Zengyan Bai, Ke Shi, Hongwei Liu, Zhinan Ye, Suqian Xu
{"title":"Dexmedetomidine use and short-term all-cause mortality in ICU patients with ischemic stroke and atrial fibrillation: a propensity-matched analysis from MIMIC-IV.","authors":"Zengyan Bai, Ke Shi, Hongwei Liu, Zhinan Ye, Suqian Xu","doi":"10.1159/ene/acsag008","DOIUrl":"https://doi.org/10.1159/ene/acsag008","url":null,"abstract":"<p><p>Introduction Preclinical studies have demonstrated that dexmedetomidine (DEX), a sedative widely used in intensive care units (ICUs), exerts neuroprotective effects. However, its potential protective role and underlying mechanisms in ischemic stroke patients with atrial fibrillation (AF) remain unclear. The study's objective was to assess the connection between the early administration of DEX and short-term mortality from all causes in patients with ischemic stroke and AF. Methods From the MIMIC-IV database, patients with ischemic stroke and AF were identified, including those who were administered DEX and those who were not. The primary endpoint was 7-day all-cause mortality, and the secondary endpoint was 90-day all-cause mortality. Propensity score matching (PSM) was utilized to ensure baseline covariates were balanced. Cox proportional hazards models and Kaplan-Meier survival curves and were utilized to evaluate the association between dexmedetomidine (DEX) use and mortality, with subgroup analyses conducted to ensure the findings' robustness. Results In total, 1,355 patients with ischemic stroke and AF participated, with 253 (18.7%) receiving DEX. After PSM, 208 pairs were matched. In the fully adjusted model, dexmedetomidine (DEX) administration was significantly associated with lower all-cause mortality compared with the control group at both 7 days (before PSM, HR=0.36, 95%CI: 0.19-0.68, P=0.002; after PSM, HR=0.26, 95%CI: 0.12-0.54, P<0.001) and 90 days (before PSM, HR=0.84, 95%CI: 0.66-1.07, P=0.162; after PSM, HR=0.72, 95%CI: 0.53- 0.97, P=0.031). The subgroup analyses revealed that DEX's protective effect was stronger in patients aged 70 and above, females, those with hypertension, and those without heart failure or chronic kidney disease. Kaplan-Meier survival curves consistently illustrated that DEX users had greater survival probabilities both before and after matching. Conclusion In patients with ischemic stroke complicated by AF, DEX administration is connected to decreased mortality, underlining the possible importance of DEX in ICU settings.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149192078","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Elisa Bianchi, E Beghi, Anisia Carlino, Federica Cerri, Christian Lunetta, Eugenio Vitelli, Ammar Al-Chalabi, Maurizio A Leone, Elisabetta Pupillo
{"title":"Needs and Expectations of ALS Patients and Caregivers: An Integrated Perspective.","authors":"Elisa Bianchi, E Beghi, Anisia Carlino, Federica Cerri, Christian Lunetta, Eugenio Vitelli, Ammar Al-Chalabi, Maurizio A Leone, Elisabetta Pupillo","doi":"10.1159/ene/acsag005","DOIUrl":"https://doi.org/10.1159/ene/acsag005","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease requiring complex multidisciplinary care. As disability advances, patients' and caregivers' needs may diverge.</p><p><strong>Objectives: </strong>to assess needs, priorities and satisfaction with care among people with ALS (pALS) and their caregivers; to examine the association between satisfaction and Quality of life (QoL); to explore differences according to clinical characteristics; to understand the Italian ALS community's willingness to engage in research.</p><p><strong>Methods: </strong>In this cross-sectional multicentre study, 154 pALS and 211 caregivers were recruited (July 2021-November 2022). Structured interviews explored information received, care priorities, research expectations and satisfaction. Answers to the interview were compared between patients and caregivers and between different patients' subgroups with the chi-square or the Fisher's exact test. The scores of QoL questionnaires were compared between satisfied vs. unsatisfied with the Wilcoxon-Mann-Whitney test in patients (ALSAQ-40) and caregivers (SF-36).</p><p><strong>Results: </strong>Patients prioritized finding a cure (95%) and overcoming disease-related limitations (72%) over safe and tolerable drugs (55%), similarly to caregivers. Research on psychological and emotional support was rated more important by caregivers (70%) than patients (49%). QoL was higher among satisfied patients, with better ALSAQ-40 scores in mobility, daily activities, communication and emotional domains. Caregivers needing urgent psychological support were fewer among the satisfied (35%) than the not satisfied (50%), and SF-36 mental health scores were also higher in satisfied caregivers.</p><p><strong>Conclusions: </strong>Incorporating both pALS and caregiver perspectives into clinical practice and research planning may enhance patient- and family-centered care, improve QoL, and better align healthcare research and resources with societal needs.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149192158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrei Ionut Cucu, Amelian Bobu, Andreas Georg Nerlich, Victor Asensi, Antonio Perciaccante, Raffaella Bianucci
{"title":"Evidence of a possible Parry-Romberg Syndrome in an 18th-19th Century Orthodox Votive Plaque.","authors":"Andrei Ionut Cucu, Amelian Bobu, Andreas Georg Nerlich, Victor Asensi, Antonio Perciaccante, Raffaella Bianucci","doi":"10.1159/ene/acsag007","DOIUrl":"https://doi.org/10.1159/ene/acsag007","url":null,"abstract":"<p><p>Votive offerings (ex-voto) are material objects deposited in sacred spaces as expressions of gratitude, petitions for divine assistance, or as part of consecration rituals. In Eastern Orthodox Christianity, particularly in Greece and the Balkan region, these offerings frequently take the form of tamata; these are small metal votive plaques depicting body parts or organs often rendered with a high degree of realism. Here we report previously unreported tama depicting a child/young adolescent with features suggestive of Parry-Romberg syndrome also known as progressive hemifacial atrophy. The 18th-19th century collection of votive offerings from the Orthodox Monastery of Florești (Romania) was examined. Among the seven identified objects, one tama portrays a child/adolescent, approximately 10-15 years of age, in the shape of an angel, with evident pathological features. The artifact was carefully examined by a multidisciplinary team following the International Guidelines for Iconodiagnosis in order to establish a consensus diagnosis and to determine the level of iconodiagnostic evidence. The votive plaque displays right-sided hemifacial atrophy, ipsilateral enophthalmos, and atrophy of the right upper and lower limbs. A deliberate reduction in the size of the right wing compared to the contralateral side is also evident. Correlation of these morphological features with clinical data from the medical literature supports the iconodiagnostic hypothesis of Parry-Romberg syndrome with possible ipsilateral extension to both the upper and lower limbs (level II iconodiagnostic evidence). This tama may represent an early Orthodox votive depiction of Parry-Romberg syndrome. This finding highlights the value of votive collections as interdisciplinary sources for the historical documentation of rare diseases and for the application of iconodiagnosis within a religious context.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149192000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Charlotte Robitaille, Vincent Alvarez, Kaspar Schindler, Stephan Rüegg, Jan Novy, Andrea O Rossetti
{"title":"Changes in clinical management related to continuous versus routine EEG in critically ill adults: analysis of a randomized trial.","authors":"Charlotte Robitaille, Vincent Alvarez, Kaspar Schindler, Stephan Rüegg, Jan Novy, Andrea O Rossetti","doi":"10.1159/ene/acsag010","DOIUrl":"https://doi.org/10.1159/ene/acsag010","url":null,"abstract":"<p><strong>Introduction: </strong>Continuous electroencephalogram (cEEG) is increasingly utilized in the intensive care unit for consciousness-impaired patients with various conditions. It is, however, costly in terms of technical and human resources, and its impact on prognosis compared with routine EEG (rEEG) remains debated. We aimed to characterize the effect of cEEG vs rEEG on clinical management modifications, and to determine if these correlated with a better outcome.</p><p><strong>Methods: </strong>This is an ancillary analysis using data of CERTA (NCT03129438), a randomized trial comparing cEEG versus repeated rEEG in critically ill adults with altered consciousness. Clinical management modifications due to the EEG intervention, pre-defined as performed within 60 hours of EEG start, were prospectively recorded (anti-seizure medication -ASM, sedation, new radiological examination, new surgical intervention, withdrawal of life sustaining therapy). Correlation with the EEG type were analyzed by 2x2 tables; Mantel-Haenszel tests explored the impact on mortality and functional outcome at 6 months.</p><p><strong>Results: </strong>The trial included 364 patients completing follow-up (182 each in the cEEG and rEEG groups). While 58.2% of cEEG and 45.6% of rEEG patients had any management change (p= 0.018), ASM were modified in 22.0% (cEEG) and 12.1% (rEEG) (p = 0.012)); other clinical interventions were comparable across EEG groups. Adjusting for ASM modifications, mortality (p=0.845) and functional outcome (p=0.619) remained similar between the EEG groups.</p><p><strong>Conclusion: </strong>In critically ill adults, cEEG is associated with more clinical management modifications compared to repeated rEEG, particularly regarding ASM, but this does not seem to translate into improved patients' outcomes.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180362","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Clayton Rawson, Yashieta Somani, Michael Karsy, Mehrdad Pahlevani
{"title":"Idiopathic Intracranial Hypertension, Basic Concepts and Emerging Molecular, Genetic, and Immunological Therapies.","authors":"Clayton Rawson, Yashieta Somani, Michael Karsy, Mehrdad Pahlevani","doi":"10.1159/ene/acsag015","DOIUrl":"https://doi.org/10.1159/ene/acsag015","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic intracranial hypertension (IIH) is a chronic disorder characterized by elevated intracranial pressure (ICP) without an identifiable intracranial mass lesion or abnormal cerebrospinal fluid (CSF) composition. Traditionally viewed as a disorder of impaired CSF absorption or venous outflow obstruction, IIH predominantly affects women of reproductive age with obesity and remains a major cause of preventable visual loss. Emerging evidence, however, suggests that IIH is driven by a broader biologic framework involving dysregulated CSF secretion, metabolic and endocrine dysfunction, immune activation, and genetic susceptibility.</p><p><strong>Summary: </strong>This narrative review synthesizes recent advances in the molecular and cellular pathophysiology of IIH and examines emerging mechanism-based therapeutic strategies. Particular emphasis is placed on choroid plexus biology and hormonally regulated CSF hypersecretion mediated through glucocorticoid metabolism via 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) and androgen signaling pathways that modulate Na⁺/K⁺-ATPase activity. Distinct metabolic features of IIH, including visceral adiposity, insulin resistance, and adipose tissue inflammation, are integrated with evidence of systemic and central immune dysregulation. Recent genetic and biobank-based studies further support IIH-specific biological risk extending beyond obesity alone and implicate pathways involved in steroid metabolism, ion transport, vascular biology, and glymphatic regulation. We additionally review translational and clinical evidence supporting targeted therapies, including glucagon-like peptide-1 (GLP-1) receptor agonists and 11β-HSD1 inhibitors, which demonstrate ICP-lowering effects that may occur partially independent of weight loss.</p><p><strong>Key messages: </strong>Accumulating mechanistic evidence supports a paradigm shift in IIH from a diagnosis of exclusion toward a biologically defined neuroendocrine disorder characterized by dysregulated CSF secretion, endocrine and metabolic dysfunction, inflammation, and impaired glymphatic regulation. Advances in molecular biology and translational therapeutics are enabling the development of targeted, disease-modifying therapies and may facilitate future precision medicine approaches for IIH management.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149148154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Subsequent Migraine and Tension Headache After Pelvic Inflammatory Disease: A Longitudinal Follow-up Study of 381,122 Women.","authors":"JhenWu Lai, Mu-N Liu, Tzeng-Ji Chen, Shih-Jen Tsai, Mu-Hong Chen","doi":"10.1159/ene/acsag012","DOIUrl":"https://doi.org/10.1159/ene/acsag012","url":null,"abstract":"<p><strong>Background: </strong>Pelvic inflammatory disease (PID) is a common systemic inflammatory condition affecting women of reproductive age. Although systemic inflammation has been implicated in primary headache disorders, associations between PID and migraine, as well as tension headache, remain unclear.</p><p><strong>Objective: </strong>To investigate whether women with a history of PID have an increased risk of developing migraine or tension headache.</p><p><strong>Methods: </strong>Using Taiwan's National Health Insurance Research Database, 190,561 women with PID were identified from 1998 to 2011 and individually matched 1:1 to PID-free controls on demographics and pain-related comorbidities. Incident migraine and tension headache were ascertained from the index date through 2013.</p><p><strong>Results: </strong>Stratified Cox proportional hazards models demonstrated that PID was associated with a 2.11-fold increased risk of migraine (95% confidence interval: 1.89-2.36) and a 3.60-fold increased risk of tension headache (3.18-4.09) adjusted for age, Charlson Comorbidity Index, and healthcare utilization. The strongest association was observed for migraine with aura (hazard ratio = 3.78).</p><p><strong>Conclusions: </strong>PID is associated with significantly elevated long-term risks of both migraine and tension headache. These findings highlight the potential neurological sequelae of systemic inflammation and central sensitization triggered by PID.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The orexinergic system in Alzheimer's disease: from sleep disruption to accelerated neurodegeneration.","authors":"Claudio Liguori, Matteo Carpi","doi":"10.1159/ene/acsag009","DOIUrl":"https://doi.org/10.1159/ene/acsag009","url":null,"abstract":"<p><strong>Background: </strong>Sleep-wake dysregulation is increasingly recognized as an early and clinically relevant feature of Alzheimer's disease (AD), with growing evidence supporting a bidirectional relationship between disturbed sleep and AD pathophysiology. Sleep fragmentation, reduced slow-wave and REM sleep may contribute to amyloid-β (Aβ) accumulation by increasing Aβ production during wakefulness and diminishing glymphatic clearance during sleep, while emerging amyloid pathology can further disrupt sleep architecture and circadian sleep-wake rhythm, reinforcing a feed-forward cycle.</p><p><strong>Summary: </strong>Within this framework, the orexinergic system, centered in the hypothalamus and crucial for arousal and sleep-wake stability, has been implicated as a mechanistic link between hyperarousal, impaired sleep consolidation, and altered amyloid dynamics. Preclinical studies suggested that orexin-A signaling can exacerbate Aβ and tau pathology and impair synaptic function, whereas biomarker studies reported high cerebrospinal fluid orexin-A levels in mild cognitive impairment and dementia due to AD. These observations raise the hypothesis that targeting orexin signaling and improving sleep may represent actionable strategies within the AD continuum.</p><p><strong>Key messages: </strong>Clinical evidence indicates that behavioral and pharmacological interventions can improve sleep in at-risk populations, yet robust proof of disease-modifying effects on AD biomarkers or clinical progression remains limited. Ongoing trials of dual orexin receptor antagonists may clarify whether sustained modulation of sleep-wake regulation can influence AD-related biology beyond symptomatic sleep benefits.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149106163","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Georges Guinon (1859-1932) and His Overlooked Contribution to the Description of Gilles de la Tourette Syndrome.","authors":"Olivier Walusinski","doi":"10.1159/ene/acsag006","DOIUrl":"10.1159/ene/acsag006","url":null,"abstract":"<p><p>In January 1886, Georges Guinon, who succeeded Georges Gilles de la Tourette as a resident under Jean-Martin Charcot, expanded the initial description of chronic tic disorder by adding obsessive-compulsive symptoms to the clinical picture of the new nosographic entity. This biography aims to honor the memory and contribution of Georges Guinon, a figure forgotten by the history of neurology.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148944579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Predictors of mortality in acute large ischemic stroke patients after endovascular thrombectomy.","authors":"Huiyan Zhang, Nizhen Yu, Jie Yang, Mingchao Wu, Minghua Cao, Changwei Guo, Linyu Li, Wenjie Zi, Dahong Yang, Shunfu Jiang","doi":"10.1159/ene/acsag003","DOIUrl":"https://doi.org/10.1159/ene/acsag003","url":null,"abstract":"<p><strong>Objective: </strong>Endovascular thrombectomy (EVT) has been recommended as first-line therapy for patients with acute large-core ischemic stroke, yet the mortality remains high. We aimed to identify independent predictors of death within 90 days after EVT in this population.</p><p><strong>Methods: </strong>This is a multicenter cohort study across 38 Chinese comprehensive stroke centers (November 2021-February 2023). Acute ischemic stroke patients with anterior circulation large vessel occlusion with ASPECTS ≤5, who underwent EVT were included. Independent mortality predictors of 90-day mortality were identified using multivariate logistic regression.</p><p><strong>Results: </strong>Among 490 eligible patients with large core infarct included in this analysis, 205 (41.8 %) died within 90 days. In multivariable analysis, advancing age (adjusted OR 1.04, 95% CI 1.02-1.06; p < 0.001), admission hyperglycemia (adjusted OR 1.22, 95% CI 1.12-1.33; p < 0.001), higher NIHSS score on admission (adjusted OR 1.10, 95% CI 1.04-1.15; p < 0.001), lower ASPECTS on admission (adjusted OR 0.74, 95% CI 0.64-0.86; p < 0.001), unsuccessful reperfusion (eTICI<2b; adjusted OR 0.78, 95% CI 0.67-0.91), lower mTICI grade (p = 0.002), and poor collateral status (ASITN/SIR grade ≤2; adjusted OR 0.61, 95% CI 0.47-0.78, p <0.001) were independently associated with an increased risk of death within 90 days. SICH (p = 0.012), herniation (p < 0.001) and craniectomy (p = 0.001) were identified as independent in-hospital clinical predictors of mortality.</p><p><strong>Conclusions: </strong>In addition to non-modifiable factors (age and baseline stroke severity), admission hyperglycaemia, reperfusion success and collateral status are key modifiable predictors. Early intervention of cerebral edema and hemorrhagic complications may improve survival in this high-risk population.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neural Substrates of Amusia: Insights from a Narrative Review of Pure Acquired Amusia Cases.","authors":"Masayuki Satoh","doi":"10.1159/ene/acsag004","DOIUrl":"https://doi.org/10.1159/ene/acsag004","url":null,"abstract":"<p><strong>Background: </strong>Approximately 100 cases of amusia have been documented to date. However, the neural mechanisms underlying amusia remain insufficiently understood.</p><p><strong>Aims: </strong>This narrative review aims to synthesize existing literature on amusia, with a particular focus on reported cases of \"pure acquired amusia\" which means the acquired selective impairment of music processing in the brain.</p><p><strong>Methods: </strong>The clinical characteristics of documented cases with pure acquired amusia were comprehensively reviewed and summarized. Furthermore, common lesion sites were identified by analyzing the lesion data from cases, and the crucial regions for music processing was estimated.</p><p><strong>Results: </strong>Eleven cases with pure acquired amusia were identified. Analyses of symptoms and lesion sites revealed following things: first, most of cases with pure acquired amusia was caused by right hemisphere lesion. Second, there are three brain regions which damaged in many cases, that is superior temporal gyrus, insula, isthmus, and temporal pole. Third, until now, pure cases with musical alexia and/or agraphia have not been reported.</p><p><strong>Conclusion: </strong>These findings suggest that there are some brain regions which play a crucial role in music processing: the right superior temporal gyrus, the isthmus, the insula, and the temporal pole.</p>","PeriodicalId":12065,"journal":{"name":"European Neurology","volume":" ","pages":"1"},"PeriodicalIF":2.0,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148864347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}