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Background: 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. Objective: 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. Methods: 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 according to the Poser criteria. We calculated prevalence ratios, for patients affected by MS who were living and resident in the study area on December 31, 2002. Crude and age- and sex-specific incidence ratios were computed for the period from January 1, 1993, to December 31, 2002. Results: The prevalence of definite MS rose in 20 years from 69.2 (retrospective prevalence rate) to 165.8/100,000 population. We calculated the incidence of definite MS for the period 1970–2000. These rates calculated for 5-year periods increased from 2.3 to 9.2/100,000/year. Conclusion: This survey shows the highest prevalence and incidence figures of MS in the Mediterranean area and confirms central Sicily as a very-high-risk area for MS.

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 indicate gender differences regarding the onset and the prognosis of PD. Most of the analytical studies explored the relationship between PD and exogenous estrogens. Only three studies investigated the role of endogenous estrogens in the risk of developing PD. These studies reported an increased risk of PD in conditions causing an early reduction in endogenous estrogens (early menopause, reduced fertile life length). Longer cumulative length of pregnancies has also been associated with an increased PD risk. A lack of consensus still exists on the effect of the type of menopause (surgical vs. natural) on PD risk. Finally, the effect of postmenopausal estrogen replacement therapy is still debated. Inconsistencies across studies are in part explained by the complexity of the mechanisms of action of sexual hormones and by the paucity of analytical studies.

Background There are only 2 small sample studies investigating allodynia in the pediatric population. The aim of this study was to evaluate the frequency of allodynia during cephalalgic attacks in a juvenile population with primary headaches and its association with other symptoms of migraine. Methods We reviewed all medical records of patients with primary headache consecutively seen during a 2-year period. Frequency of allodynia was evaluated, by means of a questionnaire, consisting of 6 questions (for example: Do you avoid touching your head when you have a migraine attack?). Results Two hundred thirty children suffering from primary headache were seen during the study period. Two hundred two children were affected by migraine, 28 (12.2%) by other primary headaches. Migraineurs significantly more frequently complained of allodynia compared to other primary headaches (37% vs 0%). At multivariate analysis, allodynia was significantly associated with pain aggravated by physical activity (adjusted odds ratio [ORa ] 2.0, 95% confidence interval [CI] 1.0, 3.8), phonophobia (ORa 2.3, 95% CI 1.0, 5.1), and nausea (ORa 1.9, 95% CI 1.0, 3.7). Conclusion According to our data, allodynia is common during pediatric migraine attacks. The association between allodynia and physical activity, nausea and phonophobia are supported by studies on adult population and suggests specific physiopathological mechanisms.

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 = 0.006) and RR (RR = 0.63, 95% CI = 0.40–0.94) decreased during the three trimesters of pregnancy. RR increased in the first 3 months of puerperium, although this was not statistically significant (RR = 1.36, 95% CI = 0.79–2.20). Conclusion – Our study confirms that in MS the relapse rate decreases throughout pregnancy and increases during puerperium. This suggests a complex interplay between hormonal and immune factors.

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 categories and risk factors. We included 222 PD patients. Frequency of cancer was 6.8% for cases, 12.6% for controls. PD patients had a decreased risk for neoplasms (adjusted OR, 0.4; 95% confidence interval [CI], 0.2–0.7). Risk was reduced only for women (adjusted OR, 0.3; 95% CI, 0.1–0.7). PD patients had a decreased risk both for malignant (adjusted OR, 0.6; 95% CI, 0.1–2.5) and nonmalignant neoplasms (adjusted OR, 0.3; 95% CI, 0.1–0.7). Still, risk was decreased for endocrine-related tumors (adjusted OR, 0.3; 95% CI, 0.1–0.9) and non–endocrine-related tumors (adjusted OR, 0.4; 95% CI, 0.1–0.9). Our study confirms the inverse association between PD and neoplasms reported in previous epidemiologic studies. © 2004 Movement Disorder Society

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 Monreale city for 1992–2000 compared to 1981–1991. Prevalence rates were similar to those of the previous follow-up study. Intervals between onset of symptoms and diagnosis seemed shorter than in prior studies. There is no evidence that the high prevalence and incidence rates have changed in this interval but numbers are too small for firm statements. These findings indicate that in Monreale city MS prevalence is stable and confirm Sicily as a high-risk area for MS.

Aim Migraines in children younger than 7 years of age have received limited attention in the published literature. The aim of this study is to describe the characteristics of migraine phenotypes in children younger than 7 years, and to compare them with migraines in children older than 7 years of age. Method We reviewed all standard clinical files, collected over 4 years, related to children with a diagnosis of primary headache. We included all children younger than 7 years diagnosed with migraine in our study. Results A total of 374 children (188 males, 186 females) were affected by migraine with/without aura: 40 of these patients (10.7%; 20 males, 20 females; mean age 5y 7mo, SD 1y 2mo) where younger than 7 years old. The frequencies of the main migraine features in the younger age group were similar to those of children older than 7 years, with the exception of a shorter duration of migraine and reduced frequency of attacks. Interpretation In children younger than 7 years of age, the clinical phenotype of migraine is similar to that seen in older children. We propose that there is a general genetic migraine susceptibility that, in the presence of activating environmental factors, may induce typical attacks of migraine in individuals already predisposed to migraine attacks. Therefore, different modules induce different clinical features within the different age groups, but there is no difference in the frequencies of clinical phenotypes between the two age groups.

N.A.

The frequency of cranial autonomic symptoms in children affected by primary headaches is uncertain. The aim of our study was to estimate the frequency of symptoms in pediatric headaches and correlate it with main migraine characteristics. A questionnaire investigating the presence of cranial autonomic symptoms was administered to all children with primary headache for 2 years. A total of 230 children with primary headache (105 males, 125 females) were included. Two hundred two children were affected by migraine and 28 (12.2%) by other primary headaches. Cranial autonomic symptoms were significantly complained by migraineurs (55% vs 17.8%) ( P < .001) and by children with higher frequency of migraine attacks (odds ratio = 2.6, confidence interval = 1.4-4.7, P = .001). Our findings show that cranial autonomic symptoms are rather common during pediatric migraine attacks. The association between cranial autonomic symptoms and higher frequency of attacks might suggest the role of the trigeminal-autonomic reflex in migraine pathophysiology.

The effectiveness of cognitive rehabilitation (CR) in Parkinson’s disease (PD) is in its relative infancy, and nowadays there is insufficient information to support evidence-based clinical protocols. This study is aimed at testing a validated therapeutic strategy characterized by intensive computer-based attention-training program tailored to attention deficits. We further investigated the presence of synaptic plasticity by means of functional magnetic resonance imaging (fMRI). Using a randomized controlled study, we enrolled eight PD patients who underwent a CR program (Experimental group) and seven clinically/demographically-matched PD patients who underwent a placebo intervention (Control group). Brain activity was assessed using an 8-min resting state (RS) fMRI acquisition. Independent component analysis and statistical parametric mapping were used to assess the effect of CR on brain function. Significant effects were detected both at a phenotypic and at an intermediate phenotypic level. After CR, the Experimental group, in comparison with the Control group, showed a specific enhanced performance in cognitive performance as assessed by the SDMT and digit span forward. RS fMRI analysis for all networks revealed two significant groups (Experimental vs Control) × time (T0 vs T1) interaction effects on the analysis of the attention (superior parietal cortex) and central executive neural networks (dorsolateral prefrontal cortex). We demonstrated that intensive CR tailored for the impaired abilities impacts neural plasticity and improves some aspects of cognitive deficits of PD patients. The reported neurophysiological and behavioural effects corroborate the benefits of our therapeutic approach, which might have a reliable application in clinical management of cognitive deficits.

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.

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.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.A significant improvement of gait, as assessed by the Stand Walk Sit test, with reduction in number and duration of FOG episodes, along with a significant reduction in the Unified Parkinson's Disease Rating Scale score, were observed after anodal stimulation. Beneficial effects were more evident after the entire 5-day stimulation session, and persisted until the end of the observation period.Anodal transcranial direct current stimulation of the motor cortex is safe and has therapeutic potential in PD patients with FOG.

Background: Knowledge about sleep complaints of caregivers of patients with Alzheimer's disease (AD) and Parkinson's disease (PD) is limited, and we lack information about the relationship between caregivers’ sleep problems and their quality of life (QoL).Methods: We evaluated subjective sleep quality and its relationship to QoL in a group of 80 caregivers of patients with AD (ADCG, n = 40) and PD (PDCG, n = 40), and in 150 controls. Information about night-time complaints was collected using the Pittsburgh Sleep Quality Index (PSQI). QoL was measured using the McGill QoL Questionnaire.Results: Eighteen ADCG (45%), 22 PDCG (55%), and 45 (30%) controls reported poor sleep quality. Mean global PSQI score of PDCG (6.25 ± 3.9) was not significantly different from that of ADCG (5.8 ± 3.5; p = 0.67). However, both PDCG and ADCG scored significantly higher than control group (4.3 ± 3.1; p < 0.01). ADCG frequently reported difficulties falling asleep (72.5%) and disturbed sleep (100%). PDCG reported reduced subjective sleep quality (80%) and increased sleep disturbances (100%). Poor sleep quality was associated with depressive symptoms and correlated with QoL in caregivers of both groups, particularly the psychological symptoms domain.Conclusions: Among caregivers of patients with AD and PD, poor sleep quality is frequent and significantly linked to QoL and depressive symptoms. Identifying the nature of sleep disturbances not only in patients but also in their caregivers is important as appropriate treatment may lead to a better management of the needs of families coping with these patients.

Transient topographical amnesia (TTA) is the temporary inability to find one's way in familiar or unfamiliar surroundings due to the inability to use well known environmental landmarks for route finding. The syndrome has not been described as having any obvious aetiology and has been thought to be caused by a vascular deficit in right hemispheric structures which are crucial for topographic recognition, i.e. parietal association and parahippocampal cortex. The patient described in the present study complained of several critical episodes of TTA and tonic rigidity of the left limbs. Neuropsychological assessment was normal except for a deficit in spatial memory tasks. Magnetic resonance (MR) imaging of the brain showed an angioma at the border of areas 24d and 23 of the right cingulate cortex. Because area 23 is strategically located in a network that links the parietal associative (area 7a) and parahippocampal cortices, and because these cortical areas are involved in topographical orienting processes, we suggest that a transient functional inactivation of the network caused by epileptic discharges spreading from the damaged cingulate cortex towards the parahippocampal and parietal association cortex could account for the spatial disorder. Similar discharges spreading from area 24d towards the primary motor cortex and/or the spinal cord could account for the episodes of tonic rigidity of the left limbs.

Background: 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. Methods: 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 according to their percentile distribution. Results: TC levels were available for 215 patients (89.6%), while LDLC levels were available for 184 patients (76.7%). The risk of HT significantly increased with decreasing levels of TC (p for trend = 0.03) and LDLC (p for trend = 0.01). In multivariate analysis, the risk of HT was significantly higher in the groups of patients with the lowest TC (OR 2.8, 95% CI 1.0–8.9, p = 0.05) and LDLC (OR 5.0, 95% CI 1.2–20.1, p = 0.002) values compared to those with the highest ones. Conclusion: We confirm that lower TC and lower LDLC levels are associated with an increased risk of HT. As none of our patients received thrombolytic therapy, the results of our study provide baseline information about the natural history of HT.

Background: 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. Methods: 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, education and working activity were calculated. Regression models were used to analyze the relationship between the investigated variables and cognitive performance. Results: A total of 1,837 persons agreed to participate (1,062 women and 775 men). Distribution of MMSE showed a highly significant inverse linear trend related to the increasing age (p < 0.0001) and to the degree of education (p < 0.0001), both in men and women. We observed a significant association between higher education and a better cognitive performance (OR 6.91; CI 3.24–14.76) and an inverse association between manual kind of occupation versus not manual and a poorer cognitive status (OR 0.82; CI 0.67–0.997). Height was associated with decreased cognitive performances in women (OR = 1.03; CI = 1.01–1.05) but not in men (OR = 0.997; CI = 0.993–1.002). Conclusions: These findings provide a feature of cognitive performances in Sicily, measured by MMSE scores in individuals aged 65 years or older.

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 low activity of ADAMTS13 was not observed and ADAMTS-13 antibodies were absent. Neurological symptoms and patient's condition were completely resolved by plasma exchange therapy in addition to high dose of methylprednisolone.

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 Conclusions: Our study showed that (i) in children, RES is a highly specific sign for migraine. In addition, the evidence of an association of RES with some migraine features partially provoked by the parasympathetic system supports the hypothesis of a shared pathophysiological background (e.g. via the activation of the trigeminal-autonomic reflex).

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 involved in PD pathogenesis. The present review will focus on two HSPs, HSP70 and HSP90, with the aim of specifying their role in PD pathogenesis.

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 irreversible abnormalities.

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 examinations and cultures, serology, and molecular techniques were normal. Tacrolimus treatment was stopped. A cerebral biopsy of the lesion showed a sub acute necrotizing process. In the following months, cognitive status of the patient tended to improve although he remained hemiplegic, while serial MRI confirmed the tendency to the recovery of the lesion that was still present 1 year after. The present observation describes a progressive encephalopathy associated with immune suppression with an unusual feature and permanent brain damage.

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 cell cycle, a fact that allows us to assume their interaction in tumorigenesis. Understanding the nature of these processes may help researchers find new and more efficacious therapeutic approaches for both diseases.

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 significantly different after 2 years. The correlation between MRI data at baseline and their variation during the follow-up showed that lower basal gray matter atrophy was significantly related with higher progression of gray matter atrophy during follow-up. The correlation between MRI parameters and disease duration showed that gray matter atrophy rate decreased with increasing disease duration, whereas the rate of white matter atrophy had a constant pattern. Lower basal gray matter atrophy was associated with increased probability of developing gray matter atrophy at follow-up, whereas gray matter atrophy progression over 2 years and new T2 lesion load were risk factors for whole brain atrophy progression. Conclusions In MS, brain atrophy occurs even after a relatively short period of time and in patients with limited progression of disability. Short-term brain atrophy progression rates differ across tissue compartments, as gray matter atrophy results more pronounced than white matter atrophy and appears to be a early phenomenon in the MS-related disease progression.

Background: 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. Methods: 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. Results: We included 240 patients (125 males, 52%), mean age at admission was 72.5 years. HT was observed in 29 patients (12%). At univariate analysis, consciousness impairment at admission (OR 5.6, 95% CI 1.3–28.2), the presence of early CT signs (OR 2.4, 95% CI 1.1–5.3), infarcts of medium-large size (OR 11.3, 95% CI 4.1–30.8), cardioembolic stroke (OR 2.3, 95% CI 1.1–5.2) and low total cholesterol levels (OR 3.3, 95% CI 1.3–8.2) were significantly associated with HT. At multivariate analysis, only infarct size (OR 10.2, 95% CI 3.2–32.1) was still significantly associated with HT. Conclusions: Frequency of HT in our study was 12%. Consistently with previous results, HT was associated with the size of ischemic area. As patients included in our study did not receive thrombolytic therapy, our results are applicable to those patients whom clinicians, working in a hospital setting, usually deal with.

Blood levels of total homocysteine (tHcy), cysteine (Cys), total and reduced glutathione (tGSH and rGSH), folic acid (FA), and vitamin B(12) (B(12) change during ischemic stroke as accompaniment of the tissue damage. The relationship between these changes remains scantly investigated. We evaluated the variation of these molecules in the 48 h after acute large artery atherothrombotic stroke (LAAS) and searched for the presence of matched variation of them. The study involved 50 subjects affected by acute LAAS and 49 healthy controls. Plasma levels of tHcy and Cys were significantly higher and serum levels of FA and B(12) and plasma levels of rGSH were significantly lower in the patients than in the control group. Acute LAAS was associated with increased Hcy-decreased tGSH and decreased FA/tGSH. Pathways involved in cellular stress and in tissue repair are activated during acute LAAS.

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 (OR = 0.99; CI = 0.94–1.03; P = 0.51). Conclusions: These results did not confirm the previously reported association between PD and BMI. Population characteristics and methodological issues may partially account for the differences observed between the present study and the others.

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 patients with Gly2019Ser mutation in the LRRK2 gene, whereas it was impaired in all patients with idiopathic PD. MIBG was preserved in all control subjects. Our study shows that myocardial MIGB uptake was normal in 8 of 14 patients with genetic PD, suggesting that cardiac sympathetic denervation occurs less frequently in genetic PD than in idiopathic PD. Our findings also demonstrate that MIGB uptake has a heterogeneous pattern in genetic PD, because it was differently impaired in patients with different mutations in the same gene or with the same gene mutation. (c) 2007 Movement Disorder Society.

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 chi(2) analyses. Crude and adjusted odds ratios (OR) and 95% confidence intervals (CI) were calculated by logistic regression models. Mean height was significantly lower in persons affected by PD compared to controls (P = 0.03). Difference was significant only in men (P = 0.001). Logistic regression models showed an inverse association between height and PD (OR 0.35; CI 0.16, 0.79; P0.01 comparing individuals in the highest percentiles of height with those in the lowest). Our results indicate an association between height and PD in men. Considering that dopamine sensitivity in the hypothalamic-pituitary axis is related to adult height, our findings suggest a relationship between PD and factors modulating somatic growth early in life.

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. Prevalence for all types of parkinsonism was 156.3/100,000 (95% CI 99.4–234.8). Fourteen subjects fulfilled diagnostic criteria for PD giving a crude prevalence of 104.2/100,000 (95% CI 59.4–170.7) and 422.5/100,000 in the population aged 60 years and over. Conclusions Prevalence of all types of parkinsonism and PD found in the Aeolian Archipelago is lower than that previously reported in Sicily.

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.

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.

Multiple sclerosis (MS) is a cell-mediated autoimmune disease characterized by type-1 cytokine production. Environmental and individual genetic background might influence this response particularly in cytokine gene polymorphisms. We evaluated whether polymorphisms of interleukin (IL)-10, IL-12, and tumor necrosis factor (TNF)-alpha genes, which might play a role in MS pathogenesis, are associated with MS susceptibility. Genotype frequencies for all the analyzed polymorphisms were not differently distributed between cases and controls. It is reasonable to suppose that the cytokine single-nucleotide polymorphisms (SNPs) studied must be considered against a larger genetic background involving other functional SNPs of Th1 regulator elements such as IL-21 and IL-23.

Purpose: Very little is known about short-term mortality after acute symptomatic seizure. One study found an increased mortality in the first year after acute symptomatic seizure, like mortality following acute symptomatic status epilepticus. Methods: We studied mortality in the first 30 days after an acute symptomatic seizure in two cohorts. In Washington Heights, New York City, we reviewed the medical records of all adults aged 20 years and older seen at Columbia Presbyterian Medical Center from January 1, 1990 through December 13, 1994 to identify incident acute symptomatic seizure. In Rochester, Minnesota, the medical records of all Rochester residents were reviewed to identify incident acute symptomatic seizure from January 1, 1965 through December 31, 1984. Case fatality (CF) and standardized mortality ratio (SMR) were calculated for deaths in the first 30 days. Results: A total of 323 adults were identified in Washington Heights and 428 in Rochester. The CF was 20% in both cohorts. CF was greatly increased in the people aged 65 years and older (28.4% in Washington Heights and 40.5% in Rochester) versus younger individuals (17.7% inWashington Heights and 11.2% in Rochester). In both cohorts, theSMRwas greatly increased overall (102.1 in Washington Heights and 149.4 in Rochester), and separately for males, females, all etiologies except head trauma in Washington Heights, and younger individuals. In older individuals, the SMR was increased in Washington Heights but not in Rochester. Conclusion: Acute symptomatic seizures are associated with increased mortality in the first 30 days. It is unknown how seizures contribute to this mortality or whether mortality is due solely to the underlying medical condition. KeyWords: Acute symptomatic seizure—Mortality.

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

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, cigarette smoking, trauma, polio vaccination) was analysed by the calculation of the odds ratio. Results Forty-eight participants met the adopted diagnostic criteria for PPS, giving a prevalence of 31.0%. The prevalence rate was significantly higher in women than in men ( p = 0.02). Logistic regression analyses revealed a significant inverse association with onset of poliomyelitis at over 12 months of age (OR 0.33; CI 0.14–0.79) a higher degree of education (OR 0.20; CI 0.07–0.79), and a significant association with the presence of other diseases (OR 9.86; CI 3.69–26.34). Conclusions In our survey one-third of patients with prior poliomyelitis had PPS. Higher age at onset of poliomyelitis is inversely associated with PPS. The association with other diseases may indicate that a chronic physical stress, particularly in already weak motor units, can contribute to the development of signs and symptoms of PPS. Our results also suggest the impact of socio-economic conditions on the risk of PPS.

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 categories and risk factors. We included 222 PD patients. Frequency of cancer was 6.8% for cases, 12.6% for controls. PD patients had a decreased risk for neoplasms (adjusted OR, 0.4; 95% confidence interval [CI], 0.2-0.7). Risk was reduced only for women (adjusted OR, 0.3; 95% CI, 0.1-0.7). PD patients had a decreased risk both for malignant (adjusted OR, 0.6; 95% CI, 0.1-2.5) and nonmalignant neoplasms (adjusted OR, 0.3; 95% CI, 0.1-0.7). Still, risk was decreased for endocrine-related tumors (adjusted OR, 0.3; 95% CI, 0.1-0.9) and non-endocrine-related tumors (adjusted OR, 0.4; 95% CI, 0.1-0.9). Our study confirms the inverse association between PD and neoplasms reported in previous epidemiologic studies.

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.

The operational and diagnostic criteria for migraine and all other headache disorders released in 1988 by the International Headache Society are universally considered reliable and exhaustive. These criteria, however, cannot be considered as satisfactory for population-based studies on migraine prevalence, especially if adolescents are the subjects of the study. Using these diagnostic criteria, we conducted an epidemiological study in order to assess the prevalence of migraine headache in a student population aged 11 to 14 years. Our survey made it possible to code IHS 1.1 (migraine without aura) in 2.35%, IHS 1.2 (migraine with aura) in 0.62%, IHS 1.7 (migrainous disorders not fulfilling migraine criteria) in 1.52%, and IHS 13 (headache not classifiable) in 1.38% of the examined pupils. In adolescents, the low prevalence estimates of migraine headache coded IHS 1.1 and the relatively high prevalence estimates of headaches coded IHS 1.7 and IHS 13 have appeared to be a consequence of the rigidity of some operational diagnostic criteria of the recent IHS classification rather than of the geographical, environmental, or socioeconomical pecularities of the cohort. Therefore, in order to improve the reliability and the exhaustiveness of the IHS classification by increasing its sensitivity, we believe that minor modifications of the diagnostic criteria are necessary. Within these revised criteria, the subitem “moderate or severe intensity” of pain headache should become mandatory, whereas the lower limit of the criterion “duration of pain” should be reduced to 1 hour.

Two cases of type I ACM are described, one of which presented with dizziness in late childhood (case 1), the other with mild intention tremor in adulthood (case 2). Cerebellar ectopia should be considered in monosymptomatic patients even in the absence of other symptoms and signs of C.N.S. dysfunction. Magnetic resonance imaging of the craniocervical junction should be performed because it may be diagnostic for type I ACM.

The influence of the ventral tegmental area (VTA) (dopaminergic A10 group neurons) on flight behaviour, induced by hypothalamic stimulation, was studied in the cat. Co-stimulation of hypothalamus and VTA (ipsi- or contralateral) induced an increase of the flight latency. Slow-motion analysis of flight behaviour showed that this increase was due to the augmentation of the fixation latency (FL) whereas the upright latency (UL) was not modified. Sulpiride injection (50 mg/kg i.p.) provoked the disappearance of VTA effect without affecting the basal behavioural sequence. The results show that DA A10 group neurons increase the attentive component of the flight reaction, suggesting a possible influence of the DA A10 system on the mechanisms modulating focused attention in the animal.

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 pair univariate analysis and conditional logistic regression analyses were used to calculate adjusted odds ratio (OR), 95% CI, and two-tailed p values for the investigated variables. Results: PD was significantly associated with a fertile life length shorter than 36 years (OR 2.07; 95% CI 1.00 to 4.30) and a cumulative length of pregnancies longer than 30 months (OR 2.19; 95% CI 1.22 to 3.91). An inverse association between PD and surgical menopause (adjusted OR 0.30; 95% CI 0.13 to 0.77) was also found. Conclusions: An association between factors reducing estrogen stimulation during life and PD was found. These results support the hypothesis that endogenous estrogens play a role in the development of PD.