Living with chronic illness scale: international validation of a new self-report measure in Parkinson's disease

Understanding how a person lives with a chronic illness, such as Parkinson's disease (PD), is necessary to provide individualized care and professionals role in person-centered care at clinical and community levels is paramount. The present study was aimed to analyze the psychometric properties of the Living with Chronic Illness-PD Scale (EC-PC) in a wide Spanish-speaking population with PD. International cross-sectional study with retest was carried out with 324 patients from four Latin American countries and Spain. Feasibility, acceptability, scaling assumptions, reliability, precision, and construct validity were tested. The study included 324 patients, with age (mean±s.d.) 66.67±10.68 years. None of the EC-PC items had missing values and all acceptability parameters fulfilled the standard criteria. Around two-third of the items (61.54%) met scaling assumptions standards. Concerning internal consistency, Cronbach's alpha values were 0.68-0.88; item-total correlation was >0.30, except for two items; item homogeneity index was >0.30, and inter-item correlation values 0.14-0.76. Intraclass correlation coefficient for EC-PC stability was 0.76 and standard error of measurement (s.e.m.) for precision was 8.60 (for a EC-PC s.d.=18.57). EC-PC presented strong correlation with social support (rS=0.61) and moderate correlation with life satisfaction (rS=0.46). Weak and negligible correlations were found with the other scales. Internal validity correlations ranged from 0.46 to 0.78. EC-PC total scores were significantly different for each severity level based on Hoehn and Yahr and Clinical Impression of Severity Index, but not for Patient Global Impression of Severity. The EC-PC has satisfactory acceptability, reliability, precision, and validity to evaluate living with PD.S

Living with chronic illness scale: international validation of a new self-report measure in Parkinson’s disease

Understanding how a person lives with a chronic illness, such as Parkinson’s disease (PD), is necessary to provide individualized care and professionals role in person-centered care at clinical and community levels is paramount. The present study was aimed to analyze the psychometric properties of the Living with Chronic Illness-PD Scale (EC-PC) in a wide Spanish-speaking population with PD. International cross-sectional study with retest was carried out with 324 patients from four Latin American countries and Spain. Feasibility, acceptability, scaling assumptions, reliability, precision, and construct validity were tested. The study included 324 patients, with age (mean±s.d.) 66.67±10.68 years. None of the EC-PC items had missing values and all acceptability parameters fulfilled the standard criteria. Around two-third of the items (61.54%) met scaling assumptions standards. Concerning internal consistency, Cronbach’s alpha values were 0.68–0.88; item-total correlation was >0.30, except for two items; item homogeneity index was >0.30, and inter-item correlation values 0.14–0.76. Intraclass correlation coefficient for EC-PC stability was 0.76 and standard error of measurement (s.e.m.) for precision was 8.60 (for a EC-PC s.d.=18.57). EC-PC presented strong correlation with social support (rS=0.61) and moderate correlation with life satisfaction (rS=0.46). Weak and negligible correlations were found with the other scales. Internal validity correlations ranged from 0.46 to 0.78. EC-PC total scores were significantly different for each severity level based on Hoehn and Yahr and Clinical Impression of Severity Index, but not for Patient Global Impression of Severity. The EC-PC has satisfactory acceptability, reliability, precision, and validity to evaluate living with PD

Analysis of four scales for global severity evaluation in Parkinson’s disease

Global evaluations of Parkinson?s disease (PD) severity are available, but their concordance and accuracy have not been previously tested. The present international, cross-sectional study was aimed at determining the agreement level among four global scales for PD (Hoehn and Yahr, HY; Clinical Global Impression of Severity, CGIS; Clinical Impression of Severity Index, CISI-PD; and Patient Global Impression of Severity, PGIS) and identifying which of them better correlates with itemized PD assessments. Assessments included additional scales for evaluation of the movement impairment, disability, affective disorders, and quality of life. Spearman correlation coefficients, weighted and generalized kappa, and Kendall?s concordance coefficient were used. Four hundred thirty three PD patients, 66% in HY stages 2 or 3, mean disease duration 8.8 years, were analyzed. Correlation between the global scales ranged from 0.60 (HY with PGIS) to 0.91 (CGIS with CISI-PD). Kendall?s coefficient of concordance resulted 0.76 (P<0.0001). HY and CISI-PD showed the highest association with age, disease duration, and levodopa-equivalent daily dose, and CISI-PD with measures of PD manifestations, disability, and quality of life. PGIS and CISI-PD correlated similarly with anxiety and depression scores. The lowest agreement in classifying patients as mild, moderate, or severe was observed between PGIS and HY or CISI-PD (58%) and the highest between CGIS and CISI-PD (84.3%). The four PD global severity scales agree moderately to strongly among them; clinician-based ratings estimate PD severity, as established by other measures, better than PGIS; and the CISI-PD showed the highest association with measures of impairment, disability, and quality of life.Fil: Martinez Martin, Pablo. Universidad Carlos III de Madrid. Instituto de Salud; EspañaFil: Rojo Abuin, José Manuel. Consejo Superior de Investigaciones Cientificas. Centro de Ciencias Humanas y Sociales. Instituto de Historia.; EspañaFil: Rodríguez Violante, Mayela. Instituto Nacional de Neurología y Neurocirugía; MéxicoFil: Serrano Dueñas, Marcos. Pontificia Universidad Católica del Ecuador; EcuadorFil: Garreto, Nélida Susana. Universidad de Buenos Aires. Facultad de Medicina. Centro Universitario de Neurologia "dr. Jose Maria Ramos Mejia".; ArgentinaFil: Martínez Castrillo, Juan Carlos. Instituto Ramón y Cajal de Investigación Sanitaria; EspañaFil: Campos Arillo, Víctor. Hospital Xanit International; EspañaFil: Fernández, William. Universidad Nacional de Colombia; ColombiaFil: Chaná Cuevas, Pedro. Universidad de Santiago de Chile. Facultad de Humanidades. Instituto de Ciencias Biomédicas.; ChileFil: Arakaki, Tomoko. Universidad de Buenos Aires. Facultad de Medicina. Centro Universitario de Neurologia "dr. Jose Maria Ramos Mejia".; Argentina. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; ArgentinaFil: Alvarez, Mario Gustavo. Centro Internacional de Restauración Neurológica ; CubaFil: Pedroso Ibañez, Ivonne. Centro Internacional de Restauración Neurológica ; CubaFil: Rodríguez Blázquez , Carmen. Universidad Carlos III de Madrid. Instituto de Salud; EspañaFil: Ray Chaudhuri , Kallol. National Parkinson Foundation International Centre of Excellence; Reino UnidoFil: Merello, Marcelo Jorge. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentin

Expanded and Independent Spanish Validation of the MDS-Non Motor Rating Scale

[Background] The Movement Disorder Society-sponsored Non-motor Rating Scale (MDS-NMS) assess the severity and disability caused by non-motor symptoms (NMS) in Parkinson's disease (PD).[Objective] This article encapsulates the formal process for completing this program and the data on the first officially approved non-English version of the MDS-NMS (Spanish).[Methods] The MDS-NMS translation program involves four steps: translation and back-translation; cognitive pre-testing to ensure that raters and patients understand the scale and are comfortable with its content; field testing of the finalized version; analysis of the factor structure of the tested version against the original English language version for the nine domains that could be analyzed in a confirmatory factor analysis. To be designated an “Official MDS translation,” the confirmatory factor analysis Comparative Fit Index had to be ≥0.90.[Results] The Spanish MDS-NMS was tested in 364 native-Spanish-speaking patients with PD from seven countries. For all subjects with fully computable data with all domains of the MDS-NMS (n = 349), the Comparative Fit Index was ≥0.90 for the nine eligible domains. Missing data were negligible and moderate floor effect (42.90%) was found for the Non-Motor Fluctuations subscale. Item homogeneity coefficient was adequate, and the correlation of the MDS-NMS domains with other measures for related constructs was acceptable (rs ≥ 0.50).[Conclusions] The Spanish version of the MDS-NMS followed the IPMDS Translation Program protocol, reached the criterion to be designated as an Official Translation, and is now available on the MDS website.Grant support from the International Parkinson and Movement Disorder SocietyPeer reviewe

Systematic Collaborative Reanalysis of Genomic Data Improves Diagnostic Yield in Neurologic Rare Diseases

Altres ajuts: Generalitat de Catalunya, Departament de Salut; Generalitat de Catalunya, Departament d'Empresa i Coneixement i CERCA Program; Ministerio de Ciencia e Innovación; Instituto Nacional de Bioinformática; ELIXIR Implementation Studies (CNAG-CRG); Centro de Investigaciones Biomédicas en Red de Enfermedades Raras; Centro de Excelencia Severo Ochoa; European Regional Development Fund (FEDER).Many patients experiencing a rare disease remain undiagnosed even after genomic testing. Reanalysis of existing genomic data has shown to increase diagnostic yield, although there are few systematic and comprehensive reanalysis efforts that enable collaborative interpretation and future reinterpretation. The Undiagnosed Rare Disease Program of Catalonia project collated previously inconclusive good quality genomic data (panels, exomes, and genomes) and standardized phenotypic profiles from 323 families (543 individuals) with a neurologic rare disease. The data were reanalyzed systematically to identify relatedness, runs of homozygosity, consanguinity, single-nucleotide variants, insertions and deletions, and copy number variants. Data were shared and collaboratively interpreted within the consortium through a customized Genome-Phenome Analysis Platform, which also enables future data reinterpretation. Reanalysis of existing genomic data provided a diagnosis for 20.7% of the patients, including 1.8% diagnosed after the generation of additional genomic data to identify a second pathogenic heterozygous variant. Diagnostic rate was significantly higher for family-based exome/genome reanalysis compared with singleton panels. Most new diagnoses were attributable to recent gene-disease associations (50.8%), additional or improved bioinformatic analysis (19.7%), and standardized phenotyping data integrated within the Undiagnosed Rare Disease Program of Catalonia Genome-Phenome Analysis Platform functionalities (18%)

Treatment with tocilizumab or corticosteroids for COVID-19 patients with hyperinflammatory state: a multicentre cohort study (SAM-COVID-19)

Objectives: The objective of this study was to estimate the association between tocilizumab or corticosteroids and the risk of intubation or death in patients with coronavirus disease 19 (COVID-19) with a hyperinflammatory state according to clinical and laboratory parameters. Methods: A cohort study was performed in 60 Spanish hospitals including 778 patients with COVID-19 and clinical and laboratory data indicative of a hyperinflammatory state. Treatment was mainly with tocilizumab, an intermediate-high dose of corticosteroids (IHDC), a pulse dose of corticosteroids (PDC), combination therapy, or no treatment. Primary outcome was intubation or death; follow-up was 21 days. Propensity score-adjusted estimations using Cox regression (logistic regression if needed) were calculated. Propensity scores were used as confounders, matching variables and for the inverse probability of treatment weights (IPTWs). Results: In all, 88, 117, 78 and 151 patients treated with tocilizumab, IHDC, PDC, and combination therapy, respectively, were compared with 344 untreated patients. The primary endpoint occurred in 10 (11.4%), 27 (23.1%), 12 (15.4%), 40 (25.6%) and 69 (21.1%), respectively. The IPTW-based hazard ratios (odds ratio for combination therapy) for the primary endpoint were 0.32 (95%CI 0.22-0.47; p < 0.001) for tocilizumab, 0.82 (0.71-1.30; p 0.82) for IHDC, 0.61 (0.43-0.86; p 0.006) for PDC, and 1.17 (0.86-1.58; p 0.30) for combination therapy. Other applications of the propensity score provided similar results, but were not significant for PDC. Tocilizumab was also associated with lower hazard of death alone in IPTW analysis (0.07; 0.02-0.17; p < 0.001). Conclusions: Tocilizumab might be useful in COVID-19 patients with a hyperinflammatory state and should be prioritized for randomized trials in this situatio

New diagnostic criteria for neurocysticercosis: reliability and validity

Objective: The diagnosis of neurocysticercosis (NCC) remains problematic because of the heterogeneity of its clinical, immunological, and imaging characteristics. Our aim was to develop and assess a new set of diagnostic criteria for NCC, which might allow for the accurate detection of, and differentiation between, parenchymal and extraparenchymal disease. Methods: A group of Latin American NCC experts developed by consensus a new set of diagnostic criteria for NCC. A multicenter, retrospective study was then conducted to validate it. The reference standard for diagnosis of active NCC was the disappearance or reduction of cysts after anthelmintic treatment. In total, three pairs of independent neurologists blinded to the diagnosis evaluated 93 cases (with NCC) and 93 controls (without NCC) using the new diagnostic criteria. Mixed-effects logistic regression models were used to estimate sensitivity and specificity. Results: Inter-rater reliability (kappa) of diagnosis among evaluators was 0.60. For diagnosis of NCC versus no NCC, the new criteria had a sensitivity of 93.2% and specificity of 81.4%. For parenchymal NCC, the new criteria had a sensitivity of 89.8% and specificity of 80.7% and for extraparenchymal NCC, the new criteria had a sensitivity of 65.9% and specificity of 94.9%. Interpretation: These criteria have acceptable reliability and validity and could be a new tool for clinicians and researchers. An advantage of the new criteria is that they consider parasite location (ie, parenchymal or extraparenchymal), which is an important factor determining the clinical, immunological, and radiological presentation of the disease, and importantly, its treatment and prognosis

Evaluation of the metric properties of the WHODAS 2.0, WHODAS-S, and RADS in the assessment of disability in Parkinsonian patients

Objectives: Parkinson's disease is the second most prevalent progressive neurodegenerative disease and causes considerable disability in patients. We conducted a cross-sectional analytical study to examine the metric properties of the World Health Organization Disability Assessment Schedule 2.0 (WHODAS-2); the 12-item World Health Organization Disability Assessment Schedule (WHODAS-S) and the Rapid Assessment of Disability Scale (RADS) in assessing disability in Parkinsonian patients. Patients and methods: Patients with cognitive impairment, neurological disorder, or disability due to any condition other than PD were excluded. One hundred sixty-eight consecutive patients were assessed in ON state. The following attributes were evaluated: data quality and acceptability, reliability, and construct (convergent and known-groups) validity. Testretest reliability was analyzed in fifty-six patients. Results: Out of 168 patients, 65.4% were men's, 96 (57.1%) at stage III of Hoehn and Yahr. One hundred fifty-one patients lived independently in the community, 102 lived with their spouses, 108 were retired, and 48 were still working. Cronbach's alpha exceeded the minimum requirement of 0.70 for the three scales. The SEM obtained was, also for the three scales, higher than the ½ of the standard deviation value. The validity for known groups showed that all domains were significantly different in both WHODAS-S and RADS. The stability of the scale was evaluated with the test-retest (ICC). The results for the WHODAS-2 ≤ 0.002; for the WHODAS-S were p ≤ 0.000]; and for the RADS were p ≤ 0.000]. Conclusion: The RADS is by far the fastest scale to use. All three scales showed suitable metric properties. Based on the results obtained, a one-way analysis of variance (ANOVA) was performed. Isolates 1 and 2 produced a higher amount of EPS with molasses, isolate 3 with glycerol and isolate 4 with sucrose as a carbon source. In terms of temperature, isolates 1, 3 and 4 produced a greater amount of EPS between 27-35 ° C, while isolate 2 at 42 ° C. According to the pH, the best production was observed at a neutral and slightly acidic pH. Finally, regarding salinity, it was observed that isolate 1 and 2 produced a higher amount of EPS at a concentration of 0.5 M sodium chloride, and isolate 3 and 4 with 0 M sodium chloride. The results show that EPS production is influenced by the source of carbon and environmental conditions. The best performance in EPS production is obtained at least under an abiotic stress condition. These changes represent adaptive changes of bacteria against conditions of abiotic stress. Their tolerance depends on the fact that these microorganisms can be used in the improvement of the clay soil structure to favor bioremediation processes in the Amazon

Clinical and radiological profile of neuromyelitis optica spectrum disorders in an Ecuadorian cohort

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a complex disease characterized by a severe inflammation of the central nervous system (CNS). This disease typically manifests with recurrent optic neuritis (ON) and acute transverse myelitis (ATM). The clinical and radiological spectrum of NMOSD is little known in Latin America (LATAM) and few reports have been published in the literature so far. In Ecuador, no reports on NMOSD have been published. For this reason we aimed to assess the demographic, clinical and imaging characteristics of patients with NMOSD from third level hospitals from Ecuador. Methods: This is a descriptive study in which we assessed medical reports of patients with inflammatory demyelinating diseases who were attended in third level hospitals from Ecuador in 2017. Then we applied the 2015 diagnostic criteria, those patients who met the new NMOSD diagnostic criteria were selected and analyzed. Additionally, exploratory sub-analyses were subsequently carried out. Results: We identified 59 patients with NMOSD, the relative frequency of NMOSD was 15.9%. The multiple sclerosis (MS) /NMOSD ratio was 5.2:1. Twenty four percent of patients were newly defined as having NMOSD when 2015 criteria was applied. The median time to diagnoses was shorter by the 2015 criteria than 2006 criteria (p<0.001). NMOSD was more prevalent in women (female/male ratio 4.4:1). The disease onset was more frequent at the fourth decade of life. The most common symptoms at the disease onset were ON and the association of ON with ATM. The mean of expanded disability status scale (EDSS) was 4.8 (SD±1.8). Concomitant autoimmune diseases were infrequent in this population (11.9%). The brain magnetic resonance imaging (MRI) abnormalities were present in 25.7% of patients at disease onset. Spinal cord MRI showed longitudinally extensive transverse myelitis (LETM) in 91.5% of cases. Recurrent NMOSD was frequent in this cohort (88%). Positivity for antibodies against aquaporin-4 (AQP4-IgG) which was measured through indirect immunofluorescence assay (IIF) was identified in 81% of the patients tested. Patients with seronegative AQP4-IgG had higher grade of disability than seropositive patients (p<0.05). Ninety eight percent of patients received treatment with immunosuppressive drugs. Three patients died due to gastric cancer (1 patient) and infectious diseases (2 patients). Conclusions: This is the first descriptive study in an Ecuadorian cohort of patients with NMOSD. We show a wide epidemiological, clinical and radiological spectrum of NMOSD

Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito

Background: Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants. So far in Ecuador, we do not know what the prevalence or incidence is, and only one case report has been written. Case presentation: We present a case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito. The average age of symptom onset in our patients was 58.8 years. The male to female ratio was 1:1. Two patients began with cognitive/behavioral symptoms, while 4 patients began with focal neurological signs; 1 case with ataxia, 2 with gait disorders and 1 with vertigo and headache. All of the patients had the clinical features established by the World Health Organization. In addition, the entire cohort was positive for the 14-3-3 protein in cerebrospinal fluid, and had high signal abnormalities in caudate and putamen nucleus in DWI and FLAIR IRM. Only in one case, did we reach a definitive diagnosis through a pathological study. All other cases had a probable diagnosis. In this series of cases, 6 out of 6 patients died. The average time from the onset of the symptoms to death in this cohort was 13 months. Conclusion: This is the first report of a series of cases of Creutzfeldt-Jakob disease in Quito. Although definitive diagnosis must be histopathological, there are ancillary tests currently available that have allowed us to obtain a diagnosis of the disease