BMC Neurology[JOURNAL]
Sun
200 papers
Predictors of in-hospital case-fatality in patients with stroke at the National Hospital of the Kyrgyz Republic: a retrospective cohort study.
Choi H, Choi J, Maksatbekov C … +2 more , Tologonov B, Kim K
BMC Neurol · 2026 May · PMID 42216139 · Full text
BACKGROUND: Stroke-related mortality remains disproportionately high in Central Asia, with Kyrgyz Republic among the most affected countries. However, evidence on predictors of in-hospital death in this setting remains l...
BACKGROUND: Stroke-related mortality remains disproportionately high in Central Asia, with Kyrgyz Republic among the most affected countries. However, evidence on predictors of in-hospital death in this setting remains limited, particularly for comparative analyses of ischemic and hemorrhagic stroke. Identifying context-specific determinants of in-hospital death is essential for informing targeted strategies to improve acute stroke care and outcomes in resource-constrained health systems. METHODS: This retrospective cohort study included 1,259 patients with stroke (1,033 ischemic and 226 hemorrhagic) admitted in 2024 to the Neurology Department No. 2 of the National Hospital of the Kyrgyz Republic. Sociodemographic, lifestyle, medical and admission-related characteristics were extracted. Multivariable logistic regression was applied separately to ischemic and hemorrhagic stroke to identify independent predictors of in-hospital case-fatality. RESULTS: In-hospital case-fatality was 9.6% for ischemic and 21.7% for hemorrhagic stroke. In ischemic stroke, high Modified Early Warning Score (MEWS) (aOR = 75.1, 95% CI 29.18-193.21) and medium MEWS (aOR = 12.5, 95% CI 6.54-23.95) were strongest predictors of in-hospital case-fatality. Higher risk was noted among Bishkek residents (aOR = 4.2, 95% CI 1.72-9.94), those with prior myocardial infarction (aOR = 2.1, 95% CI 1.05-4.18) and ambulance admissions (aOR = 2.9, 95% CI 1.16-6.97). Overweight (aOR = 0.5, 95% CI 0.25-0.98) and hyperlipidemia (aOR = 0.5, 95% CI 0.26-0.84) were inversely associated with in-hospital case-fatality. In hemorrhagic stroke, high (aOR = 51.3, 95% CI 12.73-206.73) and medium (aOR = 7.5, 95% CI 2.23-25.40) MEWS, female sex (aOR = 2.8, 95% CI 1.09-7.36) and prior myocardial infarction (aOR = 5.94, 95% CI 1.45-24.29) increased case-fatality. CONCLUSION: This study provides early real-world, hospital-based evidence on predictors of in-hospital case-fatality among stroke patients in the Kyrgyz Republic. Early physiological risk stratification and targeted interventions for high-risk groups could improve outcomes and guide stroke care in low- and middle-income country settings.
From profound hypothermia to malignant hyperpyrexia: a case report of biphasic thermoregulatory crisis following acute high cervical spinal cord injury.
Ekanayaka N, De Silva S
BMC Neurol · 2026 May · PMID 42216138 · Full text
BACKGROUND: Thermoregulatory dysfunction is a well-recognized yet underreported consequence of high cervical spinal cord injury (SCI). Patients may present with extreme temperature disturbances, ranging from profound hyp...
BACKGROUND: Thermoregulatory dysfunction is a well-recognized yet underreported consequence of high cervical spinal cord injury (SCI). Patients may present with extreme temperature disturbances, ranging from profound hypothermia to malignant hyperthermia, reflecting severe autonomic dysregulation. Early recognition is crucial, as these abnormalities can rapidly become life-threatening. CASE PRESENTATION: A 55-year-old previously healthy man was found unconscious outdoors in the rain. Initial assessment revealed profound hypothermia (31 °C), bradycardia (48 bpm), hypotension (70/50 mmHg), and absent shivering. He was resuscitated with warmed intravenous fluids, external rewarming, hydration and vasopressor support. Neurological evaluation after stabilization demonstrated complete quadriparesis with sensory loss below C5 and bowel and bladder involvement (ASIA Grade A). Imaging revealed C5-C6 retrolisthesis with spinal cord compression. During hospital monitoring, he developed rapid-onset hyperpyrexia (42.2 °C) without sweating, refractory to conventional cooling, ultimately resulting in cardio-respiratory arrest. Extensive evaluation excluded infectious, metabolic, and drug-related causes, suggesting neurogenic/ quad fever secondary to high cervical SCI. CONCLUSIONS: This case illustrates the biphasic and rapidly progressive nature of autonomic thermoregulatory dysfunction in acute high cervical SCI. Neurogenic fever, though uncommon, may occur within hours and is associated with high morbidity and mortality. Continuous temperature monitoring, early hemodynamic stabilization, and high clinical suspicion are critical, particularly in resource-limited settings.
Combined ipsilateral peak systolic velocity and ASPECTS are associated with early neurological deterioration after endovascular thrombectomy: a prospective cohort study.
Zhou S, Chen W, Yin M … +9 more , Hu J, He Z, Lai X, Guo H, Kuang J, Liu M, Yang H, Cheng Z, Tu J
BMC Neurol · 2026 May · PMID 42210127 · Full text
PURPOSE: This study aimed to assess whether a predictive model combining the Alberta Stroke Program Early CT Score (ASPECTS) and post-endovascular thrombectomy (EVT) transcranial Doppler (TCD) parameters could improve th...
PURPOSE: This study aimed to assess whether a predictive model combining the Alberta Stroke Program Early CT Score (ASPECTS) and post-endovascular thrombectomy (EVT) transcranial Doppler (TCD) parameters could improve the prediction of early neurological deterioration (END) in patients with large artery occlusion (LAO) stroke after successful recanalization. METHODS: In this prospective study, we enrolled 87 consecutive patients with anterior circulation LAO who achieved successful recanalization (modified Thrombolysis in Cerebral Infarction [mTICI] grade ≥2b), underwent post-EVT TCD monitoring within 48 h, and had baseline ASPECTS assessment. Bilateral middle cerebral artery (MCA) flow velocities were measured, and key hemodynamic indices were subsequently calculated. The prespecified primary outcome was END within 72 h; secondary outcomes included functional status at 90 days. RESULTS: Among the 87 successfully recanalized patients, END occurred in 21.8% (n = 19) within 72 h after EVT. Compared with the non-END group, patients who developed END exhibited significantly higher ipsilateral MCA flow velocities, including peak systolic, end-diastolic, and mean flow velocities, as well as a significantly higher ipsilateral-to-contralateral mean flow velocity ratio. Multivariable analysis identified lower baseline ASPECTS (odds ratio [OR], 0.50, 95% confidence interval [CI], 0.31-0.80) and higher ipsilateral peak systolic velocity (iPSV) (OR, 2.15, 95% CI, 1.08-4.28) as independent predictors of END. Restricted cubic spline analysis suggested an approximately linear association between iPSV and END risk. A combined model incorporating iPSV and ASPECTS demonstrated superior discriminative performance in predicting END (area under the curve [AUC], 0.801; 95% CI, 0.700-0.902), outperforming either parameter alone. CONCLUSION: A model integrating post-EVT hemodynamic (iPSV) and baseline imaging (ASPECTS) data significantly improves the prediction of END after successful EVT. This multimodal approach, which showed superior discriminative ability (AUC = 0.801), may facilitate early risk stratification and support tailored clinical management. TRIAL REGISTRATION: This prospective observational study was registered with the National Clinical Research Center of China on June 19, 2024 (Registration No. MR-36-24-042352). The study was registered during the enrollment period.
Comparative anatomical and clinical evaluation of three surgical spaces for contralateral internal carotid artery C7-segment aneurysm clipping: the role of the subchiasmatic-preinfundibular space.
Wang X, Wang S, Gao S … +9 more , Wang X, Wu Y, Du H, Zhang C, Zhao L, Liu X, Liu X, Zhang G, Wu J
BMC Neurol · 2026 May · PMID 42204509 · Full text
OBJECTIVE: This study aims to anatomically define and clinically evaluate the subchiasmatic-preinfundibular space (SCPIS) as an alternative surgical space for clipping contralateral ICA C7-segment aneurysms, by comparing...
OBJECTIVE: This study aims to anatomically define and clinically evaluate the subchiasmatic-preinfundibular space (SCPIS) as an alternative surgical space for clipping contralateral ICA C7-segment aneurysms, by comparing its outcomes with those of the standard prechiasmatic space (PCS) and contralateral opticocarotid space (OCS). METHODS: Basic Research: Five cadaveric heads were dissected via the pterional approach. Contralateral ICA exposure was measured through three spaces- PCS, contralateral OCS), and SCPIS-before and after anterior clinoid process (ACP) drilling. Clinical Research: A retrospective analysis of 53 patients with multiple intracranial aneurysms (MIAs) treated via unilateral craniotomy compared outcomes across three groups: SCPIS (Group 1), PCS (Group 2), and contralateral OCS (Group 3). Postoperative aneurysm occlusion rates, complications, discharge mRS scores, and 6-month neurological deficits were assessed. RESULTS: The lengths of the contralateral ICA exposure increased significantly post-ACP drilling: PCS (5.62 ± 0.91 mm vs. 7.11 ± 0.84 mm, p < 0.001), contralateral OCS (4.44 ± 2.33 mm vs. 6.03 ± 2.30 mm, p = 0.001), and SCPIS (4.98 ± 1.91 mm vs. 6.67 ± 2.01 mm, p < 0.001). Significant increases were also observed in the lengths of the inferior border of the optic chiasm and/or optic nerves, the deep superior border of the diaphragma sellae, and the area of this space after ACP drilling (p < 0.05). SCPIS clipping showed no significant differences in hospitalization duration, complications (pneumonia, electrolyte disturbances, optic nerve injury, cerebral infarction), or discharge mRS scores (p > 0.05) compared to other approaches. The rate of complete angiographic occlusion of the contralateral aneurysms was comparable among the three groups (PCS: 88.6%, OCS: 90.9%, SCPIS: 85.7%; p = 0.92). Six-month neurological outcomes were satisfactory. CONCLUSION: SCPIS is a viable alternative for contralateral ICA C7-segment aneurysm clipping. ACP drilling improves surgical access, and the approach is safe and effective.
Telitacicept as a potential therapeutic alternative in autoantibody-negative probable autoimmune encephalitis: a case report.
Xu C, Zhang Y, Chen J
BMC Neurol · 2026 May · PMID 42204505 · Full text
BACKGROUND: The diagnosis and treatment of autoantibody-negative autoimmune encephalitis (AbNAE) remain challenging due to the absence of definitive serological markers and established treatment strategies. CASE PRESENTA...
BACKGROUND: The diagnosis and treatment of autoantibody-negative autoimmune encephalitis (AbNAE) remain challenging due to the absence of definitive serological markers and established treatment strategies. CASE PRESENTATION: We present a case of a 54-year-old male with acute memory impairment. Cerebrospinal fluid (CSF) analysis and cranial magnetic resonance imaging (MRI) revealed features consistent with neuroinflammation. However, the autoimmune encephalitis (AE) antibody panel in both CSF and serum returned negative results. Initially misdiagnosed as viral encephalitis (VE), the patient received a three-week antiviral regimen. Subsequently, the patient developed seizures and neuropsychiatric symptoms, with concurrent deterioration on serial electroencephalography (EEG) and MRI. Repeat AE antibody testing remained negative. The diagnosis of autoantibody-negative but probable autoimmune encephalitis (ANPRA), a subtype of AbNAE, was established. Despite prompt initiation of first-line immunotherapies, including high-dose corticosteroids and intravenous immunoglobulin (IVIg), the patient's neurological status continued to decline. Therefore, subcutaneous telitacicept (160 mg weekly) was administered for four consecutive weeks. This intervention led to progressive neurological improvement without clinically significant adverse events. CONCLUSION: This case highlights the aggressive nature and suboptimal response to conventional immunotherapy in ANPRA, and suggests that telitacicept may represent a viable therapeutic alternative for this challenging condition.
Health-related outcomes among adults with cerebral palsy: a scoping review.
Kim YW, Jeong HN, Choi GW … +1 more , Chang SJ
BMC Neurol · 2026 May · PMID 42204471 · Full text
BACKGROUND: Advances in medical technology have extended the life expectancy of individuals with cerebral palsy (CP), resulting in a growing adult population facing long-term physical, psychological, and social health-re...
BACKGROUND: Advances in medical technology have extended the life expectancy of individuals with cerebral palsy (CP), resulting in a growing adult population facing long-term physical, psychological, and social health-related challenges. This underscores the need for a comprehensive understanding of their health-related outcomes. OBJECTIVE: This study aimed to identify and map the health-related outcomes assessed and the corresponding measurement instruments used in studies involving adults with CP. METHODS: Nine electronic databases were searched. The final search was completed on May 26, 2025. The studies were screened according to predefined criteria. To map the identified outcomes, data were organized into health-outcome levels: domain, sub-domain, outcome, and measurement. Frequency analysis was then performed to summarize the distribution of outcomes and measurement instruments across the included studies. RESULTS: A total of 16,820 records were identified (16,811 through database searches and 9 through manual searches), of which 159 studies met the inclusion criteria. The analysis identified four domains, 18 sub-domains, and 67 outcomes, represented by 348 distinct measurement instruments. Most studies focused on younger adults, and research on older adults was underrepresented. Most of the outcomes were concentrated within the physical domain. Mental, social, and overall domains were less frequently addressed, and CP-specific measurement instruments were rarely applied. CONCLUSIONS: This study provides an overview of the health-related outcomes reported in adults with CP. It underscores the need for the broader inclusion of older adults, continued expansion into non-physical domains, where research interests have been increasing but remain limited, and the consistent use of common measurement instruments, together with the development and wider application of CP-specific measurement instruments.
Posterior-to-anterior periventricular white matter hyperintensity area ratio: a supportive MRI feature for differentiating cerebral amyloid angiopathy in lobar intracerebral hemorrhage.
Zhang Y, Wang S, Wu X … +3 more , Zhong Y, Sun S, Ju Y
BMC Neurol · 2026 May · PMID 42192316 · Full text
BACKGROUND: Diagnosing cerebral amyloid angiopathy (CAA) in primary lobar intracerebral hemorrhage (ICH) remains challenging using routine MRI markers. We investigated a quantitative posterior-dominant pattern of periven...
BACKGROUND: Diagnosing cerebral amyloid angiopathy (CAA) in primary lobar intracerebral hemorrhage (ICH) remains challenging using routine MRI markers. We investigated a quantitative posterior-dominant pattern of periventricular white matter hyperintensity (PWMH) as a potential supportive imaging feature for CAA within the Boston v2.0 framework. METHODS: We retrospectively analyzed 229 patients aged ≥ 50 years with primary lobar ICH, classified as CAA (n = 123) or CAA-negative (n = 106) according to the Boston criteria v2.0. On FLAIR, the largest anterior and posterior periventricular WMH areas were measured bilaterally, and the posterior-to-anterior PWMH area ratio (PA-AR) was derived. Multivariable logistic regression models were developed, and diagnostic performance was evaluated using ROC analysis. RESULTS: Patients with CAA showed a greater posterior PWMH burden and higher PA-AR than CAA-negative controls (median 1.69 vs. 0.79; p < 0.001). Adding PA-AR to the covariate-adjusted model improved discrimination for CAA (AUC 0.804; bootstrap mean AUC 0.813, 95% CI 0.759-0.867). In the predefined diagnostic "gray zone" subgroup, PA-AR remained informative. The AUC was 0.887 in univariable analysis and 0.888 after age adjustment. PA-AR was also higher in patients with posterior lobar CMBs than in those with no CMBs or mixed-location hemorrhage. CONCLUSIONS: A posterior-dominant periventricular WMH pattern, quantified by PA-AR, is associated with CAA in lobar ICH. This pattern is also related to posterior-predominant lobar CMB topography. PA-AR may serve as a supportive MRI feature that complements Boston criteria v2.0 markers for identifying CAA within a real-world mixed lobar ICH population, particularly in diagnostically challenging patients.
Seasonal variation in inflammatory biomarkers and blood pressure among patients with ischemic stroke: the influence of clinical confounding factors.
Zhang X, Jiang C, Yang Q … +4 more , Ha X, Xie Z, Zuang C, Wu H
BMC Neurol · 2026 May · PMID 42186035 · Full text
BACKGROUND: Seasonal variation has been implicated in the occurrence of ischemic stroke; however, evidence regarding seasonal differences in inflammatory biomarkers remains inconsistent. This study aimed to investigate s...
BACKGROUND: Seasonal variation has been implicated in the occurrence of ischemic stroke; however, evidence regarding seasonal differences in inflammatory biomarkers remains inconsistent. This study aimed to investigate seasonal patterns of inflammatory markers and blood pressure (BP) in patients with acute ischemic stroke and to evaluate whether these differences are independent of clinical confounders. METHODS: This single-center retrospective study included 168 patients with acute ischemic stroke admitted during summer and winter. Clinical characteristics and laboratory indicators-including sCD40L, lipoprotein-associated phospholipase A2 (Lp-PLA2), high-sensitivity C-reactive protein (hs-CRP), blood pressure, and blood glucose-were collected. Stroke severity and etiology were assessed using the NIHSS score and TOAST classification. Between-group comparisons were performed using independent samples t-tests or Mann-Whitney U tests, as appropriate. Analysis of covariance (ANCOVA) was conducted to adjust for potential confounders, including age, sex, hypertension, diabetes mellitus, and NIHSS score, and to assess interaction effects. RESULTS: In unadjusted analyses, patients admitted in winter had significantly higher levels of systolic blood pressure (SBP), sCD40L, Lp-PLA2, and hs-CRP compared with those admitted in summer (all P < 0.05). No significant difference in NIHSS scores was observed between groups (P = 0.636), while TOAST classification differed significantly (P = 0.005). However, after adjustment using ANCOVA, the previously observed associations between season and inflammatory biomarkers or SBP were attenuated and no longer statistically significant (all P > 0.05), suggesting potential confounding by baseline clinical characteristics. Notably, a significant interaction between season and hypertension was observed for SBP (P < 0.05), suggesting that seasonal effects on blood pressure may differ according to hypertension status. CONCLUSION: Although unadjusted analyses suggested higher inflammatory marker levels and SBP in winter, these differences were not independent after controlling for confounding factors. These findings suggest that the observed seasonal differences may be largely explained by patient-level characteristics rather than being solely attributable to seasonal classification. However, the observed interaction between season and hypertension highlights potential heterogeneity in blood pressure responses, which may have implications for individualized risk management.
Diagnostic accuracy of drawing tests and their combinations for post-stroke cognitive impairment in patients with lower education.
Wang Y, Wang Y, Xiang Y … +3 more , Fang F, Yan N, Hu Y
BMC Neurol · 2026 May · PMID 42186008 · Full text
BACKGROUND: Post-stroke cognitive impairment (PSCI) is highly prevalent among ischemic stroke patients in China, a population who received less education compared to Western counterparts. Commonly used cognitive assessme...
BACKGROUND: Post-stroke cognitive impairment (PSCI) is highly prevalent among ischemic stroke patients in China, a population who received less education compared to Western counterparts. Commonly used cognitive assessments often affected by educational levels. Drawing tests may mitigate educational bias, but the diagnostic accuracy of combined drawing tests remains understudied and unclear. METHODS: The diagnostic accuracy of the Cube Copying Test (CCT), Clock Drawing Test (CDT), and Pentagon Copying Test (PCT), and their various combinations was evaluated against the Montreal Cognitive Assessment (MoCA) as the reference standard. Ischemic stroke patients at a hospital in Chongqing, China, were enrolled between January 2022 and June 2025. Receiver operating characteristic (ROC) curves and the area under the curve (AUC) were used to assess diagnostic accuracy. RESULTS: Among 413 participants, 260 were in the acute phase (mean age was 64.3 years, and 47.7% had primary education) and 153 in the convalescent phase (mean age was 64.7 years, and 33.3% had primary education). PSCI was identified in 61.2% and 60.8% of acute and convalescent patients, respectively. Among individual tests, the CDT showed the highest AUC in both phases (acute: 0.83, 95% CI: 0.78, 0.88; convalescent: 0.78, 95% CI: 0.72, 0.86). For test combinations, the full battery (CCT + CDT+PCT) achieved the highest AUC in the acute phase (0.85, 95% CI: 0.80, 0.90), with a sensitivity of 80.5% (95% CI: 73.3%, 86.2%) and a specificity of 83.2% (95% CI: 74.1%, 90.0%); while the CCT + CDT combination performed the best in the convalescent phase (0.82, 95% CI: 0.75, 0.88), with a sensitivity of 78.5% (95% CI: 68.5%, 86.1%) and a specificity of 71.7% (95% CI: 58.4%, 82.2%). These two combinations exhibited AUC > 0.8 across sex and age subgroups. In acute patients, the full battery showed better AUC in those with primary (0.88, 95% CI: 0.80, 0.97) and secondary education (0.84, 95% CI: 0.76, 0.92) compared to higher education (0.74, 95% CI: 0.57, 0.90). This educational gradient was less evident in convalescent patients (primary school: 0.72, 95% CI: 0.52, 0.93; secondary school: 0.83, 95% CI: 0.75, 0.92; ≥university: 0.65, 95% CI: 0.42, 0.88). CONCLUSION: Combined drawing tests demonstrate good diagnostic accuracy for PSCI, with their de-linguistic design reducing educational bias, particularly in acute patients. The CCT + CDT+PCT combination is recommended for acute-phase PSCI screening, while CCT + CDT may suffice during convalescence. These findings suggest that combined drawing tests may offer practical tools for rapid PSCI screening in stroke patients with lower education.
Morphological brain changes in high-altitude careers and spaceflights: a systematic review and meta-analysis.
Mohamed MI, Dean YE, Rath S … +4 more , Rehman AU, Aleem MA, Hamza A, Abdelall NA
BMC Neurol · 2026 May · PMID 42186000 · Full text
BACKGROUND AND AIM: Occupational exposure to high-altitude environments, such as those experienced by pilots and astronauts, has been associated with unique challenges, including neurocognitive implications. White matter...
BACKGROUND AND AIM: Occupational exposure to high-altitude environments, such as those experienced by pilots and astronauts, has been associated with unique challenges, including neurocognitive implications. White matter hyperintensities (WMH) detected through neuroimaging have emerged as potential markers of brain pathology in individuals with high-altitude careers. This meta-analysis aimed to explore the association between occupational high-altitude exposure and WMH burden. METHODS: A systematic literature search was conducted following PRISMA guidelines in databases such as PubMed, Scopus, Web of Science, and Cochrane. Studies reporting WMH in pilots were included without language or date restrictions. Data extraction and quality assessment were performed independently by two reviewers. Pooled analyses were conducted using R software, and the risk of bias was assessed using the Newcastle-Ottawa scale. RESULTS: A total of 1,036 articles were screened, with five studies meeting the inclusion criteria. These studies included 441 individuals, comprising 264 pilots and 177 controls. Pilots exhibited a significantly higher mean number (MD: 3.57, 95% CI: 1.71 - 5.43, p-value = 0.0002) and volume (MD: 0.09, 95% CI: 0.04 - 0.14, p-value = 0.0001) of WMH compared to non-flying personnel. No heterogeneity was observed in the analyses. CONCLUSION: Occupational exposure to high altitudes is associated with a higher prevalence and volume of WMH, highlighting the possible need for regular screening and monitoring of individuals in high-altitude careers to mitigate the risk of cerebral diseases, and their implications. This meta-analysis underscores the importance of addressing the neurocognitive consequences of high-altitude professions in aviation and space exploration industries.
Impact of genetic polymorphisms in the interleukin-18 and interleukin-21 genes on the risk and clinical outcomes of multiple sclerosis.
Bakr NM, Hashim NA, El-Eshmawy MA … +1 more , El-Baz HA
BMC Neurol · 2026 May · PMID 42185985 · Full text
BACKGROUND: This study was designed to investigate the association of IL-18-137G/C (rs187238) and IL-21-1472G/T (rs2055979) single nucleotide polymorphisms (SNPs) with the risk of MS, the disease progression, and related...
BACKGROUND: This study was designed to investigate the association of IL-18-137G/C (rs187238) and IL-21-1472G/T (rs2055979) single nucleotide polymorphisms (SNPs) with the risk of MS, the disease progression, and related clinical features. METHODS: In this study, 271 Egyptian individuals participated, including 131 MS cases (relapsing-remitting (RR-MS) cases [N = 99], and progressive MS cases [N = 32]) and 140 unrelated healthy controls. The association between IL-18-137G/C (rs187238) and IL-21-1472G/T (rs2055979) single nucleotide polymorphisms (SNPs) and the risk of MS and the disease progression, as well as the clinical features including the disability progression and the severity, were analyzed by using polymerase chain reaction (PCR) methods. RESULTS: The GC genotype and C allele of the IL-18 SNP were linked to susceptibility to MS, and the genotypic forms (GC and CC genotypes) and allelic form (C allele) were linked to an elevated risk of the development of progressive MS. Regression investigation further strengthened the notion that this SNP is a predictor of the disability of the disease according to the Disability Status Scale (EDSS) values. For the IL-21-1472G/T SNP, the TT genotype and T allele in the Egyptian cohort were related to the reduced risk of progressive MS when comparing the two diseased forms; however, they were not associated with the disability progression and severity of the disease. CONCLUSION: The IL-18-137G/C SNP might be a possible risk determinant of the susceptibility as well as the progression of MS. In contrast, the IL-21-1472G/T SNP could protect against progressive MS.
Evaluating the performance of ChatGPT in responding to myotonic dystrophy type 1 patient inquiries: a specialist physician-based study.
Çelik G, Hekim HH, Filiz MB … +1 more , Toraman NF
BMC Neurol · 2026 May · PMID 42185983 · Full text
BACKGROUND: Advancements in artificial intelligence have led to the widespread use of large language models such as ChatGPT in healthcare communication. Myotonic dystrophy type 1, a chronic and multisystemic neuromuscula...
BACKGROUND: Advancements in artificial intelligence have led to the widespread use of large language models such as ChatGPT in healthcare communication. Myotonic dystrophy type 1, a chronic and multisystemic neuromuscular disorder, poses significant challenges for patients in understanding disease progression, symptom management, and future planning. Given the scarcity of specialized resources for rare diseases, AI-based tools may offer valuable support by delivering accessible and relevant information. This study aimed to evaluate ChatGPT-4o's performance in addressing patient-centered questions about myotonic dystrophy type 1. METHODS: This descriptive methodological study utilized ten frequently asked patient-style questions concerning myotonic dystrophy type 1, posed to ChatGPT-4o in Turkish using a standardized zero-shot prompting approach. A total of 53 specialist physicians in Physical Medicine and Rehabilitation and Neurology assessed the responses using a structured rubric across five domains: accuracy, currency, comprehensiveness, usefulness, and understandability. Each response was scored from 1 to 5. RESULTS: ChatGPT-4o achieved high specialist physicians' satisfaction across all domains, with over 80% of responses rated ≥ 4. Accuracy received the highest score (85.7%), followed by usefulness and comprehensiveness (above 82%). Currency and understandability were rated slightly lower (81.9% and 81.5%, respectively). CONCLUSION: ChatGPT-4o generated responses that were generally accurate, relevant, and understandable, demonstrating promise as an accessible resource for rare disease contexts such as myotonic dystrophy type 1. It also showed strong potential as a supportive informational tool for specialist physicians. Ongoing model refinement and integration with up-to-date clinical data are essential to optimize its performance and ensure its safe, equitable use in healthcare communication.
The role of health literacy in medication adherence among people living with multiple sclerosis: a cross-sectional study in Isfahan, Iran.
Kanani MM, Abolhassani S, Nazari F
BMC Neurol · 2026 May · PMID 42185979 · Full text
BACKGROUND: Multiple Sclerosis (MS) is a chronic demyelinating disease of the central nervous system with a growing prevalence. While various disease-modifying treatments (DMTs) are available, adherence to DMTs has remai...
BACKGROUND: Multiple Sclerosis (MS) is a chronic demyelinating disease of the central nervous system with a growing prevalence. While various disease-modifying treatments (DMTs) are available, adherence to DMTs has remained a challenge in the management of MS. Among the previously known factors associated with adherence to DMTs, health literacy as an essential factor in health-related behaviors and a contributor to treatment adherence in other chronic diseases, has not been studied. This study aims to determine the role of health literacy in medication adherence among people living with MS. METHODS: The current study is a multicenter cross-sectional study conducted in two centers affiliated with the Isfahan University of Medical Sciences. A total of 317 participants were recruited using random systematic sampling. The participants were assessed for eligibility via the phone call. Data was then collected via a form for demographic and disease-related information, a revised version of the Multiple Sclerosis Treatment Adherence Questionnaire (rMS-TAQ), and the Health Literacy Questionnaire for Multiple Sclerosis patients (MSHLQ). Statistical analysis included descriptive statistics, Spearman's correlation, and stepwise linear regression. Data analysis was carried out by IBM SPSS Statistics version 24. RESULTS: 76.3% of participants were adherent (doses missed = 0). The health literacy score in MSHLQ was moderately associated with medication adherence score in rMS-TAQ (Spearman's rho = 0.553, p < 0.001). A final regression model was found significant (F(3, 316) = 43.877, p < 0.001), which predicted adherence score in rMS-TAQ based on total health literacy score, the score in the 'knowledge of caring for the disease' subscale of MSHLQ, and income. CONCLUSION: Health literacy is associated with better adherence to DMTs. Disease knowledge may play a mediating role in the relationship between health literacy and medication adherence in people living with MS.
Retinal hemodynamic and caliber biomarkers for the assessment of cerebral small vessel disease burden: insights from fundus fluorescein angiography.
Xu L, Xiong X, Zhou Y … +4 more , Zhao X, Wu Y, Ye C, Meng Z
BMC Neurol · 2026 May · PMID 42185800 · Full text
BACKGROUND: Retinal microvasculature serves as a promising window to assess cerebral small vessel disease (CSVD). However, the association between comprehensive fundus fluorescein angiography (FFA) biomarkers and the tot...
BACKGROUND: Retinal microvasculature serves as a promising window to assess cerebral small vessel disease (CSVD). However, the association between comprehensive fundus fluorescein angiography (FFA) biomarkers and the total CSVD burden score remains to be fully elucidated. AIMES: To investigate the relationship between the total CSVD burden score and FFA-derived retinal biomarkers, and to identify independent retinal indicators for evaluating CSVD severity. METHODS: This retrospective study enrolled 120 patients. The CSVD burden score was evaluated based on lacunes of presumed vascular origin, white matter hyperintensities, enlarged perivascular spaces, and cerebral microbleeds. FFA parameters included dynamic hemodynamic indicators (arm-retinal circulation time [ARCT], retinal circulation time [V1-ARCT], retinal venous transit time [V2-V1]) and static vascular caliber indicators (central retinal artery equivalent [CRAE], central retinal vein equivalent [CRVE], arteriolar-to-venular ratio [AVR]), as well as arteriovenous nicking and vascular leakage. Correlation and multivariable regression analyses were performed. RESULTS: Across CSVD burden groups, significant differences were detected in age, hypertension, hyperlipidemia, key neuroimaging markers, ARCT, V1-ARCT, CRAE, CRVE, and AVR, whereas gender, diabetes, arteriovenous nicking, vascular leakage, and V2-V1 showed no significant differences. Spearman analysis showed that the CSVD burden score was negatively correlated with CRAE and AVR, and positively correlated with CRVE and V1-ARCT. Multivariable analysis identified CRAE, CRVE, and V1-ARCT as independent factors associated with the CSVD burden score: lower CRAE, higher CRVE, and prolonged V1-ARCT were related to higher CSVD burden. CONCLUSION: Static vascular caliber (CRAE, CRVE) and dynamic retinal circulation time (V1-ARCT) are independently associated with CSVD burden. The combined use of these FFA biomarkers provides a promising tool for the early assessment and noninvasive monitoring of CSVD, supporting further clinical translation.
Association between creatinine-to-cystatin C ratio and ALSFRS-R across clinical phenotypes.
Fujiwara Y, Hashiguchi A, Yamashiro S … +7 more , Seno H, Ohya Y, Yasutomi D, Fujisaki N, Kido M, Suwazono S, Tokashiki T
BMC Neurol · 2026 May · PMID 42185781 · Full text
BACKGROUND: Reliable and accessible biomarkers for amyotrophic lateral sclerosis (ALS) are scarce. Creatinine (Cre) reflects muscle mass, whereas cystatin C (CysC) may reflect neurodegeneration without being directly inf...
BACKGROUND: Reliable and accessible biomarkers for amyotrophic lateral sclerosis (ALS) are scarce. Creatinine (Cre) reflects muscle mass, whereas cystatin C (CysC) may reflect neurodegeneration without being directly influenced by muscle mass; however, both have limitations. We aimed to investigate whether the creatinine-to-cystatin C ratio (Cre/CysC) was cross-sectionally associated with functional status in patients with ALS. METHODS: We retrospectively analyzed 30 patients diagnosed with ALS at the National Organization Hospital Okinawa Hospital between 2021 and 2024. Baseline ALS Functional Rating Scale-Revised (ALSFRS-R) scores and serum Cre and CysC levels were recorded. Associations with the ALSFRS-R were assessed using Spearman's correlation, with subgroup analyses by sex, site of onset, age at diagnosis, body mass index (BMI), and diagnostic delay. Multivariable analyses were performed to examine the independent association between Cre/CysC and ALSFRS-R while accounting for relevant clinical covariates. RESULTS: Cre/CysC showed a stronger cross-sectional correlation with ALSFRS-R (r=0.648, p = 0.0001) than Cre alone (r =0.427) or CysC (r =-0.119). Exploratory subgroup analyses showed generally positive associations in several subgroups, although no statistically significant association was observed in the small bulbar-onset subgroup. In multivariable analysis adjusted for age at onset and diagnostic delay, Cre/CysC remained independently associated with ALSFRS-R (β = 20.1, 95% CI 6.41-33.9, p = 0.006). Given the small sample size and cross-sectional design, these findings should be interpreted as exploratory. CONCLUSIONS: Cre/CysC showed a stronger cross-sectional association with functional status than either marker alone. Because it is derived from routine laboratory tests, Cre/CysC may represent a simple exploratory measure associated with functional status in ALS. However, the present findings do not establish prognostic utility or fully account for disease stage and biological heterogeneity. Prospective longitudinal studies incorporating disease progression measures and broader clinical and genetic characterization are warranted.
Temporal changes in hemoglobin-to-red blood cell distribution width ratio and its association with in-hospital all-cause mortality in patients with intracerebral hemorrhage: a retrospective analysis across MIMIC-IV and eICU databases.
Huang Y, Liang H, Tang S
BMC Neurol · 2026 May · PMID 42178542 · Full text
BACKGROUND: Intracerebral hemorrhage (ICH) is associated with poor prognosis, characterized by high acute mortality and long-term disability. Real-time risk stratification using dynamic biomarkers is urgently needed to i...
BACKGROUND: Intracerebral hemorrhage (ICH) is associated with poor prognosis, characterized by high acute mortality and long-term disability. Real-time risk stratification using dynamic biomarkers is urgently needed to improve patient outcomes. This study aimed to explore the association between temporal changes in the hemoglobin-to-red blood cell distribution width ratio (HRR) and in-hospital all-cause mortality in patients with ICH. METHODS: We retrospectively analyzed 2,447 ICH patients from MIMIC-IV and externally validated findings in 3,142 patients from eICU. Cox regression analyzed the association between HRR (baseline and daily dynamic values) and in-hospital mortality. Restricted cubic spline (RCS) models assessed the non-linear relationship between baseline HRR and in-hospital mortality. An optimal baseline HRR cutoff for risk stratification was identified using the maximally selected log-rank statistic and subsequently externally validated. Pearson correlation analysis was applied to evaluate associations between daily HRR and in-hospital mortality, and receiver operating characteristic (ROC) curves were used to examine the time-specific predictive performance of serial HRR values. RESULTS: HRR exhibited a consistent inverse association with in-hospital all-cause mortality in both cohorts. In fully adjusted Cox models, continuous baseline HRR was associated with lower mortality risk (HR approximately 0.51 in both cohorts), and the highest HRR quartile was associated with a reduced mortality risk compared with the lowest quartile. Both survivors and non-survivors showed progressive HRR declines during 14-day hospitalization. Non-survivors had more pronounced declines (daily reduction approximately 0.017 vs. 0.014 in MIMIC-IV; P for trend difference < 0.001) and consistently lower HRR levels at all time points. Daily HRR was inversely correlated with mortality throughout hospitalization (adjusted HRs < 1.0, P < 0.05), with the strength of this inverse association increasing notably from Day 1 (r = -0.141) to Day 4 (r = -0.225). HRR demonstrated predictive performance comparable to Hb and RDW across time points. In the MIMIC-IV test set, all indicators presented a temporal upward trend within the initial few days, with AUCs reaching 0.631 (HRR), 0.594 (Hb) and 0.630 (RDW) at Day 1, and 0.663, 0.632 and 0.645 at Day 3, respectively. Calibration curves revealed poor predictive performance with overestimation of mortality risk. A baseline HRR cutoff of ≤ 0.74 identified high-risk patients, who had significantly higher mortality (25.14% vs. 12.93% in MIMIC-IV; 17.64% vs. 12.72% in eICU, all P < 0.001). CONCLUSIONS: This study preliminarily suggests that temporal changes in HRR are associated with in-hospital all-cause mortality in ICH patients. HRR may serve as a complementary dynamic prognostic marker with predictive performance comparable to Hb and RDW, though its utility is limited by poor calibration. Further prospective validation in dedicated ICH cohorts with severity and imaging data is warranted, and future studies should consider underlying diseases and inflammatory status.
Assessing the role of Subacute Encephalopathy with Seizures in Alcoholics (SESA) syndrome in the development of status epilepticus: a case report and systematic review with individual patients' analysis.
Dasara M, Dono F, Evangelista G … +5 more , Quintieri P, Cipollone S, Liviello D, Ferrante R, Sensi SL
BMC Neurol · 2026 May · PMID 42178529 · Full text
BACKGROUND: Subacute Encephalopathy with Seizures in Alcoholics (SESA) is a rare neurological syndrome characterized by altered mental status, periodic EEG discharges, and neuroimaging abnormalities, typically occurring...
BACKGROUND: Subacute Encephalopathy with Seizures in Alcoholics (SESA) is a rare neurological syndrome characterized by altered mental status, periodic EEG discharges, and neuroimaging abnormalities, typically occurring in individuals with chronic alcohol use. Its progression to status epilepticus (SE), including refractory and super-refractory forms, is poorly documented. METHODS: We present a super-refractory status epilepticus (SRSE) case in a patient with confirmed SESA and conduct a systematic review of the literature to explore the relationship between SESA and SE. A systematic literature review was performed using MEDLINE (accessed through PubMed), Scopus, EMBASE and Google Scholar, in accordance with the PRISMA guidelines. Data were extracted on demographics, EEG and imaging findings, treatment approaches, and outcomes. RESULTS: Our patient developed SRSE in the setting of chronic hepatic dysfunction. The patients responded to a combination of propofol and ketamine and obtained long-term seizure control with vagus nerve stimulation (VNS). Type 3 oligoclonal bands were detected in the CSF, although it is considered a non-specific marker of neuroinflammation. The literature review identified 7 cases of SE in SESA, with variable clinical presentations. While levetiracetam and valproic acid were the most employed treatments, benzodiazepines were notably absent from first-line protocols. Phenytoin and lacosamide resulted as favorable alternatives. CONCLUSION: SE in the context of SESA requires tailored therapeutic strategies due to hepatic comorbidities and diagnostic ambiguity with NCSE. Our report supports the cautious use of propofol and ketamine in SRSE, as well as the efficacy of VNS in long-term seizure control.
Neurite orientation dispersion and density imaging identifies microstructural alterations in patients with cerebral microbleeds and correlations with cognitive impairment.
Li F, Hu Q
BMC Neurol · 2026 May · PMID 42177420 · Full text
BACKGROUND: Neurite Orientation Dispersion and Density Imaging (NODDI) has advanced the study of cortical microstructure. However, its application to participants with cerebral microbleeds (CMBs) to explore cognitive imp...
BACKGROUND: Neurite Orientation Dispersion and Density Imaging (NODDI) has advanced the study of cortical microstructure. However, its application to participants with cerebral microbleeds (CMBs) to explore cognitive impairment mechanisms and potential imaging biomarkers remains underexplored. METHODS: This observational study used NODDI to assess cortical microstructural changes in three groups: 52 participants with cerebral small vessel disease and CMBs (CSVD-c), 78 CSVD participants without CMBs (CSVD-n), and 37 healthy controls. Multivariable regression analysis examined the correlation between altered NODDI metrics, CMB severity, and cognitive function. Cox regression analysis evaluated the association between altered NODDI metrics and dementia risk. RESULTS: The CSVD-c group showed a significant decrease in Intracellular Volume Fraction (ICVF) in the bilateral caudate nucleus and an increase in Isotropic volume fraction (ISOVF) in the right precuneus compared to both the controls and CSVD-n groups. Decreased ICVF in the bilateral caudate nucleus correlated with the number of CMBs (P = 0.023), lower logical memory scores (P = 0.003), and higher Rey Auditory Verbal Learning Test and Clinical Dementia Rating-Sum of Boxes scores (P = 0.011; P < 0.001). Increased ISOVF in the right precuneus was significantly associated with a higher risk of dementia (P = 0.039), but this significance was lost after adjusting for age, sex and years of education. CONCLUSION: Participants with CMBs exhibit decreased ICVF in the bilateral caudate nucleus and increased ISOVF in the right precuneus, associated with cognitive impairment and increased dementia risk. NODDI metrics may serve as valuable markers for investigating CMB-related cognitive impairment.
Deep medullary vein drainage and unfavorable outcomes in stroke patients after endovascular therapy: a retrospective study.
Wei J, Lu T, Li S … +3 more , Li J, Fan Y, Zeng J
BMC Neurol · 2026 May · PMID 42177404 · Full text
BACKGROUND: Ischemic stroke due to middle cerebral artery (MCA) or internal carotid artery (ICA) occlusion often involves deep white matter, which drains through deep medullary veins (DMVs). Whether disruption of the DMV...
BACKGROUND: Ischemic stroke due to middle cerebral artery (MCA) or internal carotid artery (ICA) occlusion often involves deep white matter, which drains through deep medullary veins (DMVs). Whether disruption of the DMVs drainage contributes to unfavorable outcomes following endovascular thrombectomy (EVT) remains unknown. This study aimed to investigate the association between DMVs continuity and post-EVT prognosis. METHODS: In this retrospective observational study from two stroke centers, we included stroke patients due to MCA or ICA occlusion who underwent EVT with successful recanalization between January 2023 and December 2024. The modified Rankin Scale (mRS) was recorded at 90 days. DMVs continuity was assessed on susceptibility weighted imaging (SWI) after EVT. Clinical data were analyzed using multivariable logistic regression and receiver operating characteristic (ROC) curves. RESULTS: A total of 61 patients was included (mean age: 65.4 ± 12.2 years; 63.9% male). Thirty patients (49.2%) achieved unfavorable outcomes (mRS 2-6). Compared to the favorable group, ipsilateral DMVs discontinuity was more frequent in the unfavorable group (70.0% vs. 41.9%, P = 0.040). DMV discontinuity remained independently associated with unfavorable outcomes post-EVT after adjusting for confounders (aOR = 3.23, 95% CI: 1.12-9.30, P = 0.030). ROC curve analysis indicated that the combined model of DMVs continuity status, baseline NIHSS score, and arterial collateral status showed good predictive value for prognosis post-EVT (AUC = 0.81, sensitivity = 73.3%, specificity = 80.6%). CONCLUSIONS: Impaired DMVs drainage is associated with unfavorable outcomes after EVT in ischemic stroke patients with MCA or ICA occlusion.
Aortic stiffness and cardiac function in middle-aged women with ischemic stroke with and without migraine.
Ali M, Holswilder G, van der Weerd N … +13 more , van Os HJA, Linstra KM, van Harten TW, Te Kiefte BJC, Laveaux SDM, Velthuis BK, Kruyt ND, Westenberg JJM, MaassenVanDenBrink A, Terwindt GM, Lamb HJ, Wermer MJH, CREW consortium
BMC Neurol · 2026 May · PMID 42174501 · Full text
INTRODUCTION: Migraine increases the risk of ischemic stroke and cardiovascular disease in women, potentially due to systemic vascular dysfunction. We investigated whether migraine is associated with increased aortic sti...
INTRODUCTION: Migraine increases the risk of ischemic stroke and cardiovascular disease in women, potentially due to systemic vascular dysfunction. We investigated whether migraine is associated with increased aortic stiffness and impaired cardiac function as markers of macrovascular dysfunction in middle-aged women with ischemic stroke. METHODS: This cross-sectional study included three groups of women aged 40-60 years with: (I) ischemic stroke, (II) ischemic stroke with migraine, and (III) no history of stroke or migraine. Aortic stiffness was measured by assessing aortic arch pulse wave velocity (arch PWV) using high-temporal 2D-phase contrast MRI. Cardiac function was evaluated through left ventricular measures, including stroke volume (SV), cardiac output (CO), and ejection fraction (EF), from two orthogonal cine biplane long-axis (2-/4-Chamber) acquisitions. Multivariable linear regression was used to estimate mean differences in arch PWV and left ventricular function, comparing groups 1 and 2 with group 3 as the reference. RESULTS: Among 118 included women (mean age 51 years [SD 5]), (I) 44 had ischemic stroke, (II) 44 had both ischemic stroke and migraine, and (III) 30 had no stroke or migraine. In the reference group (group III), mean (SD) values were 6.97 (1.05) m/s for arch PWV, 95.91 (15.82) mL for SV, 6.73 (1.61) L/min for CO, and 64.90 (5.96)% for EF. Comparisons of groups I and II with group III showed no statistically significant mean differences in arch PWV, SV, CO, or EF. Although mean arch PWV was slightly higher in group II (7.11 [1.53] m/s), the adjusted mean difference (-0.03 m/s; 95%CI: -0.60 to 0.54 m/s) was not statistically significant. Small, non-significant reductions in SV, CO, and EF were also observed in the ischemic stroke groups compared with the reference group. CONCLUSIONS: The increased risk of ischemic stroke in middle-aged women with migraine was not associated with differences in arch PWV or left ventricular function in this cohort. We did not detect evidence that macrovascular dysfunction plays a key role in linking migraine and cardiovascular risk, supporting other studies that point to a potential role for microvascular dysfunction.
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