Acta Neurologica Scandinavica最新文献

{"title":"Cognitive Theory of Mind Deficit Associated with Executive Dysfunction in Cervical Dystonia","authors":"Christin Kupper,&nbsp;Hannah Lochmann,&nbsp;Michelle Wyrobnik,&nbsp;Oliver Steiner,&nbsp;Fabian Klostermann","doi":"10.1155/2023/8873653","DOIUrl":"10.1155/2023/8873653","url":null,"abstract":"<div>\u0000 <p><i>Introduction</i>. Cervical dystonia (CD) is viewed as a circumscribed movement disorder. However, beyond motor symptoms, it can imply subtle cognitive change, among others with respect to theory of mind (ToM) capacities. Here, affective and cognitive ToM performances and potential relations to other mental functions were investigated to refine the concept of social cognitive dysfunction in CD. <i>Methods</i>. 20 persons with CD were clinically assessed, together with 20 healthy controls engaged in overview cognitive testing, executive function (EF) tasks, and the Faux Pas Recognition Test (FPRT) as well as the Reading the Mind in the Eyes Tests (RMET) addressing cognitive and affective ToM functions, respectively. <i>Results</i>. Persons with CD showed lower cognitive, but not affective ToM performance than controls. Further, they had abnormally low word production in verbal fluency (VF) tasks, imposing high EF demands. Generally, ToM performance correlated with VF and, further, with the decreased quality of life score in persons with CD. <i>Conclusion</i>. Cognitive ToM deficits seem to occur in the context of executive dysfunction in CD. They belong to an underrecognized spectrum of nonmotor symptoms of likely clinical relevance.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/8873653","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138590792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sarcopenia and Hypoxia in Patients with Obstructive Sleep Apnea","authors":"Jinseung Kim,&nbsp;Ho-Joon Lee,&nbsp;Dong Ah. Lee,&nbsp;Kang Min Park","doi":"10.1155/2023/4310809","DOIUrl":"10.1155/2023/4310809","url":null,"abstract":"<div>\u0000 <p><i>Objectives</i>. Obstructive sleep apnea (OSA) is associated with a variety of health issues. Both OSA and sarcopenia are associated with metabolic disorders; however, there is limited literature assessing the correlation between them. Therefore, we aimed to investigate sarcopenia using temporal muscle thickness (TMT) in patients with obstructive sleep apnea (OSA) and the association between sarcopenia and hypoxia. <i>Methods</i>. We enrolled patients with OSA and healthy controls. Both groups underwent brain magnetic resonance imaging (MRI) scans, including three-dimensional T1-weighted imaging. TMT, a marker for sarcopenia, was obtained based on the T1-weighted imaging and compared between the groups. Additionally, we analyzed the correlation between TMT and clinical factors in patients with OSA. <i>Results</i>. In total, 40 patients with OSA and 52 healthy controls were evaluated. There was no difference in TMT between patients with OSA and healthy controls (10.425 ± 2.13 vs. 10.400 ± 1.94 mm, <i>p</i> = 0.953). In the correlation analysis, age was negatively correlated with TMT (<i>r</i> = −0.356, <i>p</i> = 0.023), and lowest oxygen saturation (<i>r</i> = −0.558, <i>p</i> &lt; 0.001) in patients with OSA. <i>Conclusion</i>. Our results demonstrated negative correlations between TMT and lowest oxygen saturation in the patients with OSA. These findings suggest potential relationships between sarcopenia and hypoxia in patients with OSA. Thus, these results underline the importance of maintaining oxygen saturation in patients with OSA by implementing active treatment. This study also demonstrates the feasibility of sarcopenia assessment by measuring TMT through conventional head MRI in patients with various neurological disorders.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/4310809","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138596059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Real-Life Effectiveness and Tolerability of Brivaracetam in Focal to Bilateral and Primary Generalized Tonic-Clonic Seizures","authors":"Elena Fonseca,&nbsp;Ariadna Gifreu,&nbsp;Javier Abril Jaramillo,&nbsp;Luis Redondo Vergé,&nbsp;Manuel Quintana,&nbsp;Sofía Lallana,&nbsp;Samuel López-Maza,&nbsp;Laura Abraira,&nbsp;Daniel Campos-Fernández,&nbsp;Estevo Santamarina,&nbsp;Juan Rodríguez Uranga,&nbsp;Manuel Toledo","doi":"10.1155/2023/6636730","DOIUrl":"10.1155/2023/6636730","url":null,"abstract":"<div>\u0000 <p><i>Purpose</i>. Brivaracetam (BRV), an antiseizure medication indicated for focal-onset seizures, has shown efficacy in the treatment of focal to bilateral tonic-clonic seizures (FBTCS). We aimed to determine the effectiveness and safety of BRV in patients with FBTCS and generalized tonic-clonic seizures (GTCS). <i>Methods</i>. We performed a multicenter, retrospective, longitudinal study in adult patients with epilepsy who experienced at least one FBTCS or GTCS before starting BRV (baseline visit). Data were collected from consecutive outpatient visits over a 4-year period. All patients had been followed for at least 3 months before the baseline visit and completed a minimum follow-up of 3 months after starting BRV. Response (≥50% reduction in FBTCS/GTCS frequency) and retention rates, as well as seizure freedom and presence of adverse events at 3, 6, and 12 months, were recorded as outcome measures. <i>Results</i>. 114 patients were included (mean age 36.3 ± 18.0 years, 52% male, 36.6% genetic generalized epilepsy); 94 had a 12-month follow-up period. At 12 months’ follow-up, the response rate was 83%, and 73.4% of patients were FBTCS/GTCS-free. Retention was 79% at 12 months. Adverse events occurred in 29.8% of patients, the most common being drowsiness (14.9%). No significant differences were found in response rates between FBTCS and GTCS. Drug resistance was independently associated with lower response and seizure freedom rates at follow-up. The absence of a titration period predicted seizure freedom and response at 3 months. <i>Conclusions</i>. BRV is an effective and well-tolerated treatment in patients with focal to bilateral tonic-clonic seizures and generalized tonic-clonic seizures.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/6636730","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138603736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex Differences in the Association between Hemoglobin A1c and Cerebral White Matter Lesions in the General Japanese Population","authors":"Honghao Li,&nbsp;Jing Yu,&nbsp;Shougang Guo","doi":"10.1155/2023/9580116","DOIUrl":"10.1155/2023/9580116","url":null,"abstract":"<div>\u0000 <p>The influence of diabetes and associated sex differences on cerebral white matter lesions (WML) is unclear. We used data from a cross-sectional study uploaded to the DATADRYAD website by Shinkawa et al. to investigate differences in the association between hemoglobin A1c (HbA1c) levels and cerebral WML between men and women. The average age of all participants was 56.4 ± 11.5 years old, and approximately 51.89% of them were men. A linear relationship between HbA1c and cerebral WML was detected in men. Fully adjusted binary logistic regression showed no association of HbA1c with cerebral WML in men. A nonlinear relationship between HbA1c and cerebral WML was detected in women, whose cutoff point was 5.6%. The effect sizes and confidence intervals of the left and right sides of the inflection point were OR = 0.21 (95% CI 0.06, 0.69, <i>P</i> = 0.0098) and OR = 3.5 (95% CI 1.50, 8.15, <i>P</i> = 0.0037), respectively. In the higher HbA1c group, further subgroup analysis showed a stronger association between HbA1c and cerebral WML in women (OR = 3.83, 95% CI 1.68, 8.72 <i>P</i> = 0.0014) than in men (OR = 1.02, 95% CI 0.76, 1.36 <i>P</i> = 0.8986) (<i>P</i> for interaction with sex was 0.0004). A stronger effect of HbA1c on the risk of cerebral WML in women than in men was found in the higher HbA1c group.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/9580116","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141150579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum Biomarkers of Olfactory Identification Deficits in Patients with Parkinson’s Disease","authors":"Fu-Jia Li,&nbsp;Yang-Dan-Yu Li,&nbsp;Xu Liu,&nbsp;Jie Zu,&nbsp;Wei Zhang,&nbsp;Qi-Hua Xiao,&nbsp;Xue-Bin Niu,&nbsp;Li Du,&nbsp;Chen-Chen Cui,&nbsp;Ru-Yu Zhang,&nbsp;Xiao-Qing He,&nbsp;Gui-Yun Cui,&nbsp;Chuan-Ying Xu","doi":"10.1155/2023/8860125","DOIUrl":"10.1155/2023/8860125","url":null,"abstract":"<div>\u0000 <p>To investigate whether glial fibrillary acidic protein (GFAP), neurofilament light chain (NFL), and 12 cytokines can serve as serum biomarkers of olfactory identification dysfunction in patients with Parkinson’s disease (PD). GFAP and NFL levels were measured in 75 patients with PD and 36 healthy controls (HCs). The levels of 12 cytokines were assayed in 41 patients with PD. The 16-item Sniffin’ Sticks test and the Mini-Mental State Examination (MMSE) were used to assess olfactory identification ability and cognitive function, respectively. Linear regression models were applied to control for confounding effects. Receiver operating characteristic curves were used to examine the diagnostic accuracy of serum NFL, GFAP, and interleukin-6 (IL-6) levels. The cut-off value for the SS-16 test in diagnosing dysosmia was equal to 9.5 points. Serum GFAP levels were higher in patients with PD with olfactory identification dysfunction than in those without. GFAP, NFL, and IL-6 levels were correlated with SS-16 scores. Moreover, combining these three biomarkers yielded the best-fitting model for distinguishing patients with PD with or without dysosmia. We found a prominent indirect effect of GFAP on MMSE scores through its contribution to SS-16 scores. GFAP, NFL, and IL-6 can serve as serum biomarkers for olfactory identification dysfunctions in PD. We inferred that astrogliosis might promote the occurrence of dysosmia by releasing proinflammatory factors and causing neuronal damage and may indirectly impair cognition through its effect on olfactory function.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/8860125","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139275457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis and Prognosis in Disorders of Consciousness: An Active Paradigm fMRI Study","authors":"Laura Schnetzer,&nbsp;Verena S. Schätzle,&nbsp;Lisa Kronbichler,&nbsp;Jürgen Bergmann,&nbsp;Stefan Leis,&nbsp;Alexander B. Kunz,&nbsp;Julia S. Crone,&nbsp;Eugen Trinka,&nbsp;Martin Kronbichler","doi":"10.1155/2023/3991087","DOIUrl":"10.1155/2023/3991087","url":null,"abstract":"<div>\u0000 <p>Diagnoses in patients with disorders of consciousness are prone to misdiagnosis; thus, research has sought approaches to increase reliability, for instance, with functional MRI. By applying a motor imagery task, patients showing covert command following despite the absence of behavioural signs of awareness can be identified as being in a cognitive motor dissociation. This study seeks to determine the proportion of patients, with unresponsive wakefulness syndrome and minimally conscious state, who display covert command following. Moreover, the prognostic value of the improved diagnosis and different methodical approaches to analyse the functional MRI data were evaluated. 73 disorder of consciousness patients (35 unresponsive, 35 minimally conscious, and three already recovered) underwent weekly standardized behavioural assessments with the coma-recovery scale—revised and one functional MRI examination comparing their brain activations in the supplementary motor area between phases of imaging playing tennis and rest. 27 healthy controls served as a control group. The data was evaluated using different region-of-interest analyses (one- and two-tailed small-volume correction and region-of-interest exploration approaches) and a whole-brain analysis. Based on the one-tailed small volume correction data, seven patients, all of nontraumatic aetiology, showed covert command following. The one-tailed region-of-interest exploration identified three additional responders. 10 patients showed significantly more activation during rest than during the imagery paradigm (negative responders). 40% of patients (minimally conscious patients being three times more likely) showed significant activations in the whole brain analysis. Besides, no significant further associations were found between covert command following and clinical parameters. The analyses showed that the tennis paradigm could identify patients with cognitive motor dissociation with a nontraumatic aetiology, but our data failed to show any short-term prognostic validity. The relevance of negative responders and activated regions outside of the region of interest should be further investigated.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/3991087","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136281946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of Dual- versus Single-Antiplatelet Therapy on Early Neurological Deterioration in Minor Stroke of Undetermined Cause","authors":"Bingdong Xu,&nbsp;Xiufeng Xin,&nbsp;Yan Ding,&nbsp;Anding Xu,&nbsp;Yusheng Zhang","doi":"10.1155/2023/5532467","DOIUrl":"10.1155/2023/5532467","url":null,"abstract":"<div>\u0000 <p><i>Background</i>. There is insufficient evidence about the suitability of dual-antiplatelet therapy (DAPT) for different stroke subtypes. We aimed to determine the relationship between DAPT and early neurological deterioration (END) in patients with minor stroke of undetermined cause. <i>Methods</i>. We retrospectively collected data on patients with minor stroke treated with aspirin alone or in combination with clopidogrel and aspirin. Efficacy was the incidence of END defined as the National Institutes of Health Stroke Scale score increase of ≥2 within 7 days after admission. Safety was defined as the rate of any bleeding event. These were investigated in subtypes including the stroke of undetermined cause (SUC), large artery atherosclerosis (LAA), cardioembolism (CE), and small artery occlusion (SAO). <i>Results</i>. 442 patients were assigned to the SUC (<i>n</i> = 91), LAA (<i>n</i> = 157), CE (<i>n</i> = 30), and SAO (<i>n</i> = 164) groups. The incidences of END were not significantly different between patients treated with dual- versus single-antiplatelet therapy in any stroke subtypes: LAA, 17.6% vs. 12.1% (<i>P</i> = 0.348); CE, 0% vs. 20.0% (<i>P</i> = 0.224); SAO, 8.8% vs. 2.4% (<i>P</i> = 0.093); and SUC, 13.6% vs. 2.1% (<i>P</i> = 0.053). Multivariable analysis showed that after adjusting for confounding factors, DAPT was the independent factor associated with END (odds ratio 13.39, 95% confidence interval (1.16-154.81), <i>P</i> = 0.038) in the SUC group, rather than the LAA, CE, and SAO groups. <i>Conclusion</i>. Combined clopidogrel and aspirin is a risk factor for the rate of END only in minor stroke patients with the SUC subtype. This suggests that cryptogenic stroke may not be suitable for DAPT in the acute phase.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/5532467","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135567916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Instruments and Parameters for Evaluating Upper Limb Motor Fatigability in Individuals with Neuromuscular Diseases: Systematic Review","authors":"Danila Cristina Petian-Alonso,&nbsp;Camila Scarpino Barboza Franco,&nbsp;Emanuela Juvenal Martins,&nbsp;Karoliny Lisandra Teixeira Cruz,&nbsp;Roberta de Fátima Carreira Moreira Padovez,&nbsp;Ana Claudia Mattiello-Sverzut","doi":"10.1155/2023/8373917","DOIUrl":"10.1155/2023/8373917","url":null,"abstract":"<div>\u0000 <p><i>Background</i>. Neuromuscular diseases present a set of clinical and pathological disabilities that include muscle weakness and atrophy, perception of fatigue, fatigability, and contracture. Motor fatigability compromises the ability of the individual to generate muscle strength and perform their daily activities. Quantitative measures of strength and motor fatigability are important to determine the clinical progression of the disease and the response to the proposed treatments. Thus, the aim of this study was to identify the equipment and protocols frequently used to assess upper limb motor fatigability in patients with neuromuscular disease. <i>Methods</i>. Information such as equipment used to induce motor fatigability, body segment or joint studied, movement analyzed, type of contraction, and protocol utilized for the test was analyzed. Joanna Briggs Scale and Newcastle-Ottawa Scale assessed the methodological quality of the studies. In addition, a checklist was prepared by the research group to assess the protocols presented in the referred studies. <i>Results</i>. The isokinetic and handgrip dynamometers were the most utilized equipment to induce motor fatigability. 83% of the studies had a design with low methodological rigor and half of them with high risk of bias. In the analysis of the protocols utilized to induce motor fatigability, one study was classified as regular and the other ones as good. <i>Conclusion</i>. The methodological topics to assess motor fatigability were incompletely described considering the electrophysiological and biomechanical approach. Although the motor fatigability in the upper limb was evaluated using isokinetic and handgrip equipment, the absence of a gold standard protocol still compromises the understanding of clinical progression and responses to the treatments in the neuromuscular diseases. This trial is registered with CRD42021206934.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/8373917","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135853765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erratum to “Changes in Retinal Thickness and Brain Volume during 6.8-Year Escalating Therapy for Multiple Sclerosis”","authors":"Max Borgström,&nbsp;Mats Fredrikson,&nbsp;Magnus Vrethem,&nbsp;Pierfrancesco Mirabelli,&nbsp;Hans Link,&nbsp;Yumin Huang-Link","doi":"10.1155/2023/9764870","DOIUrl":"10.1155/2023/9764870","url":null,"abstract":"<p>In the article titled “Changes in Retinal Thickness and Brain Volume during 6.8-Year Escalating Therapy for Multiple Sclerosis” [<span>1</span>], incorrect versions of Figures 1(a) and 1(b) were published due to an error in the production process. Specifically, “baseline” and “high-efficacy DMT” on the <i>x</i>-axis are incorrectly labelled. In addition, the <i>x</i>-axis labels are displaced from the corresponding bar chart columns. The correct figures are shown below.</p>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/9764870","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139319954","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Understanding the Cross-Talk between the Ceramide Biosynthetic Pathway and Mitochondrial Function in Multiple Sclerosis and Demyelinating Disorders","authors":"Amarjeet,&nbsp;Raja Babu,&nbsp;Abhipsa Mohapatra,&nbsp;Bhaskaranand Pancholi,&nbsp;Debapriya Garabadu,&nbsp;Anjali Sharma,&nbsp;Ashwani Sharma,&nbsp;Md. A. K. Azad","doi":"10.1155/2023/7398037","DOIUrl":"10.1155/2023/7398037","url":null,"abstract":"<div>\u0000 <p>Myelin is a spiral compilation of uniformly thick membranes around the axon in an alternating fashion, and it is formed by a complicated process known as myelination. Myelin sheaths are responsible for various physiological functions such as metabolism, rapid nerve conduction, and maintaining ionic and water homeostasis in the brain. Lipid is one of the major components in the myelin, which includes cholesterol, ceramide, and their derivatives, such as galactosylceramide, sulfatide, and gangliosides. Ceramide and its derivatives are synthesised by various ceramide biosynthetic pathways such as de novo, salvage, sphingomyelinase, and recycling of exogenous ceramide. At an appropriate level, ceramide facilitates the development of the nervous system, cell proliferation, autophagy, and apoptosis, which are responsible for normal functioning, but when the level is altered from normal, it results in mitochondrial dysfunction or cell death through autophagy and apoptosis. The ceramide level increases, especially in the mitochondria. Ceramide level increases in response to oxidative stress which is mediated by inflammatory cytokines. Due to mitochondrial dysfunction, an energy-deficient condition is created because of disruption in the electron transport chain, which results in the death of neurons and glial cells, which subsequently cause demyelination and degeneration of axon. Losing myelin while axons remain relatively intact is the characteristic feature of demyelinating diseases. The primary element of demyelinating disorder is damage, malfunction, failure, or death of mitochondria. These disturbances may occur due to direct or indirect interaction of ceramide with mitochondria. There are several risk factors for demyelination, such as viruses, bacteria, fungi, trauma, obesity, vitamin D deficiency, and genetic and environmental factors. Thus, the review is mainly aimed towards the interaction between ceramide and mitochondria during demyelination.</p>\u0000 </div>","PeriodicalId":6939,"journal":{"name":"Acta Neurologica Scandinavica","volume":"2023 1","pages":""},"PeriodicalIF":2.9,"publicationDate":"2023-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1155/2023/7398037","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136013855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}