159 results on '"Giovanna Calandra-Buonaura"'
Search Results
2. Ictal Bradycardia and Asystole in Sleep-Related Hypermotor Epilepsy: A Study of 200 Patients
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Lorenzo Muccioli, Giulia Bruschi, Lorenzo Ferri, Anna Scarabello, Lisa Taruffi, Lidia Di Vito, Barbara Mostacci, Federica Provini, Giovanna Calandra-Buonaura, Paolo Tinuper, Laura Licchetta, and Francesca Bisulli
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arrhythmia ,heart ,seizure ,polygraphy ,focal cortical dysplasia (FCD) ,MRI ,Medicine - Abstract
Background: Ictal bradycardia (IB) and asystole (IA) represent a rare but potentially harmful feature of epileptic seizures. The aim of this study was to study IB/IA in patients with sleep-related hypermotor epilepsy (SHE). Methods: We retrospectively included cases with video-EEG-confirmed SHE who attended our Institute up to January 2021. We reviewed the ictal polysomnography recordings focusing on ECG and identified cases with IB (R-R interval ≥ 2 s or a ≥10% decrease of baseline heart rate) and IA (R-R interval ≥ 4 s). Results: We included 200 patients (123 males, 61.5%), with a mean age of 42 ± 16 years. Twenty patients (20%) had focal cortical dysplasia (FCD) on brain MRI. Eighteen (out of 104 tested, 17.3%) carried pathogenic variants (mTOR pathway, n = 10, nAchR subunits, n = 4, KCNT1, n = 4). We identified IB/IA in four cases (2%): three had IA (mean 10 s) and one had IB. Three patients had FCD (left fronto-insular region, left amygdala, right mid-temporal gyrus) and two had pathogenic variants in DEPDC5; both features were more prevalent in patients with IB/IA than those without (p = 0.003 and p = 0.037, respectively). Conclusions: We identified IB/IA in 2% of patients with SHE and showed that this subgroup more frequently had FCD on brain MRI and pathogenic variants in genes related to the mTOR pathway.
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- 2024
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3. Case report: Bilateral double beta peak activity is influenced by stimulation, levodopa concentrations, and motor tasks, in a Parkinson’s disease patient on chronic deep brain stimulation
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Giulia Giannini, Luca Baldelli, Gaetano Leogrande, Ilaria Cani, Paolo Mantovani, Giovanna Lopane, Pietro Cortelli, Giovanna Calandra-Buonaura, and Alfredo Conti
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Parkinson’s disease ,deep brain stimulation ,local field potentials ,beta band frequency ,case report ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
IntroductionSubthalamic (STN) local field potentials (LFPs) in the beta band are considered potential biomarkers for closed-loop deep brain stimulation (DBS) in Parkinson’s disease (PD). The beta band is further dissected into low-and high-frequency components with somewhat different functions, although their concomitance and association in the single patient is far to be defined. We present a 56-year-old male PD patient undergoing DBS showing a double-beta peak activity on both sides. The aim of the study was to investigate how low-and high-beta peaks were influenced by plasma levodopa (L-dopa) levels, stimulation, and motor performances.MethodsA systematic evaluation of raw LFPs, plasma L-dopa levels, and motor tasks was performed in the following four conditions: OFF medications/ON stimulation, OFF medications/OFF stimulation, ON medications/OFF stimulation, and ON medications/ON stimulation.ResultsThe analysis of the LFP spectra suggests the following results: (1) the high-beta peak was suppressed by stimulation, while the low-beta peak showed a partial and not consistent response to stimulation; (2) the high-beta peak is also influenced by plasma L-dopa concentration, showing a progressive amplitude increment concordant with plasma L-dopa levels, while the low-beta peak shows a different behavir; and (3) motor performances seem to impact beta peaks behavior.ConclusionThis single exploratory case study illustrates a complex behavior of low-and high-beta peaks in a PD patient, in response to stimulation, L-dopa plasma levels, and motor performances. Our results suggest the importance to investigate patient-specific individual LFP patterns in view of upcoming closed-loop stimulation.
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- 2023
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4. Heart Rate Variability as a Tool for Seizure Prediction: A Scoping Review
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Federico Mason, Anna Scarabello, Lisa Taruffi, Elena Pasini, Giovanna Calandra-Buonaura, Luca Vignatelli, and Francesca Bisulli
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seizure detection ,seizure prediction ,heart rate variability ,epilepsy ,Medicine - Abstract
The most critical burden for People with Epilepsy (PwE) is represented by seizures, the unpredictability of which severely impacts quality of life. The design of real-time warning systems that can detect or even predict ictal events would enhance seizure management, leading to high benefits for PwE and their caregivers. In the past, various research works highlighted that seizure onset is anticipated by significant changes in autonomic cardiac control, which can be assessed through heart rate variability (HRV). This manuscript conducted a scoping review of the literature analyzing HRV-based methods for detecting or predicting ictal events. An initial search on the PubMed database returned 402 papers, 72 of which met the inclusion criteria and were included in the review. These results suggest that seizure detection is more accurate in neonatal and pediatric patients due to more significant autonomic modifications during the ictal transitions. In addition, conventional metrics are often incapable of capturing cardiac autonomic variations and should be replaced with more advanced methodologies, considering non-linear HRV features and machine learning tools for processing them. Finally, studies investigating wearable systems for heart monitoring denoted how HRV constitutes an efficient biomarker for seizure detection in patients presenting significant alterations in autonomic cardiac control during ictal events.
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- 2024
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5. Cognitive profile in idiopathic autonomic failure: relation with white matter hyperintensities and neurofilament levels
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Ilaria Cani, Luisa Sambati, Fiorina Bartiromo, Gian Maria Asioli, Simone Baiardi, Laura M. B. Belotti, Giulia Giannini, Pietro Guaraldi, Corinne Quadalti, Luciano Romano, Raffaele Lodi, Piero Parchi, Pietro Cortelli, Caterina Tonon, and Giovanna Calandra‐Buonaura
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Neurosciences. Biological psychiatry. Neuropsychiatry ,RC321-571 ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Abstract Objective To disclose the nature of cognitive deficits in a cohort of patients with idiopathic autonomic failure (IAF) by exploring the relation among cognitive functions, cardiovascular autonomic failure (AF) and clinical progression to another α‐synucleinopathy (phenoconversion). Methods We retrospectively identified all patients with a clinical diagnosis of IAF who underwent a comprehensive neuropsychological evaluation, clinical examination and cardiovascular autonomic tests from the IAF‐BO cohort. Brain magnetic resonance imaging (MRI) studies and cerebrospinal fluid (CSF) analysis, including neurofilament light chain (NfL), Alzheimer disease core biomarkers, and α‐synuclein seeding activity were further evaluated when available. Correlations among cognitive functions, clinical features, cardiovascular AF, cerebral white matter hyperintensities (WMH) load, and CSF biomarkers were estimated using Spearman correlation coefficient. Results Thirteen out of 30 (43%) patients with IAF displayed cognitive deficits (CI) mainly concerning executive functioning. Seven out of 30 (23%) met the criteria for mild cognitive impairment (MCI). The diagnosis of CI and MCI was not associated with phenoconversion or autonomic function parameters, including duration and severity of neurogenic orthostatic hypotension, presence and severity of supine hypertension, and nocturnal dipper profile. Twenty patients underwent a brain MRI and CSF analysis. MCI was related to WMH load (r = 0.549) and NfL levels (r = 0.656), while autonomic function parameters were not associated with either WMH or NfL levels. Interpretation Cardiovascular AF and phenoconversion, underlying the spreading of neurodegeneration to the central nervous system, were not independent drivers of cognitive dysfunction in IAF. We identified WMH load and NfL levels as potential biomarkers of the neural network disruption associated with cognitive impairment in patients with IAF.
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- 2022
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6. Early downregulation of hsa-miR-144-3p in serum from drug-naïve Parkinson’s disease patients
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Elisa Zago, Alessandra Dal Molin, Giovanna Maria Dimitri, Luciano Xumerle, Chiara Pirazzini, Maria Giulia Bacalini, Maria Giovanna Maturo, Tiago Azevedo, Simeon Spasov, Pilar Gómez-Garre, María Teresa Periñán, Silvia Jesús, Luca Baldelli, Luisa Sambati, Giovanna Calandra-Buonaura, Paolo Garagnani, Federica Provini, Pietro Cortelli, Pablo Mir, Claudia Trenkwalder, Brit Mollenhauer, Claudio Franceschi, Pietro Liò, Christine Nardini, and PROPAG-AGEING Consortium
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Medicine ,Science - Abstract
Abstract Advanced age represents one of the major risk factors for Parkinson’s Disease. Recent biomedical studies posit a role for microRNAs, also known to be remodelled during ageing. However, the relationship between microRNA remodelling and ageing in Parkinson’s Disease, has not been fully elucidated. Therefore, the aim of the present study is to unravel the relevance of microRNAs as biomarkers of Parkinson’s Disease within the ageing framework. We employed Next Generation Sequencing to profile serum microRNAs from samples informative for Parkinson’s Disease (recently diagnosed, drug-naïve) and healthy ageing (centenarians) plus healthy controls, age-matched with Parkinson’s Disease patients. Potential microRNA candidates markers, emerging from the combination of differential expression and network analyses, were further validated in an independent cohort including both drug-naïve and advanced Parkinson’s Disease patients, and healthy siblings of Parkinson’s Disease patients at higher genetic risk for developing the disease. While we did not find evidences of microRNAs co-regulated in Parkinson’s Disease and ageing, we report that hsa-miR-144-3p is consistently down-regulated in early Parkinson’s Disease patients. Moreover, interestingly, functional analysis revealed that hsa-miR-144-3p is involved in the regulation of coagulation, a process known to be altered in Parkinson’s Disease. Our results consistently show the down-regulation of hsa-mir144-3p in early Parkinson’s Disease, robustly confirmed across a variety of analytical and experimental analyses. These promising results ask for further research to unveil the functional details of the involvement of hsa-mir144-3p in Parkinson’s Disease.
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- 2022
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7. Data-driven clustering of combined Functional Motor Disorders based on the Italian registry
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Giovanni Mostile, Christian Geroin, Roberto Erro, Antonina Luca, Enrico Marcuzzo, Paolo Barone, Roberto Ceravolo, Sonia Mazzucchi, Andrea Pilotto, Alessandro Padovani, Luigi Michele Romito, Roberto Eleopra, Carlo Dallocchio, Carla Arbasino, Francesco Bono, Pietro Antonio Bruno, Benedetta Demartini, Orsola Gambini, Nicola Modugno, Enrica Olivola, Laura Bonanni, Alberto Albanese, Gina Ferrazzano, Rosa De Micco, Maurizio Zibetti, Giovanna Calandra-Buonaura, Martina Petracca, Francesca Morgante, Marcello Esposito, Antonio Pisani, Paolo Manganotti, Fabrizio Stocchi, Mario Coletti Moja, Ilaria Antonella Di Vico, Lucia Tesolin, Francesco De Bertoldi, Tommaso Ercoli, Giovanni Defazio, Mario Zappia, Alessandra Nicoletti, and Michele Tinazzi
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cluster analysis ,clinical phenotypes ,Functional Motor Disorders ,data-driven phenotyping ,functional neurological disorder ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
IntroductionFunctional Motor Disorders (FMDs) represent nosological entities with no clear phenotypic characterization, especially in patients with multiple (combined FMDs) motor manifestations. A data-driven approach using cluster analysis of clinical data has been proposed as an analytic method to obtain non-hierarchical unbiased classifications. The study aimed to identify clinical subtypes of combined FMDs using a data-driven approach to overcome possible limits related to “a priori” classifications and clinical overlapping.MethodsData were obtained by the Italian Registry of Functional Motor Disorders. Patients identified with multiple or “combined” FMDs by standardized clinical assessments were selected to be analyzed. Non-hierarchical cluster analysis was performed based on FMDs phenomenology. Multivariate analysis was then performed after adjustment for principal confounding variables.ResultsFrom a study population of n = 410 subjects with FMDs, we selected n = 188 subjects [women: 133 (70.7%); age: 47.9 ± 14.4 years; disease duration: 6.4 ± 7.7 years] presenting combined FMDs to be analyzed. Based on motor phenotype, two independent clusters were identified: Cluster C1 (n = 82; 43.6%) and Cluster C2 (n = 106; 56.4%). Cluster C1 was characterized by functional tremor plus parkinsonism as the main clinical phenotype. Cluster C2 mainly included subjects with functional weakness. Cluster C1 included older subjects suffering from anxiety who were more treated with botulinum toxin and antiepileptics. Cluster C2 included younger subjects referring to different associated symptoms, such as pain, headache, and visual disturbances, who were more treated with antidepressants.ConclusionUsing a data-driven approach of clinical data from the Italian registry, we differentiated clinical subtypes among combined FMDs to be validated by prospective studies.
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- 2022
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8. Neurofilament light chain and α-synuclein RT-QuIC as differential diagnostic biomarkers in parkinsonisms and related syndromes
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Corinne Quadalti, Giovanna Calandra-Buonaura, Simone Baiardi, Andrea Mastrangelo, Marcello Rossi, Corrado Zenesini, Giulia Giannini, Niccolò Candelise, Luisa Sambati, Barbara Polischi, Giuseppe Plazzi, Sabina Capellari, Pietro Cortelli, and Piero Parchi
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Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Abstract Neurofilament light chain (NfL) and α-synuclein oligomeric seeds (α-syn-s) are promising biomarkers for patients with parkinsonism. We assessed their performance in discriminating Parkinson disease (PD) from atypical parkinsonisms (APDs) and evaluated the association between NfL levels and clinical measures of disease severity. We measured NfL in cerebrospinal fluid (CSF) and/or plasma by immunoassays and α-syn-s in CSF by real-time quaking-induced conversion (RT-QuIC) in patients with PD (n = 153), multiple system atrophy (MSA) (n = 80), progressive supranuclear palsy/cortico-basal syndrome (PSP/CBS) (n = 58), dementia with Lewy bodies (n = 64), isolated REM-sleep behaviour disorder (n = 19), and isolated autonomic failure (n = 30). Measures of disease severity included disease duration, UPDRS-III score, Hoehn and Yahr stage, orthostatic hypotension, MMSE score, and CSF amyloid-beta profile. Both CSF NfL (cNfL) and plasma NfL (pNfL) levels were markedly elevated in APDs, and allowed differentiation with PD (vs. APDs, cNfL AUC 0.96; pNfL AUC 0.95; vs. MSA cNfL AUC 0.99; pNfL AUC 0.97; vs. PSP/CBS cNfL AUC 0.94; pNfL AUC 0.94). RT-QuIC detected α-syn-s in 91.4% of PD, but only 2.5% of APDs (all MSA). In PD/PDD, motor scales significantly correlated with cNfL levels. Although pNfL and both cNfL and α-syn-s accurately distinguished PD from APDs, the combined assessment of CSF markers provided a higher diagnostic value (PD vs. APDs AUC 0.97; vs. MSA AUC 0.97; vs. PSP/CBS AUC 0.99) than RT-QuIC alone (p = 0.047 vs. APDs; p = 0.002 vs MSA; p = 0.007 vs PSP/CBS), or cNfL alone (p = 0.011 vs. APDs; p = 0.751 vs MSA; p = 0.0001 vs. PSP/CBS). The results support the use of these assays in specialised clinics.
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- 2021
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9. Heterogeneity of prodromal Parkinson symptoms in siblings of Parkinson disease patients
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Luca Baldelli, Sebastian Schade, Silvia Jesús, Sebastian R. Schreglmann, Luisa Sambati, Pilar Gómez-Garre, Claire Halsband, Giovanna Calandra-Buonaura, Astrid Daniela Adarmes-Gómez, Friederike Sixel-Döring, Corrado Zenesini, Chiara Pirazzini, Paolo Garagnani, Maria Giulia Bacalini, Kailash P. Bhatia, Pietro Cortelli, Brit Mollenhauer, Claudio Franceschi, PROPAG-AGEING consortium, Pablo Mir, Claudia Trenkwalder, and Federica Provini
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Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Abstract A prodromal phase of Parkinson’s disease (PD) may precede motor manifestations by decades. PD patients’ siblings are at higher risk for PD, but the prevalence and distribution of prodromal symptoms are unknown. The study objectives were (1) to assess motor and non-motor features estimating prodromal PD probability in PD siblings recruited within the European PROPAG-AGEING project; (2) to compare motor and non-motor symptoms to the well-established DeNoPa cohort. 340 PD siblings from three sites (Bologna, Seville, Kassel/Goettingen) underwent clinical and neurological evaluations of PD markers. The German part of the cohort was compared with German de novo PD patients (dnPDs) and healthy controls (CTRs) from DeNoPa. Fifteen (4.4%) siblings presented with subtle signs of motor impairment, with MDS-UPDRS-III scores not clinically different from CTRs. Symptoms of orthostatic hypotension were present in 47 siblings (13.8%), no different to CTRs (p = 0.072). No differences were found for olfaction and overall cognition; German-siblings performed worse than CTRs in visuospatial-executive and language tasks. 3/147 siblings had video-polysomnography-confirmed REM sleep behavior disorder (RBD), none was positive on the RBD Screening Questionnaire. 173/300 siblings had
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- 2021
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10. The Indirect Impact of COVID-19 on Major Clinical Outcomes of People With Parkinson's Disease or Parkinsonism: A Cohort Study
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Luca Vignatelli, Flavia Baccari, Laura Maria Beatrice Belotti, Corrado Zenesini, Elisa Baldin, Giovanna Calandra-Buonaura, Pietro Cortelli, Carlo Descovich, Giulia Giannini, Maria Guarino, Giuseppe Loddo, Stefania Alessandra Nassetti, Luisa Sambati, Cesa Scaglione, Susanna Trombetti, Roberto D'Alessandro, and Francesco Nonino
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COVID-19 ,Parkinson's disease ,parkinsonism ,cohort studies ,physiotherapy ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
BackgroundThe indirect impact of the COVID-19 epidemic on major clinical outcomes of people with Parkinson's disease (PD) or other parkinsonism is unknown.ObjectivesThe study aimed to (1) describe changes in healthcare services during the first epidemic bout in people with PD or parkinsonism; (2) compare the occurrence of hospitalization for any PD-related major clinical outcomes in 2020 with 2019; (3) investigate the factors, including changes in healthcare services, associated with major clinical outcomes and death.MethodsAll healthcare services of the province of Bologna and major clinical outcomes were assessed through a record linkage study (ParkLink Bologna) using clinical data and health databases. Same analyses were performed in a random cohort of controls matched for age, sex, district of residence, and comorbidities with the ParkLink cohort (ratio of 1:10).ResultsA cohort of subjects with PD (759) or other parkinsonism (192) was included together with a cohort of controls (9,226). All indicators of healthcare services dropped at least below 50% during the lockdown period in all cohorts, mostly impacting physiotherapy in people with PD (−93%, 95% CI 88–96%). In 2020, compared to 2019, a three-fold risk of major injuries (RR 3.0, 95% CI 1.5–6.2) and infections (RR 3.3, 95% CI 1.5–7.2), excluding COVID-19, was observed only in people with PD, and neither in people with parkinsonism nor in controls. Decreased physiotherapy was associated with the occurrence of at least one major clinical outcome (OR 3.3, 95% CI 1.1–9.8) in people with PD. Experiencing at least one major clinical outcome was the strongest risk factor for death (OR 30.4, 95% CI 11.1–83.4) in people with PD.ConclusionsDuring the first COVID-19 epidemic peak, healthcare services were drastically reduced in a province of northern Italy, regardless of the disease condition. However, compared to 2019, in 2020, only people with PD had a higher risk of major clinical outcomes, that were associated with higher mortality. Strategies to maintain physical activity in people with PD should be implemented in possible future health emergencies.
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- 2022
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11. Diagnostic accuracy of quantitative susceptibility mapping in multiple system atrophy: The impact of echo time and the potential of histogram analysis
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Marta Lancione, Matteo Cencini, Mauro Costagli, Graziella Donatelli, Michela Tosetti, Giulia Giannini, Roberta Zangaglia, Giovanna Calandra-Buonaura, Claudio Pacchetti, Pietro Cortelli, and Mirco Cosottini
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Quantitative susceptibility mapping ,Echo-time-dependent QSM ,Multiple system atrophy ,QSM optimization ,QSM diagnostic accuracy ,Histogram analysis ,Computer applications to medicine. Medical informatics ,R858-859.7 ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
The non-invasive quantification of iron stores via Quantitative Susceptibility Mapping (QSM) could play an important role in the diagnosis and the differential diagnosis of atypical Parkinsonisms. However, the susceptibility (χ) values measured via QSM depend on echo time (TE). This effect relates to the microstructural organization within the voxel, whose composition can be altered by the disease. Moreover, pathological iron deposition in a brain area may not be spatially uniform, and conventional Region of Interest (ROI)-based analysis may fail in detecting alterations. Therefore, in this work we evaluated the impact of echo time on the diagnostic accuracy of QSM on a population of patients with Multiple System Atrophy (MSA) of either Parkinsonian (MSAp) or cerebellar (MSAc) phenotypes. In addition, we tested the potential of histogram analysis to improve QSM classification accuracy.We enrolled 32 patients (19 MSAp and 13 MSAc) and 16 healthy controls, who underwent a 7T MRI session including a gradient-recalled multi-echo sequence for χ mapping. Nine histogram features were extracted from the χ maps computed for each TE in atlas-based ROIs covering deep brain nuclei, and compared among groups.Alterations of susceptibility distribution were found in the Putamen, Substantia Nigra, Globus Pallidus and Caudate Nucleus for MSAp and in the Substantia Nigra and Dentate Nucleus for MSAc. Increased iron deposition was observed in a larger number of ROIs for the two shortest TEs and the standard deviation, the 75th and the 90th percentile were the most informative features yielding excellent diagnostic accuracy with area under the ROC curve > 0.9.In conclusion, short TEs may enhance QSM diagnostic performances, as they can capture variations in rapidly-decaying contributions of high χ sources. The analysis of histogram features allowed to reveal fine heterogeneities in the spatial distribution of susceptibility alteration, otherwise undetected by a simple evaluation of ROI χ mean values.
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- 2022
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12. REM Sleep Behaviour Disorder in Multiple System Atrophy: From Prodromal to Progression of Disease
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Giulia Giannini, Federica Provini, Pietro Cortelli, and Giovanna Calandra-Buonaura
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disease progression ,sleep disorders ,phenoconversion ,prodromic phase ,review ,autonomic failure ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
A higher frequency of motor and breathing sleep-related disorders in multiple system atrophy (MSA) populations is reported. REM sleep behaviour disorder (RBD) is one of the most robust markers of an underlying alpha-synucleinopathy. Although a large corpus of literature documented the higher prevalence of RBD in MSA, few studies have systematically investigated the prevalence of RBD as mode of disease onset and its role in disease progression. Moreover, there has been increasing interest in phenoconversion into synucleinopathies of cohorts of patients with isolated RBD (iRBD). Finally, some studies investigated RBD as predictive factor of conversion in isolated autonomic failure, a synucleinopathy presenting with autonomic failure as the sole clinical manifestation that could convert to a manifest central nervous system synucleinopathy. As the field of neurodegenerative disorders moves increasingly towards developing disease-modifying therapies, detecting individuals in the prodromal stage of these synucleinopathies becomes crucial. The aims of this review are to summarise (1) the prevalence of RBD during the course of MSA and as presenting feature of MSA (iRBD), (2) the RBD features in MSA, (3) MSA progression and prognosis in the subgroup of patients with RBD predating disease onset, and (4) the prevalence of MSA conversion in iRBD cohorts. Moreover, we summarise previous results on the role of RBD in the context of isolated autonomic failure as marker of phenoconversion to other synucleinopathies and, in particular, to MSA.
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- 2021
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13. Cognitive Profile and Its Evolution in a Cohort of Multiple System Atrophy Patients
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Luisa Sambati, Giovanna Calandra-Buonaura, Giulia Giannini, Ilaria Cani, Federica Provini, Roberto Poda, Federico Oppi, Michelangelo Stanzani Maserati, and Pietro Cortelli
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mild cognitive impairment ,multiple system atrophy (MSA) ,cognition ,neuropsychology ,dementia ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Introduction: Cognitive decline is not a characteristic feature of multiple system atrophy (MSA), but recent evidence suggests cognitive impairment as an integral part of the disease. We aim to describe the cognitive profile and its progression in a cohort of patients with MSA.Methods: We retrospectively selected patients referred to our department with a clinical diagnosis of MSA who were evaluated at least once a year during the course of the disease and underwent a comprehensive neuropsychological evaluation.Results: At the first evaluation (T0), 37 out of 60 patients (62%) were cognitively impaired, mainly (76%) in attention and executive functioning. Thirteen patients were impaired in one cognitive domain and 24 in more than one cognitive domain. Six out of the 24 had dementia. Twenty patients underwent a follow-up evaluation (T1) after a mean of 16.6 ± 9.3 months from the first evaluation (T0). Eight out of 20 patients were cognitively normal at both T0 and T1. Seven out of 12 patients presented with stable cognitive impairment at T1, while cognitive decline progressed in five patients. Patients with progression in cognitive decline performed significantly worse at T0 than cognitively stable patients. Education was significantly different between patients with and without cognitive impairment. No other differences in demographic and clinical variables and autonomic or sleep disturbances were found. Patients with dementia were older at disease onset and at T0 and had lower education and disease duration at T0 compared to those in other groups.Conclusions: In patients with MSA, we observed three different cognitive profiles: normal cognition, stable selective attention-executive deficits, and progressive cognitive deficits evolving to dementia. The detection of cognitive impairment in patients with suspected MSA suggests the need for comprehensive and longitudinal neuropsychological evaluation.
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- 2020
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14. Mathematical modeling and parameter estimation of levodopa motor response in patients with parkinson disease.
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Mauro Ursino, Elisa Magosso, Giovanna Lopane, Giovanna Calandra-Buonaura, Pietro Cortelli, and Manuela Contin
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Medicine ,Science - Abstract
Parkinson disease (PD) is characterized by a clear beneficial motor response to levodopa (LD) treatment. However, with disease progression and longer LD exposure, drug-related motor fluctuations usually occur. Recognition of the individual relationship between LD concentration and its effect may be difficult, due to the complexity and variability of the mechanisms involved. This work proposes an innovative procedure for the automatic estimation of LD pharmacokinetics and pharmacodynamics parameters, by a biologically-inspired mathematical model. An original issue, compared with previous similar studies, is that the model comprises not only a compartmental description of LD pharmacokinetics in plasma and its effect on the striatal neurons, but also a neurocomputational model of basal ganglia action selection. Parameter estimation was achieved on 26 patients (13 with stable and 13 with fluctuating LD response) to mimic plasma LD concentration and alternate finger tapping frequency along four hours after LD administration, automatically minimizing a cost function of the difference between simulated and clinical data points. Results show that individual data can be satisfactorily simulated in all patients and that significant differences exist in the estimated parameters between the two groups. Specifically, the drug removal rate from the effect compartment, and the Hill coefficient of the concentration-effect relationship were significantly higher in the fluctuating than in the stable group. The model, with individualized parameters, may be used to reach a deeper comprehension of the PD mechanisms, mimic the effect of medication, and, based on the predicted neural responses, plan the correct management and design innovative therapeutic procedures.
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- 2020
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15. L-Dopa Modulation of Brain Connectivity in Parkinson’s Disease Patients: A Pilot EEG-fMRI Study
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Stefania Evangelisti, Francesca Pittau, Claudia Testa, Giovanni Rizzo, Laura Ludovica Gramegna, Lorenzo Ferri, Ana Coito, Pietro Cortelli, Giovanna Calandra-Buonaura, Fabio Bisquoli, Claudio Bianchini, David Neil Manners, Lia Talozzi, Caterina Tonon, Raffaele Lodi, and Paolo Tinuper
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Parkinson’s disease ,EEG-fMRI ,functional connectivity ,L-dopa ,pilot study ,Neurosciences. Biological psychiatry. Neuropsychiatry ,RC321-571 - Abstract
Studies of functional neurosurgery and electroencephalography in Parkinson’s disease have demonstrated abnormally synchronous activity between basal ganglia and motor cortex. Functional neuroimaging studies investigated brain dysfunction during motor task or resting state and primarily have shown altered patterns of activation and connectivity for motor areas. L-dopa administration relatively normalized these functional alterations. The aim of this pilot study was to examine the effects of L-dopa administration on functional connectivity in early-stage PD, as revealed by simultaneous recording of functional magnetic resonance imaging (fMRI) and electroencephalographic (EEG) data. Six patients with diagnosis of probable PD underwent EEG-fMRI acquisitions (1.5 T MR scanner and 64-channel cap) before and immediately after the intake of L-dopa. Regions of interest in the primary motor and sensorimotor regions were used for resting state fMRI analysis. From the EEG data, weighted partial directed coherence was computed in the inverse space after the removal of gradient and cardioballistic artifacts. fMRI results showed that the intake of L-dopa increased functional connectivity within the sensorimotor network, and between motor areas and both attention and default mode networks. EEG connectivity among regions of the motor network did not change significantly, while regions of the default mode network showed a strong tendency to increase their outflow toward the rest of the brain. This pilot study provided a first insight into the potentiality of simultaneous EEG-fMRI acquisitions in PD patients, showing for both techniques the analogous direction of increased connectivity after L-dopa intake, mainly involving motor, dorsal attention and default mode networks.
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- 2019
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16. Accuracy of MR markers for differentiating Progressive Supranuclear Palsy from Parkinson's disease
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Stefano Zanigni, Giovanna Calandra-Buonaura, David Neil Manners, Claudia Testa, Dino Gibertoni, Stefania Evangelisti, Luisa Sambati, Maria Guarino, Patrizia De Massis, Laura Ludovica Gramegna, Claudio Bianchini, Paola Rucci, Pietro Cortelli, Raffaele Lodi, and Caterina Tonon
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Progressive Supranuclear Palsy ,Parkinson's disease ,MRI ,Morphometry ,DTI ,Computer applications to medicine. Medical informatics ,R858-859.7 ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Background: Advanced brain MR techniques are useful tools for differentiating Progressive Supranuclear Palsy from Parkinson's disease, although time-consuming and unlikely to be used all together in routine clinical work. We aimed to compare the diagnostic accuracy of quantitative morphometric, volumetric and DTI metrics for differentiating Progressive Supranuclear Palsy-Richardson's Syndrome from Parkinson's disease. Methods: 23 Progressive Supranuclear Palsy-Richardson's Syndrome and 42 Parkinson's disease patients underwent a standardized 1.5T brain MR protocol comprising high-resolution T1W1 and DTI sequences. Brainstem and cerebellar peduncles morphometry, automated volumetric analysis of brain deep gray matter and DTI metric analyses of specific brain structures were carried out. We determined diagnostic accuracy, sensitivity and specificity of MR-markers with respect to the clinical diagnosis by using univariate receiver operating characteristics curve analyses. Age-adjusted multivariate receiver operating characteristics analyses were then conducted including only MR-markers with a sensitivity and specificity exceeding 80%. Results: Morphometric markers (midbrain area, pons to midbrain area ratio and MR Parkinsonism Index), DTI parameters (infratentorial structures) and volumetric analysis (thalamus, putamen and pallidus nuclei) presented moderate to high diagnostic accuracy in discriminating Progressive Supranuclear Palsy-Richardson's Syndrome from Parkinson's disease, with midbrain area showing the highest diagnostic accuracy (99%) (mean ± standard deviation: 75.87 ± 16.95 mm2 vs 132.45 ± 20.94 mm2, respectively; p
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- 2016
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17. Iodine-123-meta-iodobenzylguanidine Myocardial Scintigraphy in Isolated Autonomic Failure: Potential Red Flag for Future Multiple System Atrophy
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Francesca Baschieri, Giovanna Calandra-Buonaura, Annagrazia Cecere, Giorgio Barletta, Manuela Contin, Piero Parchi, and Pietro Cortelli
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autonomic failure ,Parkinson’s disease ,multiple system atrophy ,Iodine-123-meta-iodobenzylguanidine myocardial scintigraphy ,orthostatic hypotension ,Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Pure autonomic failure is challenging as it can be the presenting feature of a central nervous system syncleinopathy such as Parkinson’s disease (PD) or multiple system atrophy (MSA). Because the prognosis of MSA and PD is so different, predictive features for a possible conversion can be extremely valuable. In this paper, we report three cases (two with autopsy-proven diagnosis) that had isolated AF for many years before converting to MSA or PD. Of all the tests that were performed during the premotor stage, Iodine-123-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy was predictive of the conversion to MSA. We suggest that MIBG myocardial scintigraphy, when performed in patients with isolated AF, may be a valuable predictor of conversion to MSA. On the contrary, the role of such test in parkinsonian patients irrespective of the presence of AF is still to be clarified.
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- 2017
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18. Rotigotine Objectively Improves Sleep in Parkinson’s Disease: An Open-Label Pilot Study with Actigraphic Recording
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Giovanna Calandra-Buonaura, Pietro Guaraldi, Andrea Doria, Stefano Zanigni, Stefania Nassetti, Valentina Favoni, Sabina Cevoli, Federica Provini, and Pietro Cortelli
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Neurology. Diseases of the nervous system ,RC346-429 - Abstract
Sleep disturbances represent important predictors of poor quality of life (QoL) in Parkinson’s disease (PD). This open-label pilot study aimed to objectively assess, by means of actigraphic recording, effect of rotigotine on sleep in PD patients with self-reported sleep complaints. 15 PD patients underwent one-week actigraphic recording before (T0) and during (T1) rotigotine treatment, which was titrated to the dose subjectively improving motor symptoms (4–8 mg/24 h). Sleep disturbances, daytime sleepiness, cognitive performance, QoL, and depression were also evaluated with questionnaires. Actigraphic recordings showed a significant reduction in nocturnal motor activity and mean duration of wake episodes after sleep onset during rotigotine treatment compared to baseline. In 10 patients presenting objective evidence of poor sleep quality at T0 (sleep efficiency ≤ 85%), rotigotine also significantly improved other sleep parameters and further reduced nocturnal motor activity and mean duration of wake episodes. A significant decrease in number and duration of daytime sleep episodes was also observed at T1. Finally we confirmed that rotigotine significantly improves perceived sleep quality and QoL. Our study showed for the first time that rotigotine is associated with an objective improvement of nocturnal and diurnal sleep disturbances in PD patients with self-reported sleep complaints. This study is registered with AIFA-observational study registry number 12021.
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- 2016
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19. Cognitive function in peripheral autonomic disorders.
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Pietro Guaraldi, Roberto Poda, Giovanna Calandra-Buonaura, Laura Solieri, Luisa Sambati, Roberto Gallassi, and Pietro Cortelli
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Medicine ,Science - Abstract
Objectiveaims of the current study were 1) to evaluate global cognitive function in patients with autonomic failure (AF) of peripheral origin and 2) to investigate the effect of a documented fall in blood pressure (BP) fulfilling the criteria for orthostatic hypotension (OH) on cognitive performances.Methodswe assessed 12 consecutive patients (10 males, 68±7 years old) with pure AF (PAF) or autoimmune autonomic neuropathy (AAN) and 12 age- and gender-matched controls. All patients had no clinical signs of central nervous system involvement and normal brain CT/MRI scan. Cognitive function was assessed on two consecutive days in 3 conditions: on day 1, while sitting, by means of a comprehensive battery of neuropsychological tests; on day 2, while tilted (HUT) and during supine rest (supine) in a randomized manner. BP and heart rate (HR) were continuously recorded non-invasively for the whole duration of the examination.Resultspatients with PAF or AAN displayed a preserved global cognitive function while sitting. However, compared to supine assessment, during HUT patients scored significantly worse during the Trail Making Test A and B, Barrage test, Analogies test, Immediate Visual Memory, Span Forward and Span Backward test. Pathological scores, with regard to Italian normative range values, were observed only during HUT in the Barrage test and in the Analogies test in 3 and 6 patients respectively. On the contrary, in healthy controls, results to neuropsychological tests were not significantly different, during HUT compared to supine rest.Conclusionsthese data demonstrate that patients with PAF and AAN present a normal sitting global cognitive evaluation. However, their executive functions worsen significantly during the orthostatic challenge, possibly because of transient frontal lobes hypoperfusion.
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- 2014
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20. Assessing Synergy/Redundancy of Baroreflex and Non-Baroreflex Components of the Cardiac Control during Sleep.
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Beatrice Cairo, Vlasta Bari, Beatrice De Maria, Emanuele Vaini, Pietro Guaraldi, Daniela Lucini, Massimo Pagani, Federica Provini, Giovanna Calandra-Buonaura, Pietro Cortelli, and Alberto Porta
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- 2019
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21. Screening for impulse control disorders in Parkinson’s disease and dopamine agonist use: a study of pharmacokinetic and psychological risk factors
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Manuela Contin, Giovanna Lopane, Luca Marini, Susan Mohamed, Luisa Sambati, Patrizia De Massis, Maria Guarino, Simonetta Sermi, Chiara Persichella, Pietro Cortelli, and Giovanna Calandra-Buonaura
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Psychiatry and Mental health ,Neurology (clinical) ,Dermatology ,General Medicine - Abstract
Dopamine agonist (DA) use is considered the main risk factor for impulse control disorder (ICD) development in Parkinson's disease (PD). Besides DAs, personality traits and cognitive features may represent risk factors for ICDs. The primary aim of this study was to investigate differences in DA plasma concentrations in PD patients with and without a positive screening for ICDs according to validated tools. The secondary aim was to compare the psychological profile between ICD positive and negative screened patients.PD patients receiving chronic DA therapy were screened for ICDs according to the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease (QUIP). Blood samples for measurement of DA (pramipexole, ropinirole, rotigotine) trough plasma concentrations were drawn in the morning, at mean 16-19 h from the last DA dose. Patients' psychological profile was investigated by Millon Clinical Multiaxal Inventory III and Barratt Impulsiveness Scale (BIS-11).One hundred and five PD patients were enrolled. Forty-one patients (39%) were QUIP positive, mainly for binge eating and hobbyism. Median plasma concentrations of pramipexole (n = 71, 66%), ropinirole (n = 21, 19%), and rotigotine (n = 16, 15%) were similar between QUIP positive and negative patients. QUIP positive patients showed higher motor impulsiveness (p = 0.04) and tended to higher total impulsiveness (p = 0.05).This is the first prospective study to evaluate the relationship between DA plasma concentrations and ICDs risk in PD patients. DA plasma levels were overlapping between QUIP positive and negative patients. BIS-11, particularly the motor impulsiveness subscale, might be a useful screening tool in PD patients eligible for DA therapy.
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- 2022
22. How resistant are levodopa‐resistant axial symptoms? Response of freezing, posture, and voice to increasing levodopa intestinal infusion rates in Parkinson disease
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Gabriele Imbalzano, Domiziana Rinaldi, Giovanna Calandra‐Buonaura, Manuela Contin, Federica Amato, Giulia Giannini, Luisa Sambati, Claudia Ledda, Alberto Romagnolo, Gabriella Olmo, Pietro Cortelli, Maurizio Zibetti, Leonardo Lopiano, Carlo Alberto Artusi, Imbalzano G., Rinaldi D., Calandra Buonaura G., Contin M., Amato F., Giannini G., Sambati L., Ledda C., Romagnolo A., Olmo G., Cortelli P., Zibetti M., Lopiano L., and Artusi C.A.
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Parkinson disease ,axial symptoms ,freezing of gait ,levodopa ,posture ,parkinson’s disease ,Neurology ,axial symptom ,Neurology (clinical) - Abstract
Background and purpose: Treatment of freezing of gait (FoG) and other Parkinson disease (PD) axial symptoms is challenging. Systematic assessments of axial symptoms at progressively increasing levodopa doses are lacking. We sought to analyze the resistance to high levodopa doses of FoG, posture, speech, and altered gait features presenting in daily-ON therapeutic condition. Methods: We performed a pre-/postinterventional study including patients treated with levodopa/carbidopa intestinal gel infusion (LCIG) with disabling FoG in daily-ON condition. Patients were evaluated at their usual LCIG infusion rate (T1), and 1 h after 1.5× (T2) and 2× (T3) increase of the LCIG infusion rate by quantitative outcome measures. The number of FoG episodes (primary outcome), posture, speech, and gait features were objectively quantified during a standardized test by a blinded rater. Changes in motor symptoms, dyskinesia, and plasma levodopa concentrations were also analyzed. Results: We evaluated 16 patients with a mean age of 69 ± 9.4 years and treated with LCIG for a mean of 2.2± 2.1 years. FoG improved in 83.3% of patients by increasing the levodopa doses. The number of FoG episodes significantly decreased (mean=2.3 at T1, 1.7 at T2, 1.2 at T3; p= 0.013). Posture and speech features did not show significant changes, whereas stride length (p= 0.049), turn duration (p= 0.001), and turn velocity (p= 0.024) significantly improved on doubling the levodopa infusion rate. Conclusions: In a short-term evaluation, the increase of LCIG dose can improve "dopa-resistant" FoG and gait issues in most advanced PD patients with overall good control of motor symptoms in the absence of clinically significant dyskinesia.
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- 2022
23. Wavelet analysis of the Valsalva maneuver: Methodology validation and application to pathological subjects.
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Ivan Corazza, Stefano Giancaterino, Giorgio Barletta, Annagrazia Cecere, Pietro Guaraldi, Giovanna Calandra-Buonaura, Romano Zannoli, and Pietro Cortelli
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- 2017
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24. Clinical autonomic nervous system laboratories in Europe: A joint survey of the European Academy of Neurology and the European Federation of Autonomic Societies: A joint survey of the European Academy of Neurology and the European Federation of Autonomic Societies
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Mario, Habek, Fabian, Leys, Magdalena, Krbot Skorić, Diogo, Reis Carneiro, Giovanna, Calandra-Buonaura, Jennifer, Camaradou, Giacomo, Chiaro, Pietro, Cortelli, Cristian, Falup-Pecurariu, Roberta, Granata, Pietro, Guaraldi, Raimund, Helbok, Max J, Hilz, Valeria, Iodice, Jens, Jordan, Evert C A, Kaal, Anita, Kamondi, Anne, Pavy Le Traon, Isabel, Rocha, Johann, Sellner, Jean Michel, Senard, Astrid, Terkelsen, Gregor K, Wenning, Thomas, Berger, Roland D, Thijs, Walter, Struhal, Alessandra, Fanciulli, and Werner J, Z'Graggen
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Autonomic Nervous System Diseases ,Neurology ,Surveys and Questionnaires ,Humans ,Laboratories ,Autonomic Nervous System - Abstract
Disorders of the autonomic nervous system (ANS) are common conditions, but it is unclear whether access to ANS healthcare provision is homogeneous across European countries. The aim of this study was to identify neurology-driven or interdisciplinary clinical ANS laboratories in Europe, describe their characteristics and explore regional differences.We contacted the European national ANS and neurological societies, as well as members of our professional network, to identify clinical ANS laboratories in each country and invite them to answer a web-based survey.We identified 84 laboratories in 22 countries and 46 (55%) answered the survey. All laboratories perform cardiovascular autonomic function tests, and 83% also perform sweat tests. Testing for catecholamines and autoantibodies are performed in 63% and 56% of laboratories, and epidermal nerve fiber density analysis in 63%. Each laboratory is staffed by a median of two consultants, one resident, one technician and one nurse. The median (interquartile range [IQR]) number of head-up tilt tests/laboratory/year is 105 (49-251). Reflex syncope and neurogenic orthostatic hypotension are the most frequently diagnosed cardiovascular ANS disorders. Thirty-five centers (76%) have an ANS outpatient clinic, with a median (IQR) of 200 (100-360) outpatient visits/year; 42 centers (91%) also offer inpatient care (median 20 [IQR 4-110] inpatient stays/year). Forty-one laboratories (89%) are involved in research activities. We observed a significant difference in the geographical distribution of ANS services among European regions: 11 out of 12 countries from North/West Europe have at least one ANS laboratory versus 11 out of 21 from South/East/Greater Europe (p = 0.021).This survey highlights disparities in the availability of healthcare services for people with ANS disorders across European countries, stressing the need for improved access to specialized care in South, East and Greater Europe.
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- 2022
25. Pervasive and diffuse muscle activity during REM sleep and non-REM sleep characterises multiple system atrophy in comparison with Parkinson's disease
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Alessandro Silvani, Luca Baldelli, Giulia Giannini, Pietro Guaraldi, Luisa Sambati, Annagrazia Cecere, Francesco Mignani, Pietro Cortelli, Giovanna Calandra‐Buonaura, and Federica Provini
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Behavioral Neuroscience ,Cognitive Neuroscience ,General Medicine - Abstract
Multiple system atrophy (MSA) and Parkinson's disease (PD) may share overlapping features particularly at early disease stage, including sleep alterations, but have profoundly different prognoses. Certain sleep phenomena and disorders of motor control are more prevalent in multiple system atrophy, such as REM sleep behaviour disorder (RBD). We quantitatively tested whether pervasive muscle activity during sleep occurs in subjects with multiple system atrophy versus Parkinson's disease. Laboratory polysomnographic studies were performed in 50 consecutive subjects with Parkinson's disease and 26 age- and gender-matched subjects with multiple system atrophy at5 years from disease onset. The distributions of normalised electromyographic activity of submentalis, wrist extensor, and tibialis anterior muscles in different wake-sleep states during the night were analysed. Subjects with multiple system atrophy had significantly higher activity of submentalis, wrist extensor, and tibialis anterior muscles than subjects with Parkinson's disease during non-REM sleep, including separately in stages N1, N2, and N3, and during REM sleep, but not during nocturnal wakefulness. The activity of wrist extensor and tibialis anterior muscles during non-REM sleep and the activity of tibialis anterior muscles during REM sleep were also significantly higher in subjects with multiple system atrophy and RBD than in subjects with Parkinson's disease and RBD. In conclusion, with respect to Parkinson's disease, multiple system atrophy is characterised by a pervasive and diffuse muscle overactivity that involves axial and limb muscles and occurs not only during REM sleep, but also during non-REM sleep and between subjects with comorbid RBD.
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- 2022
26. A new integrated instrumental approach to autonomic nervous system assessment.
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Ivan Corazza, Giorgio Barletta, Pietro Guaraldi, Annagrazia Cecere, Giovanna Calandra-Buonaura, E. Altini, Romano Zannoli, and Pietro Cortelli
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- 2014
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27. Autonomic dysfunction in progressive supranuclear palsy
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Francesca Baschieri, Maria Vitiello, Pietro Cortelli, Giovanna Calandra-Buonaura, Francesca Morgante, Baschieri F., Vitiello M., Cortelli P., Calandra Buonaura G., and Morgante F.
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Neurology ,Orthostatic hypotension ,Parkinsonian Disorders ,Photophobia ,Progressive supranuclear palsy ,Autonomic dysfunction ,Urinary incontinence ,Humans ,Neurodegenerative Diseases ,Neurology (clinical) ,Supranuclear Palsy, Progressive ,Primary Dysautonomias ,Autonomic Nervous System - Abstract
Background The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results. There is a need for a better characterization of autonomic dysfunction in PSP, to enhance our understanding of this highly disabling neurodegenerative disease including patients’ needs and possibly be of value for clinicians in the differential diagnosis among Parkinsonian syndromes. Methods We applied a systematic methodology to review existing literature on Pubmed regarding autonomic nervous system involvement in PSP. Results PSP reported quite frequently symptoms suggestive of autonomic dysfunction in all domains. Cardiovascular autonomic testing showed in some cases a certain degree of impairment (never severe). There was some evidence suggesting bladder dysfunction particularly in the storage phase. Dysphagia and constipation were the most common gastrointestinal symptoms. Instrumental tests seemed to confirm sudomotor and pupillomotor disturbances. Conclusions PSP patients frequently reported visceral symptoms, however objective testing showed that not always these reflected actual autonomic impairment. Further studies are needed to better delineate autonomic profile and its prognostic role in PSP.
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- 2022
28. Quantitative Assessment of Motor Response to a Low Subacute Levodopa Dose in the Differential Diagnosis of Parkinsonisms at Disease Onset: Data from the BoProPark Cohort
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Giovanna Calandra-Buonaura, Pietro Cortelli, Manuela Contin, Giovanna Lopane, Susan Mohamed, Luisa Sambati, Contin M., Lopane G., Cortelli P., Sambati L., Mohamed S., and Calandra Buonaura G.
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0301 basic medicine ,kinetics-dynamic ,Research Report ,Levodopa ,medicine.medical_specialty ,Parkinson's disease ,Gastroenterology ,Antiparkinson Agents ,Diagnosis, Differential ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Parkinsonian Disorders ,alternate finger tapping test ,Internal medicine ,medicine ,Humans ,kinetics-dynamics ,Prospective Studies ,Keywords: Levodopa ,Benserazide ,Receiver operating characteristic ,business.industry ,medicine.disease ,atypical parkinsonisms ,atypical parkinsonism ,Prospective Studie ,030104 developmental biology ,Carbidopa ,Antiparkinson Agent ,Finger tapping ,Cohort ,Parkinson’s disease ,Neurology (clinical) ,Differential diagnosis ,business ,030217 neurology & neurosurgery ,medicine.drug ,Human - Abstract
Background: Differential diagnosis between Parkinson’s disease (PD) and atypical parkinsonisms (APs) may be difficult at disease onset. The response to levodopa (LD) is a key supportive feature but its definition is largely empirical. Studies evaluating this issue by quantitative tests are scanty. Objective: We aimed to assess the utility of a subacute low LD dose kinetic-dynamic test in the differential diagnosis between PD and APs. It was applied at the baseline of a prospective follow-up in patients with parkinsonian signs within three years of disease motor onset (“BoProPark” cohort) and eventually diagnosed as PD or APs according to consensus criteria. Methods: Patients under at least 3-month LD therapy received a first morning fasting dose of LD/benserazide or carbidopa (100/25 mg) and underwent simultaneous serial assessments of plasma LD concentration and alternate finger tapping frequency up to 3 h. The main outcome was the extent of LD motor response, calculated by the area under the 3 h tapping effect–time curve (AUC_ETap). A receiver operating characteristic (ROC) curve analysis was performed to establish the optimal AUC_ETap cut-off to differentiate PD and APs. Results: The first 100 consecutive “BoProPark” patients were analyzed. Forty-seven patients were classified as possible, 37 as probable PD and 16 as APs. AUC_ETap medians were similar in the PD subgroups but reduced to a third in APs (p 2186 [(tap/min) x min], with a sensitivity of 92% and a specificity of 75%. Accuracy of the test was 0.85 (95% CI 0.74–0.95), p
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- 2021
29. Testing cardiovascular autonomic function in the COVID-19 era: lessons from Bologna’s Autonomic Unit
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Francesca Baschieri, Pietro Cortelli, Federica Provini, Pietro Guaraldi, Giovanna Calandra-Buonaura, Giorgio Barletta, Guaraldi P., Barletta G., Baschieri F., Calandra Buonaura G., Provini F., and Cortelli P.
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Autonomic function ,Coronavirus disease 2019 (COVID-19) ,Health Personnel ,Coronaviru ,Pneumonia, Viral ,Clinical Neurology ,Diagnostic Techniques, Cardiovascular ,Autonomic unit ,Autonomic Nervous System ,Unit (housing) ,Autonomic units ,Betacoronavirus ,Pandemic ,Health care ,medicine ,Humans ,Personal protective equipment ,Pandemics ,Personal Protective Equipment ,Coronavirus Infection ,Endocrine and Autonomic Systems ,business.industry ,SARS-CoV-2 ,International health ,COVID-19 ,medicine.disease ,Coronavirus ,Italy ,Cardiovascular autonomic testing ,Test performance ,Neurology (clinical) ,Medical emergency ,business ,Coronavirus Infections ,Human ,Research Article - Abstract
The coronavirus disease 2019 (COVID-19) pandemic has changed the way most medical procedures are performed. Autonomic units, as well as other healthcare sectors, are required to undergo a thorough reorganization of the protocols in order to guarantee the safety of patients and healthcare staff. Cardiovascular autonomic function testing (CAFT) is necessary in certain situations; however, it poses several concerns which need to be addressed. Here, we provide some practical advice based on current national and international health authorities’ recommendations and our experience about how to perform CAFT during the COVID-19 emergency. We examine aspects regarding patients, healthcare staff, laboratory preparation, and test performance.
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- 2020
30. Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies
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Simone Baiardi, Niccolò Candelise, Anna Ladogana, Sabina Capellari, Byron Caughey, Elena Antelmi, Giovanna Calandra-Buonaura, Piero Parchi, Giuseppe Plazzi, Giulia Giannini, Andrew G. Hughson, Marcello Rossi, Christina D. Orrù, Angela Mammana, Pietro Cortelli, Rossi M., Candelise N., Baiardi S., Capellari S., Giannini G., Orru C.D., Antelmi E., Mammana A., Hughson A.G., Calandra-Buonaura G., Ladogana A., Plazzi G., Cortelli P., Caughey B., and Parchi P.
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Lewy Body Disease ,Pathology ,medicine.medical_specialty ,Parkinson's disease ,Synucleinopathies ,diagnosis ,prion disease ,Prion disease ,multiple system atrophy ,Sensitivity and Specificity ,REM sleep behavior disorder ,Pathology and Forensic Medicine ,Cellular and Molecular Neuroscience ,Biomarker ,Diagnosis ,Multiple system atrophy ,Parkinson’s disease ,α-Synuclein ,mental disorders ,medicine ,Humans ,Dementia ,Cognitive decline ,Original Paper ,Lewy body ,business.industry ,Dementia with Lewy bodies ,Parkinsonism ,Correction ,medicine.disease ,nervous system diseases ,Spectrometry, Fluorescence ,alpha-Synuclein ,biomarker ,Neurology (clinical) ,business ,Diagnosi - Abstract
The clinical diagnosis of synucleinopathies, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), is challenging, especially at an early disease stage, due to the heterogeneous and often non-specific clinical manifestations. The discovery of reliable specific markers for synucleinopathies would consequently be of great aid to the diagnosis and management of these disorders. Real-Time Quaking-Induced Conversion (RT-QuIC) is an ultrasensitive technique that has been previously used to detect self-templating amyloidogenic proteins in the cerebrospinal fluid (CSF) and other biospecimens in prion disease and synucleinopathies. Using a wild-type recombinant α-synuclein as a substrate, we applied RT-QuIC to a large cohort of 439 CSF samples from clinically well-characterized, or post-mortem verified patients with parkinsonism or dementia. Of significance, we also studied patients with isolated REM sleep behavior disorder (iRBD) (n = 18) and pure autonomic failure (PAF) (n = 28), representing clinical syndromes that are often caused by a synucleinopathy, and may precede the appearance of parkinsonism or cognitive decline. The results show that our RT-QuIC assay can accurately detect α-synuclein seeding activity across the spectrum of Lewy Body (LB)-related disorders (LBD), including DLB, PD, iRBD, and PAF, with an overall sensitivity of 95.3%. In contrast, all but two patients with MSA showed no α-synuclein seeding activity in the applied experimental setting. The analysis of the fluorescence response reflecting the amount of α-synuclein seeds revealed no significant differences between the clinical syndromes associated with LB pathology. Finally, the assay demonstrated 98% specificity in a neuropathological cohort of 101 cases lacking LB pathology. In conclusion, α-synuclein RT-QuIC provides an accurate marker of synucleinopathies linked to LB pathology and may have a pivotal role in the early discrimination and management of affected patients. The finding of no α-synuclein seeding activity in MSA seems to support the current view that MSA and LBD are associated with different conformational strains of α-synuclein. Electronic supplementary material The online version of this article (10.1007/s00401-020-02160-8) contains supplementary material, which is available to authorized users.
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- 2020
31. Progression and prognosis in multiple system atrophy presenting with REM behavior disorder
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Annagrazia Cecere, Federica Provini, Giorgio Barletta, Pietro Guaraldi, Giulia Giannini, Andrea Droghini, Francesco Mignani, Giovanna Calandra-Buonaura, Vincenzo Mastrangelo, Pietro Cortelli, Giannini G., Mastrangelo V., Provini F., Droghini A., Cecere A., Barletta G., Mignani F., Guaraldi P., Cortelli P., and Calandra Buonaura G.
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Male ,Pediatrics ,medicine.medical_specialty ,Stridor ,REM Sleep Behavior Disorder ,Disease ,REM sleep behavior disorder ,03 medical and health sciences ,Orthostatic vital signs ,0302 clinical medicine ,Atrophy ,Prevalence ,medicine ,Humans ,Aged ,Retrospective Studies ,030214 geriatrics ,business.industry ,Parkinsonism ,Retrospective cohort study ,Middle Aged ,Multiple System Atrophy ,Prognosis ,medicine.disease ,Disease Presentation ,Disease Progression ,Female ,Neurology (clinical) ,Multiple system atrophy Autonomic diseases Cohort studies Natural history studies (prognosis) REM sleep behaviour disorder ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
ObjectivesTo investigate (1) the prevalence of REM sleep behavior disorder (RBD) as mode of disease onset in a cohort of patients with multiple system atrophy (MSA) and (2) disease progression and prognosis in patients with MSA with RBD predating (pre-RBD) and following (post-RBD) disease onset.MethodsWe retrospectively identified all patients with a clinical diagnosis of MSA evaluated at least once a year during the disease course. Type of onset was defined by the first reported motor or autonomic symptom/sign related to MSA. The occurrence of symptoms/signs and milestone of disease progression, and their latency from disease onset, were collected. Survival data were calculated. RBD was confirmed by video-polysomnography.ResultsOf a total of 158 patients, pre-RBD represented the mode of disease onset in 27% of patients, preceding disease onset according to the international criteria with a median of 3 (2–5) years. Comparing pre-RBD and post-RBD patients, the first group showed an increased prevalence of autonomic onset of disease, a reduced prevalence of parkinsonism, an earlier onset of stridor, pyramidal signs, symptomatic orthostatic hypotension, urinary dysfunction, severe dysphagia, and wheelchair dependency. The risk of death was higher in patients with pre-RBD.ConclusionsIn our MSA cohort, RBD represented the most frequent mode of disease presentation. A more rapid progression of disease was observed in the pre-RBD group. These findings suggested a careful assessment of sleep disorders to early recognize RBD and a closer follow-up of autonomic dysfunction and stridor in patients with pre-RBD.
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- 2020
32. The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy
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Gregor K. Wenning, Iva Stankovic, Luca Vignatelli, Alessandra Fanciulli, Giovanna Calandra‐Buonaura, Klaus Seppi, Jose‐Alberto Palma, Wassilios G. Meissner, Florian Krismer, Daniela Berg, Pietro Cortelli, Roy Freeman, Glenda Halliday, Günter Höglinger, Anthony Lang, Helen Ling, Irene Litvan, Phillip Low, Yasuo Miki, Jalesh Panicker, Maria Teresa Pellecchia, Niall Quinn, Ryuji Sakakibara, Maria Stamelou, Eduardo Tolosa, Shoji Tsuji, Tom Warner, Werner Poewe, Horacio Kaufmann, Wenning, Gregor K, Stankovic, Iva, Vignatelli, Luca, Fanciulli, Alessandra, Calandra-Buonaura, Giovanna, Seppi, Klau, Palma, Jose-Alberto, Meissner, Wassilios G, Krismer, Florian, Berg, Daniela, Cortelli, Pietro, Freeman, Roy, Halliday, Glenda, Höglinger, Günter, Lang, Anthony, Ling, Helen, Litvan, Irene, Low, Phillip, Miki, Yasuo, Panicker, Jalesh, Pellecchia, Maria Teresa, Quinn, Niall, Sakakibara, Ryuji, Stamelou, Maria, Tolosa, Eduardo, Tsuji, Shoji, Warner, Tom, Poewe, Werner, and Kaufmann, Horacio
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Consensus ,diagnosis ,Clinical Sciences ,multiple system atrophy ,Consensu ,Rare Diseases ,pathology [Brain] ,pathology [Multiple System Atrophy] ,Humans ,ddc:610 ,Prospective Studies ,screening and diagnosis ,Neurology & Neurosurgery ,Prevention ,Neurosciences ,Brain ,Human Movement and Sports Sciences ,Multiple System Atrophy ,diagnostic criteria ,Magnetic Resonance Imaging ,Brain Disorders ,4.1 Discovery and preclinical testing of markers and technologies ,Detection ,diagnosi ,Prospective Studie ,diagnosis [Multiple System Atrophy] ,Neurology ,Neurology (clinical) ,4.2 Evaluation of markers and technologies ,Human - Abstract
BackgroundThe second consensus criteria for the diagnosis of multiple system atrophy (MSA) are widely recognized as the reference standard for clinical research, but lack sensitivity to diagnose the disease at early stages.ObjectiveTo develop novel Movement Disorder Society (MDS) criteria for MSA diagnosis using an evidence-based and consensus-based methodology.MethodsWe identified shortcomings of the second consensus criteria for MSA diagnosis and conducted a systematic literature review to answer predefined questions on clinical presentation and diagnostic tools relevant for MSA diagnosis. The criteria were developed and later optimized using two Delphi rounds within the MSA Criteria Revision Task Force, a survey for MDS membership, and a virtual Consensus Conference.ResultsThe criteria for neuropathologically established MSA remain unchanged. For a clinical MSA diagnosis a new category of clinically established MSA is introduced, aiming for maximum specificity with acceptable sensitivity. A category of clinically probable MSA is defined to enhance sensitivity while maintaining specificity. A research category of possible prodromal MSA is designed to capture patients in the earliest stages when symptoms and signs are present, but do not meet the threshold for clinically established or clinically probable MSA. Brain magnetic resonance imaging markers suggestive of MSA are required for the diagnosis of clinically established MSA. The number of research biomarkers that support all clinical diagnostic categories will likely grow.ConclusionsThis set of MDS MSA diagnostic criteria aims at improving the diagnostic accuracy, particularly in early disease stages. It requires validation in a prospective clinical and a clinicopathological study. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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- 2022
33. Tracheostomy is associated with increased survival in Multiple System Atrophy patients with stridor
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Giulia Giannini, Federica Provini, Ilaria Cani, Annagrazia Cecere, Francesco Mignani, Pietro Guaraldi, Cristian Vincenzo Francesco Di Mirto, Pietro Cortelli, Giovanna Calandra‐Buonaura, Giannini, Giulia, Provini, Federica, Cani, Ilaria, Cecere, Annagrazia, Mignani, Francesco, Guaraldi, Pietro, Di Mirto, Cristian Vincenzo Francesco, Cortelli, Pietro, and Calandra-Buonaura, Giovanna
- Subjects
Male ,Multiple System Atrophy ,stridor ,Follow-Up Studie ,Cohort Studies ,Prospective Studie ,Tracheostomy ,Neurology ,Retrospective Studie ,cohort study ,Humans ,Neurology (clinical) ,movement disorder ,Prospective Studies ,Cohort Studie ,Respiratory Sound ,prognosi ,Human ,Follow-Up Studies ,Respiratory Sounds ,Retrospective Studies - Abstract
Stridor treatment in multiple system atrophy (MSA) mainly comprises tracheostomy or continuous positive airway pressure (CPAP), but guidelines for the use of these treatments are lacking. The aim of the study was to evaluate the predictive value of stridor treatment in an MSA cohort.This is a retrospective and prospective monocentric cohort study including MSA patients evaluated at least once a year during the disease course. Stridor was video-polysomnography confirmed. The time of stridor treatment (CPAP or tracheostomy) and latency from stridor onset were collected. Survival and predictors of survival were calculated.A total of 182 (107 males, mean age at disease onset 57.3 ± 8.4 years) MSA patients were included in the study; 141 were deceased at the time of study. Of the total sample, 75 patients were diagnosed with stridor: 22 patients were treated with tracheostomy and 29 with CPAP, whilst 24 patients did not receive treatment. Treatment with tracheostomy showed longer survival compared with both treatment with CPAP or no treatment (incidence rate of death 12 vs. 21 vs. 23 per 100 person-years, respectively). Tracheostomy remained an independent factor associated with longer survival (hazard ratio 0.38, p = 0.029), also after adjustment for other confounders and latency for stridor treatment.This is the largest monocentric and long-term follow-up study comparing survival between tracheostomy and CPAP in MSA patients with stridor. Treatment with tracheostomy showed longer survival compared with both treatment with CPAP or no treatment. A careful multidisciplinary approach is required for the management of MSA patients with stridor.
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- 2022
34. Disorders of arousal in 4 older men: evidence from clinical practice
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Giorgio Buzzi, Greta Mainieri, Giovanna Calandra-Buonaura, Federica Fragiacomo, Susanna Mondini, Federica Provini, Giuseppe Loddo, Loddo G., Fragiacomo F., Mainieri G., Mondini S., Buzzi G., Calandra Buonaura G., and Provini F.
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Adult ,Male ,Pulmonary and Respiratory Medicine ,sleepwalking ,Polysomnography ,Somnambulism ,REM sleep behavior disorder ,Night Terrors ,Nocturnal ,confusional arousal ,Confusional arousal ,Arousal ,sleep terror ,disorders of arousal ,medicine ,Humans ,Aged ,business.industry ,parasomnia ,Sleep terror ,medicine.disease ,Scientific Investigations ,Clinical Practice ,Neurology ,Sleepwalking ,Sleep Arousal Disorders ,Neurology (clinical) ,business ,Clinical psychology - Abstract
STUDY OBJECTIVES: To describe clinical and video-polysomnographic features of disorders of arousal (DoA) in older adults. METHODS: Four consecutive male patients with nocturnal motor behaviors underwent a clinical interview, neurologic examination, laboratory tests, brain magnetic resonance imaging, and nocturnal in-laboratory or 24- to 48-hour home video polysomnography. The patients repeated an evaluation after 6 months of follow-up, including a 48-hour home video polysomnography in 2 patients. RESULTS: The patients were aged 65-72 years, and 1 patient has Parkinson disease. Sleep-related behavioral episodes had begun from 12-55 years before our observation. Three patients presented with a positive family history for DoA. Sleep motor episodes were described as suddenly raising the head or trunk, sitting in bed, screaming, speaking, gesturing, and sleepwalking (in 1 patient). When questioned, all patients seemed confused, rarely reporting any dream-like content. We recorded 25 DoA episodes of different intensity and complexity arising from nonrapid eye movement sleep. The semiology of the episodes was similar to that described in younger patients, consisting of sleep terrors and confusional arousals. All patients presented with physiological rapid eye movement sleep muscle atonia. Medication therapy reduced the frequency of the episodes in 2/4 patients. CONCLUSIONS: DoA may begin in adulthood and persist or arise in older adults, sometimes causing sleep-related injuries. Motor patterns of DoA in older adults are similar to those of younger patients. A combined clinical examination and video polysomnography recording are crucial in establishing a definitive diagnosis of nocturnal motor behavior in all older adults and especially in those affected by neurodegenerative diseases. CITATION: Loddo G, Fragiacomo F, Mainieri G, etal. Disorders of arousal in 4 older men: evidence from clinical practice. J Clin Sleep Med. 2022;18(1):129-136.
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- 2022
35. Do demographic and clinical features and comorbidities affect the risk of spread to an additional body site in functional motor disorders?
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Tommaso Ercoli, Michele Tinazzi, Christian Geroin, Enrico Marcuzzo, Roberto Erro, Sofia Cuoco, Roberto Ceravolo, Sonia Mazzucchi, Andrea Pilotto, Alessandro Padovani, Luigi Michele Romito, Roberto Eleopra, Mario Zappia, Alessandra Nicoletti, Carlo Dallocchio, Carla Arbasino, Francesco Bono, Giorgio Spano, Benedetta Demartini, Orsola Gambini, Nicola Modugno, Enrica Olivola, Laura Bonanni, Alberto Albanese, Gina Ferrazzano, Alessandro Tessitore, Leonardo Lopiano, Giovanna Calandra-Buonaura, Martina Petracca, Francesca Morgante, Marcello Esposito, Antonio Pisani, Paolo Manganotti, Lucia Tesolin, Francesco Teatini, Fabrizio Stocchi, Giovanni Defazio, Ercoli, Tommaso, Tinazzi, Michele, Geroin, Christian, Marcuzzo, Enrico, Erro, Roberto, Cuoco, Sofia, Ceravolo, Roberto, Mazzucchi, Sonia, Pilotto, Andrea, Padovani, Alessandro, Romito, Luigi Michele, Eleopra, Roberto, Zappia, Mario, Nicoletti, Alessandra, Dallocchio, Carlo, Arbasino, Carla, Bono, Francesco, Spano, Giorgio, Demartini, Benedetta, Gambini, Orsola, Modugno, Nicola, Olivola, Enrica, Bonanni, Laura, Albanese, Alberto, Ferrazzano, Gina, Tessitore, Alessandro, Lopiano, Leonardo, Calandra-Buonaura, Giovanna, Petracca, Martina, Morgante, Francesca, Esposito, Marcello, Pisani, Antonio, Manganotti, Paolo, Tesolin, Lucia, Teatini, Francesco, Stocchi, Fabrizio, and Defazio, Giovanni
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Phenotypic change ,Movement Disorders ,Spread ,Motor Disorders ,Functional motor disorders ,Functional neurological disorders ,Outcome ,Demography ,Humans ,Psychiatry and Mental health ,Neurology ,Neurology (clinical) ,Functional motor disorder ,Motor Disorder ,Functional neurological disorder ,Biological Psychiatry ,Human - Abstract
The aim of this study is to assess changes in the body distribution and the semeiology of functional motor disorder (FMD) in patients who reported only one or more than one body site affected at FMD onset. Data were obtained from the Italian Registry of Functional Motor Disorders, which included patients with a diagnosis of clinically definite FMDs. The relationship between FMD features and spread to other body sites was estimated by multivariate Cox regression analysis. We identified 201 (49%) patients who reported only one body site affected at FMD onset and 209 (51%) who reported multiple body sites affected at onset. FMD spread from the initial site to another site in 43/201 (21.4%) patients over 5.7 ± 7.1 years in those with only one site affected at FMD onset; FMD spread to an another body site in 29/209 (13.8%) over 5.5 ± 6.5 years. The spread of FMD was associated with non-motor functional symptoms and psychiatric comorbidities only in the patients with one body site affected at FMD onset. Our findings provide novel insight into the natural history of FMD. The number of body sites affected at onset does not seem to have a consistent influence on the risk of spread. Furthermore, our findings suggest that psychiatric comorbidities and non-motor functional symptoms may predict the spread of FMD symptoms, at least in patients with one body site affected at onset.
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- 2022
36. The Unified Multiple System Atrophy Rating Scale: Status, Critique, and Recommendations
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Florian Krismer, Jose‐Alberto Palma, Giovanna Calandra‐Buonaura, Iva Stankovic, Luca Vignatelli, Anna‐Karin Berger, Cristian Falup‐Pecurariu, Alexandra Foubert‐Samier, Günter Höglinger, Horacio Kaufmann, Larry Kellerman, Han‐Joon Kim, Thomas Klockgether, Johannes Levin, Pablo Martinez‐Martin, Tiago A. Mestre, Maria Teresa Pellecchia, Susan Perlman, Irfan Qureshi, Olivier Rascol, Anette Schrag, Klaus Seppi, Huifang Shang, Glenn T. Stebbins, Gregor K. Wenning, Wolfgang Singer, Wassilios G. Meissner, Krismer, Florian, Palma, Jose-Alberto, Calandra-Buonaura, Giovanna, Stankovic, Iva, Vignatelli, Luca, Berger, Anna-Karin, Falup-Pecurariu, Cristian, Foubert-Samier, Alexandra, Höglinger, Günter, Kaufmann, Horacio, Kellerman, Larry, Kim, Han-Joon, Klockgether, Thoma, Levin, Johanne, Martinez-Martin, Pablo, Mestre, Tiago A, Pellecchia, Maria Teresa, Perlman, Susan, Qureshi, Irfan, Rascol, Olivier, Schrag, Anette, Seppi, Klau, Shang, Huifang, Stebbins, Glenn T, Wenning, Gregor K, Singer, Wolfgang, and Meissner, Wassilios G
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Disability Evaluation ,Neurology ,diagnosis [Multiple System Atrophy] ,Humans ,Neurology (clinical) ,ddc:610 ,Multiple System Atrophy ,Severity of Illness Index ,MSA, SCALE, CRITIQUE, Recommendations - Abstract
The Unified Multiple System Atrophy (MSA) Rating Scale was developed to provide a surrogate marker of disease severity and clinical progression in patients with MSA. It is comprised of four subscales: UMSARS-I (12 items) rates patient-reported functional disability; UMSARS-II (14 items) assesses motor impairment based on a clinical examination; UMSARS-III records blood pressure and heart rate in the supine and standing positions; and UMSARS-IV (1 item) rates chore-based disability. Strengths of the UMSARS include its wide acceptance in the field, the comprehensive coverage of motor symptoms and its clinimetric properties (including reliability and validity). However, with its increasing use, potential areas of improvement in the UMSARS have become apparent. To address these limitations, a task force, involving clinicians, researchers, patient groups, and industry representatives, has recently been endorsed by the International Parkinson’s Disease and Movement Disorders Society. The present viewpoint summarizes strengths and weaknesses of the UMSARS and suggests a roadmap to develop an improved MSA clinical outcome assessment.
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- 2022
37. Functional gait disorders: Demographic and clinical correlations
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Christian Geroin, Benedetta Demartini, Alessandra Nicoletti, Gina Ferrazzano, Luigi Romito, Michele Tinazzi, Paolo Manganotti, Alessandro Padovani, Laura Bonanni, Alberto Albanese, Tommaso Ercoli, Roberto Ceravolo, Carla Arbasino, Elisabetta Zanolin, Giovanni Defazio, Leonardo Lopiano, Francesca Morgante, Roberto Erro, Nicola Modugno, Enrica Olivola, Marcello Esposito, Andrea Pilotto, Mario Zappia, Orsola Gambini, Enrico Marcuzzo, Carlo Dallocchio, Lucia Tesolin, Giuseppe Magro, Alessandro Tessitore, Sonia Mazzucchi, Fabrizio Stocchi, Francesco Bono, Martina Petracca, Sofia Cuoco, Antonio Pisani, Francesco Teatini, Roberto Eleopra, Giovanna Calandra-Buonaura, Tinazzi M., Pilotto A., Morgante F., Marcuzzo E., Cuoco S., Ceravolo R., Mazzucchi S., Padovani A., Romito L.M., Eleopra R., Nicoletti A., Dallocchio C., Arbasino C., Bono F., Magro G., Demartini B., Gambini O., Modugno N., Olivola E., Bonanni L., Zanolin E., Albanese A., Ferrazzano G., Tessitore A., Lopiano L., Calandra Buonaura G., Petracca M., Esposito M., Pisani A., Manganotti P., Tesolin L., Teatini F., Defazio G., Ercoli T., Stocchi F., Erro R., Zappia M., and Geroin C.
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Adult ,Male ,medicine.medical_specialty ,Slow gait ,Movement disorders ,Motor Disorders ,Internal medicine ,Knee-buckling ,medicine ,80 and over ,Neurologic ,Humans ,Gait disorders ,Gait Disorders ,Motor Disorder ,Functional gait disorder ,Astasia-abasia ,Gait Disorders, Neurologic ,Functional gait disorders ,Functional neurological disorders ,Aged ,Demography ,Aged, 80 and over ,Cross-Sectional Studie ,business.industry ,Cross-Sectional Studies ,Female ,Italy ,Middle Aged ,Regression Analysis ,Odds ratio ,Visual symptoms ,Gait ,Confidence interval ,Neurology ,Observational study ,Neurology (clinical) ,Geriatrics and Gerontology ,medicine.symptom ,business ,Functional neurological disorder ,Human - Abstract
Objective\ud We aimed to describe the prevalence and clinical-demographical features of patients with functional gait disorders (FGDs) and to compare them to patients with functional motor disorders (FMDs) without FGDs (No-FGDs).\ud \ud Methods\ud In this multicenter observational study, we enrolled patients with a clinically definite diagnosis of FMDs in 25 tertiary movement disorders centers in Italy. Each subject with FMDs underwent a comprehensive clinical assessment, including screening for different subtypes of functional gait disorders. Multivariate regression models were implemented in order to estimate the adjusted odds ratio (OR; 95% confidence interval) of having FGDs in relation to sociodemographic and clinical characteristics.\ud \ud Results\ud Out of 410 FMDs, 26.6% (n = 109) of patients exhibited FGDs. The most frequent FGDs were slow gait (n = 43, 39.4%), astasia-abasia (n = 26, 23.8%), and knee buckling (n = 24, 22%). They exhibited single FGDs in 51.4% (n = 56) or complex FGDs (more than one type of FGDs) in 48.6% (n = 53) of cases. On multivariate regression analysis, the presence of FGDs was more likely associated with older age (OR 1.03, 95% CI 1.01–1.04), functional visual symptoms (OR 2.19, 95% CI 1.08–4.45), and the diagnosis of somatic symptoms disorder (OR 2.97, 95% CI 1.08–8.17). FGDs were also more likely to undergo physiotherapy (OR 1.81, 95% CI 1.08–3.03).\ud \ud Conclusions\ud People with FMDs may present with different and overlapping types of FGDs, which may occur in older age. The association of FGDs with functional visual symptoms and somatic symptoms disorder opens up to new avenues to the understanding of the neural mechanisms of these disorders.
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- 2021
38. Neurogenic orthostatic hypotension in early stage Parkinson's disease: New insights from the first 105 patients of the BoProPark study
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Pietro Cortelli, Giorgio Barletta, Giovanna Calandra-Buonaura, Francesca Baschieri, Luisa Sambati, Pietro Guaraldi, Baschieri F., Sambati L., Guaraldi P., Barletta G., Cortelli P., and Calandra Buonaura G.
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Male ,medicine.medical_specialty ,Parkinson's disease ,Valsalva Maneuver ,Cardiovascular autonomic failure ,Diaphragmatic breathing ,Blood Pressure ,Disease ,Asymptomatic ,Orthostatic vital signs ,Hypotension, Orthostatic ,Tilt-Table Test ,Internal medicine ,Reflex ,medicine ,Humans ,Prospective Studies ,Prospective cohort study ,Pure autonomic failure ,Aged ,Orthostatic intolerance symptom ,Orthostatic hypotension ,Hand Strength ,business.industry ,Parkinson Disease ,Middle Aged ,medicine.disease ,Neurology ,Cardiovascular autonomic testing ,Cohort ,Standing Position ,Female ,Neurology (clinical) ,Geriatrics and Gerontology ,medicine.symptom ,business - Abstract
Objectives The prevalence of neurogenic orthostatic hypotension (NOH, due to cardiovascular autonomic failure) at early stage of Parkinson's disease (PD) is unknown. The aims of this study are to prospectively evaluate in a cohort of PD patients recruited within 3 years from motor onset (1) cardiovascular autonomic functions by means of cardiovascular reflex tests (CRTs) and the occurrence of NOH; (2) the frequency of orthostatic symptoms with a validated questionnaire. Methods We included the first 105 PD patients of the prospective “BoProPark” study. Each patient underwent CRTs (head up tilt test; Valsalva manoeuvre; deep breathing; cold face test and handgrip test) under continuous blood pressure monitoring according to standardized procedures and SCOPA-Aut questionnaire at baseline (T0) and after 16 months (T1). A group of 50 age- and sex-matched controls was used for comparison. Results At T0 (mean age 61 ± 9 years, disease duration 19 ± 9 months) NOH was detected in 4/105 (3.8%) patients, whereas at T1 in 8/105 (7.6%). CRTs responses assessing sympathetic function were impaired at T0 in PD patients compared to controls and progressively worsened at T1. Only 1 patient at T0 and 3 at T1 with NOH reported orthostatic symptoms with low frequency, while the majority of patients reporting these symptoms did not have OH at testing. Conclusions Our prospective study shows that NOH is not common at early PD stage. Asymptomatic mild sympathetic impairment was observed at first evaluation and progressed with disease evolution. Secondary OH may account for the higher prevalence of OH in PD reported so far.
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- 2021
39. A prospective evaluation of clinical and instrumental features before and after ventriculo-peritoneal shunt in patients with idiopathic Normal pressure hydrocephalus: The Bologna PRO-Hydro study
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Raffaele Agati, Vito Antonio Piserchia, Luca Albini-Riccioli, Monica Nicola, Stefania Magnoni, Federico Oppi, Sabina Capellari, Giulia Giannini, Antonella Supino, Alberto Ferrari, Tiziana Urli, Luisa Sambati, Giovanna Calandra-Buonaura, Margherita Merola, Sabina Cevoli, David Milletti, Piero Parchi, Anna Federica Marliani, Giorgio Palandri, Carmelo Lucio Sturiale, Pietro Cortelli, Lorenzo Chiari, Michelangelo Stanzani-Maserati, Paolo Mantovani, Giannini G., Palandri G., Ferrari A., Oppi F., Milletti D., Albini-Riccioli L., Mantovani P., Magnoni S., Chiari L., Cortelli P., Cevoli S., Agati R., Calandra-Buonaura G., Capellari S., Parchi P., Stanzani-Maserati M., Marliani A.F., Merola M., Piserchia V.A., Sambati L., Sturiale C., Supino A., Nicola M., and Urli T.
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Idiopathic Normal pressure hydrocephalu ,Male ,0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,Movement disorders ,Disease ,Clinical practice ,Spinal Puncture ,Ventriculoperitoneal Shunt ,Neuropsychological features ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Prospective Studies ,Ventriculo-peritoneal shunt ,Movement disorder ,Aged ,Ventriculo peritoneal shunt ,business.industry ,Patient Selection ,Neuropsychological feature ,Observational prospective study ,Neuropsychology ,Gait ,Hydrocephalus, Normal Pressure ,Shunting ,Treatment Outcome ,030104 developmental biology ,Neurology ,Gait analysis ,Idiopathic Normal pressure hydrocephalus ,(Idiopathic) normal pressure hydrocephalus ,Female ,Neurology (clinical) ,Geriatrics and Gerontology ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Introduction Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex and often misdiagnosed syndrome, whose major challenge is to identify which patients will benefit from surgery. Previous studies reported a variability in positive surgery response. The role of tap test(TT) in screening patients suitable for shunting is controversial. The primary aim of this study was to describe the clinical/instrumental features and their longitudinal progression after surgery in iNPH patients. Secondarily, we aimed to investigate the response of the three iNPH domains and the best time of outcome assessment after TT. Methods Patients compatible with iNPH underwent a 3-T-MRI and an inpatients program with TT including standardized clinical evaluations, neuropsychological assessments and instrumental gait analysis pre- and after-(24-h and 72-h) TT. The multidisciplinary team selected candidates for surgery. Patients were evaluated 6- and 12-months after surgery. Results A total of 154 consecutive patients were included from 2015 to 2018, 76 with an iNPH diagnosis (43 underwent surgery, 35 were evaluated after 6-months). Clinical and instrumented quantitative gait measures and urinary symptoms improved over time along with some neuropsychological functions. Concerning pre and post-TT analyses, the three iNPH domains showed a different response after TT, the delayed motor assessment was more appropriate than the early one and the instrumental measures highlighted the motor improvement. Conclusion: iNPH patients improved after surgery, when accurately selected. A multidisciplinary team focused on this disease and a standardized protocol helped in achieving a correct diagnosis and management of iNPH. Our results could impact the management of this disease.
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- 2019
40. Management of supine hypertension in patients with neurogenic orthostatic hypotension
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Gregor K. Wenning, Anne Pavy-Le Traon, Pietro Cortelli, Italo Biaggioni, Roland D. Thijs, Horacio Kaufmann, Hannes Reuter, Satish R. Raj, Alessandra Fanciulli, Giovanna Calandra-Buonaura, Max J. Hilz, Jens Jordan, Lucy Norcliffe-Kaufmann, Gert Mayer, Konstantinos Tsioufis, J. Gert van Dijk, David Robertson, Sabine Eschlboeck, Heinz Lahrmann, William P. Cheshire, Guido Grassi, Jens Tank, Giuseppe Mancia, Walter Struhal, Isabel Rocha, Jordan, J, Fanciulli, A, Tank, J, Calandra-Buonaura, G, Cheshire, W, Cortelli, P, Eschlboeck, S, Grassi, G, Hilz, M, Kaufmann, H, Lahrmann, H, Mancia, G, Mayer, G, Norcliffe-Kaufmann, L, Pavy-Le Traon, A, Raj, S, Robertson, D, Rocha, I, Reuter, H, Struhal, W, Thijs, R, Tsioufis, K, Gert van Dijk, J, Wenning, G, Biaggioni, I, Jordan J., Fanciulli A., Tank J., Calandra-Buonaura G., Cheshire W.P., Cortelli P., Eschlboeck S., Grassi G., Hilz M.J., Kaufmann H., Lahrmann H., Mancia G., Mayer G., Norcliffe-Kaufmann L., Pavy-Le Traon A., Raj S.R., Robertson D., Rocha I., Reuter H., Struhal W., Thijs R.D., Tsioufis K.P., Gert Van Dijk J., Wenning G.K., and Biaggioni I.
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medicine.medical_specialty ,Physiology ,Supine hypertension ,Disease ,030204 cardiovascular system & hematology ,Essential hypertension ,orthostatic hypotension ,Hypotension, Orthostatic ,03 medical and health sciences ,Orthostatic vital signs ,0302 clinical medicine ,Quality of life ,Internal medicine ,Supine Position ,Internal Medicine ,Humans ,Medicine ,In patient ,030212 general & internal medicine ,Pure autonomic failure ,Societies, Medical ,autonomic failure ,business.industry ,medicine.disease ,supine hypertension ,Hypertension ,Quality of Life ,Cardiology ,neuropathy ,Cardiology and Cardiovascular Medicine ,business ,Risk assessment - Abstract
Supine hypertension commonly occurs in patients with neurogenic orthostatic hypotension due to autonomic failure. Supine hypertension promotes nocturnal sodium excretion and orthostatic hypotension, thus, interfering with quality of life. Perusal of the literature on essential hypertension and smaller scale investigations in autonomic failure patients also suggest that supine hypertension may predispose to cardiovascular and renal disease. These reasons provide a rationale for treating supine hypertension. Yet, treatment of supine hypertension, be it through nonpharmacological or pharmacological approaches, may exacerbate orthostatic hypotension when patients get up during the night. Fall-related complications may occur. More research is needed to define the magnitude of the deleterious effects of supine hypertension on cardiovascular, cerebrovascular, and renal morbidity and mortality. Integration of more precise cardiovascular risk assessment, efficacy, and safety data, and the prognosis of the underlying condition causing autonomic failure is required for individualized management recommendations.
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- 2019
41. Stridor-related gray matter alterations in multiple system atrophy: A pilot study
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Stefano Ratti, Federica Provini, Annagrazia Cecere, Stefania Evangelisti, Lia Talozzi, Caterina Tonon, David Neil Manners, Giovanna Calandra-Buonaura, Giulia Giannini, Claudia Testa, Raffaele Lodi, Pietro Cortelli, Testa, Claudia, Calandra-Buonaura, Giovanna, Evangelisti, Stefania, Giannini, Giulia, Provini, Federica, Ratti, Stefano, Cecere, Annagrazia, Talozzi, Lia, Manners, David Neil, Lodi, Raffaele, Tonon, Caterina, and Cortelli, Pietro
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Adult ,Male ,Voxel based morphometry ,0301 basic medicine ,Cerebellum ,Pathology ,medicine.medical_specialty ,Neurology ,Polysomnography ,Stridor ,Pilot Projects ,03 medical and health sciences ,0302 clinical medicine ,Atrophy ,stomatognathic system ,mental disorders ,otorhinolaryngologic diseases ,Humans ,Medicine ,Gray Matter ,Aged ,Respiratory Sounds ,Retrospective Studies ,Aged, 80 and over ,business.industry ,Brain ,Multiple system atrophy ,Voxel-based morphometry ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Pathophysiology ,nervous system diseases ,030104 developmental biology ,medicine.anatomical_structure ,nervous system ,Laryngeal Muscle ,Female ,Neurology (clinical) ,Geriatrics and Gerontology ,medicine.symptom ,business ,030217 neurology & neurosurgery ,Dystonic disorder - Abstract
Introduction: The neuroanatomical substrate of stridor associated with Multiple System Atrophy (MSA) remains unclear. We evaluated stridor-related gray matter (GM) changes in MSA. Methods: 36 MSA patients underwent standardized nocturnal video-polysomnography and brain MRI. Differences in GM density between MSA patients with and without stridor and a sample of 22 matched healthy controls were evaluated with Voxel Based Morphometry protocol supplemented by a specific tool (SUIT) for analysing infratentorial structures. Results: Stridor was confirmed in 14 patients (10 MSA-cerebellar variant; 10 M; mean ± SD age = 61.6 ± 8.9years; disease duration = 5.2 ± 2.9years) and absent in 22 (11 MSA-cerebellar variant; 18 M; age = 61.4 ± 9.9years; disease duration = 4.8 ± 3.4years). Compared to MSA without stridor, patients with stridor showed higher GM density in the cerebellum (p < 0.05, corrected for the MSA-cerebellar variant and uncorrected when considering both MSA-variants) and lower in the striatum (p < 0.05, uncorrected). Conclusions: This preliminary study has demonstrated for the first time in MSA stridor-related GM changes in striatal and cerebellar regions. Abnormalities in these regions were previously reported in dystonic disorders affecting laryngeal muscles, suggesting the hypothesis that stridor pathophysiology is dystonia-related. These results need however to be confirmed in a larger sample of patients.
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- 2019
42. Clinical pharmacokinetics of pramipexole, ropinirole and rotigotine in patients with Parkinson's disease
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Giovanna Calandra-Buonaura, Alessandro Perrone, Manuela Contin, Roberto Riva, Sabina Capellari, Giovanna Lopane, Stefania Nassetti, Susan Mohamed, Luisa Sambati, Cesa Scaglione, Patrizia De Massis, Pietro Cortelli, Contin, Manuela, Lopane, Giovanna, Mohamed, Susan, Calandra-Buonaura, Giovanna, Capellari, Sabina, De Massis, Patrizia, Nassetti, Stefania, Perrone, Alessandro, Riva, Roberto, Sambati, Luisa, Scaglione, Cesa, and Cortelli, Pietro
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Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Indoles ,Parkinson's disease ,Tetrahydronaphthalenes ,Clinical pharmacokinetic ,Urology ,Thiophenes ,Single Center ,03 medical and health sciences ,Pramipexole ,Sex Factors ,0302 clinical medicine ,Pharmacokinetics ,medicine ,Humans ,In patient ,Aged ,Morning ,Dopaminergic agonist ,Aged, 80 and over ,business.industry ,Age Factors ,Parkinson Disease ,Rotigotine ,Middle Aged ,medicine.disease ,030104 developmental biology ,Ropinirole ,Neurology ,Delayed-Action Preparations ,Dopamine Agonists ,Female ,Neurology (clinical) ,Geriatrics and Gerontology ,business ,030217 neurology & neurosurgery ,medicine.drug - Abstract
Introduction: Pramipexole (PRA), ropinirole (ROP) and rotigotine (ROT) are non-ergoline dopaminergic agonists (DAs) used to treat Parkinson's disease (PD). Clinical pharmacokinetics of DAs is poorly characterized in PD. The main purpose of our study was to investigate the effect of dose, age and sex on steady-state plasma concentrations of DAs in real life PD patients on chronic DAs therapy. Methods: The study was single center, open and prospective. Blood samples for measurement of DAs plasma concentrations were drawn in the morning, at a median 18-h distance from the last DA dose. Results: Ninety-one patients treated with PRA, 50 with ROP and 37 with ROT were enrolled in the study. Plasma concentration of DAs significantly correlated with weight-adjusted daily dose in all subgroups, although at a given dose, matched plasma concentrations highly varied among patients. Median PRA plasma concentration-to-daily dose ratio (C/D) [(ng/mL)/(mg/kg/d)] was 68% higher in patients >65 years than ≤65 years (158 vs 94, p < 0.001), while was not affected by age in ROP and ROT subgroups. No sex-mediated differences in C/D ratios were observed in any group. Conclusion: These are the first observations on DAs pharmacokinetics in PD patients’ everyday clinical practice. Of relevance, patients over 65yrs may require about one third of PRA dose compared to under 65yrs to achieve the same plasma concentration. Due to the high intersubject variability in plasma concentrations at the same dosage, we speculate that monitoring of plasma DAs might be helpful in the individualization of treatment in selected patients.
- Published
- 2019
43. Persistence of Facio‐Skeletal Myorhythmia During Sleep in anti‐IgLON5 Disease
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Vincenzo Mastrangelo, Joan Santamaria, Pietro Cortelli, Federica Provini, Gian Maria Asioli, Giovanna Calandra-Buonaura, Carles Gaig, Giulia Pierangeli, Asioli G.M., Calandra Buonaura G., Mastrangelo V., Pierangeli G., Gaig C., Santamaria J., Cortelli P., and Provini F.
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Persistence (psychology) ,Pediatrics ,medicine.medical_specialty ,Movement disorders ,Whipple disease ,business.industry ,Whipple Disease ,autoimmune ,Disease ,Case Reports ,Sleep in non-human animals ,Neurology ,hyperkinetic ,medicine ,movement disorder ,Neurology (clinical) ,medicine.symptom ,business ,sleep disorder - Abstract
Myorhythmia is a hyperkinetic movement disorder characterized by slow, repetitive, rhythmic, cranial and limb contractions, typically disappearing during sleep. Infectious, autoimmune or vascular lesions involving brainstem or diencephalic structures are the most common etiologies.1 Anti-IgLON5 disease was originally reported as a progressive neurological syndrome characterized by a preeminent sleep disorder, variably associated with bulbar dysfunctions, gait instability, oculomotor abnormalities and cognitive decline.2, 3 Since initial descriptions, several hyperkinetic movement disorders have been described, including oro-facial myorhythmia in few cases.4, 5 We describe a case of anti-IgLON5 disease-related myorhythmia involving both facial and limb muscles, persisting during physiological and pathological sleep.
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- 2021
44. Circadian and state-dependent core body temperature in people with spinal cord injury
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Francesca Baschieri, Federica Provini, Annagrazia Cecere, Pietro Cortelli, Pietro Guaraldi, Giuseppe De Scisciolo, Giorgio Barletta, Monica Chiogna, Giovanna Calandra-Buonaura, Baschieri, Francesca, Guaraldi, Pietro, Provini, Federica, Chiogna, Monica, Barletta, Giorgio, Cecere, Annagrazia, De Scisciolo, Giuseppe, Cortelli, Pietro, and Calandra-Buonaura, Giovanna
- Subjects
030506 rehabilitation ,Sympathetic nervous system ,Non-rapid eye movement sleep ,Body Temperature ,03 medical and health sciences ,0302 clinical medicine ,None ,Humans ,Medicine ,Prospective Studies ,Circadian rhythm ,Spinal cord injury ,Spinal Cord Injuries ,Slow-wave sleep ,Core (anatomy) ,Sleep Stages ,business.industry ,General Medicine ,Thermoregulation ,medicine.disease ,Circadian Rhythm ,medicine.anatomical_structure ,Neurology ,Anesthesia ,Neurology (clinical) ,Sleep ,0305 other medical science ,business ,030217 neurology & neurosurgery - Abstract
Prospective cohort study. To analyze the circadian rhythm and state-dependent modulation of core body temperature (Tcore) in individuals with spinal cord injury (SCI) under controlled environmental conditions. Institute of the Neurological Sciences of Bologna, Italy. We assessed 48-h rectal Tcore and sleep–wake cycle by means of video-polygraphic recording in five cervical SCI (cSCI), seven thoracic SCI (tSCI), and seven healthy controls under controlled environmental conditions. cSCI showed higher night-time Tcore values with reduced nocturnal decrease, higher MESOR and earlier acrophase compared with tSCI and controls (p
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- 2021
45. Neurofilament light chain and α-synuclein RT-QuIC as differential diagnostic biomarkers in parkinsonisms and related syndromes
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Piero Parchi, Niccolò Candelise, Giuseppe Plazzi, Barbara Polischi, Sabina Capellari, Simone Baiardi, Giulia Giannini, Marcello Rossi, Giovanna Calandra-Buonaura, Pietro Cortelli, Luisa Sambati, Corinne Quadalti, Andrea Mastrangelo, Corrado Zenesini, Quadalti C., Calandra Buonaura G., Baiardi S., Mastrangelo A., Rossi M., Zenesini C., Giannini G., Candelise N., Sambati L., Polischi B., Plazzi G., Capellari S., Cortelli P., and Parchi P.
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medicine.medical_specialty ,Parkinson's disease ,Gastroenterology ,Article ,Progressive supranuclear palsy ,Cellular and Molecular Neuroscience ,Orthostatic vital signs ,Atrophy ,Cerebrospinal fluid ,Internal medicine ,medicine ,α-synuclein oligomeric seed ,Pure autonomic failure ,RC346-429 ,Neurofilament light chain ,parkinsonism ,α-syn-s ,business.industry ,Dementia with Lewy bodies ,Parkinsonism ,Diagnostic markers ,medicine.disease ,nervous system diseases ,NfL ,Neurology ,biomarker ,Neurology (clinical) ,Neurology. Diseases of the nervous system ,business - Abstract
Neurofilament light chain (NfL) and α-synuclein oligomeric seeds (α-syn-s) are promising biomarkers for patients with parkinsonism. We assessed their performance in discriminating Parkinson disease (PD) from atypical parkinsonisms (APDs) and evaluated the association between NfL levels and clinical measures of disease severity. We measured NfL in cerebrospinal fluid (CSF) and/or plasma by immunoassays and α-syn-s in CSF by real-time quaking-induced conversion (RT-QuIC) in patients with PD (n = 153), multiple system atrophy (MSA) (n = 80), progressive supranuclear palsy/cortico-basal syndrome (PSP/CBS) (n = 58), dementia with Lewy bodies (n = 64), isolated REM-sleep behaviour disorder (n = 19), and isolated autonomic failure (n = 30). Measures of disease severity included disease duration, UPDRS-III score, Hoehn and Yahr stage, orthostatic hypotension, MMSE score, and CSF amyloid-beta profile. Both CSF NfL (cNfL) and plasma NfL (pNfL) levels were markedly elevated in APDs, and allowed differentiation with PD (vs. APDs, cNfL AUC 0.96; pNfL AUC 0.95; vs. MSA cNfL AUC 0.99; pNfL AUC 0.97; vs. PSP/CBS cNfL AUC 0.94; pNfL AUC 0.94). RT-QuIC detected α-syn-s in 91.4% of PD, but only 2.5% of APDs (all MSA). In PD/PDD, motor scales significantly correlated with cNfL levels. Although pNfL and both cNfL and α-syn-s accurately distinguished PD from APDs, the combined assessment of CSF markers provided a higher diagnostic value (PD vs. APDs AUC 0.97; vs. MSA AUC 0.97; vs. PSP/CBS AUC 0.99) than RT-QuIC alone (p = 0.047 vs. APDs; p = 0.002 vs MSA; p = 0.007 vs PSP/CBS), or cNfL alone (p = 0.011 vs. APDs; p = 0.751 vs MSA; p = 0.0001 vs. PSP/CBS). The results support the use of these assays in specialised clinics.
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- 2021
46. Autonomic dysfunction during sleep in Parkinson's disease
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Pietro Cortelli, Francesca Baschieri, Giovanna Calandra-Buonaura, and Pietro Guaraldi
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medicine.medical_specialty ,Physical medicine and rehabilitation ,Parkinson's disease ,business.industry ,medicine ,business ,medicine.disease ,Sleep in non-human animals - Published
- 2021
47. Fatal Familial Insomnia
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Luca Baldelli, Giovanna Calandra-Buonaura, Federica Provini, and Pietro Cortelli
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- 2021
48. Premotor antidepressants use differs according to Parkinson's disease subtype: A cohort study
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Roberto D'Alessandro, Corrado Zenesini, Elisa Baldin, Giuseppe Bonavina, Giovanna Calandra-Buonaura, Pietro Cortelli, Giovanni Fabbri, Maria Guarino, Stefania Alessandra Nassetti, Roberta Pantieri, Giuseppe Samoggia, Francesco Nonino, Luca Vignatelli, Emanuela Azzoni, Francesca Baschieri, Laura Maria Beatrice Belotti, Marzio Bellan, Lidia Bettelli, Sabina Capellari, Sabina Cevoli, Piero de Carolis, Carlo Descovich, Danilo Di Diodoro, Renata Ferrara, Anna Sandra Gabellini, Giulia Giannini, Pietro Guaraldi, Fabiola Lucchi, Barbara Mostacci, Gaetano Procaccianti, Rita Rinaldi, Giovanni Rizzo, Tommaso Sacquegna, Luisa Sambati, Cesa Scaglione, Elisa Stivanello, Antonella Tempestini, Carmelina Trocino, Susanna Trombetti, D'Alessandro R., Zenesini C., Baldin E., Bonavina G., Calandra Buonaura G., Cortelli P., Fabbri G., Guarino M., Nassetti S.A., Pantieri R., Samoggia G., Nonino F., Vignatelli L., Azzoni E., Baschieri F., Beatrice Belotti L.M., Bellan M., Bettelli L., Capellari S., Cevoli S., de Carolis P., Descovich C., Diodoro D.D., Ferrara R., Gabellini A.S., Giannini G., Guaraldi P., Lucchi F., Mostacci B., Procaccianti G., Rinaldi R., Rizzo G., Sacquegna T., Sambati L., Scaglione C., Stivanello E., Tempestini A., Trocino C., and Trombetti S.
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Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Parkinson's disease ,Prodromal symptoms ,Population ,Disease ,Cohort Studies ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Tremor ,Humans ,Medicine ,education ,Depression (differential diagnoses) ,Aged ,education.field_of_study ,business.industry ,Depression ,Hazard ratio ,Antidepressive agent ,Parkinson Disease ,Odds ratio ,Middle Aged ,medicine.disease ,Antidepressive Agents ,nervous system diseases ,030104 developmental biology ,Italy ,Neurology ,Antidepressant ,Female ,Neurology (clinical) ,Geriatrics and Gerontology ,business ,030217 neurology & neurosurgery ,Cohort studie ,Cohort study - Abstract
Background Depression is more frequently associated with akinetic-rigid/postural instability gait difficulty subtypes of Parkinson's disease than with tremor-dominant subtype. Objectives The aim of the study is to investigate the frequency of exposure to antidepressant drugs, as proxy of depression, before motor onset according to Parkinson's disease subtypes. Method Based on a historical cohort design, the exposure to antidepressant drugs before Parkinson's disease motor onset was obtained from the drug prescription database and assessed in the resident population of the Local Healthcare Trust of Bologna (443,117 subjects older than 35 years). Diagnosis of Parkinson's disease and subtype (tremor dominant, non-tremor dominant) at onset were recorded by neurologists and obtained from the “ParkLink Bologna” record linkage system. Exposure to antidepressants was compared both to the general population and between the two subtypes. Results From 2006 to 2018, 198 patients had a tremor dominant subtype at onset whereas 450 did not. Comparison with the general population for antidepressant exposure showed an adjusted hazard ratio of 0.86 (95% CI 0.44–1.70) for the tremor dominant subtype and 1.66 (1.16–2.39) for the non-tremor dominant subtype. Comparison of non-tremor dominant with tremor dominant subtypes showed an adjusted odds ratio of 1.86 (1.05–3.95) for antidepressant exposure. Conclusions In our study, non-tremor dominant Parkinson's disease at onset was significantly associated with exposure to antidepressants in comparison to the general population and in comparison with the tremor dominant subtype. These results support the hypothesis of different biological substrates for different Parkinson's disease subtypes even before motor onset.
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- 2021
49. Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment
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Giovanna Calandra-Buonaura, Enrico Alfonsi, Luca Vignatelli, Eduardo E. Benarroch, Giulia Giannini, Alex Iranzo, Phillip A. Low, Paolo Martinelli, Federica Provini, Niall Quinn, Eduardo Tolosa, Gregor K. Wenning, Giovanni Abbruzzese, Pamela Bower, Angelo Antonini, Kailash P. Bhatia, Jacopo Bonavita, Maria Teresa Pellecchia, Nicole Pizzorni, François Tison, Imad Ghorayeb, Wassilios G. Meissner, Tetsutaro Ozawa, Claudio Pacchetti, Nicolò Gabriele Pozzi, Claudio Vicini, Antonio Schindler, Pietro Cortelli, Horacio Kaufmann, Calandra Buonaura G., Alfonsi E., Vignatelli L., Benarroch E.E., Giannini G., Iranzo A., Low P.A., Martinelli P., Provini F., Quinn N., Tolosa E., Wenning G.K., Abbruzzese G., Bower P., Antonini A., Bhatia K.P., Bonavita J., Pellecchia M.T., Pizzorni N., Tison F., Ghorayeb I., Meissner W.G., Ozawa T., Pacchetti C., Pozzi N.G., Vicini C., Schindler A., Cortelli P., and Kaufmann H.
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0301 basic medicine ,Consensus development conference ,Dysphagia ,Multiple system atrophy ,Prognosis ,Deglutition Disorders ,Humans ,Multiple System Atrophy ,nervous system diseases ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,Neurology ,nervous system ,stomatognathic system ,otorhinolaryngologic diseases ,Neurology (clinical) ,Geriatrics and Gerontology ,030217 neurology & neurosurgery - Abstract
Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.
- Published
- 2021
50. Risk of Hospitalization and Death for COVID-19 in People with Parkinson's Disease or Parkinsonism
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Giovanni Fabbri, Laura Maria Beatrice Belotti, Giovanna Calandra-Buonaura, Carlo Descovich, Susanna Trombetti, Corrado Zenesini, Francesco Nonino, Roberta Pantieri, Luca Vignatelli, Cesa Scaglione, Giuseppe Samoggia, Giuseppe Bonavina, Elisa Baldin, Roberto D'Alessandro, Giulia Giannini, Maria Guarino, Pietro Cortelli, Vignatelli, Luca, Zenesini, Corrado, Belotti, Laura M B, Baldin, Elisa, Bonavina, Giuseppe, Calandra-Buonaura, Giovanna, Cortelli, Pietro, Descovich, Carlo, Fabbri, Giovanni, Giannini, Giulia, Guarino, Maria, Pantieri, Roberta, Samoggia, Giuseppe, Scaglione, Cesa, Trombetti, Susanna, D'Alessandro, Roberto, and Nonino, Francesco
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Adult ,Male ,Risk ,0301 basic medicine ,medicine.medical_specialty ,Parkinson's disease ,Clinical Neurology ,Cohort Studies ,03 medical and health sciences ,Patient Admission ,0302 clinical medicine ,Parkinsonian Disorders ,COVID‐19 ,Internal medicine ,Case fatality rate ,Humans ,Medicine ,Risk factor ,Research Articles ,parkinsonism ,Aged ,Aged, 80 and over ,business.industry ,Mortality rate ,Parkinsonism ,Hazard ratio ,COVID-19 ,Parkinson Disease ,Length of Stay ,Middle Aged ,medicine.disease ,frail elderly ,Nursing Homes ,nervous system diseases ,Hospitalization ,030104 developmental biology ,Italy ,Neurology ,Cohort ,Female ,Neurology (clinical) ,business ,030217 neurology & neurosurgery ,Research Article ,Cohort study ,cohort studie - Abstract
Background The risk of COVID‐19 and related death in people with Parkinson's disease or parkinsonism is uncertain. The aim of the study was to assess the risk of hospitalization for COVID‐19 and death in a cohort of patients with Parkinson's disease or parkinsonism compared with a control population cohort, during the epidemic bout (March–May 2020) in Bologna, northern Italy. Methods Participants of the ParkLink study with the clinical diagnosis of Parkinson's disease or parkinsonism and people anonymously matched (ratio 1:10) for sex, age, district, and Charlson Index were included. The hospital admission rate for COVID‐19 (February 26–May 31, 2020) and the death rate for any cause were the outcomes of interest. Results The ParkLink cohort included 696 subjects with Parkinson's disease and 184 with parkinsonism, and the control cohort had 8590 subjects. The 3‐month hospitalization rate for COVID‐19 was 0.6% in Parkinson's disease, 3.3% in parkinsonism, and 0.7% in controls. The adjusted hazard ratio (age, sex, district, Charlson Index) was 0.8 (95% CI, 0.3–2.3, P = 0.74) in Parkinson's disease and 3.3 (1.4–7.6, P = 0.006) in parkinsonism compared with controls. Twenty‐nine of the infected subjects died; 30‐day fatality rate was 35.1%, without difference among the 3 groups. Six of 10 Parkinson's disease/parkinsonism patients had the infection during hospitalization or in a nursing home. Conclusions Parkinson's disease per se probably is not a risk factor for COVID‐19 hospitalization. Conversely, parkinsonism is an independent risk factor probably because of a more severe health status, entailing higher care dependence and placement in high‐infection‐risk accommodations. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC. on behalf of International Parkinson and Movement Disorder Society.
- Published
- 2021
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