Neurologia最新文献

{"title":"The clinical characteristics of Hirayama disease in females","authors":"Hongwei Wang ,&nbsp;Wei Lei ,&nbsp;Ye Tian ,&nbsp;Jianwei Wu ,&nbsp;Xiaosheng Ma ,&nbsp;Feizhou Lyu ,&nbsp;Xinlei Xia ,&nbsp;Jingjuan Liang ,&nbsp;Jianyuan Jiang ,&nbsp;Hongli Wang","doi":"10.1016/j.nrl.2022.06.009","DOIUrl":"10.1016/j.nrl.2022.06.009","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":19300,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 792-801"},"PeriodicalIF":2.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142660389","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}

{"title":"Rare neurological and neuropsychiatric manifestations of scrub typhus: a case series of 10 cases","authors":"Ritwik Ghosh ,&nbsp;Arpan Mandal ,&nbsp;Moisés León-Ruiz ,&nbsp;Dipayan Roy ,&nbsp;Shambaditya Das ,&nbsp;Souvik Dubey ,&nbsp;Julián Benito-León","doi":"10.1016/j.nrl.2022.06.006","DOIUrl":"10.1016/j.nrl.2022.06.006","url":null,"abstract":"<div><h3>Introduction</h3><div>Scrub typhus is a potentially life-threatening but curable disease that can produce multi-organ failure. Neurological manifestations in scrub typhus have gained attention recently, where the entire neural axis except the myoneural junction can be involved. Although the pathogenesis of neurological involvement has not been established, immune-mediated mechanisms are suspected. This article reports the clinicopathological features of scrub typhus cases presenting several rare neurological and neuropsychiatric manifestations.</div></div><div><h3>Methods</h3><div>Three hundred fifty-four serologically confirmed scrub typhus cases were admitted to the Department of General Medicine of Burdwan Medical College and Hospital (West Bengal, India) between May 2018 and May 2022. There were 50 patients who had predominantly neurological manifestations. Of these 50 cases, ten patients presented with extremely rare neurological manifestations.</div></div><div><h3>Results</h3><div>We report 10 cases of scrub typhus (four men and six women) who presented with complex neurological pictures (posterior reversible encephalopathy syndrome, Opalski syndrome, parkinsonism, cerebellitis, isolated opsoclonus, acute transverse myelitis, myositis, polyradiculoneuropathy with cranial neuropathy, acute transient behavioral changes, and fibromyalgia). Immune-mediated mechanisms might have mediated the pathogenesis of most cases following scrub typhus infection.</div></div><div><h3>Conclusion</h3><div>From a clinicopathological point of view, each case was unique in its presentation and treatment response. In any acute onset neurological disorders associated with febrile illness in the tropics or subtropics, scrub typhus infection should be included in the differential diagnosis, despite the absence of eschar and unremarkable neuroimaging findings. This otherwise curable disease may result in multi-organ dysfunction syndrome and death if the diagnosis is delayed.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 766-780"},"PeriodicalIF":2.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142659707","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}

{"title":"Insomnio fatal esporádico: fenotipo PSP-like rápidamente progresivo","authors":"J.C. Romero-Fábrega ,&nbsp;R. Lorenzo-López ,&nbsp;E. Rivas-Infante ,&nbsp;F. Escamilla-Sevilla ,&nbsp;M. Rashki ,&nbsp;A. Mínguez-Castellanos ,&nbsp;A. Carvajal-Hernández","doi":"10.1016/j.nrl.2023.05.004","DOIUrl":"10.1016/j.nrl.2023.05.004","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 820-823"},"PeriodicalIF":2.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139825264","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}

{"title":"The prevalence, incidence, and clinical assessment of neuromyelitis optica spectrum disorder in patients with demyelinating diseases","authors":"M.A. Mireles-Ramírez ,&nbsp;I.E. Velázquez-Brizuela ,&nbsp;N. Sánchez-Rosales ,&nbsp;Y. Márquez-Pedroza ,&nbsp;M.R. Hernandez-Preciado ,&nbsp;G. Gabriel Ortiz","doi":"10.1016/j.nrl.2022.06.004","DOIUrl":"10.1016/j.nrl.2022.06.004","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":19300,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 743-748"},"PeriodicalIF":2.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142659613","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}

{"title":"Test cognitivos breves como herramienta de decisión en Atención Primaria. Estudio poblacional y de validación","authors":"M. Tainta ,&nbsp;A. Iriondo ,&nbsp;M. Ecay-Torres ,&nbsp;A. Estanga ,&nbsp;M. de Arriba ,&nbsp;M. Barandiaran ,&nbsp;M. Clerigue ,&nbsp;M. Garcia-Sebastian ,&nbsp;J. Villanua ,&nbsp;A. Izagirre ,&nbsp;J. Saldias ,&nbsp;A. Aramburu ,&nbsp;J. Taboada ,&nbsp;J. Múgica ,&nbsp;A. Barandiaran ,&nbsp;A. Arrospide ,&nbsp;J. Mar ,&nbsp;P. Martinez-Lage","doi":"10.1016/j.nrl.2022.05.006","DOIUrl":"10.1016/j.nrl.2022.05.006","url":null,"abstract":"<div><h3>Introduction and objectives</h3><div>Brief cognitive tests (BCT) are used in primary care (PC) for the detection of cognitive impairment (CI). Still, there are little data on their diagnostic utility (DU) in a community setting. This work evaluates the DU at the population level of Fototest, T@M, AD8 questionnaire and MMSE. It provides new cut-off points (CoP) validated in a CI early detection program.</div></div><div><h3>Material and methods</h3><div>In the population and validation samples, the evaluation was carried out in 2<!--> <!-->phases, a first of screening and administration of BCT and a second of clinical diagnosis, blinded to the results of the BCT, applying the current NIA-AA criteria. The DU of BCT in the population sample was evaluated with the area under the ROC curve (aROC). Youden index and the CoP with the best specificity that ensured a sensitivity of 80% were used to decide on the most appropriate CoP. The sensitivity, specificity, and predictive values for these CoP were calculated in the validation sample.</div></div><div><h3>Results</h3><div>260 participants (23.1% with CI) from the population sample and 177 (42.4% with CI) from the validation sample were included. The Fototest has the best UD at the population level (aROC 0.851), which improves with the combination of Fototest and AD8 (aROC 0.875). The proposed CoP are AD8≥1, Fototest≤35, T@M≤40, and MMSE≤26.</div></div><div><h3>Conclusion</h3><div>BCT are helpful in detecting CI in PC. This work supports the use of more demanding PoC.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 9","pages":"Pages 781-791"},"PeriodicalIF":2.9,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46387487","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}

{"title":"Ischemic encephalopathic debut of CADASIL, a case report: It is better to be safe than sorry","authors":"","doi":"10.1016/j.nrl.2023.02.002","DOIUrl":"10.1016/j.nrl.2023.02.002","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 712-715"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139635794","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}

{"title":"Post-COVID-19 memory complaints: Prevalence and associated factors","authors":"","doi":"10.1016/j.nrl.2022.03.007","DOIUrl":"10.1016/j.nrl.2022.03.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Memory complaints resulting from COVID-19 may have a significant impact on the survivors’ quality of life. Unfortunately, there is insufficient information available on memory loss and its relationship to COVID-19. Therefore, the purpose of this research was to determine the prevalence of memory complaints in post-COVID-19 patients and to find potential contributing factors.</div></div><div><h3>Method</h3><div>A cross-sectional survey was conducted on 401 individuals who had previously been diagnosed with COVID-19 at four COVID testing centers situated across Bangladesh. The MAC-Q questionnaire was used to evaluate memory. A binary logistic regression model was fit to study the variables related to memory complaints, with a <em>p</em>-value of &lt;0.05 deemed statistically significant.</div></div><div><h3>Result</h3><div>Memory complaints was prevalent in 19.2% of the post-COVID patients. Individual predictor analysis revealed that among the treatment modalities, steroids and antibiotics were associated with impaired memory. Multiple logistic regression showed that individuals who recovered from COVID-19 within six to twelve months were more likely to have memory deficits. Even though age, sex, oxygen demand, and hospitalization were not linked with memory complaints, rural residents exhibited more significant memory complaints than urban residents.</div></div><div><h3>Conclusion</h3><div>Nearly one-fifth of the COVID-19 patients suffer from various degrees of memory complaints within one year. However, no association was found between COVID-19 severity to memory complaints.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 651-657"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9020525/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78133866","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}

{"title":"Unilateral MRIgFUS thalamotomy: Long-term follow-up in fragile X-associated tremor/ataxia syndrome","authors":"","doi":"10.1016/j.nrl.2023.11.002","DOIUrl":"10.1016/j.nrl.2023.11.002","url":null,"abstract":"","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 710-712"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139637715","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}

{"title":"Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital","authors":"","doi":"10.1016/j.nrl.2022.04.006","DOIUrl":"10.1016/j.nrl.2022.04.006","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":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 658-665"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48736380","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}

{"title":"Incidencia y prevalencia de la esclerosis múltiple en España. Una revisión sistemática","authors":"","doi":"10.1016/j.nrl.2022.02.006","DOIUrl":"10.1016/j.nrl.2022.02.006","url":null,"abstract":"<div><h3>Introduction</h3><div>Greater understanding of the prevalence and incidence of multiple sclerosis in Spain and their temporal trends is necessary to improve the allocation of healthcare resources and to study aetiological factors.</div></div><div><h3>Methods</h3><div>We performed a systematic search of the MedLine database and reviewed the reference lists of the articles gathered. We collected studies reporting prevalence or incidence rates of multiple sclerosis in any geographical location in Spain, with no time limits. In 70% of cases, data were extracted by 2 researchers (FGL and EAC); any discrepancies were resolved by consensus.</div></div><div><h3>Results</h3><div>We identified 51 prevalence and 33 incidence studies published between 1968 and 2018. In the adjusted analysis, the number of prevalent cases per 100 000 population increased by 26.6 (95% confidence interval [CI], 21.5-31.8) every 10 years. After adjusting for year and latitude, the number of incident cases per 100 000 population increased by 1.34 (95% CI, 0.98-1.69) every 10 years. We observed a trend toward higher prevalence and incidence rates at higher latitudes.</div></div><div><h3>Conclusions</h3><div>The prevalence of multiple sclerosis in Spain has increased in recent decades, although case ascertainment appears to be incomplete in many studies. Incidence rates have also increased, but this may be due to recent improvements in the detection of new cases.</div></div>","PeriodicalId":19300,"journal":{"name":"Neurologia","volume":"39 8","pages":"Pages 639-650"},"PeriodicalIF":2.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48872997","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}