BMJ Neurology Open最新文献

Background: Acute ischaemic stroke (AIS) is a leading cause of mortality and disability globally, with volume contracted state (VCS), as indicated by an elevated blood urea nitrogen to creatinine (BUN/Cr) ratio, potentially influencing outcomes. This study investigates the association between VCS and clinical outcomes in patients with AIS due to large vessel occlusion (LVO).

Methods: A retrospective cohort study was conducted involving 298 patients with LVO-AIS from two comprehensive stroke centres. Patients were divided into two groups based on BUN/Cr ratio: ≤20 (n=205) and >20 (n=93). Primary outcomes included 90-day mortality and unfavourable functional outcomes, defined as a modified Rankin Scale score of 3-6. Secondary outcomes included the successful reperfusion, haemorrhagic transformation and National Institutes of Health Stroke Scale score at discharge.

Results: Patients with a BUN/Cr ratio >20 had significantly higher 90-day mortality (35% vs 13%, p<0.001) and this association remained significant after adjusting for confounding factors (OR 2.20; 95% CI 1.11 to 4.39; p=0.024). However, VCS was not significantly associated with unfavourable functional outcomes at 90 days (OR 1.28; 95% CI 0.67 to 2.51; p=0.46). Age and initial stroke severity were more strongly associated with long-term functional outcomes.

Conclusions: VCS is associated with higher odds of 90-day mortality in patients with LVO-AIS but not with unfavourable functional outcomes. These findings suggest the need for further research into the role of hydration management in improving survival in patients with AIS, potentially informing future treatment protocols.

Abstract:

Background: Detecting an anterior circulation large-vessel occlusion (aLVO) as a cause of stroke during emergency calls could optimise dispatch, particularly in rural areas. Numerous stroke severity scales assessing motor and cortical signs have been designed for direct patient contact; however, none have been purposely designed for emergency calls. This study aimed to prospectively validate the novel Freiburg Neurointerventional Score (FRENS) in patients presenting with a suspected acute stroke to detect aLVO.

Methods: Patients aged ≥18 years with suspected acute stroke brought directly to our tertiary hospital by paramedics, between May 2021 and 2022, were included in this study. We used the FRENS and National Institutes of Health Stroke Scale (NIHSS) to analyse the FRENS convergent validity. We evaluated and compared the predictive ability and test characteristics of the FRENS and five established NIHSS-based stroke severity scales for the detection of an aLVO stroke. We analysed the number of steps required for the completion of the FRENS algorithm.

Results: Among the 1042 analysed patients, the FRENS items showed high convergent validity with corresponding NIHSS items. A positive FRENS was statistically significantly correlated with an aLVO stroke. The FRENS had the highest sensitivity and negative predictive values for an aLVO stroke and the highest sensitivity value for M2 occlusions. The FRENS provided results after two and all four steps, in 71% and <3% of cases, respectively.

Conclusions: The FRENS enabled the rapid, sensitive detection of an aLVO in patients with suspected acute stroke. Designed for use in emergency calls, the FRENS requires translation and validation in a prehospital setting.

Background: Degenerative cervical myelopathy (DCM) is the most common form of atraumatic spinal cord injury globally. Clinical guidelines regarding surgery for patients with mild DCM and minimal symptoms remain uncertain. This study aims to identify imaging and clinical predictors of neurological deterioration in mild DCM and explore pathophysiological correlates to guide clinical decision-making.

Methods: Patients with mild DCM underwent advanced MRI scans that included T2-weighted, diffusion tensor imaging and magnetisation transfer (MT) sequences, along with clinical outcome measures at baseline and 6-month intervals after enrolment. Quantitative MRI (qMRI) metrics were derived above and below maximally compressed cervical levels (MCCLs). Various machine learning (ML) models were trained to predict 6 month neurological deterioration, followed by global and local model interpretation to assess feature importance.

Results: A total of 49 patients were followed for a maximum of 2 years, contributing 110 6-month data entries. Neurological deterioration occurred in 38% of cases. The best-performing ML model, combining clinical and qMRI metrics, achieved a balanced accuracy of 83%, and an area under curve-receiver operating characteristic of 0.87. Key predictors included MT ratio (demyelination) above the MCCL in the dorsal and ventral funiculi and moderate tingling in the arm, shoulder or hand. qMRI metrics significantly improved predictive performance compared to models using only clinical (bal. acc=68.1%) or imaging data (bal. acc=57.4%).

Conclusions: Reduced myelin content in the dorsal and ventral funiculi above the site of compression, combined with sensory deficits in the hands and gait/balance disturbances, predicts 6-month neurological deterioration in mild DCM and may warrant early surgical intervention.

Objective: This study investigated the effects of early treatment and pathophysiology on eosinophilic granulomatosis with polyangiitis neuropathy (EGPA-N).

Methods: Twenty-six consecutive patients with EGPA-N were diagnosed and treated within a day of admission and underwent clinical analysis. Peripheral nerve recovery rates were evaluated after early treatment by identifying the damaged peripheral nerve through detailed neurological findings.

Results: The eosinophil count at onset was significantly correlated with the total number of damaged nerves. There was a strong correlation between the timing of treatment and the recovery rate in patients who started treatment within 50 days, as the recovery rate did not increase after 50 days of treatment. Antineutrophil cytoplasmic antibodies (ANCA)-negative cases showed significantly higher recovery rates than ANCA-positive cases. Vasculitis was detected in 67% of ANCA-positive and 29% of ANCA-negative patients in the sural nerve and skin biopsy specimen. In addition, infiltration of eosinophils into peripheral nerve tissues was observed in 40% of ANCA-negative patients, whereas it was absent in ANCA-positive patients. Intrafascicular oedema was found in 95% of all patients.

Discussion: Our results suggest three pathological pathways: (1) ischaemic peripheral nerve due to vasculitis mainly in ANCA-positive cases, (2) direct infiltration and degranulation of eosinophils in ANCA-negative cases and (3) progression of axonal ischaemia due to intrafascicular oedema in both cases. The study also found that ANCA-negative cases exhibited better responsiveness to acute-phase treatment than ANCA-positive cases. It is essential to treat patients with EGPA-N as early as possible because the patients could recover time-dependently within 50 days of the onset.

Objectives: Functional neurological disorder (FND) is a complex disorder, recently attracting much research into aetiology and treatment. However, there is limited research on the patient's lived experience. This paper addresses this gap to ask: 'What is the subjective life experience of adult patients living with FND?'

Methods: From 1980 to 2020, Medline, PsycInfo, Scopus, Science Direct, PubMed, CINAHL and Embase were searched for English language qualitative adult research. The disciplines used general medicine, psychiatry, physiotherapy, nursing, neurology, psychosomatic medicine and occupational therapy. The qualitative literature search included book chapters, theses, fellowship reports and conference articles as well as peer-reviewed scientific journals.The Critical Appraisal Skills Programme tool was used to assess 33 papers, with eight papers included in the final synthesis. Nine additional papers, suggested during review, were evaluated but excluded from synthesis, though incorporated elsewhere in the paper. Two authors used an integrative immersion approach to identify the literature's main themes using line-by-line and top-down methods.

Results: Eight main themes were identified: lost, body-mind dualism, preceding stressful events, relatedness, stigma, the battle or fight, the burden and losses of the illness and trust versus mistrust. From these emerged a central overarching theme of relationally regulated selves, which posits the essence of the lived experience of FND as responding to stressful experiences within a relational, regulatory context.

Conclusions: The prevalent themes give valuable insight into the lived experience of FND and the impact of stressors, past and present, and the relational environment in the development of and recovery from the disorder. Further research is needed to support the formulation of the patient experience and cocreated recovery pathways.

Background: Early diagnosis of degenerative cervical myelopathy (DCM) is often challenging due to subtle, non-specific symptoms, limited disease awareness and a lack of definitive diagnostic criteria. As primary care physicians are typically the first to encounter patients with early DCM, equipping them with effective screening tools is crucial for reducing diagnostic delays and improving patient outcomes. This systematic review evaluates the efficacy of quantitative screening methods for DCM that can be implemented in primary care settings.

Methods: A systematic search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted across PubMed, Embase and Cochrane Library up to July 2024 using keywords relevant to DCM screening. Studies were included if they evaluated the sensitivity and specificity of DCM screening tools applicable to primary care settings. Study quality was assessed using the Quality Assessment of Diagnostic Accuracy Studies-2 tool.

Results: The search identified 14 studies evaluating 18 screening methods for DCM. Questionnaires consistently showed high diagnostic accuracy, with Youden indices exceeding 0.60, while only three out of nine conventional physical performance tests met the same threshold. Sensor-assisted tests, particularly those using advanced technology like finger-wearable gyro sensors, exhibited the highest diagnostic accuracy but present challenges related to accessibility and learning curves.

Conclusion: This review highlights the potential of quantitative screening methods for early DCM detection in primary care. While questionnaires and conventional tests are effective and accessible, sensor-assisted tests offer greater accuracy but face implementation challenges. A tailored, multifaceted approach is crucial for improving outcomes. Future research should focus on validating these tools in diverse populations and standardising diagnostic criteria.

Objective: Diffusion tensor imaging (DTI) showed promising results in diagnosing upper limb neuropathies, but its value in patients with foot drop due to peroneal neuropathy has not yet been investigated. We aim to establish reference values for DTI metrics of the healthy peroneal nerve and to evaluate differences in DTI metrics between patients and healthy controls.

Methods: Diffusion-weighted images (DWI) from 22 pathological nerves, 14 asymptomatic patients' nerves and 65 healthy peroneal nerves were processed for quantitative assessment of fractional anisotropy (FA), radial diffusivity (RD), axial diffusivity and mean diffusivity. Clinical baseline and follow-up data were prospectively collected for all patients.

Results: Mean patient FA values (0.40, SD 0.08) were significantly lower compared with healthy controls (mean FA 0.44, SD 0.06). Mean patient RD values (0.98 10^-3 mm²/s, SD 0.21 10^-3 mm²/s) were significantly higher compared with healthy controls (mean RD 0.85 10^-3 mm²/s, SD 0.16 10^-3 mm²/s). FA values were significantly lower in patients with severe foot drop (mean FA 0.40, SD 0.06) compared with non-severe foot drop (mean FA 0.48, SD 0.05).

Conclusion: Based on these results, DTI appears to aid in the differential diagnostic process of patients with peroneal neuropathy. Future studies should focus on automation of DWI processing, confirm the results in larger patient groups and try to establish reliable cut-off values for DTI metrics.

Background: Longitudinal studies investigating cognitive function changes in patients with progressive supranuclear palsy (PSP) are limited. The variability of cognitive impairment across clinical subtypes of PSP remains unclear.

Objective: This study aimed to compare the longitudinal changes in cognitive function between patients with PSP and Parkinson's disease (PD) and to assess differences in cognitive impairment among PSP subtypes.

Methods: A retrospective observational study was conducted using neuropsychological testing data from patients with PSP and PD admitted to our hospital.

Results: The study included 38 patients with PD and 41 patients with PSP (23 PSP-Richardson's syndrome, 14 PSP-progressive gait freezing (PSP-PGF), 3 PSP-Parkinsonism and 1 PSP-predominant corticobasal syndrome). At baseline, cognitive function was significantly lower in the PSP group than in the PD group. Over 12 months, patients with PSP exhibited significant declines in multiple cognitive domains, whereas no significant changes were observed in the PD group. Among PSP subtypes, PSP-RS showed a faster rate of cognitive decline than PD, while PSP-PGF demonstrated a lower progression than PSP-RS.

Conclusion: PSP is associated with progressive cognitive impairment, with rates of decline varying by subtype. PSP-PGF exhibited a slower progression than PSP-RS. Clinical management should consider subtype-specific differences in cognitive prognosis to tailor treatment and care.

Objective: This study aimed to elucidate the clinical manifestations, laboratory findings and outcomes of patients with intravascular large B cell lymphoma (IVLBCL) with neurological involvement and to differentiate IVLBCL with and without neurological involvement.

Methods: A cohort study was conducted at Siriraj Hospital, Mahidol University, Thailand, between January 2005 and September 2024. Clinical data, laboratory values and central nervous system imaging results were analysed. Categorical variables were compared using the χ² or Fisher's exact test, while continuous variables were analysed with the Mann-Whitney U test, as appropriate.

Results: Of the 30 patients with IVLBCL, 10 had neurological involvement and 20 without neurological symptoms, including myelopathy (5 patients, 50%); cognitive impairment (3 patients, 30%); seizures (2 patients, 20%); optic neuropathy, hemiparesis, homonymous hemianopia, vertigo and global aphasia (each affecting 1 patient, 10%). 60% of IVLBCL with neurological involvement had systemic symptoms, including prolonged fever, anaemia, anorexia and weight loss. MRI showed hyperintense lesions in the supratentorial, infratentorial and spinal cord with the prominent findings being longitudinally extensive cord lesions (four patients, 40.0%). The median survival time of the IVLBCL with neurological involvement was 4.1 months (95% CI: 0.0 to 17.1 months), with a 1-year survival rate of 37.5% and a 2-year survival rate of 25.0%.

Interpretation: This study highlights the distinct clinical, laboratory features and imaging of IVLBCL with neurological involvement and compares it to IVLBCL without neurological involvement. Early recognition of these findings is crucial for accurate diagnosis and improved patient outcomes despite the aggressive nature of IVLBCL.