzheimer’s disease patients and its relationship to quality of life

Bilateral ptosis and internuclear ophthalmoplegia in a case of bilateral thalamic and midbrain Infarct.

Prevalence and risk factors of post-polio syndrome in a cohort of polio survivors.

Abstract Objective To investigate frequency and associated factors of post polio syndrome (PPS) in an Italian cohort of people with prior poliomyelitis. Methods We screened subjects admitted for poliomyelitis at the paediatric hospital of the University of Palermo during the time frame 1945–1960. Patients who developed PPS were identified through a structured questionnaire and a neurologic examination. PPS diagnosis was made according to specified diagnostic criteria. Frequency of PPS was calculated in the selected cohort of polio survivors. The association with the investigated risk factors (sex, age at onset of polio, extension and severity of polio, education, associated diseases, cigare…

A population-based survey on cognitive performance in Sicily. Cognitive impairment is modified by fertile life characteristics.

Mortality in multiple sclerosis: a review

This work was undertaken to evaluate studies on mortality caused by multiple sclerosis (MS), to evaluate if useful inferences can be drawn from survival studies that can be applied to clinical practice. A literature search was carried out to find epidemiological studies on MS prognosis, survival, mortality and causes of death relevant to our aim. The World Health Organization (WHO) reports on worldwide cause-specific mortality were also considered. Studies were evaluated according to the duration of the follow-up study, the year of publication and the methodology used. We evaluated MS survival from a methodological point of view and considered if time trends could be drawn from study result…

Clinical and instrumental predictors of efficacy of natalizumab therapy in the course of multiple sclerosis: a retrospective study from a single centre

Frequency and determinants for hemorrhagic trasformation of cerebral infarction

An unexpected association between intracranial hypotension and split cord malformation

Pisa syndrome after rasagiline therapy in a patient with Parkinson’s disease

Dear Editor, We have read with interest comments by Solla et al. [1] regarding our Letter to the Editor published in NeurologicalSciences titled ‘‘Insidious onset of Pisa Syndrome afterrasagiline therapy in a patient with Parkinson’s Disease’’[2]. We thank the authors for their interest in our paper, but we think it is necessary to make some clarifications regarding the temporal relationship between the onset of Pisa Syndrome (PS) and rasagiline therapy. PS occurred in fact after and not before rasagiline treatment as stated by Solla et al. In particular, PS occurred after a time interval of 6–12 months and anyway after rasagiline was added to patient’s therapy. This long time interval is o…

Multiple sclerosis: Haemorheological pattern

BODY MASS INDEX AND PARKINSON'S DISEASE: A CASE-CONTROL STUDY

Long-term survival and outcome after first stroke in the Sicilian Aeolian Island Archipelago population.

Background: Only a restricted number of population studies based on long-term prognosis after a stroke have been published. We analyzed long-term survival and outcome in insular first-ever stroke population from the Aeolian Archipelago. Subjects and methods: From 1 July, 1999 to 30 June 2002, 62 patients with first-ever stroke were recruited to evaluate short-term incidence and outcome. Information for every patient was collected by phone interviews after 3 months, 1 year, and 4 years. Results: 30 days case fatality rate was 24.2% (95% C.I. 14.22 to 36.75). Fifty-one percent (95% C.I. 35.8 to 66.3) of patients died before last survey and 39.1% died during the first year of follow-up. Annual…

AN UNUSUAL ASSOCIATION OF MYASTHENIA GRAVIS (MG) AND PANCREATIC NEUROENDOCRINE TUMOR (PNET): A CASE REPORT

PROGRESSIVE MULTIFOCAL LEUCOENCEPHALOPATHY DURING TACROLIMUS THERAPY FOR LIVER TRANSPLANTATION

Neuronal and BBB damage induced by sera from patients with secondary progressive multiple sclerosis.

An important component of the pathogenic process of multiple sclerosis (MS) is the blood-brain barrier (BBB) damage. We recently set an in vitro model of BBB, based on a three-cell-type co-culture system, in which rat neurons and astrocytes synergistically induce brain capillary endothelial cells to form a monolayer with permeability properties resembling those of the physiological BBB. Herein we report that the serum from patients with secondary progressive multiple sclerosis (SPMS) has a damaging effect on isolated neurons. This finding suggests that neuronal damaging in MS could be a primary event and not only secondary to myelin damage, as generally assumed. SPMS serum affects the perme…

Depression and associated factors among spouse-caregivers of patients with Parkinson’s disease.

Frequency of Mitoxantrone related heart involvement in patients affected by Multiple Sclerosis

GENOTYPIC AND PHENOTIPIC FACTORS IN ISCHEMIC STROKE IN YOUNG ADULTS

Lack of association between ubiquitin carboxy-terminal hydrolase L1 gene polymorphism and PD.

In 1998, an IL93Met mutation in the ubiquitin carboxy-terminal hydrolase L1 ( UCH-L1 ) gene was identified in a German family affected by PD.1 Recently, others2-4⇓⇓ found that the S18Y polymorphism in exon 3 of UCH-L1 is associated with a low risk of PD. To verify these interesting results, we decided to design a case-control study on the S18Y polymorphism of the UCH-L1 gene using sporadic PD cases. In the meantime, as we were analyzing our samples, a case-control study5 on 142 patients with PD and 142 age- and sex-matched control subjects did not confirm the protective effect found by Maraganore et al.2 In view of these conflicting findings, we reasoned that our contribution may have some …

Multiple reversibile MR signals in course of Epstein-Barr virus encephalitis

Multiple ring-enhancing brain lesions in a ghanaian patient: a case report

Tipi diversi di cellule cerebrali rilasciano fattori di crescita attraverso vescicole extracellulari.

Identification of the novel D297fsX318 PINK1 mutation and phenotype variation in a family with early-onset Parkinson's disease

Herein we first describe a novel homozygous single nucleotide deletion in PINK1 exon 4 (889delG) which results in a loss of kinase domain on the PINK1 protein (D297fsX318). This mutation was identified in two brothers with early-onset Parkinson disease (EOPD) from a Sicilian consanguineous family. Of note, while one of the two patients developed mental deterioration and psychiatric problems, the other showed no cognitive decline. The present study supports the view that PINK1 is a pathogenic gene in some Italian families with EOPD and contributes to define the PINK1-associated phenotype. Herein we first describe a novel homozygous single nucleotide deletion in PINK1 exon 4 (889delG) which r…

Diabetes preceding Parkinson's disease onset. A case–control study

Abstract Objective To assess the association between diabetes preceding Parkinson's disease (PD) and PD. Methods PD individuals were matched to PD free individuals randomly selected from people in the same municipality as the cases. Occurrence of diabetes preceding PD onset among cases and controls was assessed through a structured questionnaire. Information regarding current and past medical treatment and other variables was also collected. We used univariate and multivariate logistic models to calculate crude and adjusted odds ratios (OR). Covariates are adjusted for included education, smoking habit, alcohol and coffee consumption. Results 318 PD individuals (165 women, 153 men) and 318 …

Tumor Diagnosis Preceding Alzheimer's Disease Onset: Is There a Link Between Cancer and Alzheimer's Disease?

Studies reporting an inverse association between Alzheimer's disease (AD) and cancer are scant. Available data are mostly based on ancillary findings of mortality data or obtained from studies evaluating frequency of neoplasms in AD patients independently if they occurred before or after AD. Moreover, some studies estimated frequencies of neoplasms in demented individuals, who were not necessarily AD patients. We estimated frequency of tumors preceding the onset of AD in AD patients and compared it to that of age- and gender-matched AD-free individuals. Occurrence of tumors preceding AD onset was assessed through a semi-structured questionnaire. Tumors were categorized as benign, malignant,…

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13.Here, we report a case of TTP with severe acute encephalopathy. Posterior leukoencephalopathy and brainstem oedema with triventricular hydrocephalus were observed on MRI. The …

Prevalence of red ear syndrome in juvenile primary headaches.

Background: Previous studies have suggested a relationship between ‘red ear syndrome’ (RES) and pediatric migraine. Aims of this study were (i) to assess the frequency, specificity and sensitivity of RES in a population of pediatric migraineurs and (ii) to establish the pathophysiological mechanisms of RES associated with migraine. Methods and results: A total of 226 children suffering from headache (aged 4–17 years) were enrolled. One hundred and seventy-two (76.4%) were affected by migraine, the remaining 54 (23.6%) by other primary headaches. RES was followed significantly more frequently by migraine (23.3%; p < .0001), and was characterized by high specificity and positive predictiv…

Age at menopause predicts age at onset of Parkinson's disease.

We investigated the association between age at onset of Parkinson's disease (PD) and fertile life characteristics in 145 women. Linear regression analyses showed a significant correlation between age at PD onset and age at menopause (P = 0.003), between age at PD onset and fertile life duration (P = 0.008), and a nonsignificant correlation between PD onset and cumulative duration of pregnancies (P = 0.23). These results support the possible role of estrogens in PD.

Occupation, education, and Parkinson's disease: A case-control study in an Italian population

Current epidemiologic data on the association between occupational exposures and Parkinson's disease (PD) are inconsistent. In a case-control study, we investigated the associations between occupation and PD and between education and PD. The cases (n = 62) were those identified in a prevalence survey (door-to-door, two-phase) of three Sicilian municipalities, as a November 1, 1987. We then randomly selected from the general population two controls for each case, matched for age ( +/- year), sex, and municipality (n = 124). Information on current and past occupations and education for cases and controls was obtained during the survey. Subjects who worked for most of their lives as farmers we…

Multiple sclerosis survival: a population-based study in Sicily

Background and purpose:  There are few population-based surveys on multiple sclerosis (MS) survival. To investigate MS survival in MS patients recruited during surveys conducted in Sicily. Methods:  Multiple sclerosis patients identified during previous surveys were randomly matched to two referent subjects by residence, year of birth, and gender. Living status was obtained by municipality records (end of follow-up June, 30th 2007) and, for the deceased, date and causes of death were searched. Kaplan-Meier plots were used to calculate differences in mortality between MS patients and referent subjects. MS risks for mortality with 95% confidence intervals (CI) were also calculated. Results:  …

Studio delle frequenze alleliche del polimorfismo +1902A/G del gene IL4R in soggetti affetti da sclerosi multipla .

ANALISI DELLA PERMEABILITÀ DI UNO STRATO DI CELLULE ENDOTELIALI DEI CAPILLARI CEREBRALI, CO-COLTIVATE CON NEURONI ED ASTROCITI.

ASTROCYTES SHED EXTRACELLULAR VESICLES THAT CONTAIN FIBROBLAST GROWTH FACTOR-2 AND VASCULAR ENDOTHELIAL GROWTH FACTOR.

An important component of the pathogenic process of multiple sclerosis (MS) is the blood-brain barrier (BBB) damage. We recently set an in vitro model of BBB, based on a three-cell-type co-culture system, in which rat neurons and astrocytes synergistically induce brain capillary endothelial cells to form a monolayer with permeability properties resembling those of the physiological BBB. Herein we report that the serum from patients with secondary progressive multiple sclerosis (SPMS) has a damaging effect on isolated neurons. This finding suggests that neuronal damaging in MS could be a primary event and not only secondary to myelin damage, as generally assumed. SPMS serum affects the perme…

Heterogeneity at the HLA-DRB1 locus and risk for multiple sclerosis.

Variation in major histocompatibility complex genes on chromosome 6p21.3, specifically the human leukocyte antigen HLA-DR2 or DRB1*1501-DQB1*0602 extended haplotype, confers risk for multiple sclerosis (MS). Previous studies of DRB1 variation and both MS susceptibility and phenotypic expression have lacked statistical power to detect modest genotypic influences, and have demonstrated conflicting results. Results derived from analyses of 1339 MS families indicate DRB1 variation influences MS susceptibility in a complex manner. DRB1*15 was strongly associated in families (P=7.8x10(-31)), and a dominant DRB1*15 dose effect was confirmed (OR=7.5, 95% CI=4.4-13.0, P<0.0001). A modest dose effect…

Differences in intercellular communication during clinical relapse and gadolinium-enhanced MRI in patients with relapsing remitting multiple sclerosis: A study of the composition of extracellular vesicles in cerebrospinal fluid

This study was designed based on the hypothesis that changes in both the levels and surface marker expression of extracellular vesicles (EVs) isolated from the cerebrospinal fluid (CSF) may be associated with the clinical form, disease activity, and severity of multiple sclerosis (MS). The analyzes were performed on subjects affected by MS or other neurological disorders. EVs, which were isolated by ultracentrifugation of CSF samples, were characterized by flow cytometry. A panel of fluorescent antibodies was used to identify the EV origin: CD4, CCR3, CCR5, CD19, and CD200, as well as isolectin IB4. The Mann–Whitney U-test and Kruskal–Wallis test were used for statistical analyzes. EVs isol…

Prevalence of stroke: A door-to-door survey in three Sicilian municipalities

As part of a door-to-door survey, we screened for stroke among the inhabitants of three Sicilian municipalities (n = 24,496 as of November 1, 1987). Neurologists then investigated those subjects suspected to have had a stroke. Diagnoses of first-ever strokes were based on specified criteria and were reviewed by an adjudication panel. We found 189 subjects who had experienced at least one completed stroke (180 definite, 9 possible); 15 strokes were hemorrhagic, 71 ischemic, and 103 uncertain. The prevalence (cases/100,000) was 771.6 in the total population and 1,893.6 in those aged 40 years or over. The prevalence increased steeply with age, was higher in men between 60 and 79 years, but was…

Dentatorubral-pallidoluysian atrophy: haplotype of Asian origin in 2 Italian families.

Return of disease activity after natalizumab therapy discontinuation in patients with multiple sclerosis: frequency and clinical and instrumental predictors of reactivation

Amyotrophic lateral sclerosis in Palermo, Italy: an epidemiological study

The incidence, prevalence and natural course of ALS were determined in the population of the province of Palermo, Italy. The average annual incidence calculated for the years 1973 through 1984, was 044./100.000 inhabitants. The prevalence rate on prevalence day December 31, 1984, was 1.67/100.000 population. The male/female ratio was 1.38. The mean age at onset was 54.3±11.02. The most common clinical form was the conventional one (61.4%); the bulbar form was more frequent among females than males. The mean duration of the disease was 33.7±35.8 months. The longest duration belongs to the pseudopolyneuritic form. The median survival was 36 months: 16 months for the bulbar, 36 months for the …

Neurons and ECM regulate occludin localization in brain endothelial cells

We report that extracellular matrix and neurons modulate the expression of occludin, one of the main components of tight junctions, by rat brain endothelial cells (RBE4.B). Of the three extracellular matrix proteins which we tested (collagen I, collagen IV, and laminin), collagen IV stimulated at the best the expression of occludin mRNA. The corresponding protein, however, was not synthesized. Significant amounts of occludin accumulated only when RBE4.B cells were cultured on collagen IV-coated inserts, in the presence of cortical neurons, plated on laminin-coated companion wells. Finally, occludin segregated at the cell periphery, only when endothelial cells were co- cultured with neurons …

DENTATO-RUBRAL PALLIDOLUYSIAN ATROPHY: CLINICAL AND GENETIC ANALYSIS OF A SICILIAN PEDIGREE

Are Multiple Domicile Changes a Risk Factor for Multiple Sclerosis?

A retrospective case-control study was conducted to estimate the possible association between risk of multiple sclerosis (MS) and the number of changes in residence before the age 20 years. Forty-one patients and 82 controls were interviewed. Using the Mantel-Haenszel estimate of the odds ratio, no association was found between the number of moves and MS.

NOVEL ROUTES OF CELL TO CELL COMUNICATIONS AMONG BRAIN CELLS

The sporadic and familial Creuzfeldt-Jacob disease in Palermo from 1996 to 2006: epidemiological, clinical and neurophysiologic characteristics.

DYSAUTONOMIC DYSFUNCTION IN PARKINSON’DISEASE BY SCOPA-AUT SCALE

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The Mini Mental State Examination (MMSE) for Cognitive Impairment in multiple sclerosis (ms): a two years Longitudinal study in 300 patients.

Cardiovascular comorbidity in multiple sclerosis patients treated with mitoxantrone therapy: a cohort study

Abstract Background Mitoxantrone (MX) has been used as second line therapy for aggressive multiple sclerosis (MS). Potential cardiotoxic effects of MX limit its use; a cumulative dose of up to 100 mg/m2, has been long considered relatively safe. We calculated the frequency of cardiac side effects in MS patients treated with MX. Methods We performed a cohort study including all MS patients treated with MX at the Neurological Department of the University Hospital of Palermo, Italy. Two hundred-sixty-four MS patients diagnosed according to validated criteria were included and followed-up until the end of September 2010. Patients were treated with MX as a second line therapy if they had no prev…

Idiopathic Parkinson's disease and diabetes: results from a case-control study.

Levetiracetam in the Treatment of Creutzfeldt-Jacob Disease.

Three years of experience : the Italian registry and safety data update

At the end of 2006, a pharmacovigilance program on natalizumab was settled by the Italian Pharmaceutical Agency, and on January 2007, multiple sclerosis patients poorly responding to the immunomodulating therapies or with an aggressive clinical form of disease from onset initiated to be registered and to receive the medication. On February 2010, almost 3,000 cases have been treated with natalizumab. The drop-out rate is 10%. Almost 800 cases received cycles of natalizumab for more than 18 months. One case of PML was reported and other adverse events are similar to those described in phase III studies. The majority of cases remained stable, while in 25% of cases, an improvement of disability…

Acute onset parkinsonism during leukoencephalopathy and CMV infection

Identification of Biomarkers in Cerebrospinal Fluid and Serum of Multiple Sclerosis Patients by Immunoproteomics Approach

Multiple sclerosis (MS) is an autoimmune inflammatory demyelinating disease of the central nervous system. At present, the molecular mechanisms causing the initiation, development and progression of MS are poorly understood, and no reliable proteinaceous disease markers are available. In this study, we used an immunoproteomics approach to identify autoreactive antibodies in the cerebrospinal fluid of MS patients to use as candidate markers with potential diagnostic value. We identified an autoreactive anti-transferrin antibody that may have a potential link with the development and progression of MS. We found this antibody at high levels also in the serum of MS patients and created an immun…

TREATMENT OF MULTIPLE SCLEROSIS WITH INTERFERON BETA IN CLINICAL PRACTICE: 2-YEAR FOLLOW-UP DATA FROM THE SOUTH ITALY MOBILE MRI PROJECT

Extracellular vesicles isolated by cerebrospinal fluid as biomarkers of inflammation in multiple sclerosis and inflammatory neurological diseases.

BODY MASS INDEX DOES NOT CHANGE BEFORE PARKINSON'S DISEASE ONSET.

Background and purpose:  Previous studies on the association between Parkinson’s disease (PD) and body mass index (BMI) have reported conflicting results. We investigated the relationship between PD and BMI by a case–control study. Methods:  PD patients were randomly matched to healthy individuals by sex and age. BMI distribution in cases has been compared with BMI of controls and odd ratios (ORs) with 95% CI were calculated. Results:  We included 318 PD patients and 318 controls. We observed no association between PD and BMI. BMI distribution in cases and controls was similar also when we adjusted for diabetes, hypercholesterolemia and the time elapsed between PD onset and the interview (O…

Extracellular vesicles as vehicles of angiogenic factors in brain cell cultures

General anaesthesia versus local anaesthesia for carotidsurgery (GALA): a multicentre, randomised controlled trial.

Background The eff ect of carotid endarterectomy in lowering the risk of stroke ipsilateral to severe atherosclerotic carotid-artery stenosis is off set by complications during or soon after surgery. We compared surgery under general anaesthesia with that under local anaesthesia because prediction and avoidance of perioperative strokes might be easier under local anaesthesia than under general anaesthesia. Methods We undertook a parallel group, multicentre, randomised controlled trial of 3526 patients with symptomatic or asymptomatic carotid stenosis from 95 centres in 24 countries. Participants were randomly assigned to surgery under general (n=1753) or local (n=1773) anaesthesia between J…

Co-culture of rat brain cells as a tool for studying cell-cell interactions

Brain capillary endothelial cells (BCECs) form the blood-brain barrier (BBB) in response to interaction with other brain cells (astrocytes, pericytes and neurons). BCECs are characterized by tight junctions (TJ), maturation and stabilization of which require different proteins, such as occludin. When co-cultured with astrocytes and neurons, BCECs were found to form a monolayer resembling the natural BBB: paracellular flux of dopamine and sucrose (i.e. compounds which are unable to cross the BBB in vivo) significantly decreased (1), while the transendothelial electrical resistance (TEER) increased. In these conditions, BCECs produced a larger amount of occludin and tended to localize it at t…

Prevalence and incidence of multiple sclerosis in the city of Monreale, Italy

The prevalence and incidence of multiple sclerosis (MS) in the city of Monreale, southern Italy were ascertained 10 years after a preliminary study in the same area. The study was undertaken in a population of 26,256 people. The patients were classified according to Poser’s criteria. The prevalence of MS on 31 December 1991 was 72.4 per 100,000 population. The incidence of MS for the period 1981–1991 was 3.3/100,000 per year. The mean period between onset and diagnosis of MS was 4.9 years for those patients found during this survey and 9.2 years for those in the first study. This study shows an increase of MS prevalence in Monreale city and a high incidence. The findings parallel the reduct…

Neuronal factors secreted through unusual pathways are probably involved in blood brain barrier (BBB) formation

FURTHER EVIDENCE OF GENETIC HETEROGENEITY IN FAMILIAL ESSENTIAL TREMOR.

MYOCARDIAL 123I-METAIODOBENZYLGUANIDINE UPTAKE IN GENETIC PARKINSON’S DISEASE.

PARKINSON’S DISEASE AND PARKINSON’S DISEASE FAMILIAL HISTORY. RESULTS OF A CASE-CONTROL STUDY

PINK mutation and the risk of parkinson's disease in family members of southern Italy

Parkinson's disease and cancer: insights for pathogenesis from epidemiology .

Epidemiological evidence suggests a reduced incidence of many common types of cancers in individuals with Parkinson's disease (PD). Parkinson's disease and cancer are two diseases that result from an excessive signaling by one of two forces driving cells to opposite directions. PD results from the excessive death of dopaminergic neurons in the substantia nigra pars compacta (SNc) in the brain, while uncontrolled growth is the key property of cancer. Parkinson's disease is a complex disorder, probably due in most of the cases to the interaction of environment and genes. Many genes responsible for familial forms of PD are supposed to have a supportive role in regulating or maintaining the cel…

NEURONAL FACTOR SECRETED THROUGH UNUSUAL PATHWAYS ARE PROBABLY INVOLVED IN BLOOD BRAIN BARRIER (BBB) FORMATION.

Height as a potential indicator of early life events predicting Parkinson's disease: A case-control study

Aim of this study was to investigate the relationship between height in young adult age and Parkinson's disease (PD) risk. We included 266 persons affected by idiopathic PD. Patients were matched by age and sex to 266 controls by a random selection from the municipality of residence. We collected information about height preceding PD from official documents where these characteristics referred to young adult age (nearly 30 years). We compared height in cases and controls by calculating differences in mean distribution and by χ2 analyses. Crude and adjusted odds ratios (OR) and 95% confidence intervals (CI) were calculated by logistic regression models. Mean height was significantly lower in…

Triiodothyronine accelerates the synthesis of synapsin I in developing neurons from fetal rat brain cultured in a synthetic medium.

The effect of Triiodothyronine (T3) on Synapsin I appearance in rat cortical neurons has been investigated in vitro. Neuronal cultures from 16-day-old fetal rat brain grown in the absence of T3, express immunohystochemically detectable Synapsin I at the 14th day in vitro. The addition of the hormone to the culture medium determines an early (at the 7th day in vitro) appearance of fluorescent dots specific for Synapsin I. © 1990 Plenum Publishing Corporation.

Protective role of heat shock proteins in Parkinson's disease.

Parkinson’s disease (PD) is the second most common neurodegenerative disease after Alzheimer’s disease. Despite a large amount of research, the pathogenetic mechanism of these diseases has not yet been clarified. Abnormal protein folding, oxidative stress, mitochondrial dysfunction, and apoptotic mechanisms have all been reported as causes of neurodegenerative diseases in association with neuroinflammatory mechanisms which, by generating deleterious molecules, could promote the cascade of events leading to neurodegeneration. Heat shock proteins (HSPs) play a central role in preventing protein misfolding and inhibiting apoptotic activity, and represent a class of proteins potentially involve…

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Mitochondrial DNA TRNACYS mutation in a family with frontotemporal dementia and Parkinson’s disease

Novel routes of cell to cell communications among brain cells

EXTRACELLULAR FACTORS INVOLVED IN THE INDUCTION OF BLOOD BRAIN BARRIER (BBB)

Predictors of caregiver burden in partners of patients with Parkinson's disease

Prevalence of cervical spondylotic radiculopathy: A door-to-door survey in a Sicilian municipality

Introduction - Because of the limited information on cervical spondylotic radiculopathy, we conducted a door-to-door two-phase survey in a Sicilian municipality. Material and methods - We first screened for cervical spondylotic radiculopathy among the inhabitants of the municipality : (N= 7653, as of the prevalence day, November 1, 1987). Study neurologists then investigated those subjects suspected to have had a cervical spondylotic radiculopathy. Diagnoses were based on specified criteria. Results - We found 27 subjects affected by CSR (17 definite, 10 possible). Prevalence (cases per 1000 population) was 3.5 in the total population ; it increased to a peak at age 50-59 years and decrease…

CARATTERIZZAZIONE DI NUOVI MARCATORI MOLECOLARI NELLA SCLEROSI MULTIPLA: UN APPROCCIO PROTEOMICO

A Door-to-Door population based survey on cognitive performance In Bagheria, Sicily.

Effetto dei terreni condizionati da neuroni ed astrociti sulle proprietà barriera di cellule di capillari cerebrali.

Frequency and Determinants for Hemorrhagic Transformation of Cerebral Infarction

Frequency of cognitive impairment and factors associated in outpatients with Parkinson’s disease

Metodo e kit per la sclerosi multipla

E’ descritta la scoperta di anticorpi anti-transferrina come biomarcatori per la sclerosi multipla da cui è stato messo a punto un metodo di diagnosi, prognosi, monitoraggio della terapia e relativo kit.

Implications for Estrogens in Parkinson's Disease: An Epidemiological Approach

Abstract: Evidence from experimental and epidemiological studies suggests a role of sex hormones in the pathogenic process leading to neurodegenerative diseases, (i.e., Alzheimer's and Parkinson's disease). The effects of sexual steroid hormones are complex and vary with the events of women's fertile life. Estrogens are supposed to influence dopamine synthesis, metabolism, and transport; however, there is no consensus regarding the direction, locus, and mechanism of the effect of estrogens on the dopaminergic system. A neuroprotective effect of estrogens has been demonstrated in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-animal models of Parkinson's disease (PD). Epidemiological st…

Implications for Estrogens in Parkinson’s Disease

Evidence from experimental and epidemiological studies suggests a role of sex hormones in the pathogenic process leading to neurodegenerative diseases, (i.e., Alzheimer's and Parkinson's disease). The effects of sexual steroid hormones are complex and vary with the events of women's fertile life. Estrogens are supposed to influence dopamine synthesis, metabolism, and transport; however, there is no consensus regarding the direction, locus, and mechanism of the effect of estrogens on the dopaminergic system. A neuroprotective effect of estrogens has been demonstrated in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-animal models of Parkinson's disease (PD). Epidemiological studies indi…

Introduzione alla neuroepidemiologia

Gender-related effect of clinical and genetic variables on the cognitive impairment in MS.

Angiogenic and/or pro-apoptotic factors are shed from brain cells via extracellular vesicles

We set a three-cell type coculture system in which neurons and astrocytes synergistically induce brain capillary endothelial cells to form a monolayer with permeability properties resembling those of the physiological blood-brain barrier (BBB) (Schiera et al., 2003; Schiera et al., 2005). On the basis of immunofluorescence, scanner electron microscopy and western blot analyses, we also suggested that both astrocytes and neurons in culture shed extracellular vesicles that contain FGF-2 and VEGF, as well as beta1-integrin, a membrane protein that can be considered a marker of shedding (Schiera et al, 2007; Proia et al., 2008). In addition, it was already known that transformed glial cells (ol…

Is season of birth associated with multiple sclerosis?

Objective - To compare the monthly distribution of multiple sclerosis (MS) patients' births with that of the Sicilian population. Background -Studies on the association between season of birth and risk of MS are scanty and controversial. Design and methods - Archives of the Institute of Neuropsychiatry of the Universities of Palermo and Catania were searched up to 1995 for definite MS patients (McDonald & Halliday criteria). The monthly distribution of MS patients' births (N=965) was compared with that of the Sicilian population (N = 5,608,307). Results -The distribution of births among MS patients compared with the general population was not different when tested by the X 2 statistic (P > …

Treatment of multiple sclerosis with interferon beta in clinical practice: 2-year follow-up data from the South Italy Mobile MRI Project.

This follow-up study assessed the 2-year clinical and magnetic resonance imaging (MRI) outcomes of patients with multiple sclerosis (MS) originally enrolled in an MRI study conducted at eight centres in south Italy (the South Italy Mobile MRI Project). Of the 597 MS patients recruited at baseline, 391 returned for the follow-up study. Of these, 363 provided 2-year clinical and MRI follow-up data, and 215 were still undergoing treatment with one of four interferon beta regimens: Avonex, 30 mcg intramuscularly once weekly; Betaferon, 250 mcg subcutaneously (sc) every other day; Rebif 22 mcg sc three times weekly (tiw; Rebif 22); or Rebif 44 mcg sc tiw (Rebif 44). Over the 2-year follow-up per…

The risk of disease worsening according to the length of exposure to IFNb in multiple sclerosis

Motor intracortical inhibition in PD: L-DOPA modulation of high-frequency rTMS effects

Dopaminergic drugs and deep brain stimulation restore cortical inhibition in Parkinson disease (PD) patients. High-frequency rTMS was also found to increase cortical inhibition in PD but its therapeutic effect is still controversial. Here we hypothesize that, if dopaminergic drugs reverse to normal cortical excitability in M1, the effect of high-frequency (hf)-rTMS in PD patients could depend on whether they are in a medicated or unmedicated state. The present study aims to explore the lasting effects of sub-threshold hf rTMS trains over M1 on cortical inhibition in patients with "on" and without "off" L-DOPA treatment. Fourteen PD patients were examined twice while "on" and "off" medicatio…

LEPROSY: A CASE REPORT FROM SICILY, ITALY

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RISK FACTORS FOR DYSKINESIAS IN PATIENTS WITH PARKINSON'S DISEASE

Genotypic and phenotypic factors in ischemic stroke in young adults

remarkably increasing incidence of multiple sclerosis. A follow-up study in Caltanissetta, Sicily

Intravenous gammaglobulin treatment in chronic progressive multiple sclerosis

Effects of More-Affected vs. Less-Affected Motor Cortex tDCS in Parkinson’s Disease

Objective. To evaluate therapeutic potential of different montages of transcranial direct current stimulation (tDCS) in Parkinson’s Disease (PD) patients with asymmetric motor symptoms. Materials and Methods. Fourteen patients with asymmetric PD underwent, while on treatment, seven separate sessions including electrophysiological and clinical evaluation at baseline and after anodal, cathodal and sham tDCS of the primary motor cortex (M1) of the two hemispheres. Changes in motor cortical excitability were evaluated by transcranial magnetic stimulation. Effects on motor symptoms were assessed by testing finger tapping and upper limb bradykinesia, and by using the Italian validated Movement Di…

Interobserver Agreement in the Diagnosis of Multiple Sclerosis

• Interobserver agreement in the clinical diagnosis of multiple sclerosis (MS) among six neurologists was evaluated. Three of them participated in a study of the clinical diagnosis of MS, the Italian Multicenter Study (IMS). The raters examined the clinical forms of MS of 50 patients randomly selected from among 430 patients recruited from the IMS. For each patient, neurologists were asked to make a diagnosis according to the McDonald-Halliday classification system of MS. The overall agreement on the diagnosis (MS present or absent) was fair, with no difference noted between the two groups of raters. Considering the six diagnostic levels instead, the reliability was higher for the neurologi…

Clinical and Instrumental Findings and Disability Progression in Primary Progressive and Progressive Relapsing Multiple Sclerosis: A Comparison Study of a Sicilian Group of Patients

The parkin gene is not a major susceptibility locus for typical late-onset Parkinson's disease

We investigated the parkin gene in 118 patients with typical Parkinson's disease (PD), i. e. in patients who had an onset of PD after the age of 45 years. The study group included 95 subjects with sporadic PD and 23 subjects from 18 families with autosomal recessive PD. No pathogenetic mutations in the parkin gene were detected either in familial or in sporadic patients. Our findings indicate that the parkin gene is not involved in the pathogenesis of classic late-onset PD.

Selective culture of rat CNS neurons in a synthetic medium.

A Chemically Defined Medium is described which facilitates the survival of neurons in primary cultures of rat cerebral hemispheres. More than 90% of all cells were identified as neurons using neurofilament as a marker in an immunocytochemical assay. In contrast, serum-supplemented medium, by stimulating nonneuronal cell proliferation, hinders the survival of neurons in culture.

Prevalence of comorbidity in Parkinson’s disease: a case-control study

Purine metabolism and multiple sclerosis: pattern varies according to disease stage and clinical form

Quality of Care and Cost-Effectiveness of ALS Interdisciplinary Centers: The Quac Study

Clinical and instrumental predictors of efficacy of Natalizumab therapy in multiple sclerosis patients: a retrospective study from a single centre

Door-to-Door Prevalence Survey of Neurological Diseases in a Sicilian Population

In three municipalities of Sicily, a prevalence survey of major neurological diseases was conducted door-to-door using screening and examination to find cases. This was the first large-scale neuroepid

A further study on the prevalence of multiple sclerosis in Sicily: Caltanissetta city

– The prevalence of probable multiple sclerosis (MS) in Caltanissetta city, Sicily, is at least 51 per 100,000. If possible multiple sclerosis patients are included, it is 54 per 100,000. This value is similar to that found in other cities in Sicily: Enna, Monreale and Agrigento. The high prevalence of multiple sclerosis in Caltanissetta confirms the previous data on Sicily and emphasises the importance of small population studies in the epidemiology of MS.

Novel ways of cell-to-cell communications among brain cells

HOMOZIGOUS DJ-1 MUTATION IN A FAMILY FROM SOUTHERN ITALY WITH AMYOTROPHIC LATERAL SCLEROSIS-PARKINSONISM-DEMENTIA COMPLEX

Frequency and determinants for hemorrhagic transformation of cerebral infarction.

&lt;i&gt;Background:&lt;/i&gt; Frequency and associated risk factors for hemorrhagic transformation (HT), a worrying complication of ischemic stroke (IS), are not clearly defined. Our aim was to estimate the overall frequency and risk factors for HT in a hospital-based population. &lt;i&gt;Methods:&lt;/i&gt; A retrospective review of medical records of patients discharged from our department during the period 2004–2006 with a diagnosis of anterior IS. Demographic, clinical and hematological information was collected. Uni- and multivariate logistic regression analyses were used to estimate risk for spontaneous HT. &lt;i&gt;Results:&lt;/i&gt; We included 240 patients (125 males, 52%), mean ag…

Incidence of multiple sclerosis in Bagheria City, Sicily, Italy.

We investigated the incidence of multiple sclerosis (MS) in a Sicilian community located at sea level. The study was a retrospective search for MS patients. Incidence was studied in the period from 1 January 1985 to 31 December 1994. We searched for definite MS patients, according to Poser's criteria, among the population resident in Bagheria (Palermo province). There were 25 subjects affected by MS, of which 20 were incident MS patients. The average annual incidence was 4.4 per 100 000 persons (n = 453 385 person-years). The incidence increased over time (1985-1989 = 3.5, 1990-1994 = 5.3). A parallel decrease of the interval between onset and diagnosis of MS was observed (1985-1989, 3.7 ye…

Myasthenia-like syndrome induced by cardiovascular agents. Report of a case

The case of a myasthenia-like syndrome induced by cardiovascular drugs is reported. The clinical and electrophysiological features of the case are discussed. © 1987 Masson Italia Periodici S.r.l.

A novel mutation of the extracellular matrix protein 1 gene (ECM1) in a patient with lipoid proteinosis (Urbach-Wiethe disease) from Sicily

Summary Background  Lipoid proteinosis (LP), also known as Urbach–Wiethe disease, is a rare autosomal recessive disorder characterized by a hoarse voice, warty skin infiltration and scarring. Mutations within the extracellular matrix protein 1 (ECM1) gene cause LP. Objectives  We report the molecular analysis of the ECM1 gene in a Sicilian patient with LP in order to extend the mutation spectrum of this genodermatosis. Methods  We studied a 32-year-old female born from consanguineous parents who was diagnosed at the age of 11 years as having LP. She has a clinical phenotype corresponding to Urbach–Wiethe disease characterized by papules/nodules, indurated plaques and sometimes ulcerated les…

The relapse rate of multiple sclerosis changes during pregnancy: a cohort study

Objective – To evaluate the influence of pregnancy and puerperium on the relapse rate of multiple sclerosis (MS). Methods – We determined retrospectively the yearly mean relapse rate (MRR) during pregnancies occurring in the course of relapsing–remitting MS. We compared the MRR of pregnancy-time with that of non-pregnancy time by paired t-test. Relative risk (RR) of relapses during the pregnancy-time was also compared with that of non-pregnancy time by χ2 analysis and 95% confidence intervals. Results – From a population of 351 women affected by clinically definite MS, only 70 reported pregnancies during their relapsing–remitting phase of MS for a total of 98 pregnancies. Both MRR (P = 0.00…

EFFECT OF SERA FROM PATIENTS WITH MULTIPLE SCLEROSIS ON A BLOOD-BRAIN BARRIER IN VITRO MODEL.

Height as an indicator of early life events predicting Parkinson's disease: a case-control study.

The dynamic properties of neuronal chromatin are modulated by triiodothyronine.

The effect of triiodothyronine (T3) on the rate of synthesis of nuclear proteins was studied during terminal differentiation of rat cortical neurons cultured in a serum-free medium. To this aim total and acid soluble nuclear proteins were analyzed by different electrophoretic techniques. Our results show that: 1) during maturation in vitro, neuronal nuclei undergo a dramatic change in the rate at which different classes of histones and high mobility group (HMG) proteins are synthesized; the synthetic activity, measured as incorporation of radioactive precursors into nuclear proteins, slows indeed down with age: especially evident is the decrease in core histones synthesis; at day 15, on the…

Risk of Parkinson’s disease in women: effect of reproductive characteristics on the age at onset

The Italian Natalizumab Registry: A 1-Year Follow-Up

SERUM URIC ACID, HOMOCYSTEINE, OXIDATIVE STRESS MARKERS AND VITAMINS IN MULTIPLE SCLEROSIS.

MORTALITY IN MULTIPLE SCLEROSIS. A POPULATION-BASED APPROACH

AN IN VITRO MODEL OF BLOOD BRAIN BARRIER (BBB).

AN UNESPECTED ASSOCIATION BETWEEN INTRACRANIAL HYPOTENSION AND SPLIT CORD MALFORMATION

Frequency and determinants for hemorrhagic transformation of posterior cerebral stroke

Abstract Background hemorrhagic transformation is a threatening ischemic stroke complication. Frequency of hemorrhagic transformation differs greatly among studies, and its risk factors have been usually studied in patients with anterior ischemic stroke who received thrombolytic therapy. We evaluated, in a hospital-based series of patients with posterior ischemic stroke not treated with thrombolysis, frequency and risk factors of hemorrhagic transformation. Patients with posterior circulation stroke were seen in our Department during the period January 2004 to December 2009. Demographic and clinical information were collected. We estimated risk for spontaneous hemorrhagic transformation by …

FIRST ITALIAN CASE WITH A DE NOVO 7666M MUTATION IN THE CACNA1A GENE ASSOCIATED WITH HEMIPLEGIC MIGRAINE/PROGRESSIVE CEREBELLAR AND EPISODIC ATAXIA

Incidence of amyotrophic lateral sclerosis in Sicily: A population based study

Our objective was to investigate incidence of amyotrophic lateral sclerosis (ALS) in Sicily, southern Italy, by means of a population based study. We included people with ALS resident in fi ve Sicilian provinces, whose onset occurred in the two-year period 2005 2006 (population at 31 December 2006: 3,481,096 inhabitants). A multisource case-fi nding procedure was adopted and patients were classifi ed as affected by ALS according to revised El Escorial criteria. During the two-year surveillance period, 97 patients meeting eligibility criteria included 57 males (58.8%) and 40 females (41.2%). Crude annual incidence rate was 1.4/100,000 person years (95% CI 1.33 1.47). The incidence rate was h…

In vitro models of BBB: a tool for the analysis of cell to cell communication in the brain

Many researchers have been trying to set in vitro models of the blood-brain barrier (BBB) aimed at analyzing, in simplified terms, the molecular mechanisms responsible for formation, maintenance and functioning of the BBB, as well as the capability of specific drugs and pro-drugs to cross BBB. We did it, starting with a simpler system of co-culture that allowed us to analyze the effects of neurons on differentiation of brain capillary endothelial cells (RBE4.B cells) in culture, and setting then a more complex model, that includes three cell types (endothelial cells, neurons and astrocytes). The reciprocal geometrical organization of brain cells in this model system is similar to the one ob…

Rey-Osterrieth Complex figure performance in patients with degenerative dementia using Boston Qualitative Scoring System

Ruolo degli estrogeni nella malattia di Parkinson

NEURONS PRODUCE FGF-2 AND VEGF SECRETE THEM AT LEST IN PART BY SHEDDING EXTRACELLULAR VESCICLES

Abstract We previously found that neurons are able to affect the ability of brain capillary endothelial cells to form in vitro a monolayer with properties resembling the blood-brain barrier. We then looked, by immunofluorescence and western analysis, for factors, produced by neurons, with the potential to influence growth and differentiation of endothelial cells. In the present paper, we report that neurons produce both vascular endothelial growth factor and fibroblast growth factor 2, two well-known angiogenic factors. More interestingly, we gained evidence that both factors are released by neurons, at least in part, by shedding of extracellular vesicles, that contain β1 integrin, a membra…

Myasthenia gravis associated with Charcot-Marie-Tooth neuropathy: report of a case

We report the case of a 24 year old woman who developed myasthenia gravis in the course of a mild form of Charcot-Marie-Tooth neuropathy. We describe the clinical manifestations together with the neurophysiological, pathological, serological findings and response to therapy and discuss the unusual association in the light of the relevant literature. © 1992 Masson Italia Periodici S.r.l.

Heart involvement in patients treated with mitoxantrone for multiple sclerosis

Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease.

Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. On the contrary, ophthalmic involvement and localisation in the central nervous system are rare.Case Report. An old man was admitted to our hospital for first seizure. Brain imaging studies revealed on the left an extra-axial thickening of the dura mater with enhancement and perilesional oedema, infiltrating the sphenoorbital fissure and an isointen…

DYSAUTOMIC DISFUNCTON OCCURS EARLY IN PARKINSON DISEASE

Multiple Sclerosis in Southern Europe: Monreale City, Italy

&lt;i&gt;Background:&lt;/i&gt; Several follow-up studies showed increasing prevalence and incidence rates for multiple sclerosis (MS). &lt;i&gt;Objective:&lt;/i&gt; To ascertain, throughout a follow-up study, the incidence and prevalence of MS in the city of Monreale, Sicily, southern Italy. &lt;i&gt;Methods:&lt;/i&gt; We calculated crude and age- and sex-specific prevalence rates on December 31, 2000, and determined incidence rates for the period January 1, 1992 to December 31, 2000. &lt;i&gt;Results:&lt;/i&gt; The prevalence of MS was 71.2 per 100,000 population (48,5/100,000 in men; 93,0/100,000 in women). The incidence rate of MS for the period 1992–2000 was 4.0/100,000 per year. &lt;i&…

THE OPTIMIZATION OF INTERFERON FOR MS STUDY: 375 MICROG INTERFERON BETA-1B IN SUBOPTIMAL RESPONDERS.

We aimed to evaluate the safety and MRI efficacy of interferon beta-1b (IFNbeta-1b) 375 microg (subcutaneously [sc] every other day [eod]) in relapsing-remitting multiple sclerosis (RRMS) patients with a suboptimal response to IFNbeta-1b 250 microg, i.e., with MRI activity or relapses. The OPTimization of Interferon for MS (OPTIMS) study was a prospective multicenter randomized phase 2 trial comprising a 6-month run-in phase (to identify suboptimal responders) and a 6-month randomized phase of open-label clinical and blinded MRI follow-up. During run-in all patients were treated with IFNbeta-1b 250 microg sc eod; during the study phase suboptimal treatment responders were randomized either …

Functional feature of a novel model of blood brain barrier: Studies on permeation of test compounds

Drug delivery to the central nervous system (CNS) is subject to the permeability limitations imposed by the blood-brain barrier (BBB). Several systems in vitro have been described to reproduce the physical and biochemical behavior of intact BBB, most of which lack the feature of the in vivo barrier. We developed a fully formed monolayer of RBE4.B immortalized rat brain microvessel endothelial cells (ECs), grown on top of polycarbonate filter inserts with cortical neuronal cells grown on the outside. Neurons induce ECs to synthesize and sort occludin to the cell periphery. Occludin localization is regulated by both compositions of the substratum and soluble signals released by cortical co-cu…

FOLLOW-UP DI ALCUNI CASI DI ATROFIA DENTATO RUBRO PALLIDO LUISIANA PROVENIENTI DA UN'AREA CIRCOSCRITTA DELLA PROVINCIA DI TRAPANI

Tumor diagnosis preceding Parkinson's disease: A case-control study

Lower cancer risk in Parkinson's disease (PD) patients compared to the general population has been reported. However, most of the studies were based on death certificates. We designed a case–control study to estimate the association of tumor preceding PD onset and PD. PD patients were matched by age and gender to PD-free individuals, randomly selected from the municipalities of residence of cases. Occurrence of tumors preceding PD onset was assessed through a structured questionnaire. Neoplasms were categorized as benign, malignant, or of uncertain classification, and endocrine-related or not. Odds ratios (OR) were calculated using conditional logistic regression and adjusted for tumor cate…

A transcranial magnetic stimulation study evaluating methylprednisolone treatment in multiple sclerosis

Objective - To investigate the efficacy of two different high doses of intravenous methylprednisolone (IVMP) during Multiple Sclerosis (MS) relapses. Background - Transcranial Magnetic Stimulation (TMS) is the most sensitive neurophysiological ascertainment to quantify motor disability, to follow the recovery from an MS relapse, and to detect the response to treatment. Design and method - Twenty-four clinically definite relapsing - remitting MS patients presenting a relapse were randomly assigned to a treatment for 5 days with IVMP 1 or 2 g/day. The response to treatment of each patient was evaluated through Expanded Disability Status Scale (EDSS), Medical Research Council (MRC) score, and …

Frequency of John Cunningham virus (JCV) DNA and of antibodies against JCV in patients affected by multiple sclerosis and their relationship with natalizumab therapy

Parkinson disease survival: a population-based study

Objective To evaluate whether the survival of patients with Parkinson disease (PD) is shorter than that of the general population. Design Survival was investigated in a cohort of patients with PD previously identified during a population-based prevalence study (prevalence day, November 1, 1987; reference follow-up date, October 31, 1995). The survival of patients with PD was compared with that of a control sample randomly selected from the same population (2 controls for each case, matched for age, sex, and study municipality). The causes of death in the 2 groups were also compared. Both univariate and multivariate survival analyses were performed to investigate the association with disease…

Prevalence of essential tremor: A door-to-door survey in Terrasini, Sicily

As part of a door-to-door neuroepidemiologic survey, we investigated the frequency and distribution of essential tremor (ET) in a Sicilian municipality. During phase 1, we administered a screening instrument for tremor to 7,653 persons residing in Terrasini (Palermo province). During phase 2, neurologists evaluated those subjects who had screened positive. The diagnoses, based on specified clinical criteria, were reviewed to increase reliability across neurologists. We found 31 subjects affected by ET (17 men, 14 women); 11 patients (35.5%) reported a familial aggregation. The prevalence of ET as of November 1, 1987, was 405.1 per 100,000 for the total population, and 1,074.9 per 100,000 fo…

Leprosy: report of a case with severe peripheral neuropathy

Leprosy (Hansen's disease) is a chronic granulomatous infectious disease, caused by Mycobacterium leprae, with cutaneous and neurological manifestations. Leprosy is very rare in Europe but some cases are reported, especially among people coming from endemic areas. Here, we report a case of Hansen's disease and emphasize the importance of a prompt diagnosis and treatment also in non-endemic areas.

A transcranial magnetic stimulation study evaluating methylprednisolone treatment in multiple sclerosis

Objective To investigate the efficacy of two different high doses of intravenous methylprednisolone (IVMP) during Multiple Sclerosis (MS) relapses. Background Transcranial Magnetic Stimulation (TMS) is the most sensitive neurophysiological ascertainment to quantify motor disability, to follow the recovery from an MS relapse, and to detect the response to treatment. Design and method Twenty-four clinically definite relapsing - remitting MS patients presenting a relapse were randomly assigned to a treatment for 5 days with IVMP 1 or 2 g/day. The response to treatment of each patient was evaluated through Expanded Disability Status Scale (EDSS), Medical Research Council (MRC) score, and TMS by…

Permeability properties of a three-cell type in vitro model of blood-brain barrier.

We previously found that RBE4.B brain capillary endothelial cells (BCECs) form a layer with blood‐brain barrier (BBB) properties if co‐cultured with neurons for at least one week. As astrocytes are known to modulate BBB functions, we further set a culture system that included RBE4.B BCECs, neurons and astrocytes. In order to test formation of BBB, we measured the amount of (3)H‐sucrose able to cross the BCEC layer in this three‐cell type model of BBB. Herein we report that both neurons and astrocytes induce a decrease in the permeability of the BCEC layer to sucrose. These effects are synergic as if BCECs are cultured with both neurons and astrocytes for 5 days, permeability to sucrose decr…

Purine metabolism and Multiple Sclerosis: different pattern according to different disease stage and different clinical form.

P422 Purine metabolism and multiple sclerosis: pattern varies according to disease stage and clinical form G. Salemi, M. Gueli, V. Cusimano, M. Lo Re, V. Lo Re, M.A. Mazzola, S. Realmuto, P. Ragonese, G. Savettieri University (Palermo, IT) Background: Serum concentration of uric acid (sUA) was variably associated with Multiple Sclerosis (MS). Many papers reported lower sUA in MS patients respect to healthy controls, expecially in course of relapse. This lower sUA was considered as a marker of oxidative stress. However, this association was not confirmed by many other papers. Recently, an increase in the concentration of sUA, hypoxanthine, xanthine, and sum of oxypurine (sPU) was reported in…

Dropped head as an unusual presenting sign of myasthenia gravis

Relationshiop between brain atrophy and lesion load and fatigue in multiple sclerosis

A case control study to evaluate the relationship between cigarette smoking or coffee consumption and multiple sclerosis

Shedding of extracellular membrane vesicles from brain cells in culture

Apolipoprotein E genotype does not influence the progression of multiple sclerosis

OBJECTIVE: To investigate the association between apolipoprotein E (APOE) polymorphisms and the progression of MS. METHODS: We investigated 428 subjects affected by clinically defined MS, with a disease duration of at least three years. We collected data concerning the age at onset of MS, clinical type, disease duration and disability according to the expanded disability status scale (EDSS). We also calculated the progression index (PI) to evaluate disease progression. APOE genotyping and the -491 A/T polymorphism of the APOE promoter were determined. RESULTS: No association was observed between the APOE epsilon4 allele and clinical characteristics of our study population. We also investiga…

The pharmacovigilance program on natalizumab in Italy: 2 years of experience.

At the end of 2006 a country-based surveillance program on natalizumab therapy in multiple sclerosis was settled in Italy by a collaborative effort of the Italian Drug Agency (AIFA) and a group of experts and neurologists appointed by the National Society of Neurology (SIN). After 2 years, 1,818 patients are registered in the database. The majority of cases (88.6%) failed the therapy with beta interferon or glatiramer acetate and had relapses or accumulated disability during immunomodulating treatment, while 11.4% of patients enrolled in the surveillance study were not previously treated with immunomodulating therapies and had a rapidly evolving clinical course. Almost 10% of the patients t…

Early and late mortality of spontaneous hemorrhagic transformation of ischemic stroke.

Background Hemorrhagic transformation (HT), a complication of ischemic stroke (IS), might influence patient's prognosis. Our aim is to evaluate, in a hospital-based series of patients not treated with thrombolysis, the relationship between HT and mortality. Methods We compared mortality of individuals with spontaneous HT with that of individuals without. Medical records of patients diagnosed with anterior IS were retrospectively reviewed. Outcome measures were 30- and 90-day survival after IS onset. Kaplan–Meier estimates were used to construct survival curves. Cox proportional hazards model was used to estimate hazard ratio (HR) for the main outcome measure (death). HT was stratified in he…

Expression of synapsin I gene in primary cultures of differentiating rat cortical neurons

Synapsin I is a neuron-specific protein which is present in two isoforms, Ia and Ib. In the last few years this protein has been demonstrated to play a central role in the regulation of neurotransmitter release and synaptic plasticity. In this paper the developmental expression of this protein has been investigated in primary neuronal cultures from fetal rat brain cortices. The presence of thyroid hormone in the culture medium stimulates an early expression of the protein without exerting any effect at the level of mRNA transcription and accumulation. These observations implicate a T3-dependent regulation of this neuron-specific gene at the level of mRNA translation. © 1995 Plenum Publishin…

Peripheral nerve involvement in chronic liver disease. Clinical and electrophysiological study.

A clinical and electrophysiological study was carried out on 19 selected patients with chronic liver disease. Clinical signs of peripheral nerve involvement were found in 4 patients (21%); while electrophysiological impairment was present in 11 patients (57.8%). These abnormalities were mostly limited to the sensory and motor fibers of the tibialis posterior nerve. Our data confirm the presence of peripheral nerve involvement in chronic liver disease, and that it may be evidenced by careful electrophysiological examination.

Further evidence of genetic heterogeneity in familial essential tremor.

Familial essential tremor (FET) is a common hereditary movement disorder with phenotypic variability and genetic heterogeneity. To date, linkage analyses revealed three loci associated to essential tremor (ET) (ETM1 on 3q13, ETM2 on 2p22-25, and a locus on 6p23). We performed a genetic analysis of these candidate chromosomal regions in a fifth-generation Italian kindred with autosomal-dominant ET. Of the 22 clinically evaluated family members, nine were affected by ET. The genetic study indicates that the ET in this family is not associated to any of the known ET loci. These findings support evidence of further genetic heterogeneity for such disease. (C) 2007 Elsevier Ltd. All rights reserv…

Mortality of multiple Sclerosis: a population based study.

An exploration of anger phenomenology in multiple sclerosis

Background and purpose:  Multiple sclerosis (MS) patients are often emotionally disturbed. We investigated anger in these patients in relation to demographic, clinical, and mood characteristics. Patients and methods:  About 195 cognitively unimpaired MS patients (150 relapsing–remitting and 45 progressive) were evaluated with the State Trait Anger Expression Inventory, the Chicago Multiscale Depression Inventory, and the State Trait Anxiety Inventory. The patients’ anger score distribution was compared with that of the normal Italian population. Correlation coefficients among scale scores were calculated and mean anger scores were compared across different groups of patients by analysis of …

APOE epsilon variation in multiple sclerosis susceptibility and disease severity: some answers

Background: Previous studies have examined the role of APOE variation in multiple sclerosis (MS), but have lacked the statistical power to detect modest genetic influences on risk and disease severity. The meta- and pooled analyses presented here utilize the largest collection, to date, of MS cases, controls, and families genotyped for the APOE epsilon polymorphism. Methods: Studies of MS and APOE were identified by searches of PubMed, Biosis, Web of Science, Cochrane Review, and Embase. When possible, authors were contacted for individual genotype data. Meta-analyses of MS case-control data and family-based analyses were performed to assess the association of APOE epsilon genotype with dis…

Stress ossidativo e neurodegenerazione.

DJ-1 mutations and parkinsonism-dementia-amyotrophic lateral sclerosis complex.

Mutations in DJ-1 gene have been recently shown to cause autosomal recessive early-onset Parkinson’s disease (EOPD) in a large Dutch family and in a small consanguineous Italian family.1 Subsequent to this initial finding, several additional DJ-1 mutations were identified in subjects with EOPD.2–6 We describe a family from southern Italy with three brothers affected by a complex disorder characterized by early-onset parkinsonism-dementia-amyotrophic lateral sclerosis (EOPD-D-ALS). The analysis of the DJ-1 gene showed a novel homozygous mutation (E163K) in exon 7 and a novel homozygous mutation (g.168_185dup) in the promoter region of this gene in living affected subjects

Cholesterol level and hemorrhagic transformation after ischemic stroke

A CASE OF JUVENILE STROKE CAUSED BY ACTINIC CERVICAL ARTERIOPATHY

Introduction: Radiation Arteriopathy is a vascular disease characterized by progressive arterial stenosis that may occur after external radiotherapy (RT) directed to a specific body region affected by malignant tumours. The arterial damage usually starts many years after the injury and the specific pathogenesis is unknown, though atherosclerosis praecox, endothelial damage, adventitial fibrosis and vasa vasorum obliteration are likely involved mechanisms. In patients with previously irradiated neck or head cancers, supra aortic arteries can be affected in their segments exposed to radiations leading to a gradual lumen narrowing. This late complication leads to a progressive stenosis of extr…

How many parkinsonian patients are suitable candidates for deep brain stimulation of subthalamic nucleus? Results of a screening questionnaire.