Journal of Neurology, Neurosurgery, and Psychiatry最新文献

Background: The natural history of clinically stable patients with Chiari I malformation (CM-I)-syringomyelia is uncertain. To understand their outcomes, we examined conservatively managed CM-I-syringomyelia patients' long-term clinical and radiological courses.

Methods: We enrolled 156 mild CM-I-syringomyelia cases (Japanese Orthopaedic Association (JOA) score ≥13) managed non-surgically between 1994 and 2014 and followed them periodically until December 2024 for significant progressive myelopathy that we termed 'obvious deterioration'. Obvious deterioration was defined as a ≥2-point decline in JOA score to less than 13. Spontaneous syrinx resolution was radiologically defined as >50% reduction in syrinx length or maximal axial diameter on T1-weighted MRI.

Results: The entire cohort had over 1401 patient-years of follow-up. 55 patients exhibited clinical deterioration, yielding an annual progression rate of 3.9%. Obstructive sleep apnoea-hypopnoea syndrome (OSAHS) (HR=1.841, 95% CI 0.999 to 3.392; p=0.049), positive Babinski sign (HR=2.252, 95% CI 1.229 to 4.125; p=0.009) and without spontaneous resolution (HR=20.308, 95% CI 4.804 to 85.849; p<0.001) independently predicted later clinical obvious deterioration. Spontaneous resolution of CM-I-syringomyelia was more frequent with cervical syringes (HR=2.12, 95% CI 1.224 to 3.674; p=0.007) and absence of OSAHS (HR=3.83, 95% CI 1.376 to 10.640; p=0.01).

Conclusion: This study showed that the natural course of myelopathy in CM-I-syringomyelia varies according to the OASHS status, Babinski sign and spontaneous syrinx resolution. Additionally, baseline characteristics, including the spinal region of the syrinx and the absence of OSAHS, correlated with spontaneous syrinx resolution.

Background: This study investigates the predictive value of baseline quantitative susceptibility mapping (QSM) metrics for assessing the risk of future symptomatic haemorrhages in patients with brainstem cavernous malformations (CMs).

Methods: From July 2020 to September 2023, a prospective multicentre cohort of 155 patients with brainstem CMs was enrolled from 12 institutions. We analysed baseline QSM metrics, including lesional mean, median, IQR and maximum susceptibility. Propensity score matching was adjusted for baseline confounders, and Cox regression models assessed haemorrhage risk. Risk stratification was performed based on thresholds determined from planned receiver operating characteristic (ROC) analyses.

Results: Postmatching cohorts (56 haemorrhage-free vs 30 haemorrhage cases) showed balanced baseline characteristics. Over a mean follow-up of 22.6 months, the baseline QSM metrics, particularly the median susceptibility (QSMmedian) (HR 58.896, 95% CI 8.544 to 405.989, p<0.001; Bonferroni-adjusted p=0.0001, k=4) and IQR of susceptibility (QSMIQR) (HR 29.754, 95% CI 6.101 to 145.119, p<0.001; Bonferroni-adjusted p=0.0001, k=4) were associated with prospective haemorrhage after adjusting for age, gender, lesion volume and prior haemorrhage. QSMmedian (area under curve (AUC)=0.759) and QSMIQR (AUC=0.740) demonstrated modest predictive performance. Risk stratification based on QSMmedian and QSMIQR demonstrated 2-year haemorrhage-free survival rates of 83.3%, 62.8% and 35.7% for the low-risk, intermediate-risk and high-risk groups, respectively. High-risk patients showed a 7.7-fold greater risk of haemorrhage compared with the low-risk group.

Conclusions: This study explored the predictive value of QSM metrics for future symptomatic haemorrhage, suggesting that QSM may serve as a complementary imaging biomarker to existing prognostic models. Further validation in larger, independent cohorts is warranted.

Background: Kappa free light chain (KFLC) index has emerged as a diagnostic biomarker for multiple sclerosis (MS). This study aims to evaluate the diagnostic accuracy of the KFLC-index in Chinese patients with MS, and its capacity to discriminate MS from myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and neuromyelitis optica spectrum disorders with aquaporin-4 antibody (AQP4+NMOSD).

Methods: 428 patients tested for KFLC-index were enrolled in the study, including 130 patients with MS, 41 with MOGAD, 25 with AQP4+NMOSD, 123 with other inflammatory or infectious neurological disorders (OIND) and 109 with non-inflammatory neurological disorders (NIND). Their oligoclonal band (OCB) results and clinical data were reviewed.

Results: KFLC-index was significantly higher in MS (20.1 (0.9-388.9)) compared with MOGAD (4.8 (0.8-56.1), p=0.003), AQP4+NMOSD (4.5 (1.5-46.4), p=0.011), OIND (2.9 (0.6-238.7), p<0.001) and NIND (1.8 (0.6-110.7), p<0.001). The optimal cut-off value for the KFLC-index to identify MS from the non-selective controls was 8.3, with an accuracy comparable to that of OCB (area under the curve 0.84 vs 0.81, p=0.249). The optimal cut-off values for differentiating MS from MOGAD and AQP4+NMOSD were 18.5 and 12.1, with performance similar to OCB (p=0.756 and 0.064). Combination of KFLC-index and OCB outperformed OCB alone in differentiating MS from non-selective controls and MOGAD (p<0.001 and p=0.044). Female (p=0.009) and higher cerebrospinal fluid leucocyte count (p<0.001) were associated with higher KFLC-index in MS.

Conclusion: KFLC-index is a valuable diagnostic tool for differentiating MS from other inflammatory demyelinating diseases.

Background: The socioeconomic burden of early onset dementia (EOD) defined as disease onset before the age of 65 years, is substantial due to its widespread disabling effects in relatively young individuals. While dementia is widely recognised as a major contributor to mortality among the elderly, only a limited number of studies have assessed survival and factors associated with prognosis specifically in EOD.

Methods: A population-based cohort study, encompassing all incident EOD cases from two defined regions in Finland. The survival and all-cause mortality rates in EOD and its subtypes were evaluated from January 2010 to December 2021. All visits at the dementia outpatient clinics were reviewed and manually re-assessed (n=12 490), resulting in 794 validated EOD cases of Alzheimer's disease (AD), frontotemporal dementia (FTD), alpha-synucleinopathy (α-SYNU) and other EOD spectra. Region-, age- and sex-matched control groups without neurodegenerative diseases were created from nonselective general population data registers (1:10 case to control ratio, 7930 controls in total).

Results: The median survival for EOD was 8.7 years, with the shortest survival in the FTD (6.9 years) and α-SYNU groups (7.0 years), followed by the AD group (9.9 years). Compared with controls, mortality was significantly higher in the total EOD group (HR=6.56, 95% CI=5.56-7.74, p<0.001). Among the dementia subtypes, FTD spectrum patients had the highest all-cause mortality risk compared with controls (HR=13.75, 95% CI=10.25-18.43, p<0.001). Male sex, older age, several comorbidities and lower level of education were associated with increased mortality, but these were not EOD-specific.

Conclusion: EOD diagnosis significantly deteriorates patients' survival, with significant variation between different diagnostic groups and in relation to patients' demographic factors.

Background: Recovery following traumatic brain injury (TBI) is highly heterogeneous. Characterising longitudinal functional trajectories may enable more individualised care and inform the trial design. This study aimed to identify distinct patterns of functional recovery in the first 12 months post-injury using the Glasgow Outcome Scale - Extended (GOSE).

Methods: Patients with available GOSE data from Transforming Research and Clinical Knowledge in Traumatic Brain Injury, a prospective, multicentre study, were included. A two-stage multiple imputation procedure addressed missingness, and trajectories were modelled using polytomous variable latent class analysis (poLCA) in both complete-case and imputed datasets.

Results: Among the 2,100 participants with TBI, poLCA identified seven distinct recovery trajectories based on GOSE scores. These included (1) death (6.2%), (2) persistent GOSE 2-5 (5.7%), (3) improvement from GOSE 3-5 to GOSE 5 by 3 months with no further gains (9.0%), (4) rapid improvement from GOSE 3-5 to GOSE 7-8 sustained through follow-up (7.0%), (5) gradual improvement from GOSE 3-6 to GOSE 5-7 over 6 months (15.3%), (6) rapid early improvement from GOSE 4-7 to GOSE 6-8 by 3 months and then plateauing (21.8%) and (7) progression from GOSE 5-8 to GOSE 7-8 within 3 months, with stable outcomes thereafter (25.1%). Recovery class membership was significantly associated with the initial Glasgow Coma Scale scores, CT severity (Marshall and Rotterdam scores) and presence of psychiatric comorbidities.

Conclusions: Our findings support a shift towards trajectory-based stratification in clinical care and research. Incorporating these patterns into prognostic modelling may improve outcome prediction, personalise rehabilitation and refine eligibility criteria for interventional trials.

Background: Intravenous immunoglobulin (IVIg) is effective in many neuroinflammatory disorders but carries a risk of thromboembolic events (TEEs). Subcutaneous immunoglobulin (SCIg) is effective, but its thromboembolic risk profile is less well-described, particularly in patients with prominent vascular risk factors. We investigated the thromboembolic risk profile of IVIg and SCIg using a large single-centre retrospective dataset of patients with neuroinflammatory disorders.

Methods: 243 patients treated with immunoglobulin over a 15-year monitoring period were analysed. Demographic information was used to calculate QRISK3 vascular risk scores. Patients were grouped based on having received IVIg-only, SCIg-only or both IVIg and SCIg (with subgroup analysis of IVIg and SCIg treatment periods). TEE incidence rates were compared. The relationship between QRISK3 and TEE likelihood was analysed.

Results: A total of 1401 patient-years immunoglobulin treatment data were obtained. The SCIg-only cohort was older with higher QRISK3 scores. More patients on IVIg (n=14) than SCIg (n=2) experienced TEEs on treatment (1.38 vs 0.52 events per 100 patient-years), but this difference was not statistically significant. IVIg-treated patients with TEEs were younger (p=0.039) than SCIg-treated TEE patients. QRISK3 scores broadly correlated with thromboembolic risk across the cohort (p=0.027), but some younger IVIg patients with low QRISK3 scores experienced TEEs indicating that IVIg but not SCIg independently increases thromboembolic risk. QRISK3 scores >10% identified 71% and 100% of TEEs in IVIg-treated and SCIg-treated patients, respectively, but are insensitive (7%) as TEEs are rare events.

Conclusions: SCIg is at least equivalent in thromboembolic risk to IVIg and may be safer for patients with existing vascular risk factors.