NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2023.10.004
V. Valadez-Barba , K. Juárez-Navarro , E. Padilla-Camberos , N.F. Díaz , J.R. Guerra-Mora , N.E. Díaz-Martínez
{"title":"Parkinson’s disease: an update on preclinical studies of induced pluripotent stem cells","authors":"V. Valadez-Barba , K. Juárez-Navarro , E. Padilla-Camberos , N.F. Díaz , J.R. Guerra-Mora , N.E. Díaz-Martínez","doi":"10.1016/j.nrleng.2023.10.004","DOIUrl":"10.1016/j.nrleng.2023.10.004","url":null,"abstract":"<div><p>Parkinson’s disease (PD) is the second most prevalent neurodegenerative disease among adults worldwide. It is characterised by the death of dopaminergic neurons in the substantia nigra pars compacta and, in some cases, presence of intracytoplasmic inclusions of α-synuclein, called Lewy bodies, a pathognomonic sign of the disease. Clinical diagnosis of PD is based on the presence of motor alterations. The treatments currently available have no neuroprotective effect. The exact causes of PD are poorly understood. Therefore, more precise preclinical models have been developed in recent years that use induced pluripotent stem cells (iPSC). In vitro studies can provide new information on PD pathogenesis and may help to identify new therapeutic targets or to develop new drugs.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 681-694"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580823000585/pdfft?md5=e319d9ba61a8f388a736001ed9d2cfac&pid=1-s2.0-S2173580823000585-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2021.04.008
M. Carmona-Abellan , R. Del Pino , A. Murueta-Goyena , M. Acera , B. Tijero , K. Berganzo , I. Gabilondo , J.C. Gómez-Esteban
{"title":"Multiple system atrophy: Clinical, evolutive and histopathological characteristics of a series of cases","authors":"M. Carmona-Abellan , R. Del Pino , A. Murueta-Goyena , M. Acera , B. Tijero , K. Berganzo , I. Gabilondo , J.C. Gómez-Esteban","doi":"10.1016/j.nrleng.2021.04.008","DOIUrl":"10.1016/j.nrleng.2021.04.008","url":null,"abstract":"<div><h3>Background and objective</h3><p>Multiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The pathologic hallmark is the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients.</p></div><div><h3>Material and methods</h3><p>Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.</p></div><div><h3>Results</h3><p>UMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes.</p></div><div><h3>Conclusion</h3><p>A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 609-616"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580822001651/pdfft?md5=95562a937a1071eb8f03be54d906914e&pid=1-s2.0-S2173580822001651-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138300849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2021.03.010
L. Amaya Pascasio , B. De La Casa-Fages , E. Esteban de Antonio , F. Grandas , R. García-Leal , F. Ruiz Juretschke
{"title":"Microvascular decompression for trigeminal neuralgia: A retrospective analysis of long-term outcomes and prognostic factors","authors":"L. Amaya Pascasio , B. De La Casa-Fages , E. Esteban de Antonio , F. Grandas , R. García-Leal , F. Ruiz Juretschke","doi":"10.1016/j.nrleng.2021.03.010","DOIUrl":"10.1016/j.nrleng.2021.03.010","url":null,"abstract":"<div><h3>Introduction</h3><p>Microvascular decompression is considered to be the most effective and only etiological surgical treatment for classical trigeminal neuralgia, relieving the neurovascular compression found in up to 95% of cases. This study aims to report the long-term outcomes and to identify prognostic factors in a series of patients with trigeminal neuralgia treated by microvascular decompression.</p></div><div><h3>Methods</h3><p>A retrospective observational study of 152 consecutive patients operated by microvascular decompression with at least six months of follow-up. The surgical results, including pain relief according to the Barrow Neurological Institute pain scale, complications and the medical treatment during the follow-up period were reviewed. Binary regression analysis was performed to identify factors associated with a good long-term outcome.</p></div><div><h3>Results</h3><p>A total of 152 patients with a mean age of 60 years and a mean follow-up of 43 months were included. At the final follow-up visit, 83% of the patients had achieved significant relief of the pain and 63% could reduce the absolute drug doses by 50% or more. The most frequent complications were wound infection (4.5%) and CSF fistula (7%). Being over 70 years of age and having paroxysmal pain were associated with a long-term pain relief.</p></div><div><h3>Conclusions</h3><p>Our results support the notion that microvascular decompression is an effective and safe therapy in patients with trigeminal neuralgia. A multidisciplinary approach with an early referral to a neurosurgical unit many be beneficial in patients who are refractory to pharmacological treatment.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 625-634"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580822001675/pdfft?md5=2ee1c0cd77437931c0b5533567fdad08&pid=1-s2.0-S2173580822001675-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138300848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2021.02.010
A. Llauradó , M. Quintana , E. Fonseca , L. Abraira , M. Toledo , M. Requena , M. Olivé , A. Ballvé , D. Campos , M. Sueiras , E. Santamarina
{"title":"Implications of starting antiepileptic treatment prior to electroencephalography in first epileptic seizures","authors":"A. Llauradó , M. Quintana , E. Fonseca , L. Abraira , M. Toledo , M. Requena , M. Olivé , A. Ballvé , D. Campos , M. Sueiras , E. Santamarina","doi":"10.1016/j.nrleng.2021.02.010","DOIUrl":"10.1016/j.nrleng.2021.02.010","url":null,"abstract":"<div><h3>Introduction</h3><p>This study aimed to determine whether the administration of antiepileptic drugs (AED) alters the likelihood of detecting epileptiform abnormalities in electroencephalographies (EEG) performed early after a first epileptic seizure.</p></div><div><h3>Methods</h3><p>We performed a retrospective, observational study including patients with a first seizure attended at our centre’s emergency department between July 2014 and November 2019. We collected clinical data, as well as technical data on the acquisition and interpretation of the EEG performed within the first 72 hours after the seizure, and the factors related with seizure recurrence.</p></div><div><h3>Results</h3><p>We recruited 155 patients with a mean (SD) age of 48.6 (22.5) years; 61.3% were men. Regarding seizure type, 51% presented tonic-clonic seizures of unknown onset and 12% presented focal to bilateral tonic-clonic seizures. Thirty-nine patients (25.2%) received AED treatment before the EEG was performed: 33 received a non-benzodiazepine AED and 6 received a benzodiazepine. Epileptiform abnormalities were observed in 29.7% of patients. Previous administration of AEDs was not significantly associated with the probability of detecting interictal epileptiform abnormalities (<em>P</em> = .25) or with the risk of recurrence within 6 months (<em>P</em> = .63).</p></div><div><h3>Conclusions</h3><p>Administration of AEDs before an early EEG following a first seizure does not decrease the likelihood of detecting epileptiform abnormalities. These findings suggest that starting AED treatment immediately in patients with a high risk of early recurrence does not imply a reduction in the diagnostic accuracy of the test.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 647-652"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580823000548/pdfft?md5=0979845b4e8c7fe319f27dc01b541223&pid=1-s2.0-S2173580823000548-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2023.10.003
U. Meza, C. Romero-Méndez, S. Sánchez-Armáss, A.A. Rodríguez-Menchaca
{"title":"Role of rafts in neurological disorders","authors":"U. Meza, C. Romero-Méndez, S. Sánchez-Armáss, A.A. Rodríguez-Menchaca","doi":"10.1016/j.nrleng.2023.10.003","DOIUrl":"10.1016/j.nrleng.2023.10.003","url":null,"abstract":"<div><h3>Introduction</h3><p>Rafts are protein-lipid structural nanodomains involved in efficient signal transduction and the modulation of physiological processes of the cell plasma membrane. Raft disruption in the nervous system has been associated with a wide range of disorders.</p></div><div><h3>Development</h3><p>We review the concept of rafts, the nervous system processes in which they are involved, and their role in diseases such as Parkinson’s disease, Alzheimer disease, and Huntington disease.</p></div><div><h3>Conclusions</h3><p>Based on the available evidence, preservation and/or reconstitution of rafts is a promising treatment strategy for a wide range of neurological disorders.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 671-680"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580823000573/pdfft?md5=0d7bd1d9d9c808ac1e722bec37aea222&pid=1-s2.0-S2173580823000573-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2023.10.002
C. Carnero-Pardo , S. López-Alcalde , M. Florido-Santiago , M. Espinosa-García , I. Rego-García , R. Calle-Calle , I. Carrera-Muñoz , R. de la Vega-Cotarelo
{"title":"Diagnostic accuracy and predictive validity of combined use of Fototest and Mini-Cog in cognitive impairment","authors":"C. Carnero-Pardo , S. López-Alcalde , M. Florido-Santiago , M. Espinosa-García , I. Rego-García , R. Calle-Calle , I. Carrera-Muñoz , R. de la Vega-Cotarelo","doi":"10.1016/j.nrleng.2023.10.002","DOIUrl":"10.1016/j.nrleng.2023.10.002","url":null,"abstract":"<div><h3>Introduction</h3><p>The Fototest and Mini-Cog include all the domains that are necessary in a cognitive assessment. This study aims to evaluate the diagnostic accuracy of the combined use of both instruments for detecting cognitive impairment.</p></div><div><h3>Methods</h3><p>We performed a phase III diagnostic accuracy study with 2 independent samples: STUDY, which included 448 participants randomly allocated to 2 datasets (BASE [80%] and TEST [20%]); and EXTERNAL, which included 61 participants. The index test was consecutive administration of the Fototest and Mini-Cog, and the reference test was formal cognitive assessment. We evaluated the diagnostic accuracy of two-step vs consecutive application of the tests and simple (Comb-Simple), logistic regression (Comb-LR), and random decision tree (Comb-RDT) models of their combined use for detecting cognitive impairment (Global Deterioration Scale score ≥ 3). We performed an exploratory analysis of the BASE dataset, selecting criteria that maximise accuracy; a pre-specified analysis was used to evaluate the selected criteria in the TEST and EXTERNAL datasets.</p></div><div><h3>Results</h3><p>The diagnostic accuracy (95% confidence interval) of the combined models in the BASE dataset (Comb-Simple: 88.3 [88.5−91.4]; Comb-LR: 91.6 [88.2−94.3]; Comb-RDT 95.2 [92.5−97.2]) was significantly higher than the individual values observed for the Mini-Cog and Fototest (81.6 [77.1−85.4] and 84.9 [80.8−88.5], respectively). These results were replicated in the TEST (Comb-Simple: 88.9; Comb-LR: 95.6; Comb-RDT: 92.2) and EXTERNAL datasets (Comb-Simple: 91.8; Comb-LR: 90.2; Comb-RDT: 88.5). Two-step application had the same diagnostic accuracy than consecutive application but required less time (mean [SD] of 197.3 s [56.7] vs 233.9 s [45.2]; <em>P</em> < .0001).</p></div><div><h3>Conclusions</h3><p>Combined application of the Fototest and Mini-Cog takes less than 4 minutes and improves the diagnostic accuracy of both instruments. Two-step application is more efficient as it requires less time while maintaining the same diagnostic accuracy.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 653-662"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S217358082300055X/pdfft?md5=c8da50250f3ffb38a8d18fd64003cd8e&pid=1-s2.0-S217358082300055X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2021.01.008
M. Alonso-Moreno, M. Ladrón-Guevara, P. Ciudad-Gutiérrez
{"title":"Systematic review of gender bias in clinical trials of monoclonal antibodies for the treatment of multiple sclerosis","authors":"M. Alonso-Moreno, M. Ladrón-Guevara, P. Ciudad-Gutiérrez","doi":"10.1016/j.nrleng.2021.01.008","DOIUrl":"10.1016/j.nrleng.2021.01.008","url":null,"abstract":"<div><h3>Introduction</h3><p>This article analyses the presence of gender bias in clinical trials of monoclonal antibodies used to treat multiple sclerosis.</p></div><div><h3>Material and methods</h3><p>We performed a systematic review of controlled clinical trials of 4 monoclonal antibodies used to treat multiple sclerosis (natalizumab, rituximab, alemtuzumab, and ocrelizumab). We searched the PubMed/MEDLINE database for articles published in English before March 2020. The study was conducted in accordance with the relevant international recommendations.</p></div><div><h3>Results</h3><p>The search identified 89 articles, 55 of which met the inclusion criteria. Of all patients included in these trials, 64.6% were women. The lead authors of 10 of the studies were women. Fifteen of the 55 studies included a sex-based analysis of the primary endpoint. Only 8 articles discussed the results separately for men and for women.</p></div><div><h3>Conclusions</h3><p>The clinical trials of these 4 monoclonal antibodies present a significant gender bias. In most cases, the primary and secondary endpoints are not analyzed according to patient sex, despite the fact that international recommendations include this as a minimum requirement for ensuring scientific validity and obtaining appropriate results for extrapolation to the wider population.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 695-706"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580822001687/pdfft?md5=921d7ebdaefccda8027f146f42850996&pid=1-s2.0-S2173580822001687-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138300852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeurologiaPub Date : 2023-11-01DOI: 10.1016/j.nrleng.2021.01.010
J. López-Gómez , B. Sacristán Enciso , M.A. Caro Miró , M.R. Querol Pascual
{"title":"Clinically isolated syndrome: Diagnosis and risk of developing clinically definite multiple sclerosis","authors":"J. López-Gómez , B. Sacristán Enciso , M.A. Caro Miró , M.R. Querol Pascual","doi":"10.1016/j.nrleng.2021.01.010","DOIUrl":"10.1016/j.nrleng.2021.01.010","url":null,"abstract":"<div><h3>Introduction</h3><p>In most cases, multiple sclerosis (MS) initially presents as clinically isolated syndrome (CIS). Differentiating CIS from other acute or subacute neurological diseases and estimating the risk of progression to clinically definite MS is essential since presenting a second episode in a short time is associated with poorer long-term prognosis.</p></div><div><h3>Development</h3><p>We conducted a literature review to evaluate the usefulness of different variables in improving diagnostic accuracy and predicting progression from CIS to MS, including magnetic resonance imaging (MRI) and such biofluid markers as oligoclonal IgG and IgM bands, lipid-specific oligoclonal IgM bands in the CSF, CSF kappa free light-chain (KFLC) index, neurofilament light chain (NfL) in the CSF and serum, and chitinase 3–like protein 1 (CHI3L1) in the CSF and serum.</p></div><div><h3>Conclusions</h3><p>Codetection of oligoclonal IgG bands and MRI lesions reduces diagnostic delays and suggests a high risk of CIS progression to MS. A KFLC index > 10.6 and CSF NfL concentrations > 1150 ng/L indicate that CIS is more likely to progress to MS within one year (40%–50%); 90% of patients with CIS and serum CHI3L1 levels > 33 ng/mL and 100% of those with lipid-specific oligoclonal IgM bands present MS within one year of CIS onset.</p></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"38 9","pages":"Pages 663-670"},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2173580823000561/pdfft?md5=9b57c5badc6455c3c4987dfc6116d5e6&pid=1-s2.0-S2173580823000561-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}