OBJECTIVES:To evaluate non-motor symptoms (NMS) occurring during ON pharmacological state and validate a new questionnaire, the Non-motor symptoms-ON scale (NoMoS-ON), exploring ON NMS in Parkinson's disease (PD). MATERIAL AND METHODS:Patients with PD were evaluated by a new questionnaire, the NoMoS-ON scale, evaluating 17 items related to the main symptoms experienced during the ON state. PD patients who experienced at least one symptom in ON were defined ON-NMS+. Internal consistency and test-retest reliability of NoMoS-ON scale were also assessed. RESULTS:One-hundred and thirty-seven PD patients were consecutively enrolled (79 men and 58 women, age 69.4 ± 9.5 years (mean ± SD)). Seventy-seven patients were ON-NMS+ (56.6 %). PD patients with short disease duration (<7 years) showed the presence of unpleasant NMS: "sleepiness", "light-headedness", "nausea/vomiting". PD patients with longer disease duration experienced pleasant non-motor features including "feel lot of energy", "feel physical well-being". ON-NMS+ were also associated with female gender (OR 2.81, 95%CI 1.37-5.77, p-value 0.005) and with motor fluctuations (OR 2.41, 95%CI 1.20-4.83, p-value 0.013). Cronbach's alpha was 0.61 and 5 items had adequate item-to-total correlations (r ≥ 0.40). Test-retest reliability was acceptable (intraclass correlation coefficient, ICC = 0.77). CONCLUSIONS:The NoMoS-ON scale is a valid, reproducible and reliable questionnaire capturing the ON NMS in PD. PD patients with disease duration shorter than 7 years showed the presence of unpleasant NMS whereas those with longer disease duration experienced pleasant non-motor features. This could help the physician in the therapy management of PD patients in different phases of their disease.

Contexte L'incidence de la sclérose en plaques (SEP) a progressivement augmenté en Europe. Nous avons précédemment analysé les taux d'incidence de la SEP dans la ville de Catane sur une période de 30 ans (1975-2005), constatant une incidence croissante. L'objectif de cette étude est de décrire les taux d'incidence et les tendances temporelles de la SEP dans la ville de Catane de 1975 à 2020. Méthodes Des cas incidents de SEP ont été constatés à l'aide des registres des principaux centres MS de la municipalité de Catane. Les patients diagnostiqués avec les critères de Poser ou McDonald's, résidents dans la ville de Catane au début de la maladie, ont été recrutés. Le risque d'incidence annuel ajusté au début était basé sur l'année du début clinique. Des taux d'incidence annualisés par âge et par sexe ont été calculés. Résultats Au total, 718 cas incidents ont été diagnostiqués dans la ville de Catane. L'incidence a fortement augmenté, passant de 1,3/100 000 personnes-années au cours de la période 1975-1979 à 7,0/100 000 au cours du quinquennat 2000-2004 et montrant un plateau après avec des taux sensiblement stables de 2004 à 2020 avec un risque d'incidence brut annuel moyen de 7,3/100 000. Le rapport moyen femmes/hommes était de 1,6 (IC à 95 %, IC 1,4-1,8, p<0,0001), allant de 0,8 (IC à 95% 0,35-1,61) en 1975-1979 à 1,8 (IC à 95 % 1,2-2,8 p<0,003) en 2016-2020. L’âge moyen au début a augmenté régulièrement, passant de 23,9±8,6 ans entre 1975 et 1979 à 36,0±11,4 ans entre 2016 et 2020. Conclusion L'augmentation de l’âge d'apparition observée pourrait être le résultat soit d'une amélioration du diagnostic, soit de l'effet de plusieurs facteurs de risque environnementaux, dont la pertinence a changé au fil des années.

Contexte La sclérose en plaques (SEP) est définie comme une sclérose en plaques de début tardive (SEP-DT) lorsque l'apparition survient après 50 ans. Dans les cohortes de SEP, la prévalence varie de 0,6 % à 12 %, mais on sait peu sur l'incidence de la SEP-DT dans la population générale. Notre objectif est d’étudier l'incidence annuelle de la SEP-DT. Méthodes La vérification des cas a été réalisée à l'aide des registres de tous les centres SEP de la province de Catane. Les patients diagnostiqués selon les critères de McDonald (2005) ou des révisions ultérieures (2010, 2017), âgés de plus de 50 ans au début de la maladie, avec apparition entre 2005 et 2020 et résidant dans la province de Catane au moment de l'apparition, ont été recrutés. Les taux d'incidence (TI) ont été calculés pour toute la période d’étude, selon le sexe, les classes d’âge et un intervalle de cinq ans. Résultats Au total, 171 patients ont été identifiés (104 femmes ; 60,8 %). L’âge moyen d'apparition était de 56±6 ans. Le TI brut annuel moyen était de 2,7/100 000 personnes-années (intervalles de confiance à 95 %, IC 2,31-3,13). Le risque d'incidence global était assez stable pendant toute la période d’étude 2005-2020 (2,7/100 000). Néanmoins, nous avons observé une augmentation du risque dans le groupe âgé de 60 à 69 ans, passant de 1,12/100 000 personnes-années entre 2005 et 2010 à 3,12 entre 2016 et 2020. En comparant le dernier quinquennat 2016-2020 au premier 2005-2010, le RTI était de 2,79 (IC à 95 % 1,13-7,81 ; p=0,01). Conclusion Bien que cette incidence croissante chez les personnes âgées puisse être due à une meilleure précision du diagnostic ainsi qu'au vieillissement de la population générale, le rôle possible de facteurs environnementaux ne peut être exclu.

Gender is an important factor influencing epidemiological and clinical features of Parkinson’s disease (PD). We aimed to evaluate gender differences in the expression of a panel of miRNAs (miR-34a-5p, miR-146a, miR-155, miR-29a, miR-106a) possibly involved in the pathophysiology or progression of disease. Serum samples were obtained from 104 PD patients (58 men and 46 women) never treated with levodopa. We measured levels of miRNAs using quantitative PCR. Correlations between miRNA expression and clinical data were assessed using the Spearman’s correlation test. We used STRING to evaluate co-expression relationship among target genes. MiR-34a-5p was significantly upregulated in PD male patients compared to PD female patients (fc: 1.62; p < 0.0001). No correlation was found with age, BMI, and disease severity, assessed by UPDRS III scale, in male and female patients. MiR-146a-5p was significantly upregulated in female as compared to male patients (fc: 3.44; p < 0.0001) and a significant correlation was also observed between disease duration and mir-146a-5p. No differences were found in the expression of miR-29a, miR-106a-5p and miR-155 between genders. Predicted target genes for miR-34a-5p and miR-146-5p and protein interactions in biological processes were reported. Our study supports the hypothesis that there are gender-specific differences in serum miRNAs expression in PD patients. Follow-up of this cohort is needed to understand if these differences may affect disease progression and response to treatment.

Introduction dysautonomic dysfunction and cognitive impairment represent the most disabling non-motor features of Parkinson's Disease (PD). Recent evidences suggest the association between Orthostatic Hypotension (OH) and PD-Dementia. However, little is known on the interactions between cardiovascular dysautonomia and Mild Cognitive Impairment (MCI). We aimed to evaluate the association between cardiovascular dysautonomia and MCI in patients with PD. Methods: non-demented PD patients belonging to the PACOS cohort underwent a comprehensive instrumental neurovegetative assessment including the study of both parasympathetic and sympathetic function (30:15 ratio, Expiratory-Inspiratory ratio [E-I] and presence of Orthostatic Hypotension [OH]). Diagnosis of MCI was made according to the MDS criteria level H. Results: we enrolled 185 PD patients of whom 102 (55.1%) were men, mean age was 64.6 +/- 9.7 years, mean disease duration of 5.6 +/- 5.5 years with a mean UPDRS-ME score of 31.7 +/- 10.9. MCI was diagnosed in 79 (42.7%) patients. OH was recorded in 52 (28.1%) patients, altered 30:15 ratio was recorded in 39 (24.1%) patients and an altered E-I ratio was found in 24 (19.1%) patients. Presence of MCI was associated with an altered 30:15 ratio (adjOR 2.83; 95%CI 1.25-6.40) but not with an altered E-I ratio, while OH was associated only with the amnestic MCI subgroup (OR 2.43; 95% CI 1.05-5.06). Conclusion: in our study sample, MCI was mainly associated with parasympathetic dysfunction in PD.

Introduction: The UPDRS-IV represents the most common screening tool to assess motor fluctuations in patients with PD despite the lack of a clinimetric validation.Objectives: We evaluated sensitivity and specificity of UPDRS-IV using a 12-h waking-day motor assessment as the gold standard.Methods: We consecutively enrolled PD patients who underwent a 12-h waking-day motor assessment in the study. Patients were clinically evaluated every 2 h for 12 h using the UPDRS-III. Motor scores were reported as a line graph and six blinded raters classified patients as having or not having motor fluctuations. The UPDRS-IV was used in order to assess the presence of predictable and unpredictable motor fluctuations according to items 36-38.Results: Sixty two PD patients were enrolled in the study. According to the raters' evaluations, 39 (62.9%) were classified as having motor fluctuations, while according to the UPDRS-IV 47 (75.8%) presented a motor fluctuation giving a sensitivity of 87.2% (95%CI 72.6-95.7) and a specificity of 43.5% (95%CI 23.2-65.5).Conclusion: Our study results confirm the high level of sensitivity with a lower level of specificity of UPDRS-IV to screen motor fluctuations in PD patients. (C) 2016 Elsevier Ltd. All rights reserved.

We really appreciated the comments by Golimstok and Basalo on our article regarding the Obsessive Compulsive Personality Disorder (OCPeD) in Progressive Supranuclear Palsy (PSP), Multiple System Atrophy, Essential tremor and in healthy subjects. In our study we reported a frequency of OCPeD in PSP patients close to that previously reported in Parkinson's disease patients, thus suggesting the possibility of a common impairment of the basal ganglia network, likely involving the orbito-frontal circuits, shared by the two movement disorders. Such an involvement could explain the repetitive behavior shown by PSP patients, since the role of the orbito-frontal circuits in grooming behavior is universally recognized [ [1] Nicoletti A. Luca A. Luca M. Donzuso G. Mostile G. Raciti L. Contrafatto D. Dibilio V. Sciacca G. Cicero C.E. Vasta R. Petralia A. Zappia M. Obsessive compulsive personality disorder in progressive supranuclear palsy, multiple system atrophy and essential tremor. Park. Relat. Disord. 2016; 30: 36-39 Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar ]. In their letter, Golimstok and Basalo pointed out that among PSP patients there may be patients suffering from frontotemporal degeneration (FTD) or corticobasal degeneration (CBD). As reported in literature, in fact, an overlapping of symptoms exists among tauopathies, so that a considerable proportion of patients diagnosed as PSP are, after autopsy, re-classified as CBD patients. As stated in our study, due to the lack of a histological confirmation of the diagnosis, a possible mis-classification of some cases cannot be totally excluded. However all our PSP patients presented parkinsonism, vertical supranuclear palsy or slowing of vertical saccades and postural instability with falls within a year of disease onset fulfilling the widely accepted diagnostic criteria proposed by Litvan [ [2] Litvan I. Agid Y. Calne D. Campbell G. Dubois B. Duvoisin R.C. Goetz C.G. Golbe L.I. Grafman J. Growdon J.H. Hallett M. Jankovic J. Quinn N.P. Tolosa E. Zee D.S. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996; 47: 1-9 Crossref PubMed Scopus (2106) Google Scholar ]. Furthermore none of the enrolled patients had signs or symptoms addressing towards a possible diagnosis of CBS (limb dystonia, limb myoclonus, apraxia, cortical sensory deficit or alien limb phenomena). According to the clinical presentation none of our PSP patients fulfilled the diagnostic criteria for CBD [ [3] Armstrong M.J. Litvan I. Lang A.E. Bak T.H. Bhatia K.P. Borroni B.B. Adam L. Dickson D.W. Dennis W. Grossman M. Hallett M. Josephs K.A. Kertesz A. Lee S.E. Miller B.L. Reich S.G. Riley D.E. David E. Tolosa E. Tröster A.I. Vidailhet M. Weiner W.J. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013; 80: 496-503 Crossref PubMed Scopus (1022) Google Scholar ].

Introduction: aim of the study was to evaluate the presence of the Obsessive Compulsive Personality Disorder (OCPeD) in Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP) and Essential Tremor (ET) and in a group of healthy subjects.Methods: patients affected by MSA, PSP and ET diagnosed according to currently accepted diagnostic criteria and a group of healthy controls were enrolled in the study. Patients with cognitive impairment were excluded from the study. The Structured Clinical Interview for Personality Disorders-II (SCID-II) has been performed to evaluate the presence of personality disorders (PeDs). The diagnosis of OCPeD was confirmed by a psychiatric interview.Results: fifteen MSA patients (8 men and 7 women; aged 62.9 +/- 7.6 years), 14 PSP patients (8 men and 6 women; aged 69.8 +/- 4.4 years), 16 ET patients (10 men and 6 women; aged 70.4 +/- 6.4 years) and 20 healthy subjects (10 men and 10 women; aged 65.5 +/- 6.0 years) were enrolled. OCPeD was recorded in 5 (35.7%) PSP patients, 2 (13.3%) MSA patients, 2 (12.5%) ET patient and 2 (10%) controls.Conclusion: a low frequency of OCPeD, close to those recorded in healthy subjects, was recorded in both MSA and ET patients. Conversely an higher frequency of OCPeD, similar to PD was found among PSP patients, supporting the possibility of an impairment of common basal ganglia network possibly involving the orbito-frontal circuits. (C) 2016 Elsevier Ltd. All rights reserved.