NeurologiaPub Date : 2024-11-01DOI: 10.1016/j.nrleng.2022.06.004
Hongwei Wang , Wei Lei , Ye Tian , Jianwei Wu , Xiaosheng Ma , Feizhou Lyu , Xinlei Xia , Jingjuan Liang , Jianyuan Jiang , Hongli Wang
{"title":"The clinical characteristics of Hirayama disease in females","authors":"Hongwei Wang , Wei Lei , Ye Tian , Jianwei Wu , Xiaosheng Ma , Feizhou Lyu , Xinlei Xia , Jingjuan Liang , Jianyuan Jiang , Hongli Wang","doi":"10.1016/j.nrleng.2022.06.004","DOIUrl":"10.1016/j.nrleng.2022.06.004","url":null,"abstract":"<div><h3>Introduction</h3><div>To characterize Hirayama disease in female patients, and increase awareness among clinicians regarding the specifics of this disease.</div></div><div><h3>Methods</h3><div>Baseline data, clinical manifestations, characteristics of cervical-flexion magnetic resonance imaging, and electromyography were collected and compared among females and males with Hirayama disease. In addition, the literature on Hirayama disease in females up to October, 2021 was searched in PubMed and the relevant data were compared with the data from our study.</div></div><div><h3>Results</h3><div>Twenty female and 40 male patients were included in this study. The average ages of onset and menarche were 14.65 and 12.75 years old. All patients suffered from muscular weakness and atrophy of the upper limb(s), with flattening and/or atrophy of the lower cervical spinal cords in cervical-flexion magnetic resonance imaging, and neurogenic patterns in the atrophic muscles as determined using electromyography. The age of onset in females was about 2 years later than the age of menarche, and the age of onset in females was 2 years earlier than that in males. There were no obvious differences in clinical presentation between males and females.</div></div><div><h3>Discussion</h3><div>Although females presented with Hirayama disease two years earlier than males, no other clinical differences were observed. Hirayama disease is likely associated with growth and development in puberty, and early identification, regardless of whether patients are male or female, is critical to optimizing prognosis.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 792-801"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40608835","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}
{"title":"Incidence of Bell’s palsy after coronavirus disease (COVID-19) vaccination: a systematic review and meta-analysis","authors":"Atena Soltanzadi , Omid Mirmosayyeb , Amin Momeni Moghaddam , Hamed Ghoshouni , Mahsa Ghajarzadeh","doi":"10.1016/j.nrleng.2023.06.002","DOIUrl":"10.1016/j.nrleng.2023.06.002","url":null,"abstract":"<div><h3>Objective</h3><div>To estimate the pooled incidence of Bell’s palsy after COVID-19 vaccination.</div></div><div><h3>Methods</h3><div>PubMed, Scopus, EMBASE, Web of Science, and Google Scholar were searched by 2 independent researchers. We also searched the grey literature including references of the references and conference abstracts. We extracted data regarding the total number of participants, first author, publication year, the country of origin, sex, type of vaccines, and the number of patients who developed Bell’s palsy after COVID-19 vaccination.</div></div><div><h3>Results</h3><div>The literature search revealed 370 articles, subsequently deleting duplicates 227 remained. After careful evaluation of the full texts, 20 articles remained for meta-analysis. The most commonly administered vaccines were Pfizer followed by Moderna.</div><div>In total, 4.54e+07 individuals received vaccines against COVID-19, and 1739 cases developed Bell’s palsy. In nine studies, controls (individuals without vaccination) were enrolled. The total number of controls was 1 809 069, of whom 203 developed Bell’s palsy. The incidence of Bell’s palsy after COVID-19 vaccines was ignorable. The odds of developing Bell’s palsy after COVID-19 vaccines was 1.02 (95% CI: 0.79-1.32) (I2 = 74.8%, <em>P</em> < .001).</div></div><div><h3>Conclusion</h3><div>The results of this systematic review and meta-analysis show that the incidence of peripheral facial palsy after COVID-19 vaccination is ignorable and vaccination does not increase the risk of developing Bell’s palsy. Maybe, Bell’s palsy is a presenting symptom of a more severe form of COVID-19, so clinicians must be aware of this.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 802-809"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9928218","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 : 2024-11-01DOI: 10.1016/j.nrleng.2022.06.002
M.A. Mireles-Ramírez , I.E. Velázquez-Brizuela , N. Sánchez-Rosales , Y. Márquez-Pedroza , M.R. Hernandez-Preciado , G. Gabriel Ortiz
{"title":"The prevalence, incidence, and clinical assessment of neuromyelitis optica spectrum disorder in patients with demyelinating diseases","authors":"M.A. Mireles-Ramírez , I.E. Velázquez-Brizuela , N. Sánchez-Rosales , Y. Márquez-Pedroza , M.R. Hernandez-Preciado , G. Gabriel Ortiz","doi":"10.1016/j.nrleng.2022.06.002","DOIUrl":"10.1016/j.nrleng.2022.06.002","url":null,"abstract":"<div><h3>Background</h3><div>Neuromyelitis optica spectrum disorder (NMOSD) is characterised by recurrent attacks of optic neuritis and transverse myelitis. The purpose of this work was to identify the incidence and prevalence of NMOSD and its clinical characteristics in the population treated for demyelinating diseases in Western Mexico.</div></div><div><h3>Material and method</h3><div>A descriptive, retrospective study was carried out in the Department of Neurology, at the Sub-specialty Medical Unit, Specialties Hospital (known by its Spanish abbreviation <em>UMAE-HE</em>), of the National Western Medical Center (<em>CMNO</em>), Mexican Institute of Social Security (<em>IMSS</em>). A review of the electronic files for all patients with a diagnosis of NMOSD in 2019, was carried out in the State of Jalisco, Mexico.</div></div><div><h3>Results</h3><div>Fifty-eight patients with NMOSD were included in the study. The incidence was 0.71/100 000 (CI 0.60-0.85) and the prevalence was 1.09/100 000 (CI 0.84-1.42). There were 79.3% women, and 20.6% were men (<em>P</em> = .01). All (100%) patients presented with anti-aquaporin-4 immunoglobulin G, and 89.6% showed seropositivity for anti-aquaporin-4 (CI 82.6-94.9). Magnetic resonance imaging was performed on 100% of patients, where 34.4% were normal, and 65.5% (38) abnormal, presenting with non-specific subcortical lesions (<em>P</em> = 0.04). The initial clinical presentation was optic neuritis (ON) in 58.6%; where 31.0% was bilateral ON, 20.7% was left ON, and 6.9% were right ON; transverse myelitis in 26.0%, area postrema syndrome (APS) in 10.3%, among others.</div></div><div><h3>Conclusions</h3><div>The incidence of NMOSD exceeds 0.71/100 000, the prevalence is low at 1.09/100 000, and NMOSD is predominantly found in women.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 743-748"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40541228","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 : 2024-10-01DOI: 10.1016/j.nrleng.2024.09.005
C. González-Mingot , A. Gil-Sánchez , R. Begué-Gómez , R. López-Ortega , B.R. Luis
{"title":"Ischemic encephalopathic debut of CADASIL, a case report: It is better to be safe than sorry","authors":"C. González-Mingot , A. Gil-Sánchez , R. Begué-Gómez , R. López-Ortega , B.R. Luis","doi":"10.1016/j.nrleng.2024.09.005","DOIUrl":"10.1016/j.nrleng.2024.09.005","url":null,"abstract":"","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 712-715"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142416091","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 : 2024-10-01DOI: 10.1016/j.nrleng.2024.09.002
J. Tenente , S. Lopes , P. Bem , M. Vila-Real , D. Ferreira , A.F. Geraldo , F. Santos
{"title":"Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital","authors":"J. Tenente , S. Lopes , P. Bem , M. Vila-Real , D. Ferreira , A.F. Geraldo , F. Santos","doi":"10.1016/j.nrleng.2024.09.002","DOIUrl":"10.1016/j.nrleng.2024.09.002","url":null,"abstract":"<div><h3>Introduction</h3><div>Cerebral venous thrombosis (CVT) is an uncommon and clinically heterogeneous cerebrovascular particularly in children, only a few published case series focused in the pediatric population.</div></div><div><h3>Patients and methods</h3><div>Retrospective single-center observational and analytical study of consecutive pediatric patients admitted in a level II Portuguese hospital with a confirmed diagnosis of CVT, from 2003 to 2021. Clinical presentation, neuroimaging findings, prothrombotic factors, treatment strategies, outcome and recanalization were documented.</div></div><div><h3>Results</h3><div>Twelve children were included (58% female). Mean age was 7.3 years. The most frequent symptoms were vomiting, headache and behavioral alterations. Infection was the triggering factor in 50% of the cases. The diagnosis of CVT was made based on imaging evidence of thrombosis through magnetic imaging resonance (MRI) with venography and/or computed tomography (CT) with venography. In 67% of cases there were multiples sinuses involved; the transverse sinus was the most affected, followed by the sigmoid sinus. In 83% of cases anticoagulant therapy was initiated with low molecular weight heparin (LMWH) and associated prothrombotic factors were investigated, with no major prothrombotic factors identified. No deaths occurred, but 30% had long-term neurological sequelae. One patient recurred 18 years later.</div></div><div><h3>Conclusion</h3><div>The results of this study are consistent with data from other published studies. MRI is the preferred imaging method for diagnosis in children by avoiding ionizing radiation and allowing identification of subjacent causes. Anticoagulation with LMWH is recommended and important to reduce mortality and sequelae. Infectious diseases are the most common trigger for CVT and can also be the cause for high morbidity and poor outcomes.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 658-665"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425825","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 : 2024-10-01DOI: 10.1016/j.nrleng.2022.09.004
{"title":"Psychometric properties of the Spanish version of the Frontal Assessment Battery (FAB-E) and normative values in a representative adult population sample","authors":"","doi":"10.1016/j.nrleng.2022.09.004","DOIUrl":"10.1016/j.nrleng.2022.09.004","url":null,"abstract":"<div><h3>Background</h3><div>The Frontal Assessment Battery is a short bedside test used to assess executive functions (EF). The aims of the present study were, first, to evaluate the psychometric proprieties of the Spanish version of the FAB (FAB-E) in a representative sample, and second, to establish cut-off points for impairment in executive function according to age and education level.</div></div><div><h3>Methods</h3><div>A sample of 798 healthy Spanish adult subjects aged 19 to 91 participated in this study. Neuropsychological assessment of participants was conducted using the FAB-E, Mini-Mental State Examination (MMSE) and Trail Making Test (TMT). We examined internal consistency, intraclass correlation, test-retest reliability, and concurrent and divergent validity. In addition, we established a cut-off point for detecting executive function impairment based on the 5th percentile by age group and education level.</div></div><div><h3>Results</h3><div>The analysis of the psychometric properties of the FAB-E showed good internal consistency (Cronbach’s α = 0.60), intraclass correlation (0.72), test-retest reliability (0.70) and concurrent and divergent validity between the TMT (<em>r</em> = −0.523), MMSE (<em>r</em> = 0.426) and the FAB-E. The cut-off points for each age group were 16 points for the ≤ 29 group, 15 points for the 30-39 group, 14 points for the 40-49 and 50-59 groups, 12 points for the 60-69 group, and 10 points for the ≥ 70 age group.</div></div><div><h3>Conclusions</h3><div>The psychometric analysis showed that the FAB-E has good validity and reliability. Thus, FAB-E may be a helpful tool to evaluate EF in a healthy Spanish population. In addition, this study provides information on reference data that will be very valuable for clinicians and researchers.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 694-700"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33497663","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 : 2024-10-01DOI: 10.1016/j.nrleng.2022.09.010
{"title":"Kernohan-Woltman notch phenomenon: an exceptional neurological picture?","authors":"","doi":"10.1016/j.nrleng.2022.09.010","DOIUrl":"10.1016/j.nrleng.2022.09.010","url":null,"abstract":"<div><h3>Introduction</h3><div>Ipsilateral hemiparesis (IH) can be defined as a paradoxical dysfunction of the first motor neuron involving the extremities on the opposite side to that expected, given the location of the triggering intracranial pathology. Compression of the corticospinal tract (CSt) along its course through the contralateral cerebral peduncle against the free edge of the tentorium, known as the Kernohan-Woltman notch phenomenon (KWNP), represents the main cause of IH.</div></div><div><h3>Methods</h3><div>This retrospective study analyses a series of 12 patients diagnosed with IH secondary to KWNP treated at our institution, including a descriptive study of epidemiological, clinical, radiological, neurophysiological, and prognostic variables.</div></div><div><h3>Results</h3><div>In 75% of the cases, symptoms had an acute or subacute onset. Initial imaging studies showed signs of significant mass effect in half of the patients, whereas magnetic resonance imaging (MRI) identified a structural lesion in the contralateral cerebral peduncle in two thirds of them. Impairment of the motor evoked potentials (MEP) was verified in 4 patients. During follow-up 7 patients experienced improvement in motor activity, and near half of the cases were classified in the first three categories of the modified Rankin scale.</div></div><div><h3>Conclusions</h3><div>In contrast to prior historical series, most of our patients developed a KWNP secondary to a traumatic mechanism. MRI represents the optimal method to identify both the classic cerebral peduncle notch and the underlying structural lesion of the CSt. The use of MEP can help to establish the diagnosis, especially in those cases lacking definite radiological findings.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 683-693"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40711319","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 : 2024-10-01DOI: 10.1016/j.nrleng.2024.09.003
S. Bermudo Fuenmayor , P.J. Serrano Castro , P. Quiroga Subirana , S. López Palmero , M.M. Requena Mullor , T. Parrón Carreño
{"title":"Design and validation of a questionnaire for monitoring neurological dysphagia and respiratory deterioration in patients with amyotrophic lateral sclerosis (DEREDELA)","authors":"S. Bermudo Fuenmayor , P.J. Serrano Castro , P. Quiroga Subirana , S. López Palmero , M.M. Requena Mullor , T. Parrón Carreño","doi":"10.1016/j.nrleng.2024.09.003","DOIUrl":"10.1016/j.nrleng.2024.09.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Amyotrophic lateral sclerosis (ALS) is a degenerative disease of unknown origin that affects the motor neurons. It has a rapid, fatal course.</div></div><div><h3>Method</h3><div>For this study, an initial questionnaire of eleven items was developed by experts in the field, who evaluated the suitability and relevance of the items.</div></div><div><h3>Results</h3><div>The questionnaire was then applied to a pilot group of 22 patients diagnosed with ALS. Confirmatory factor analysis, based on estimating maximum likelihood, confirmed the three domains detected in the exploratory factor analysis. The reliability of the scale was tested using Cronbach's <em>α</em> (0.801) and the Kaiser–Meyer–Olkin test (0.770) confirmed the construct validity.</div></div><div><h3>Conclusions</h3><div>The DEREDELA questionnaire is valid, in terms of its content, for monitoring the neurological dysphagia and respiratory deterioration suffered by patients diagnosed with ALS.</div></div>","PeriodicalId":94155,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 666-674"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142425826","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}