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 safety and effectiveness of deep brain stimulation of the subthalamic nucleus (STN-DBS) for the treatment of dystonia lack high-level evidence-based medical support. This study aimed to clarify the efficacy and safety of STN-DBS and perform a post hoc analysis comparing it with DBS of the internal globus pallidus (GPi-DBS).

Methods: This multicentre, randomised, double-blind, controlled trial included 67 patients aged 6-60 years old diagnosed with genetic or idiopathic isolated generalised or segmental dystonia. They were enrolled from seven hospitals in China and randomly assigned to undergo GPi-DBS or STN-DBS. After surgery, they were randomised to receive either neurostimulation or sham stimulation for 3 months. At the 3-month follow-up, neurostimulation was also initiated in the sham stimulation group, and all patients were followed up for more than 3 years after treatment. The primary outcome was the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) score.

Results: In the STN group, the neurostimulation subgroup exhibited significant improvement (p<0.001), which is also superior to the sham stimulation subgroup (p=0.028) at 3-month follow-up. At the 6-month and >3-year follow-ups, all patients receiving STN-DBS showed a significant improvement in BFMDRS-M scores (p<0.001). Further post hoc analysis revealed that both STN-DBS and GPi-DBS could produce similar therapeutic effects on motor symptoms (P_{6 months}=0.865, P_{>3 years}=0.905). There were no ongoing serious adverse events throughout the study.

Conclusions: For isolated generalised and segmental dystonia patients, the STN is a selectable DBS target with ensured safety and efficacy. STN-DBS and GPi-DBS may achieve comparable therapeutic effects on motor symptoms.

Trial registration number: NCT03017586.