Pub Date : 2025-03-01DOI: 10.1016/j.nrl.2023.02.003
C. Guijarro-Castro , L. Estallo-Guijarro
{"title":"Síndrome de opsoclono-mioclono y cáncer de próstata. Una entidad a tener en cuenta","authors":"C. Guijarro-Castro , L. Estallo-Guijarro","doi":"10.1016/j.nrl.2023.02.003","DOIUrl":"10.1016/j.nrl.2023.02.003","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 2","pages":"Pages 216-217"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139888115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.nrl.2023.04.002
D. Santos García , J. Pagonabarraga Mora , F. Escamilla Sevilla , P.J. García Ruiz , J. Infante Ceberio , J. Kulisevsky Bojarski , G. Linazasoro Cristóbal , M.R. Luquín Piudo , J.C. Martínez Castrillo , S. Jesús Maestre , L. Vela Desojo , F.J. Campos Lucas , F. Caballero Martínez , P. Mir , Panel of Experts Phase 1
Background
Different types of therapies were proven effective for the medical management of motor and non-motor symptoms in Parkinson’s disease (PD). We aimed to gain consensus on the dopamine agonist (DA) therapy use in different clinical scenarios of Parkinson’s disease (PD) patients.
Methods
This consensus study was based on the nominal group technique. Initially, a consensus group comprising 12 expert neurologists in the PD field identified the topics to be addressed and elaborated different evidence-based preliminary statements. Next, a panel of 48 Spanish neurologists expressed their opinion on an internet-based systematic voting program. Finally, initial ideas were reviewed and rewritten according to panel contribution and were ranked by the consensus group using a Likert-type scale. The analysis of data was carried out by using a combination of both qualitative and quantitative methods. The consensus was achieved if the statement reached ≥ 3.5 points in the voting process.
Results
The consensus group produced 76 real-world recommendations. The topics addressed included 12 statements related to DA therapy in early PD, 20 statements concerning DA treatment strategy in patients with motor complications, 11 statements associated with DA drugs and their side effects, and 33 statements regarding DA therapy in specific clinical scenarios. The consensus group did not reach a consensus on 15 statements.
Conclusion
The findings from this consensus method represent an exploratory step to help clinicians and patients in the appropriate use of DA in different stages and clinical situations of PD.
{"title":"Dopamine agonist therapy in Parkinson’s disease: Spanish expert consensus on its use in different clinical situations","authors":"D. Santos García , J. Pagonabarraga Mora , F. Escamilla Sevilla , P.J. García Ruiz , J. Infante Ceberio , J. Kulisevsky Bojarski , G. Linazasoro Cristóbal , M.R. Luquín Piudo , J.C. Martínez Castrillo , S. Jesús Maestre , L. Vela Desojo , F.J. Campos Lucas , F. Caballero Martínez , P. Mir , Panel of Experts Phase 1","doi":"10.1016/j.nrl.2023.04.002","DOIUrl":"10.1016/j.nrl.2023.04.002","url":null,"abstract":"<div><h3>Background</h3><div>Different types of therapies were proven effective for the medical management of motor and non-motor symptoms in Parkinson’s disease (PD). We aimed to gain consensus on the dopamine agonist (DA) therapy use in different clinical scenarios of Parkinson’s disease (PD) patients.</div></div><div><h3>Methods</h3><div>This consensus study was based on the nominal group technique. Initially, a consensus group comprising 12 expert neurologists in the PD field identified the topics to be addressed and elaborated different evidence-based preliminary statements. Next, a panel of 48 Spanish neurologists expressed their opinion on an internet-based systematic voting program. Finally, initial ideas were reviewed and rewritten according to panel contribution and were ranked by the consensus group using a Likert-type scale. The analysis of data was carried out by using a combination of both qualitative and quantitative methods. The consensus was achieved if the statement reached ≥ 3.5 points in the voting process.</div></div><div><h3>Results</h3><div>The consensus group produced 76 real-world recommendations. The topics addressed included 12 statements related to DA therapy in early PD, 20 statements concerning DA treatment strategy in patients with motor complications, 11 statements associated with DA drugs and their side effects, and 33 statements regarding DA therapy in specific clinical scenarios. The consensus group did not reach a consensus on 15 statements.</div></div><div><h3>Conclusion</h3><div>The findings from this consensus method represent an exploratory step to help clinicians and patients in the appropriate use of DA in different stages and clinical situations of PD.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 2","pages":"Pages 171-181"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143548367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.nrl.2022.09.007
J. Tejada García , L.L. Lezama , R. de la Fuente Blanco , A. Pérez de Prado , L. Benavente Fernández , M. Rico Santos , M.D. Fernández Couto , L. Naya Ríos , I. Couso Pazó , P.V. Alba , L. Redondo-Robles , L. López Mesonero , S. Arias-Rivas , M. Santamaría Cadavid , H. Tejada Meza , L. Horna Cañete , I. Azkune Calle , A. Pinedo Brochado , J.M. García Sánchez , I. Caballero Romero , M. Martínez Zabaleta
Introduction
There is an extending use of percutaneous closure of patent foramen ovale (PFO) as therapy for PFO-associated cryptogenic strokes. The aim of our study was to investigate the clinical practice of percutaneous closure of PFO and to analyse the variables for decision-making on the selection of patients for this procedure.
Method
A prospective observational multicentric survey was conducted using all the cases of cryptogenic stroke/transient ischemic attack associated with PFO recorded in the NORDICTUS hospital registry during the period 2018-2021. Clinical data, radiological patterns, echocardiogram data and factors related to PFO-associated stroke (thromboembolic disease and paradoxical embolism criteria) were recorded. The indication for closure was analysed according to age (≤/ > 60 years) and the characteristics of the PFO.
Results
In the group ≤60 years (n = 488), 143 patients (29.3%) underwent PFO closure. The most influential variables for this therapy were detection of a high-risk PFO (OR 4,11; IC 2,6-6,5, p< 0,001), criteria for paradoxical embolism (OR 2,61; IC 1,28-5,28; p = 0,008) and previous use of antithrombotics (OR 2,67; IC 1,38-5,18; p = 0,009). In the group >60 years old (n = 124), 24 patients had PFO closure (19%). The variables related to this option were history of pulmonary thromboembolism, predisposition to thromboembolic disease, paradoxical embolism criteria and high-risk PFO.
Conclusions
The detection of a high-risk PFO (large shunt, shunt with associated aneurysm) is the main criterion for a percutaneous closure-based therapy. Other conditions to consider in the eligibility of patients are the history of thromboembolic disease, paradoxical embolism criteria or the previous use of antithrombotics.
{"title":"Selección de pacientes para cierre percutáneo en el ictus isquémico no lacunar criptogénico asociado a foramen oval permeable. Datos del registro cooperativo NORDICTUS","authors":"J. Tejada García , L.L. Lezama , R. de la Fuente Blanco , A. Pérez de Prado , L. Benavente Fernández , M. Rico Santos , M.D. Fernández Couto , L. Naya Ríos , I. Couso Pazó , P.V. Alba , L. Redondo-Robles , L. López Mesonero , S. Arias-Rivas , M. Santamaría Cadavid , H. Tejada Meza , L. Horna Cañete , I. Azkune Calle , A. Pinedo Brochado , J.M. García Sánchez , I. Caballero Romero , M. Martínez Zabaleta","doi":"10.1016/j.nrl.2022.09.007","DOIUrl":"10.1016/j.nrl.2022.09.007","url":null,"abstract":"<div><h3>Introduction</h3><div>There is an extending use of percutaneous closure of patent foramen ovale (PFO) as therapy for PFO-associated cryptogenic strokes. The aim of our study was to investigate the clinical practice of percutaneous closure of PFO and to analyse the variables for decision-making on the selection of patients for this procedure.</div></div><div><h3>Method</h3><div>A prospective observational multicentric survey was conducted using all the cases of cryptogenic stroke/transient ischemic attack associated with PFO recorded in the NORDICTUS hospital registry during the period 2018-2021. Clinical data, radiological patterns, echocardiogram data and factors related to PFO-associated stroke (thromboembolic disease and paradoxical embolism criteria) were recorded. The indication for closure was analysed according to age (≤/<!--> <!-->> 60 years) and the characteristics of the PFO.</div></div><div><h3>Results</h3><div>In the group ≤60 years (n<!--> <!-->=<!--> <!-->488), 143 patients (29.3%) underwent PFO closure. The most influential variables for this therapy were detection of a high-risk PFO (OR 4,11; IC 2,6-6,5, p<<!--> <!-->0,001), criteria for paradoxical embolism (OR 2,61; IC 1,28-5,28; p<!--> <!-->=<!--> <!-->0,008) and previous use of antithrombotics (OR 2,67; IC 1,38-5,18; p<!--> <!-->=<!--> <!-->0,009). In the group<!--> <!-->>60 years old (n<!--> <!-->=<!--> <!-->124), 24 patients had PFO closure (19%). The variables related to this option were history of pulmonary thromboembolism, predisposition to thromboembolic disease, paradoxical embolism criteria and high-risk PFO.</div></div><div><h3>Conclusions</h3><div>The detection of a high-risk PFO (large shunt, shunt with associated aneurysm) is the main criterion for a percutaneous closure-based therapy. Other conditions to consider in the eligibility of patients are the history of thromboembolic disease, paradoxical embolism criteria or the previous use of antithrombotics.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 2","pages":"Pages 139-149"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49273719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.nrl.2023.02.004
J. Martín Prieto , E. García-Serrano Fuertes , J. Iglesias Bermejillo , A. Luna Rodríguez
{"title":"Mutaciones en el gen del colágeno tipo IV (COL4A1) como etiología infrecuente de enfermedad cerebrovascular en el adulto joven","authors":"J. Martín Prieto , E. García-Serrano Fuertes , J. Iglesias Bermejillo , A. Luna Rodríguez","doi":"10.1016/j.nrl.2023.02.004","DOIUrl":"10.1016/j.nrl.2023.02.004","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 2","pages":"Pages 217-220"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139889007","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-03-01DOI: 10.1016/j.nrl.2023.05.002
C. García-Muñoz , J.C. Hernández-Rodríguez , J.J. Pereyra-Rodriguez
Background
Mortality in Parkinson’s disease is increasing worldwide, but Spanish data need further study.
Objective
To analyse the mortality trends of Parkinson’s disease in Spain between 1981 and 2020.
Methods
This observational retrospective study assessed the Parkinson’s disease mortality data from 1981 to 2020 collected from the National Statistics Institute of Spain. Age-standardised mortality rates were analysed by age and sex groups, detecting significant mortality trends through a joinpoint analysis. Age-period-cohort effect and potential years of life lost analyses were conducted. The European standard population of 2013 was considered for the analyses.
Results
A total of 88 034 deaths were assessed. The overall age-standardised mortality rate rose throughout the period from 3.67 to 8.57 per 100 000 inhabitants. Mortality rates in men were higher than in women, 11.63 versus 6.57 deaths per 100 000 inhabitants. The sex ratio showed an increase in premature mortality in men during 2020. The overall joinpoint analysis recorded a rise in mortality, primarily since the 20th century, mainly in male and older groups, that matched with a period effect. The age effect was detected, confirming higher mortality at an older age. The analysis of potential years of life lost detected a growth in this rate, changing from 0.66 in 1981 to 1.06 in 2020.
Conclusions
Mortality data for Parkinson’s disease in Spain rose significantly in forty years. Mortality rate was higher in the male and age group above 75 years of age. The sex ratio showed premature mortality in men in 2020, which will need further study.
{"title":"Mortality rates for Parkinson’s disease are increasing in Spain. An age-period-cohort and joinpoint analysis of mortality rates from 1981 to 2020","authors":"C. García-Muñoz , J.C. Hernández-Rodríguez , J.J. Pereyra-Rodriguez","doi":"10.1016/j.nrl.2023.05.002","DOIUrl":"10.1016/j.nrl.2023.05.002","url":null,"abstract":"<div><h3>Background</h3><div>Mortality in Parkinson’s disease is increasing worldwide, but Spanish data need further study.</div></div><div><h3>Objective</h3><div>To analyse the mortality trends of Parkinson’s disease in Spain between 1981 and 2020.</div></div><div><h3>Methods</h3><div>This observational retrospective study assessed the Parkinson’s disease mortality data from 1981 to 2020 collected from the National Statistics Institute of Spain. Age-standardised mortality rates were analysed by age and sex groups, detecting significant mortality trends through a joinpoint analysis. Age-period-cohort effect and potential years of life lost analyses were conducted. The European standard population of 2013 was considered for the analyses.</div></div><div><h3>Results</h3><div>A total of 88 034 deaths were assessed. The overall age-standardised mortality rate rose throughout the period from 3.67 to 8.57 per 100 000 inhabitants. Mortality rates in men were higher than in women, 11.63 versus 6.57 deaths per 100 000 inhabitants. The sex ratio showed an increase in premature mortality in men during 2020. The overall joinpoint analysis recorded a rise in mortality, primarily since the 20th century, mainly in male and older groups, that matched with a period effect. The age effect was detected, confirming higher mortality at an older age. The analysis of potential years of life lost detected a growth in this rate, changing from 0.66 in 1981 to 1.06 in 2020.</div></div><div><h3>Conclusions</h3><div>Mortality data for Parkinson’s disease in Spain rose significantly in forty years. Mortality rate was higher in the male and age group above 75 years of age. The sex ratio showed premature mortality in men in 2020, which will need further study.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 2","pages":"Pages 161-170"},"PeriodicalIF":2.9,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143548366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.nrl.2023.06.013
M. Rubiera Valdés , O. Gutiérrez Remis , A. González Jáimez , C. Manzaneque Rodríguez , V. Chiminazzo , G. Morís
Introduction
The aim of this research is to present the clinical characteristics of Phantom Limb Pain (PLP) in patients with amputation.
Material
A retrospective cross-sectional observational study of patients with lower limb amputation is presented. Patients between 18 and 80 years of age with unilateral or bilateral amputation between the years 2015 and 2019 were included. Demographic data, medical history, data related to the amputation, and related abnormal sensations were collected.
Results
43 patients (34 men) and 53 amputees were studied, with a mean age of 62 years, with a time elapsed since amputation of 28 months. The most frequent cause of amputation was ischemic (70%). Twenty-three (60%) patients had PLP that began 1 month after amputation with a mean intensity of 3.9 on the VAS scale, in 15 patients the PLP was daily, three patients recognized the disappearance of PLP. 91% of the patients presented non-painful sensations in relation to the phantom limb. No differences were found in the development of the PLP between the 1st and 2nd amputation. A significant association was found between the development of PLP and residual limb pain.
Conclusions
PLP is a prevalent pathology among amputee patients, therefore multidisciplinary care with an active neurologic participation is essential. Studies are needed to deepen the knowledge of the factors that favour the development of PLP in order to focus early and targeted therapies to prevent the appearance of PLP.
{"title":"Características clínicas del dolor de miembro fantasma en pacientes con amputación de miembro inferior en una población española","authors":"M. Rubiera Valdés , O. Gutiérrez Remis , A. González Jáimez , C. Manzaneque Rodríguez , V. Chiminazzo , G. Morís","doi":"10.1016/j.nrl.2023.06.013","DOIUrl":"10.1016/j.nrl.2023.06.013","url":null,"abstract":"<div><h3>Introduction</h3><div>The aim of this research is to present the clinical characteristics of Phantom Limb Pain (PLP) in patients with amputation.</div></div><div><h3>Material</h3><div>A retrospective cross-sectional observational study of patients with lower limb amputation is presented. Patients between 18 and 80 years of age with unilateral or bilateral amputation between the years 2015 and 2019 were included. Demographic data, medical history, data related to the amputation, and related abnormal sensations were collected.</div></div><div><h3>Results</h3><div>43 patients (34 men) and 53 amputees were studied, with a mean age of 62 years, with a time elapsed since amputation of 28 months. The most frequent cause of amputation was ischemic (70%). Twenty-three (60%) patients had PLP that began 1 month after amputation with a mean intensity of 3.9 on the VAS scale, in 15 patients the PLP was daily, three patients recognized the disappearance of PLP. 91% of the patients presented non-painful sensations in relation to the phantom limb. No differences were found in the development of the PLP between the 1st and 2nd amputation. A significant association was found between the development of PLP and residual limb pain.</div></div><div><h3>Conclusions</h3><div>PLP is a prevalent pathology among amputee patients, therefore multidisciplinary care with an active neurologic participation is essential. Studies are needed to deepen the knowledge of the factors that favour the development of PLP in order to focus early and targeted therapies to prevent the appearance of PLP.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 3","pages":"Pages 279-289"},"PeriodicalIF":2.9,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143680825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01DOI: 10.1016/j.nrl.2022.07.003
M.-C. Boll , M. Alcaraz-Zubeldia , C. Rios , D. González-Esquivel , S. Montes
Background
Few treatments are currently available for amyotrophic lateral sclerosis (ALS). A combination of lithium carbonate and valproic acid (VPA-Li) was shown to inhibit motor neuron death and delay disease progression.
Methods
Outpatients with a typical ALS presentation were enrolled in a randomized, placebo-controlled trial to assess the efficacy of orally administered VPA-Li. Changes in a functional scale score (ALSFRS-R) and survival rate were chosen as primary outcome variables. Secondary outcome variables included BMI, respiratory monitoring, quality of life, and a global impression of the treatment.
Results
Out of 42 patients enrolled, 20 individuals receiving VPA-Li and 18 on placebo treatment were included in the final analysis. Forty-five percent of patients receiving VPA-Li completed the trial, whereas only 22.22% of patients in the placebo group attended the final visit 18 months later (P = 0.09). Major changes in the ALSFRS-R score were observed, including a decrease of 1.195 points/month in the placebo group (95% CI: 0.7869–1.6031) and of 0.5085 under VPA-Li treatment (95% CI: 0.2288–0.7882) between months 6 and 14. Adverse events included bad mouth taste, constipation, and anorexia. Survival rate, body weight, and quality of life were positive outcomes by the end of the trial despite a high sample reduction, especially in the placebo group. The inclusion of 212 subjects in each group would confirm these differences.
Conclusions
Combined VPA-Li treatment associated with slower ALS progression and better secondary outcomes. This dual treatment overcame the futility threshold and merits further investigation in ALS.
{"title":"A phase 2, double-blind, placebo-controlled trial of a valproate/lithium combination in ALS patients","authors":"M.-C. Boll , M. Alcaraz-Zubeldia , C. Rios , D. González-Esquivel , S. Montes","doi":"10.1016/j.nrl.2022.07.003","DOIUrl":"10.1016/j.nrl.2022.07.003","url":null,"abstract":"<div><h3>Background</h3><div>Few treatments are currently available for amyotrophic lateral sclerosis (ALS). A combination of lithium carbonate and valproic acid (VPA-Li) was shown to inhibit motor neuron death and delay disease progression.</div></div><div><h3>Methods</h3><div>Outpatients with a typical ALS presentation were enrolled in a randomized, placebo-controlled trial to assess the efficacy of orally administered VPA-Li. Changes in a functional scale score (ALSFRS-R) and survival rate were chosen as primary outcome variables. Secondary outcome variables included BMI, respiratory monitoring, quality of life, and a global impression of the treatment.</div></div><div><h3>Results</h3><div>Out of 42 patients enrolled, 20 individuals receiving VPA-Li and 18 on placebo treatment were included in the final analysis. Forty-five percent of patients receiving VPA-Li completed the trial, whereas only 22.22% of patients in the placebo group attended the final visit 18 months later (<em>P</em> = 0.09). Major changes in the ALSFRS-R score were observed, including a decrease of 1.195 points/month in the placebo group (95% CI: 0.7869–1.6031) and of 0.5085 under VPA-Li treatment (95% CI: 0.2288–0.7882) between months 6 and 14. Adverse events included bad mouth taste, constipation, and anorexia. Survival rate, body weight, and quality of life were positive outcomes by the end of the trial despite a high sample reduction, especially in the placebo group. The inclusion of 212 subjects in each group would confirm these differences.</div></div><div><h3>Conclusions</h3><div>Combined VPA-Li treatment associated with slower ALS progression and better secondary outcomes. This dual treatment overcame the futility threshold and merits further investigation in ALS.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 1","pages":"Pages 32-40"},"PeriodicalIF":2.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01DOI: 10.1016/j.nrl.2022.09.003
N. Montagut , S. Borrego-Écija , J. Herrero , A. Lladó , M. Balasa , E. Muñoz , F. Valldeoriola , R. Sánchez-Valle
Introduction
Currently there is no tool to quantify buccophonatory apraxia to stratify, compare and monitor patients longitudinally in an objective manner. Our aim in this study is to create a quantitative scale for buccophonatory apraxia and evaluate it in patients with the non-fluent/grammatical variant of Primary Progressive Aphasia (nfvPPA) and other neurodegenerative diseases that occur with speech and/or language problems.
Methods
The scale was designed based on useful elements in the assessment of buccophonatory apraxia and the total was quantified in seconds. The scale was administered to 64 participants with diagnoses of: nfvPPA, semantic variant of Primary Progressive Aphasia (svPPA), logopenic variant of Primary Progressive Aphasia (lvPPA), Huntington's disease, Parkinson's disease, as well as a group of healthy controls.
Results
Patients showed a significantly higher score compared to controls. The group nfvPPA had the highest mean score on the scale (429 seconds ± 278). The scale was useful to differentiate vnfPPA from svPPA and Parkinson's disease (area under curve [AUC] of 0.956 and 0.989 respectively), but less to differentiate it from Huntington's disease (AUC = 0.67) and lvPPA. There was a statistically significant relationship between total score and disease severity in nfvPPA (p < 0.029).
Conclusions
The Barcelona scale for buccophonatory apraxia could be useful to quantitatively evaluate buccophonatory apraxia in different neurodegenerative diseases, and compare patients, especially in nfvPPA.
{"title":"Escala Barcelona para la apraxia bucofonatoria: instrumento de evaluación cuantitativo","authors":"N. Montagut , S. Borrego-Écija , J. Herrero , A. Lladó , M. Balasa , E. Muñoz , F. Valldeoriola , R. Sánchez-Valle","doi":"10.1016/j.nrl.2022.09.003","DOIUrl":"10.1016/j.nrl.2022.09.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Currently there is no tool to quantify buccophonatory apraxia to stratify, compare and monitor patients longitudinally in an objective manner. Our aim in this study is to create a quantitative scale for buccophonatory apraxia and evaluate it in patients with the non-fluent/grammatical variant of Primary Progressive Aphasia (nfvPPA) and other neurodegenerative diseases that occur with speech and/or language problems.</div></div><div><h3>Methods</h3><div>The scale was designed based on useful elements in the assessment of buccophonatory apraxia and the total was quantified in seconds. The scale was administered to 64 participants with diagnoses of: nfvPPA, semantic variant of Primary Progressive Aphasia (svPPA), logopenic variant of Primary Progressive Aphasia (lvPPA), Huntington's disease, Parkinson's disease, as well as a group of healthy controls.</div></div><div><h3>Results</h3><div>Patients showed a significantly higher score compared to controls. The group nfvPPA had the highest mean score on the scale (429<!--> <!-->seconds<!--> <!-->±<!--> <!-->278). The scale was useful to differentiate vnfPPA from svPPA and Parkinson's disease (area under curve [AUC] of 0.956 and 0.989 respectively), but less to differentiate it from Huntington's disease (AUC<!--> <!-->=<!--> <!-->0.67) and lvPPA. There was a statistically significant relationship between total score and disease severity in nfvPPA (p<!--> <!--><<!--> <!-->0.029).</div></div><div><h3>Conclusions</h3><div>The Barcelona scale for buccophonatory apraxia could be useful to quantitatively evaluate buccophonatory apraxia in different neurodegenerative diseases, and compare patients, especially in nfvPPA.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 1","pages":"Pages 48-56"},"PeriodicalIF":2.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44127333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01DOI: 10.1016/j.nrl.2022.06.007
Y. Rodríguez-Agudelo , M. Chávez-Oliveros , A. Ochoa-Morales , L. Martínez-Ruano , A. Camacho-Molina , F. Paz-Rodríguez
Introduction
Huntington's disease (HD) is a neurodegenerative and hereditary disorder, due to the predictive diagnosis, incipient clinical characteristics have been described in the prodromal phase. Several studies have reported an increase in psychiatric symptoms in carriers of the HD gene without motor symptoms.
Objective
To identify psychological distress in carriers of the mutation that causes HD, without motor symptoms, utilizing the Symptom Checklist 90 (SCL-90), and to correlate with the burden and proximity of the disease.
Method
A sample of 175 participants in a HD Predictive Diagnostic Program (PDP-HD) was divided into HEP carriers (39.4%) and NPEH non-carriers (61.6%) of the HD-causing mutation. By means of mathematical formulas, the disease burden and proximity to the manifest stage in the PEH group were obtained and it was correlated with the results of the SCL-90-R.
Results
Comparing the results obtained in the SCL-90-R of the PEH and NPEH, the difference is observed in the positive somatic male index, where the PEH obtains higher average scores. The correlations between disease burden and psychological distress occur in the domains; obsessions and compulsions, interpersonal sensitivity, hostility, global severity index and positive somatic distress index. A low correlation is observed between the burden of disease and the scores obtained in psychological discomfort.
Conclusions
In general, we found that the PEH group obtained a higher score in the dimensions evaluated with the SCL-90-R, showing a relationship with the burden and differences due to the proximity of the disease. Higher scores on the SCL-90-R dimensions in carriers of the HD gene may suggest an early finding of psychological symptoms in the disease.
{"title":"Malestar psicológico en portadores y no portadores de la mutación causante de enfermedad de Huntington y su relación con la carga de la enfermedad","authors":"Y. Rodríguez-Agudelo , M. Chávez-Oliveros , A. Ochoa-Morales , L. Martínez-Ruano , A. Camacho-Molina , F. Paz-Rodríguez","doi":"10.1016/j.nrl.2022.06.007","DOIUrl":"10.1016/j.nrl.2022.06.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Huntington's disease (HD) is a neurodegenerative and hereditary disorder, due to the predictive diagnosis, incipient clinical characteristics have been described in the prodromal phase. Several studies have reported an increase in psychiatric symptoms in carriers of the HD gene without motor symptoms.</div></div><div><h3>Objective</h3><div>To identify psychological distress in carriers of the mutation that causes HD, without motor symptoms, utilizing the Symptom Checklist 90 (SCL-90), and to correlate with the burden and proximity of the disease.</div></div><div><h3>Method</h3><div>A sample of 175 participants in a HD Predictive Diagnostic Program (PDP-HD) was divided into HEP carriers (39.4%) and NPEH non-carriers (61.6%) of the HD-causing mutation. By means of mathematical formulas, the disease burden and proximity to the manifest stage in the PEH group were obtained and it was correlated with the results of the SCL-90-R.</div></div><div><h3>Results</h3><div>Comparing the results obtained in the SCL-90-R of the PEH and NPEH, the difference is observed in the positive somatic male index, where the PEH obtains higher average scores. The correlations between disease burden and psychological distress occur in the domains; obsessions and compulsions, interpersonal sensitivity, hostility, global severity index and positive somatic distress index. A low correlation is observed between the burden of disease and the scores obtained in psychological discomfort.</div></div><div><h3>Conclusions</h3><div>In general, we found that the PEH group obtained a higher score in the dimensions evaluated with the SCL-90-R, showing a relationship with the burden and differences due to the proximity of the disease. Higher scores on the SCL-90-R dimensions in carriers of the HD gene may suggest an early finding of psychological symptoms in the disease.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"40 1","pages":"Pages 1-9"},"PeriodicalIF":2.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49288507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}